Prometric exam march 2020 (1) (3).docx
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2020 Dermatology Prometric Exam 2020 متَنَـا علا ال ما ك ال علم لَنَا ِإ سبحانَ قَالُوا حكيـم ت العَليـم ال نـاك أَنـْ صدق هللا العظيم سورة البقرة: اآلية .)32( List of contents Chapter Title Pages Total 1 Cosmetology and Lasers 1 - 5 5 2 Vascular 6 - 13 8...
2020 Dermatology Prometric Exam 2020 متَنَـا علا ال ما ك ال علم لَنَا ِإ سبحانَ قَالُوا حكيـم ت العَليـم ال نـاك أَنـْ صدق هللا العظيم سورة البقرة: اآلية .)32( List of contents Chapter Title Pages Total 1 Cosmetology and Lasers 1 - 5 5 2 Vascular 6 - 13 8 3 Metabolic 14 - 21 8 4 Connective Tissues 22 - 30 9 5 Surgery 31 - 37 7 6 Bullous disease 38 - 42 5 7 Hair and Pigmentary 43 - 47 5 8 Genodermatosis 48 - 61 14 9 Nail 62 - 64 3 10 Drugs 65 - 76 12 11 Papulosquamous 77 - 87 11 12 Tumors 88 - 103 16 13 Ethics and Safety 104 - 108 5 14 Eczema and Hypersensitivity 109 - 118 10 15 Granuloma 119 - 121 3 16 Acne 122 1 17 Infection 123 - 141 19 18 Histopathology in exam 142 1 19 Stains and Markers 143 1 20 Board Vital 144- 145 2 List of figures Chapter Title Pages Total 1 Cosmetology and Lasers 146 - 147 2 2 Vascular 148 - 150 3 3 Metabolic 151 - 158 8 4 Connective Tissues 155 - 158 4 5 Surgery 159 - 161 3 6 Bullous disease 162 - 163 2 7 Hair 164 - 165 2 8 Genodermatosis 166 - 171 6 9 Nail 172 - 173 2 10 Drugs 174 - 181 8 11 Papulosquamous 182 - 186 5 12 Tumors 187 - 194 8 13 Eczema and Hypersensitivity 195 - 199 5 14 Granuloma 200 - 201 2 15 Acne 202 1 16 Infection 203 - 207 5 Cosmetology and Lasers Cosmetology and Lasers Type of filler long lasting or permanent filler: HA Collagen Silicon Fat Carboxymethyl cellulose Note:if sculptra (poly lactic acid) it is most right Sometimes: carboxy methyl acetate. Filler in nasolabial fold, which one is longest lasting filler: Hyaluronic Ca hydroxyapatite Collagen Microsperules Patient was injected by filler and botox then balnching appeared in forehead a. Hyaluronidase inj Nitroglycerin patch Massage Itching pallor and pain after injection of filler and botox first step is: Hot fomentations Nitroglycerin patch Hyalorinidase injection due to pain mentioned If pallor only filler and botox use nitroglycerin patch If pallor only use nitroglycerin patch If filler only use hot fomentation If both botox and filler and pain with pallor use hyalourinadase Picture of case of melasma in skin type 2" Salyclic acid 10% AHA 5% TCA 25% Jessener solution+ acetic acid Old male 50 years deep forehead farrows and deep nasolabial fold increase with muscle activity how to get best improvement Peel Fractional laser Filler Botox Picture of vein in arm laser removal 1320 nm chromophore a. Water HB Melanin Blanching after filler injection what is next Hot fomentation and massage due to blanching Nitroglycerin patch Hyaluorinidase injection Melasma laser Q switched ruby 694 b. Q switched Nd yag Patient treatment with UVB 1500 stop one week return on a. 1000 b. 5000 c. 1500 or (same dose) d. 2000 Acne keloidal laser Nd yag for hair removal Co2 for resurfacing حسب السؤال tanning of Causes Oxidation of melanin (early from UVA) Melanosomes Melanin increase Increase melanocytes (delayed from UVB) Lip vascular lesion laser a. Nd yag 1064 b. 585 Which type of light used in PDT in treatment of acne: Visible Red Blue Violet Note: if with ALA red light if alone blue light Which type of laser used in Haemangioma a. 755 b. 1064 c. 585 What is the pulse duration of laser used in treatment of Haemangioma? a. 0.5 b. 2 c. 8 d. 20 Note: 0.45-40 ms bolognoia Venous lake histopathology what is commonest site? Lips Picture of lips having venous lake which type of laser used in treatment a. 755 b. 1064 c. 585 d. 2940 note: electrosurgery or a hemoglobin-targeting laser is usually effective. Picture Which type of laser used in nevus of Ota? a. 755 b. 532 q-switched ND yag c. 1064 q-switched ND yag d. 595 Device used for nevus of ota must be QS so the first choice is c then b. Wavelength responsible for immediate darkening of the skin a. 320-340 b. 320-290 c. 290-200 Picture of acne keloidalis nuchae type of laser used in treatment? a. 10600 b. 1064 c. 585 d. 755 Infant with hemangioma and has cardiac problems or seizures Increase fluence Increase pulse duration Decrease wavelength Picture of child girl with birthmark above eye and have seizures laser ttt in mj/sec 2 5 10 20 Note: if asked about fluence it is 5 mj The best lesion respond to laser is: a. Nevus of ota Beckersmelanosis Blue nevus Most common complication of infraorbital Botox injection a. Festooning Photophobia Nystagmus Blindness Type of lipid in sebum when increase sebum increse Cholesterol Squalene Sebolic acid Wax esters Case of solar urticaria with wavelength a. 320 b. 450 visible light c. 280 UVA2 wavelength: a. 320-340 Wave length of immediate tanning: a. 290 b. 311 c. 340 d. 200 Enzyme involved in cell membrane: Transglutaminase lorici Most common collagen in adult skin: a. 1 3 2 5 What is the cytoskeleton in keratinocytes? a. Tonofilament Desmosome Odler bodies Hyaline granules What makes up the majority of protein envelop of keratinocyte a. Loricrin Involucrin Fillaggrin Envoplakin Vascular Vascular Infant 1 year 15 kg with hemangioma what is the treatment: 15 mg propranolol 30 mg propranolol 15mg prednisolone 30mg prednisolone Side effect of drugs used in Hemangioma Bradycardia Hypotension Sleeplessness Picture of hemangioma in infant what type of seizers Focal Contralateral Generalized. Female patient has this lesion what association اليمين عنيها فوق pws عندها بنت صورة a. Dandy walker syndrome Vermis agenesis Cerebellar atrophy Picture of very huge hemangioma what is association PHEACES (Dandy Walker syndrome) most common and cerebral hypoplasia or dysplasia Hemangioma best topical treatment a. Timolol b. Propranolol If port wine sirolimus Picture of cyanotic leg in warm and improve in cold findings Thrombocytosis SCL70 Case of vasculitis (dilated follicular opening description of granuloma faciale) treatment Dapsone Colchicine Steroid Case of Klippel trenaunay Syndrome investigation needed X ray Doppler U/S or - MRA MRI Brain MRI Search for association Cryoglobulinemia defect or mutation in a. Cd40 ligand POEM (board vital most commonly occurring with Castleman syndrome) Tufted angioma Glomeruloid hemangioma Kaposi hemangioma Picture of histopathology GMS stain with بني وكور خضرا أرضية clinically Popular Ulcer Keloid like clinical (Lobomycosis) ttt is surgical Nodule Case of Klippeltrenaunay syndrome treatment Surgical treatment for venous malformation Sclerotherapy / epiphysiodesis Flushing investigations diarrhea, bronchospasm (carcinoid syndrome): a. Urinary 5HIAA Female patient her hand hurt with warmth and decrease with cold SLE Thrombocytosis Picture of erythema abigne (description reticulate pigmentation or picture) on back what is cause Heat pads (Hot blankets) Photo of leg with rash bilateral which cryoglobulin decrease investigations (cyanotic leg pic) I With Waldenström's macroglobulinemia or multiple myeloma II Viral infections (HCV) (HIV) mixed cryoglobulinemia syndrome III Autoimmune disorders or HCV Picture of hemangioma and his brother has same lesion what is the disease a. Dandy Walker Picture of patient leg with rash management Dapsone Colchicine Steroid Patient has neutrophilia (thrombosis) without vasculitis in leg lesion looks like vasculitis treatment (mostly sweet’s syndrome): Cortisone Aspirin Marker in revoluting capillary hemangioma??? a. GLUT1 (glucose transfer) Patient with varicose veins and injected it sever pain and swelling after injection what next : Doppler Compression Note: anesthesia used tumescent anesthesia etas 2019 Child with varicose veins and one limb bigger than the other what next CT MRI Doppler Duplex Case of hemangioma in face association Arachnoid cyst Vermis agenesis Dandy walker Note: the most common is Dandy walker then cerebellar (vermis) hypoplasia or dysplasia Case of varicose vein, pain in lower leg investigations a. Doppler b. X ray Bleu nevus most common site Neck Face Limbs (hand and foot) Description of granuloma faciale ask for line of treatment a. Steroid injection intralesional Hemangioma in face (PHACES) what next investigation MRI brain Echo Ultrasound Angiography if said high risk CT Note: arterial studies due to anomalies in heart before propranolol treatment MRA due to head and on neck (posterior fossa malformation) and ophthalmology Picture (huge hemangioma association) of baby with big hemangioma what is association Dandy Walker syndrome Vermis agenesis Corpus callosum tumor Marker of involuting hemangioma all except: CD43 von Willebrand CD31 GLUT1 Bilateral vascular ulcer without vasculitis. Most common cause a. Factor V Leiden Protein C or S deficiency Antithrombin III deficiency Erythema nodosum treated with unknown medication and has eczema, what is the drug a. Potassium iodide Picture Gene mutation in vascular birthmark (girl beside her eye) a. PWS it is GNAQ gene on ch9q21 Infant with hemangioma and seizures pulse duration laser treatment: a. 0.5 2 1 20 Picture of hemangioma what common side effect to drug: Bradycardia Sleep disturbance Bronchial problems Sweet syndrome most poor prognostic sign Leukocytosis Thrombocytopenia High ferritin Painful lesion on hand (sweet $) + clinical picture + abnormal lab CBC increase WBCs increase neutrophil fever 38.5, How long white blood cells take time for recovery? 9m 6m 12m 6 years old boy with indurated nodule and neutrophil & WBC high and no vasculitis(sweet syndrome) ,what is next Blood culture Bone marrow biopsy picture histopathology EN what type a. Granulomatus septal panniculitis Septal panniculitis Granulommatous lobular panniculitis Lobular panniculitis Patient after doing angiography developed cyanotic leg mostly associated with a. Eosinophilia Neutrophilia Neutropenia Lymphocytosis Female patient 55 years old on renal dialysis complains of ulcers at tendon achilis. What treatment suitable? Prednisolone Warfarin Hyperic oxygen Sodium thiosulphate Picture of patient with lesion as plexiform neuroma on shoulder what is the association a. Scoliosis Renal hamartoma Renal cell carcinoma Brown mamiliated lesion of scalp + café au lait on body association: **in ETAS clitoral hypertrophy Scoliosis (صوره mccune Albright syndrome) Picture of patient has lesion brownish or violaceous in lower limb as if it is eczemafrom vasculitis his brother has same lesion Prothrombin gene defect Thrombin gene defect Factor 5 gene defect Male patient has this lesion after angiography , صورة جايب رجله what needed to confirm the diagnosis: Neutrophilia Eosinophilia Lymphocytosis Male patient has plaque pigmented on his leg Kaposi and hhv8 +ve need treatment a. Sirlomus Pyoderma gangernosum IVIG Cyclosporin Biologic ( infliximab, adalimumab, etanercept and ustekinumab )off label Pt with Nodular lesion at tendon achillis كلها واالختيارات vasculitis a. Erythema elevatum duitinum Another q about investigation: may be Infection (strep, hepatitis, HIV, syphilis) Gammopathy. Hemangioma treatment a. 585 b. 755 c. 10600 Histopathology of EN and what to know this patient is going to be allergic to what? a. Potassium iodide angiography: after نفس الحاله رقم 5 بس جايبين صوباع رجله الكبير patient Male 51. Eosinophilia a. Neutrophilia Creatine kinase Pancreatic panniculitis description of pathology (ghost cells) with: Lymphocytosis Neutrophilia Neutropenia Eosinophilia Itchy follicular papules with eosinophilic folliculitis invtigations : HIV CD4 CD8 patient with blue coloration on leg a. Cryoglobulin type1 Cryoglobulin type2 Cryoglobulin type3 Cryoglobulin type4 Baby and mother having the same condition left second or third rib anomalies a. Gorlin syndrome Lesion in leg without picture description of histopathology having neutrophilia no thrombosis Dapson Steroid Patient with livedo racemose + purpura and increase urea and creatinine and pulmonary problems what is type of ANCA (with MPA) Cytoplasmic Nuclear Perinuclear Note: another question asks about gene in wagner it is HLA-DPB1 gene Dilative cardiomyopathy with deep venous thrombosis and skin rash and high eosinophil (hyperesinophilic syndrome): Mepolizumab Imatinib Pemblizumab Atlizumab Case of vasculitis معاها lung affection away from lumen of airway, what type of ANA: a. Cytoplasmic Thermonuclear Nuclear What is the first indicator of rapid involuting hemangioma (RICH) a. CD 30 or 31 or 34??? b. Glucose transport Note: GLUT1 is becoming negative and CD133 is indicator Metabolic Metabolic Case of lipoid proteinosis, hoarseness of voice treatment Steroid Acitretin 60 years old patient with multiple waxy papules on the trunk and lower limb with ecchymosis and bruises and macroglossia (mostly systemic amyloidosis) what is next? Bone marrow biopsy حاجة اخر Blood PTN fixation or detection 3 Fat aspiration Abdominal fat aspiration 1 Blood or urine electrophoresis 2 Treatment of previous condition: Chemo Steroid + Melphalan Anti siRNA. Legius syndrome caused by SPRED1 gene and NF1like but no tumors, freckles, lipoma, macrocephaly, ADHD McCune–Albright by GNAS mutation, Fibrous dysplasia, Café au lait macules, including characteristic jagged "coast of Maine" borders and tendency not to cross the midline, Precocious puberty, Testicular abnormalities, Hyperthyroidism, Growth Hormone Excess, Cushing's Syndrome/ clitoral hypertrophy/scoliosis Case of sever itchy macular hyperpigmentation on the back (macular amyloidosis) investigations: Calcitonin level or thyroid scan Congo red 10 y old child normal with scars on knuckles ask: Urine porphyrin negative blood porphyrine positive Erythropoietic porphyria Congenital erythropoietic porphyria Heridetarycorporphyria Coproporphyria Varigat porphyria Hemochromatosis case (diabetes/joints /bronze) & generalized, hyperpigmentation & hepatomegaly what association? Copper accumulation C282y gene Case of Wilsons disease a. ATP7B gene In patients with clinical features suggestive of Wilson disease (e.g. abnormal liver tests combined with neurologic symptoms), we start by obtaining liver biochemical tests, a complete blood count, serum ceruloplasmin and copper levels, an ocular slit-lamp examination, and a 24-hour urinary copper excretion. Female patient with macular amyloidosis what is next Neurologist Endocrinologist Picture of Juvenile xanthogranuloma next step a. Ophthalmologist. b. Neurologist. Patient 30 years with itching interscapular brownish patch with rippling (amyloidosis) which organ associated Kidney Adrenal Thyroid Pituitary Macular amyloidosis association: a. MEN type 2 A Male patient with erythematous rash and bone pain prober site for bone x ray Arm Leg Spine if skull or cranium not present Langerhans cell histiocytosis. Skull or cranium 1st Genetic defect in litterer Siwe Mutation in BRAF gene Child with erythema and bone pain and Histological finding epithelioid granuloma and few lymphocytes what gene defect (Blau syndrome) Carb 15 TNFR1 ACE Fabry's disease defect (picture): a. GLA galactosidase A. Pt with bilateral nodules on tendon Achilles {tubers xanthoma} What investigation? a. Cholesterol 40 years female with generalized purpuric eruption all over body and sole ulcer (with path pic) MM Ulcerative LP Same question treatment Mohs MTX Cyclosporin Another q what to monitor during treatment answer is creatinine Mastocytosis aggravated drug a. Dextromethorphan Mastocytosis mutation in which axon a. (codon 816) axen 17/C(KIT) Hardness of the upper back of the patients IgG kappa IgG lambda Pt with skin colored papules and elastic skin on neck and angoid streaks on retina what to do? Introduce Aspirin Introduce Simvastatin Stop smoking Biologic. Most common in PXE Peripheral arterial disease 2 if intermittent claudication not present Cardiomyopathy Pulmonary problems Intermittent claudication. 1 child with porphyria treatment a. Phlebotomy Hydroxychloroquine IVIG Picture of hands (bullae, blister, hypertrichosis, milia) (porphyria) what is associated organism HCV patient with cutaneous amyloidosis what association a. Thyroid tumor Renal Adrenal Lung. Picture of 2 cubital fossa in adult, non-itchy, شخصتها انا xanthoma ttt Phototherapy Imiquimod Antibiotic Steroid Skin colored popular lesion in back of female, stain colloidal iron, (scleromyxdema) what next step? Immunofixation Bone marrow biopsy to exclude multiple myeloma. Perforating Disease (histopathology: collagen within a keratin plug or extruding through the epidermis), consistent clinical features (keratotic papules or umbilicated papulonodules), with renal failure, what investigation u will order? Creatinine Leukocyte Liver enzyme. Patient has nodules on olecranon ligament what is the investigation: a. Cholesterol Picture of patient with vesicular eruption on hand arm face by histopathology no inflammatory infiltrate treatment Phlebotomy if PCT Steroid IVIG Case of Hypothesia &hypohydrosis investigations: a. G6PD Chest x ray MRI CBC Mucinosis muco-polysaccharide. Scleromyxdema mutation in keratin a. Lambda b. Kappa Case of macular amyloidosis investigations: Neurological Calcitonin ال يوجد Thyroid c. Case of Langerhans cell histiocytosis investigation needed x-ray on: Arm Spine if skull or cranium not present which is more accurate Leg Cranium or skull Pathological description of angiokeratoma and what enzyme defect a. Galactosidase A An angiokeratoma would show hyperkeratosis and rete ridges encircling dilated vessels in the papillary dermis. Picture of urticaria pigmentosa the most affected a. Git (most affected) Lung CNS (Least affected genitourinary then neuroendocrine > CNS Case of (hypertrichosis & vesicles) PCT investigation needed: a. Ferritin CBC MRI جه مرتين disease Wilson Diagnosis والمره التانيه Associated b. Case of child 3 months with vomiting & brown lesions and did x-ray showed osteolytic lesions (LCH) what is the treatment: Methotrexate Cyclosporin Vincristine or vinblasten N.B for symptomatic patients with skin-only LCH oral MTX , 6-mercaptopurine, hydroxyurea , topical corticosteroids, topical nitrogen mustard, oral methotrexate, and oral thalidomide Unifocal or multifocal involvement of one organ or system, single agent prednisone, or combination of vinblastine and prednisone, curettage of bone lesions Case of scleromyxoedema waxy papules in face upper back, what to treat Rituximab Thalidoamide 2 c. IVIG 1 Infant 3 months with low weight and multiple, brown lesions did Xray and found osteolytic lesions ,chondroitin, what is the ttt Methotrexate Cyclosporin Vincristin Case of mastocytosis the least organ affection Heart Lung Git the most frequent +/BM/LN/liver/spleen (the most) CNS + genitourinary Patient complaining from recurrent abdominal pain since childhood Steroid Hydroxychloroquine Dapsone Colchicine if FMF End stage renal with perforating dis best treatment : a. NBUVB Path PUVA Oral PUVA Child 7 years with one skin colored papule in lower eye lid with a photo ask about expected Decrease muscle power increase muscle power seizures Photo of neurofibromatosis and ask about association Xanthogranulom. Case of Wilson what will you expect to find in the epidermis. a. Elastic fiber. Photo of calcinosis cutis in index ask about association Systemic sclerosis SLE Dermatomyositis Limited scleroderma Scenario of pxe ask about association no claudication in choices a. Peripheral vascular disease Case of ulcer in the thigh in end stage renal dis pt ask about treatment Wrafarin Na thiosulfate Fabry's disease (Male has erythematous purple papules in the lower abdomen and inner thigh his brother too what is depsitted or accumulating substances: Ceramide (globotriasylceramid) Mucopolysacaride Glactoaminoglyacn Connective Tissues Connective Tissue Case of photosensitivity what is the prophylactic treatment Steroid Carotene 3 Phototherapy 1 Hydroxychloroquine 2 Case of granuloma with few lymphocytes, describe patient complain erythematous plaque treatment (all biologic) (sarcoidosis) Infliximab Adalimumab Etanercept Rituximab Patient with dermatomyositis controlled on prednisolone and MTX intake started for one week of myopathy what is next Decrease steroid Increase steroid Sclerodactyly, Raynaud's phenomenon, Photosensitivity investigations (Mixed connective tissue disease) U1RNP Double strand DNA SCL 70 topoisomerase ANA Familial SLE what c deficiency (C1, C2 and C4, causes SLE) 3 5 9 1 Picture like Gottron rash on both hand and rash on ear what is treatment Cortisone Dapsone Azathioprine Case of small ulcer on digit, photosensitivity, fibrosis, pigmentation investigations a. ANA b. Ds DNA Picture of calcinosis cutis in thigh in renal patient investigations X ray Angiography Case of photosensitivity investigations a. ANA Ds DNA Anti SCL70 Description of JXG association (Juvenile xantho-granuloma) Neurofibromatosis Note: ass NF1/CML Investigations: biopsy tutton giant foamy histocytes CD68/ HMA56/ factor 13a/ S100 Type of light chain in systemic amyloidosis Kappa Lambda JXG refer to Ophthalmologist Neurologist Histopathological features of lesion showing epidermal atrophy, edematous hyalinized superficial dermis with vascular dilatation and lichenoid band like lymphocytic infiltrate with epidermal atrophy and upper dermal fibrosis what is diagnosis Lichen sclerosis et atrophicus Describing disease (may be subacute le ??) associated with photosensitivity and giving investigations of CT diseases what is associated Picture of female finger with exophytic lesion asking about treatment (Acquired digital fibrokeratoma) Surgical removal Patient with dermatomyositis controlled on prednisolone and MTX intake started for one week of myopathy what is next a. Decrease steroid b. Increase steroid Girl with itchy hyperpigmented patch on trunk histopathology of amyloidosis refer to Neuro Endocrinologist Generalized superficial nodule with atrophied surface and surface telangiectasia on its Diagnosis Disseminated DLE 1 Multiple BCC LE panniculitis 2 Another question is investigations needed a. ds DNA Case of lichen sclerosis et atrophicus a. Topical steroid (clobetasol) Tacrolimus 0.3 Tacrolimus 1 Scleroderma + lung fibrosis a. AD AR XLR XLD Note: (Topoisomerase I antibodies (formerly Scl-70)) Steroid induced myalgia: a. Decrease steroid dose Description of case of popular sarcoidosis investigations: Note: hypercalcemia / hypercalciuria / increase amyloid A / vit d / kevim test / ACE level increase in monitoring treatment Description of case of scleroderma investigations: السؤال حسب ANA Topoisomerase if systemic c. Anticentromere if limited Scenario of limited scleroderma ask about investigation a. Anticentromere Discoid lupus with follicular plugging asking for investigations ANA Tacrolimus Hydroxychloroquine Mometasone Camoflash Type of secretion of moll's gland modified apocrine sweat gland in eyelid secret sebum by Decapitation gland secretion by decapitation: Zeis Moll Montenegro Meobian Picture case have small papule at side of eye 😊brit huge dube synd. a. Renal Annular scaly lesion in extremities of old patient كبير ست صوره Phototherapy Picture hyperpigmented patch in cubital fossa, what is diagnosis? Steroid Tacrolimus c. Minocycline d. Phototherapy Case of EhlerDanol syndrome hyperextensibility skin of the neck defect in collagen, type 1 in COL5A1 and COL5A2 has what joint complication? Joint Hypermobility Hip dislocation Cause of discoid lupus have persistent scar for 3 years treatment a. Camouflage Tacrolimus Steroid Hydroxychloroquine if active No treatment Asymptomatic maculopapular lesion in the left arm, treatment a. Symptomatic Steroid Phototherapy MTX Asymptomatic skin rash on the upper and lower limbs with atrophic center a. Generalized tumid lupus Erythematous plaque on nose and has pustules on plaque. What is treatment and diagnosis Rifampicin and retinoid Minocycline Acitretin Rifampicin and dapsone Picture of morphea 10 years ago, treatment is? Steroid Tacrolimus Mofetil MTX Female with genital plaque and asymptomatic (Lichen sclerosis) what treatment a. Steroid Cutis laxa (Picture) mutation periorbital edema and sagged cheeks Fibulin 5 Pseudoxanthoma elasticum What is the protective to treatment Aspirin sometimes say stop smoking Simvastatin (for treatment not protective) pseudoxanthoma elasticum complication Peripheral Arterial disease Cardiomyopathy Pt with yellow plaque in his leg with telangiectasia and same lesion on chest Pathology > granuloma with plasma cells no cholesterol clef What is investigation? Necrobiosis lipodicuim Blood glucose Lupus pernio picture. what you expect in this patient after 10 years Shortness of breath Palpitation Liver problem female patient her hand hurt with warmth and decrease with cold (erythromelalgia), What investigation u will do? ANA Scl-70 CBC … thrombocytosis Hypertrophic DLE (interface dermatitis & thickening basement membrane) HIV Muntgaro test Fungal culture WBCs DLE picture clinical + histo description + patient asymptomatic what to do a. Assurance Hydroxychloroquine Mometasone furate Picture of child with exanthema lacy rash on face and lower limb EBV what next investigation ANA Reticulocyte Discerption of case [SCLE] investigation Ro La Pt with SLE on hydroxychloroquine since 2 yearsليه ميستجبش بدا Add acitretin Add cyclosporine Shift to rituximab Stop treatment DLE in face (picture) What is the maximum dose of the drug of choice (hydroxychloroquine) 5 mg ideal body weight 5 mg real body weight new guidelines 2016 6.5 mg ideal body weight Pt with cyanosis in fingers (Reynaud’s)and edematous with C3and c4 Low, ANA +ve, what is best treatment? Hydroxychloroquine MTX Cyclosporine Description of lesion in vagina, investigation (Lichen sclerosus et atrophicus) CT X ray Mammogram Thyroid scan Annular lesion dorsum of hand (picture) treatment Steroid if granuloma annulare Terbinafine - الباقيantifungal Lens dislocation Anterior dislocation in PXE, Marfan Posterior dislocation in homocystinuria Yellow brown ring in eye in Wilson dis Sclerodactyly, Raynaud photosensitivity investigation needed: a. U RNP Child with encoupe de Sabre best treatment a. Subcutaneous MTX Case picture of parrot and ridges in nail caused by: Rheumatoid arthritis Systemic sclerosis if not present osteoarthritis 65 y female on renal dialysis complaining from ulcer on tendoacahilis, what is suitable treatment Prednisolone Hyperic oxygen Warfarin Na thiosulfate 65 years female complaining from sudden waxy brown papules on the trunk the most associated cancer (Laser trait) Lung Stomach Liver Pancreas Female patient with brown macular rash on the cubital fossa best treatment Phototherapy Cyclosporine Topical steroid Antibiotic Erythema, edema, induration of whole face, Stiffness of both LL Photo of a finger with periungual erythema Pentoxyphillin Steroids Colchicine Slidnafine Which manifestation not associated with subacute LE a. Scarring Photosensitivity Joint affection Pt with erythema, edema and induration of the whole face with stiffness of both LL and photo of a finger with periungual erythema. Treatment Pentoxyphiline Steroids Colchicine Sildenafil Photo of linear morhea in 10 y old child what treatment will you combine with steroid NBUVB MTX Mofetil Eruptive skin colored papules with hardness in the face and upper back treatment a. Thalidomide الباقى كلهم بيولوجك Rituximab b. Atrophic lesions with telangiectasia on surface investigations ANA Skin biopsy then DIF then ANA Note: Skin biopsy then DIF then ANA, Respectively. The most common site for that disease a. Face b. Trunk Surgery Surgery Picture of Pyoderma gangrenosum (heavy discharge) ulcer with yellowish discharge dressing Alginate Hydrocolloid Hydrogel Vaseline dressing The most organ affected in liposuction a. Arm Back Leg Surgical thread 4.0 less than 5.0 in Increase knot strength Increase tensile strength Smaller needle Patient made excision of tumor beside eye and left for secondary intension Dressing used Alginate Gauze Hydrocolloid Hydrogel Patient made excision of tumor beside eye and left for secondary intension after 2 weeks copious exudates dressing used Alginate Gauze Hydrocolloid Hydrogel Picture of rounded part removed from nose and other picture of suture and flap in middle of nose Advancement Transposition Bilobed Suture in nasolabial fold linear type of flap a. Transposition flap Picture of cyanotic foot after cardio angiography what drug do this Note: if asking about cause arterial embolism by cholesterol blog dislodgment Picture of big toe cyanotic or cyanotic feet patient have catheterization what can Lymphocytosis Neutrophilia Neutropenia Eosinophilia or Eosinophilura Painful ulcer with yellowish discharge what is dressing (according to exudate quantity) Alginate Hydrogel Hydrocolloid Child with periauricular cyst for excision best antiseptic as he has hearing problems Chlorohexidine Iodine Alcohol Hexachlorophene Patient make surgery in ear complain of hear loss what type of dressing Alginate Hydrocolloids Hydrogel Vaseline dressing Patient removed tumor beside medial canthus and reached the infra orbital conjunctiva reconstruction from lateral canthus to infra orbital conjunctiva what type of flap Mustrade flap Periauricular lesion for surgical removal the patient had deafness what type of antiseptic contraindicated Ethanol Chlorohexidine Bovidine iodine A case of burn and CBC shows decrease Hb what to avoid Silver sulphadiazine cause neutropenia etas2019 Mupirocin Bacteriocin Type of antiseptic solution before face procedure Alcohol Ethanol Chlorohexidine Iodine Clinical case of two large masses on sole after excision what source of them a. Eccrine Apocrine Hair follicle Keratinocyte What site of flap to put after Moh's microsurgery to BCC beside nose? a. Cheeks Type of flap used beside nose a. Rotational b. Rhomboidal Picture of foot this lesion appeared after angiography which of the following may be elevated Creatine kinase Transferrin glutamase Alkaline phosphatase Eosinophilia Periauricular lesion near hair line for surgical removal what type of anesthesia should be used Chlorohexidine Deep cutting lesion in mm of mouth type of suture Nylon Silk Deep lesion in back sutures used Silk Nylon Prolene Polycarpone Note: 1st choice is vicryl (polyglactine) but if not present we choice prolene Blue leg after cardiac angiography a. Arterial embolism Venous embolism Cryoglobeanimea Female patient make liposuction in arms thigh back, buttocks most frequent complication: Compartimental syndrome Female patient make liposuction in all body what site of numbness Back Thigh Arm Male patient with trigeminal rhinotomy which is the remaining part Alanasi Tip of nose Nasolabial which type of flap a. Transposition Cut wound in lower lip from inside what type of suture Nylon Silk Prolene Rhomboid flap Island Pedicled flap Type of dressing in patient with secondary intention Alginate dressing Hydrocolloid dressing Hydrogel dressing Note: gauze then foam is the best Pyoderma gangrenosum treatment Cyclosporine but if systemic steroid is in answers it is the first option Infliximab Note: Biologic (infliximab, adalimumab, etanercept and ustekinumab) off label. Type of dressing a. Hydrocolloid Hydrogel Alginate Gauze Type of flap on picture a. Island pedicle flap Rotational flap Skin graft Case of cardiac patient with pacemaker make operation using: Electrocautery Poliglecaprone 15 is a. Absorbable suture Z-plasty Reorient scar Decrease length of scar Type of suture in deep wound in lower lip in child cut in an accident Silk Nylon Polyglactin 9/10 Surgical thread 4.0 has more advantage than 5.0 in Increase knot strength Increase tensile strength Smaller needle Cyanotic leg questions? Drug causing it: capecitabine/ Doxorubicin Type of cryoglobulin 1 Cause: cholesterol arterial emboli Topical anesthesia not used in ear surgery Prilocaine Bupivacaine Topical anesthesia in child having ear problem to use a. Prilocaine Male has this lesion for more than 3 months. He is on hydrochlorothiazide. asking about treatment? Intralesional steroid. Intralesional 5 fluorouracil. Surgery Interferon Same previous lesion on thumb only, asking about treatment? Intralesional steroid. Intralesional 5 fluorouracil. No need Interferon Peri auricular surgery with vestibular abnormalities, tinnitus, vertigo, what is note suitable antiseptic Chlorohexidine Dressing: Alginate if heavy exudates Hydrocolloid if moderate Hydrogel for dry Suture: Face Deep suture vicryl [polyglactin], poliglecaprone are nonabsorbable suture Superficial suture nylon, polypropylene is nonabsorbable suture Mucosa Deep suture none Surface suture silk, polyester nonabsorbable suture Back. Deep suture: vicryl (polyglactin) absorbable Trunk, Neck, limbs all same as face Notes: M plasty used to shorten scar Z plasty used to reorient scar, lengthen contracted scar, and relieve tension. S plasty used over convex area Bullous disease Bullous disease Pregnant female case bulla on trunk, flanks, umbilical, abdomen type of Ig (H. gestationus) IgG IgM C3 Pemphigus erythematosus positive ANA, C3&C4 what treatment? a. HCQ Colchicine MTX Note: if steroid is mentioned will be the correct choice Pemphigus vulgaris with clefts and protruding villi with high creatinine Steroid Cyclo Methotrexate Pemphigus vulgaris desmoglien 3 &1 positive and CD20 is zero what the ideal time for contraception in weeks: 6 9 24 48 Picture of newly born with umbilical cord stump still present and bullae around it what type of toxin Systemic toxin Local toxin Lamina densa defect in collagen Type 4 and anchoring fibrils ////////type 7 and dermal microfibrils DIF in PG IgG IgA IgM C3 Patient with vesicles and blisters hands for 8 years and in lower limb and hypertrichosis what is treatment (PCT) Phlebotomy Hydroxychloroquine Steroid Dapsone Cyclosporine Differences between HG and PUPP Pruritis Urticarial Recurrent after subsequence pregnancy Desmoglin 3 positive antibodies + multiple bullous eruption +oral treatment (??Pemphigus vulgaris) Prednisolone / or prednisolone +rituximab Patient 60 year with tense bullae no mucous membrane affection with subepidermal bullae IgG in floor what is next: Gastroendoscopy EBA…. Inflammatory bowel disease X ray A. Histopathology of blistering with IgA and neutrophil treatment a. Dapsone Histopathology of blistering with IgA and neutrophil with DM ،HTN وAF Dapsone Steroid Azathioprine IVIG Pregnant female with bullous pemphigoid takes 1 mg /kg of prednisolone with no improvement Delivery Increase dose to 2 mg/kg Azathioprine Antigen of cicatricial pemphigoid BPAG 2 col 17 laminin 5 epiligrin, alpha 6, beta 4 integrin Note: direct immune IgG, C3 linear on BM zone – indirect roof if BpAg2 floor if anti laminin 5 Cicatricial pemphigoid underlying malignancy type of Ag: Laminin 332 Cell with halo a. Merkel cell Lamina lucida a. Palm Anchoring filaments a. Lamina lucida Para neoplastic pemphigus associated with AML ALL CML CLL The most common is if in choices Non-Hodgkin lymphoma CLL Castleman’s disease Common antigen in paraneoplastic pemphigus a. BPAG 1 Plectin, Desmoplakin I, Desmoplakin II, BPAG1, Envoplakin and Periplakin. Bullous pemphigoid treatment biologic a. Rituximab Patient with bullous pemphigoid (investigation& الحالة وصف( investigation, elevated urea and creatinine Stop drug Add biologic IVIG Continue as before Most safe biologic during pregnancy treatment Infliximab Certolizumab Adalimumab Ustkinomab on treatment, then on Scenario of recurrent abdominal pain since childhood & picture of face & ears (EBA with Chron’s disease) Colchicine Dapsone c. Steroid d. Rivaroxaban Male pt with tensile bulla on arm with no mucosal lesion (EBA)since many years. Which organ will be affected (associated cancer)? Lung Stomach Liver Pancreas Pt developed bullae on arm after 3 days from starting sulfasalazine DIF –VE a. Acute generalized exanthem pustulosis Linear IgA Sneddon Sub-corneal pustular dermatosis Case DH on treatment, patient become sick with Hb=6 what type of investigation we will G6PD Reticulocyte count Cryoglobulin Salt split test separate skin at LL Lamina densa Case ------ epidermolysis bullosa Acustica associated with Hematological malignancy Pregnancy Case of Hailey Hailey disease mother and her daughter with axillary erosions and bad smell what treatment? Steroid Minocycline Others may be biologic or retinoids Granular deposition of IgA in dermal papillae along basement membrane (DH) what treatment Dapsone Bullous pemphigoid treatment biologic a. Rituximab Case of itchy skin several years hp show acantholysis with no dyskeratosis ask about lesion clinically will be Bull Plaque Papules Papulovesicular Painful blisters, generalized, WBCs 18000 CBC: neutrophilia treatment: Cyclosporine Steroid Case- child with bulla on extremities heal with scar defect where?? Sub-lamina lucida Sub-lamina densa Sub-corneal N.B. Casecokynetourain collagen 7 Hairand Pigmentary Hair and pigmentary Type of Keratin in hair follicle a. K 81 Diameter length of hair increase by day in mm a. 0.3 b. 1 Picture of androgenic alopecia in pregnant female stop drug before a. 1 month b. 6 months Picture of large area of alopecia in child scalp what is the management PUVA Intralesional steroid Minoxidil Female 3 years with female pattern hair loss take ttt preconception period of drug she takes is (note: minoxidil 2 weeks- spironolactone 1 month) 2m 4m 6m Area of loss of hair in the scalp with loss of follicular ostia and pustulosis ttt Isotretinoin + Rifampicin Isotretinoin and Dapsone Dapsone + Rifampicin Clindamycin + Rifampicin Child with large area of alopecia what type of ttt PUVA Injection steroid Minoxidil Picture of brittle hair ttt or picture of 7 years girl with brittle hair Minoxidil Iron Ca gluconate Hair epithelium stem cells (regenerating epithelium) a. Pulge Infundibulum Inner root sheath Photo mostly trichotilomania what found beside hair follicle Hemorrhage Lymphocytosis Eosinophils Neutrophils 2 photos of hair follicle female patient year complaing from receeding of frontal hair lines tt Steroid 2nd option hydroxychloroquine 1st option Minoxidil Swollen hair picture a. Iron Zinc MTX Cu (Menkes Syndrome) Twisted hair pilitorti Case of scarring alopecia with pustule best ttt: Rifampicin Tetracycline Dissecting cellulitis: follicular occlusion triad or tetrad with acne conglobate + hidradenitis + pilonidal sinus Ttt is isotretinoin case of scaring alopecia with pustules (folliculitis declvans)ttt: a. Rifampicin + Clindamycin. Isotretinoin + Rifampicin. Isotretinoin + Clindamycin. Isotretinoin + Dapsone. Folliculitis decalvans ttt Dapsone and rifampicin Isotretinion and rifampcin Hair lipid: eicosanoic Fatty acids then phytosphingosine then ceramide type of acid in hair sebum a. Sapeinic keratin type in hair a. Cystien what makes up the majority of protein envelop of keratinocyte a. Loricrin Involucrin Fillaggrin Envoplakin Picture of hair and case of pilli torti association a. Deafness b. Atopic Type of lipid in sebum when increase sebum increse Cholesterol Squalene Sebolic acid wax esters ttt of trichotillomania: a. N-acetyl cysteine Pili torti (trichscopy of twisted hair ) treatment Copper Zinc Iron Female 45years suffer from recession of frontal hair line what dose of 1st line of Ttt a. 5mg 1 mg 2 mg 3 mg Case of folliculitis decalvans (increase urea + increase creatinine , normal CBC Minocycline Doxycycline Rifampicin Isotretinoi Female with androgenetic alopecia with high DAHEA, high testosterone and rest is normal what to suspect Adrenal tumor Ovarian tumor Thyroid tumor FFA the best ttt: Minoxidil Prednisone Hydroxychloroquine History of 3 years duration in 50yrs female with oral dyschromia with Hyperkeratotic lesions on hands and Hypopigmented trunk lesions Contaminated well water Hydrocarbon Picture of female with androgenetic alopecia and high DHEA and high testosterone and the rest are normal Adrenal tumor Ovarian tumor Female with hair line recession, Ttt Cyclosporin Acitirin Hydroxychloroquine Picture of area of scalp beside ear with scales (no loss of hair & erythema) Psoriasis Tinea capitis Irritated atopic dermatitis Seborrheic dermatitis Photo of twisted hair ask about disease …pili torti Menkes disease Bjornstand syndrome Picture of hair collected as tuft (pityriasis amantacia) but with pus: a. Psoriasis Seborrheic dermatitis Tinea capitis Genodermatoses Genodermatoses Female child with dermoid cyst: If in midline MRI If in lateral eyebrow no investigation Picture of female child looks old the defect is (AR if cockyne and AD if progeria) AD AR XLD XLR Note: microcephaly is cockayne / large head is progeria Progeria…AD …. LMNA gene cockayne…AR… ercc8, ercc6 Old featured girl picture gene mutation a. Fibulin 5in cutislaxa b. fibroblast growth factor Scaly erythematous ski lesions+ hair defect distal enter in proximal gene defect (Netherton) األجابه الى تيجى صح LEKT1 or 5 SPINK a. b. FAID H Patient had defect SPRED1 most common (Legius syndrome) Neurofibroma Optic glioma Renal cell carcinoma Macrocephaly Note: if lipoma present Lesion in child head small as aplasia cutis what the defect a. Brain ectopic tissue/ neural tube defect Multiple yellowish papule in neck and axilla and angoidstreaks (PXE) Aspirin Simvastatin ttt if CVS involved Steroid Stop smoking prophylactic CVS not involved may be right Cancer colon in father and 2 sons and one son is normal incidence Zero b. 25% c. 50% d. 100% Picture of AR inheritance with lentigo which syndrome?? Tays AR (Like TTD without photosensitivity and normal gonads) Carney Pinpk syndrome Blender syndrome AD Picture of striated nail (darrier) a. AD AR XLD XLR Darrier exacerbated by a. Lithium Ichthyosis +MR + deafness (Mednik Syndrome) Keratitis with KID syndrome but no MR Enteropathy + peripheral neuropathy MEDNIK (MR-enteropathy- deafness-neuropathy-ichthyosis-keratoderma) Father + Mother are normal but baby has syndrome incidence Zero b. 25% c. 75% d. 100% Pigmentation in all body not in mucous membrane what syndrome Peutzjeugher Cornhite Canada Syndrome (pigmentation all body + mm + Git polyposis) Huziquer syndrome Leopard syndrome if not present Cowden syndrome تصحيح Ichthyosis with low estradiol AR AD XLD XLR Papule on mandibulae investigations (popular sarcoidosis) Ca ACEI Lamellar ichthyosis a. AR Picture of axillary erythematous skin papules associated with a. Medulloblastoma b. Cardiac problems If like picture Gorlin syndrome PXE associated with Cardiomyopathy Peripheral vascular disease Peripheral claudication PPK+ cancer mucous membrane affection (Howel Evans syndrome) XLR XLD AD AR Hailey and Hailey: Diagnosis (case of erythematous vesicular lesion with acantholysis mother & daughter with bad odor in intertriginous, flaccid vesicles) Investigations histopathology (acantholysis with supra-basal cleft and depilated brick wall, mild dyskeratosis &DIF is negative) Management…Minocycline Picture of nails hyperkeratotic with history of father and daughter what ttt: a. Acitretin b. Topical steroid Picture of a man with café au lait macules + lesion on scapula the most associated anomalies (Mccune Albright syndrome) Scoliosis Lung deformity Creatine kinase enzyme found in a. Dermatomyositis Child with atopy + developmental retardation + ichthyosis what is the syndrome a. Sjogren Larsson b. Netherton Child with Langerhans cell histiocytosis refer to Ophthalmologist Neuro Pediatric Orthopedic Description of a case ???Grover’s disease asks about type of lesion Popular Papulovesicular Vesicular Sjogren Larsson syndrome (MR + ichthyosis + spastic paraplegia) Picture of hand with PPK type of keratin a. K9 K10 K5 Type of cataract in atopic dermatitis a. More common post subcapsular b. More specific ant subcapsular Stimulation of KCs cycle a. IL1 b. TNF Case of Hailey-Hailey disease (mother and her daughter with axillary erosion and bad smell), what is histopathology? Acantholysis with dyskeratosis if Grover’s disease Acantholysis without dyskeratosis Case of multiple bcc with milia: Gorlin Rombo Basex (milia with trichoepithelioma and hypohydrosis) PPK (focal+ mucous patches on mouth and tongue) what’s inheritance? AD Tumor on nose as SCC or keratoacanthoma (Muir Torre syndrome)/ Mismatch repair gene: 1 2 3 4 Picture of small girl has small yellow nodule on scalp, what is another site for this lesion? Eye Liver Brain Lung Picture of old man has multiple brown patches on back and hyper keratotic palmoplantar and oral. Gorlin Darrier Picture of palms [PPK] since birth what type of keratin K6 K1 K9 Skin lesion described (hyperpigmentation) with polyposis in colon no mm affection a. Cowden disease The parent’s phenotype appears normal and the son has a disease ratio of occurrence in other siblings a. 25 % b. 50 % Picture mutation gene periorbital edema and sagged cheeks a. Fibulin 5 if Cutis laxa b. ABCC6 gene if PXE Yellowish discoloration of all toe nail associated with Liver disease Lung disease GIT disease Renal Eye Girl with dark pigmentation on lip and his father and two sisters has history of colon cancer and one sister is normal what percentage of transmission to her siblings: 0 % b. 50 % c. 100 % d. 10 % Ichthyosis since birth + mental retardation = If with atopy / Netherton syndrome If with spastic paraplegia / Sjogren Larsson syndrome If with enteropathy + neuritis / MEDNIK syndrome Hailey-Hailey disease histopathology discerption: acantholysis with dyskeratosis most common site a-Axilla Female with bifid upper 3 rib and hemivertebra in upper 3, What is the gene defect? a. Gorlin-Goltz syndrome /PTCH1 /AD Ch9 Picture of the baby 8 months with syndrome & developmental delay (Netherton syndrome) will have Food allergy. if erythematous it is Netherton Keratitis (if with Dry or scratching & MR it might be Sjogren Larsson syndrome.) Muscle weakness N.B no atopy or hair abnormalities were in answers Child has syndrome and his father and mother are normal percentage of his brother has same lesion (AR) 0 25 50 A girl with lesion beside lat canthus associated with Developmental delay Tonic clinic convulsions Contralateral motor seizure Decreased IQ Male patient 44 years old has dark red itchy lesion on arms and buttocks what is the cause AIDS Wilson disease Cokayne syndrome Vit A deficiency Female with uterine bleeding this lesion is desmin and smooth muscle positive: gene defect Telomer Krebs cycle Enzyme Baby having hypotrichosis and atopy and scaly skin his parents are normal what are the chances that the NEXT sibling while be diseased??? zero 25 50 Gingival papules of Cowden investigation a. Neck exam or thyroid scan Note: if not mentioned choose breast Type of renal cancer in birt hug dube a. Chromophobe Rash and cardiomegaly biologic hyper- eosinophilic syndrome Imatinib histopathological picture of steatocytoma ass with a. Pachyonychia congenita Subungual hyper keratosis of toe nail and focal PPK and mucous patch on tongue. (pachyonychia congenita) AD AR XLR XLD Picture of nail in boy and similar condition in his sister with high TSH and low T3, T4 asking about TTT Acitretin Fluconazole Note: if diagnosis is chronic mucocutaneous candidiasis with hypothyroidism (AR) so ttt is antifungal Other possible diagnosis: Hyperhidrotic ectodermal dysplasia with hypothyroidism and ciliary dyskinesia is a rare condition characterized by alopecia (hair loss); nail dystrophy (abnormal development of the nails); ophthalmic complications; (primary hypothyroidism); hypohidrosis;(freckles); enteropathy, and respiratory tract infections due to ciliary dyskinesia. it is thought to be inherited in an autosomal recessive manner. Treatment is generally symptomatic and supportive. Which of these granulomatous lesions in Not ass with cholesterol clefts? a. Necrobiosis lipoidica and JXG Board vital All of the following conditions contain needle-shaped clefts EXCEPT: Subcutaneous fat necrosis of the newborn Post-steroid panniculitis Cholesterol emboli Sclerema neonatorum Juvenile xanthogranuloma Explanation: Juvenile xanthogranulomas are composed of a mixed infiltrate of histiocytes, lymphocytes and eosinophils. Foamy histiocytes and Touton giant cells are characteristic. Fibrosis is prominent in older lesions. They exhibit positive staining of CD68, factor 13a, and HAM56. They do not contain needle shape clefts. All of the other conditions listed contain needle-shaped clefting. Which of the following syndromes is not a consideration for a patient presenting with the findings illustrated below? Peutz-Jeghers Laugier-Hunziker Cronkhite-Canada Carney Complex LEOPARD Syndrome Explanation: LEOPARD syndrome (lentigines, EKG abnormalities, ocular hypertelorism, pulmonic stenosis, abnormal genitalia, retardation of growth, and sensorineural deafness), presents with dark 1-5 mm lentigines appear anywhere on the skin, including the palms and sole, with a greater predilection for the neck and upper trunk. These lentigines spare the oral mucosa, thus eliminating LEOPARD syndrome from the differential diagnosis of a patient presenting with oral melanotic macules. In the POEMS syndrome, which skin finding(s) are you likely to encounter? Rapid appearance of seborrheic keratosis Glomeruloid hemangiomas, hypertrichosis and hyperpigmentation Erosive disease of the mucosa Large red to violet-brown patch Concentric erythematous lesions, often giving the appearance of grains of wood Explanation: Skin findings in the POEMS syndrome include: glomeruloid hemangiomas, cherry angiomas, sclerodermoid changes, digital clubbing, hypertrichosis, and hyperpigmentation. The POEMS acronym stands for (polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes). Osteosclerotic myeloma, Castleman’s disease, and plasmacytomas have been reported in patients with POEMS. The rapid appearance of seborrheic keratosis is called the sign of Leser-Trélat and is controversial. It may be associated with gastric carcinoma. Erosive disease of the mucosa is seen in paraneoplastic pemphigus. A large red to violet-brown patch is seen in AESOP (adenopathy, extensive skin patch overlying (a) plasmacytoma). Concentric erythematous lesions, often giving the appearance of grains of wood are the typical description of erythema gyratum repens, sometimes said to be associated with pulmonary cancer. Histiocytosis are identified with markers, select the combination of positive and negative markers associated with Langerhans cell histiocytosis. S100- CD1a- Langerin (CD207)- CD68+ Factor XIIIa+ S100+ CD1a- Langerin (CD207)- CD68+ Factor XIIIa+/- S100+ CD1a+ Langerin (CD207)- CD68+ Factor XIIIa+/- S100+ CD1a+ Langerin (CD207)+ CD68- Factor XIIIa- S100- CD1a- Langerin (CD207)- CD68+ Factor XIIIa+/- Explanation: Langerhans cell histiocytosis (LCH) is described by an immunophenotype positive for S100, CD1a and Langerin (CD207), and which contain cytoplasmic Birbeck granules. The other profiles correspond to: S100- CD1a- Langerin (CD207)- CD68+ Factor XIIIa+ for juvenile xanthogranuloma (and other non-Langerhans histiocytosis). S100+ CD1a- Langerin (CD207)- CD68+ Factor XIIIa+/- is the typical profile for Rosai-Dorfman disease. S100+ CD1a+ Langerin (CD207)- CD68+ Factor XIIIa+/- is the profile of indeterminate cell histiocytosis. S100- CD1a- Langerin (CD207)- CD68+ Factor XIIIa+/- is the profile of necrobiotic xanthogranuloma. The lesions depicted below can be associated with which of the following? Rombo syndrome Brooke-Spiegler syndrome Bazex-Dupre-Christol syndrome A & B All of the above Explanation: All of the above syndromes can present with trichoepitheliomas. Trichoepitheliomas are benign tumors that present as discrete papules along the nasolabial folds. Rombo syndrome presents with trichoepitheliomas in addition to milia, hypotrichosis, basal cell carcinomas and atrophoderma. Brooke-Spiegler syndrome presents with multiple trichoepitheliomas and cylindromas. Bazex-Dupre-Christol syndrome is an X-linked dominant disorder that presents with follicular atrophoderma, basal cell carcinomas, trichoepitheliomas, hypotrichosis and hypohidrosis. What is the most commonly occurring associated condition in patients with POEMS syndrome? Basal cell carcinoma Kaposi Sarcoma Squamous cell carcinoma Castleman’s Poroma Explanation: POEMS, also known as Crow-Fukase syndrome is a rare, paraneoplastic syndrome that manifests as polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes such as diffuse hyperpigmentation, dependent edema, skin thickening, hyperhidrosis, and hypertrichosis. It can have associated micro-venular and glomeruloid hemangiomas and can have associated Castleman’s syndrome in up to 25% of cases. Non-Langerhans histiocytosis can be classified using four groups: primarily cutaneous, usually self-resolving, primarily cutaneous, often persistent/progressive, cutaneous, with frequent systemic involvement and systemic, with skin involvement rare to unusual. Identify the non-Langerhans Cell Histiocytosis that has cutaneous manifestations with frequent systemic involvement in the following choices: Erdheim–Chester disease Hereditary progressive mucinous histiocytosis Giant cell reticulo-histiocytoma Letterer–Siwe disease Multicentric reticulohistiocytosis Explanation: Histiocytosis are classified the following way: Langerhans Cell Histiocytosis Letterer–Siwe disease Hand–Schüller–Christian disease Eosinophilic granuloma Congenital self-healing reticulohistiocytosis (Hashimoto–Pritzker disease) Non-Langerhans Cell Histiocytosis Primarily cutaneous, usually self-resolving Juvenile xanthogranuloma Benign cephalic histiocytosis Giant cell reticulohistiocytoma Generalized eruptive histiocytoma Indeterminate cell histiocytosis Primarily cutaneous, often persistent/progressive Popular xanthoma Progressive nodular histiocytoma Hereditary progressive mucinous histiocytosis Cutaneous, with frequent systemic involvement Necrobiotic xanthogranuloma Multicentric reticulohistiocytosis Rosai–Dorfman disease Xanthoma disseminatum Systemic, with skin involvement rare to unusual Erdheim–Chester disease Sea-blue histiocyte syndrome (sea-blue histiocytosis) (inherited or acquired) Massive but painless bilateral cervical lymphadenopathy, fever, anemia, elevated erythrocyte sedimentation rate, neutrophilia and a polyclonal hyper-gamma- globulinemia in a 10-year-old boy are all features that orient toward the diagnosis of which of the following choices? Eosinophilic granuloma Hand–Schüller–Christian disease Rosai–Dorfman Disease Erdheim–Chester disease Sea-blue histiocyte syndrome Explanation: Children and young adults are most commonly an affected by Rosai-Dorfman disease. It is rare. Massive, painless bilateral cervical lymphadenopathy is characteristic. Some cases are made of extranidal sites, including the skin, and can be the sole manifestation of the disease. A skin-limited form of Rosai-Dorfman disease is increasingly being recognized. In its systemic form, cutaneous lesions occur in less than 10% of cases. They are usually multiple, and are clinically nonspecific. Fever and polyclonal hypergammaglobulinemia are often seen. In the lymph nodes and the skin, emperipolesis is a common histologic feature (engulfment of intact lymphocytes and plasma cells by the histiocytes). Many lesions are asymptomatic and heal spontaneously, and do not requiring treatment. If treatment is indicated due to destructive lesions, disseminated disease or lesions causing physical compromise, the following treatments can be considered: radiotherapy, surgical excision, systemic corticosteroids and alkylating agents. There are also a few isolated reports of improvement with thalidomide. Again, due to the rarity of the disorder, there are no controlled trials to compare the options. Eosinophilic granulomas consist mainly in bone lesions. Hand–Schüller– Christian disease consists in scalp, flexural areas, trunk and gingival lesions. The classic triad is: diabetes insipidus, bone lesions and exophthalmos. Erdheim–Chester disease occurs at any age. Skin involvement is found in 25% of patients with red-brown to yellow nodules and indurated plaques on eyelids, scalp, neck, trunk and axillae. It has features of fever, bone lesions, exophthalmos, diabetes insipidus as well involvement of the lungs, kidneys, adrenals, heart, central nervous system, retroperitoneum and testes. A high mortality rate is reported. Sea-blue histiocyte syndrome is found adolescents and young adults in the inherited form. Skin involvement is rare in the inherited form. Facial macular hyperpigmentation and nodules can be found. Histiocytes contain cytoplasmic granules that stain azure blue with May–Gruenwald stain. Multiple organs are involved and it can be fatal. Nail Nail Picture of longitudinal groove in nail (show parrot peek) in Synovitis Osteoarthritis Rheumatoid arthriti If longitudal groove >> papilloma or myxoid cyst If myxoid cyst is ass with osteoarthritis If parrot beak >>systemic sclerosis or repeated trauma (peripheral neuropathy) Picture of hyperkeratotic nail slightly bending downward what is association: a. Osteoarthritis Rheumatoid arthritis Psoriasis Oil spot in nail a. Bed Matrix Plate Nail pitting a. Matrix What forming distal part of nail a. Ventral nail fold Dorsal part of nail plate a. Proximal matrix Distal matrix Nail bed Nail fold Onycholysis due to Doxycycline or Tetracyclines Picture of 10 nails affected (Psoriasis??) associated with a. Arthritis problem Same previous picture ttt MTX Acitretin Intralesional steroid Etanercept If only bed: Topical If matrix: IL steroids (few nails) Acitretin (many nails ) If other indication (like arthritis): MTX, Biologics, Cys A. Nail lesion in patient and his sister 3 fingers ttt 1. Acitretin 2. Antifungal If pachyonychia congenitaز Psoriasis in nail and palm ttt a. Acitretin b. MTX Best treatment nail psoriasis May be biologic: Adalimumab / Etanercept Single band of longitudinal erythronychia Distal margin may be fissured common benign tumors? Onychopapilloma Myxoid cyst ( (description). What complication a. Osteoarthritis Photo of Darier's nail Psoriatic nail If Acitretin not in choices choose MTX Picture of nails dystrophic hyperkeratotic with history of boy & sister with hypothy
