Genetic Disorders Quiz: Legius Syndrome, McCune-Albright Syndrome, and Macular Amyloidosis

ProtectiveFlashback avatar
ProtectiveFlashback
·
·
Download

Start Quiz

Study Flashcards

411 Questions

What is the expected finding in the epidermis of a case of Wilson's disease?

Ceramide (globotriaosylceramid)

In a case of Fabry's disease, what are the deposited or accumulating substances?

Ceramide (globotriaosylceramid)

What is the prophylactic treatment for a case of photosensitivity?

Hydroxychloroquine

In a case of familial SLE, which C deficiency is associated with the condition?

C4

In systemic amyloidosis, what type of light chain is typically involved?

Lambda

In a case of scleroderma with lung fibrosis, what type of antibodies should be investigated?

Anti-SCL70

Which enzyme is involved in the immediate darkening of the skin?

311

What is the most common collagen in adult skin?

3

In the treatment of hemangioma in a 1-year-old infant weighing 15 kg, what is the appropriate dosage of propranolol?

30 mg propranolol

What makes up the majority of the protein envelope of keratinocytes?

Loricrin

In the case of vasculitis with dilated follicular opening (granuloma faciale), what is the recommended treatment?

Dapsone

What is the most likely cause of erythema abigne on the back?

Heat pads (Hot blankets)

Which section of the Dermatology Prometric Exam would cover the topic of 'Vascular'?

Vascular

What is the total number of pages dedicated to the 'Connective Tissues' section in the Dermatology Prometric Exam?

9

Which part of the Dermatology Prometric Exam includes information on 'Ethics and Safety'?

Ethics and Safety

What is the title of the section that includes information on 'Granuloma' in the Dermatology Prometric Exam?

Granuloma

Which type of filler is described as long-lasting or permanent in the 'Cosmetology and Lasers' section of the Dermatology Prometric Exam?

Silicon

In a patient with varicose veins and severe pain and swelling after injection, what is the next appropriate step?

Doppler Compression

In a child with varicose veins and one limb larger than the other, what is the next appropriate step?

Doppler ultrasound

In a case of varicose vein with pain in the lower leg, which investigation is most appropriate?

Doppler ultrasound

A patient with bilateral vascular ulcer but no vasculitis. What is the most common cause?

Factor V Leiden

In a patient treated for erythema nodosum with an unknown medication and presenting with eczema, what is the likely drug used for treatment?

Potassium iodide

In a patient with livedo racemosa, purpura, increased urea and creatinine, and pulmonary problems, what type of ANCA (with MPA) is likely to be present?

Cytoplasmic ANCA

In a patient with macular amyloidosis, which investigation would be most appropriate?

MRI

In a case of Langerhans cell histiocytosis, which investigation would be most accurate if the skull or cranium is not present?

Leg X-ray

In a patient with skin-colored papules and elastic skin on the neck and angioid streaks on the retina, what would be the most appropriate next step?

Biologic therapy

In a case of macular amyloidosis, what would be the most relevant investigation?

Not applicable

In a patient with vesicular eruption on the hand, arm, and face with no inflammatory infiltrate by histopathology, what would be the most appropriate treatment?

Phlebotomy if PCT is present

In a case of scleromyxoedema with waxy papules on the face and upper back, what would be the most appropriate treatment?

Not applicable

A patient with Reynaud's phenomenon, cyanosis in fingers, edema, low C3 and C4, and positive ANA would most likely benefit from which treatment?

Hydroxychloroquine

What is the maximum recommended dose of hydroxychloroquine for a patient with discoid lupus erythematosus (DLE)?

New guidelines recommend 6.5 mg based on ideal body weight

A 10-year-old child presents with linear morphea. What treatment would you combine with steroids for this condition?

NBUVB (Narrowband UVB)

What is the most common site for lichen sclerosus et atrophicus?

Trunk

After Moh's microsurgery to remove basal cell carcinoma (BCC) beside the nose, what type of flap would be used?

Cheeks

In liposuction, which organ is most affected?

Abdomen

A patient with a painful ulcer and yellowish discharge would benefit most from which type of dressing, based on exudate quantity?

Alginate

What dressing would be used for a copious exudate after excising a tumor beside the eye and leaving it for secondary intention for two weeks?

Hydrocolloid

What is the most common cause of bilateral vascular ulcer without vasculitis?

Factor V Leiden

In a patient with sweet syndrome presenting with a painful lesion on the hand and abnormal lab results, how long does it take for white blood cells to recover?

6 months

In a 6-year-old boy with indurated nodule and high neutrophil & WBC count but no vasculitis, what is the next appropriate step?

Histopathology EN

What is the association of a patient with a lesion brownish or violaceous in the lower limb and a similar lesion in his brother?

Factor 5 gene defect

In a male patient with plaque pigmented on his leg and positive Kaposi and hhv8, what treatment is indicated?

Sirolimus

What is the most appropriate next step for a male patient aged 51 with cyanotic leg after angiography to confirm the diagnosis?

Eosinophilia

A 60-year-old patient with waxy papules, ecchymosis, and macroglossia may be suspected of having systemic amyloidosis. What is the next step?

Blood or urine electrophoresis

What is the total number of pages dedicated to the 'Surgery' section in the Dermatology Prometric Exam?

7

In the 'Vascular' section of the Dermatology Prometric Exam, which chapter has the highest number of pages?

Infection

In the 'Ethics and Safety' section, how many pages are dedicated to 'Ethics and Safety' in the Dermatology Prometric Exam?

5

What is the total number of pages dedicated to the 'Granuloma' section in the Dermatology Prometric Exam?

3

In the 'Eczema and Hypersensitivity' section, how many pages are dedicated to 'Eczema and Hypersensitivity' in the Dermatology Prometric Exam?

5

In the 'Vascular' section, which chapter has the lowest number of pages?

Acne

What is the total number of pages dedicated to the 'Metabolic' section in the Dermatology Prometric Exam?

21

In the 'Genodermatosis' section, how many pages are dedicated to 'Genodermatosis' in the Dermatology Prometric Exam?

14

How many chapters are there in the Dermatology Prometric Exam that have a single page?

3

What is the total number of pages dedicated to the 'Board Vital' section in the Dermatology Prometric Exam?

2

Which enzyme is involved in the immediate darkening of the skin?

Melanin increase

What is the pulse duration of laser used in treatment of Haemangioma?

2

In a patient with solar urticaria, which wavelength is likely to be associated with the condition?

280 UVA2

What type of filler is the longest lasting filler?

Ca hydroxyapatite

For the treatment of a nevus of Ota, which type of laser is commonly used?

1064 q-switched ND yag

Which wavelength is responsible for immediate tanning of the skin?

311

What is the most common collagen in adult skin?

Type 1

In the treatment of melasma, which type of laser is commonly used with ALA and red light?

Red

What makes up the majority of the protein envelope of keratinocytes?

Fillaggrin

What type of laser is commonly used in PDT for the treatment of acne?

Visible

What is the maximum dose of hydroxychloroquine for a patient with discoid lupus erythematosus (DLE)?

6.5 mg ideal body weight

In a patient with cyanosis in fingers (Reynaud’s), edema, low C3 and C4, and positive ANA, what is the best treatment?

Cyclosporine

What is the most common site for lichen sclerosus et atrophicus?

Face

A 65-year-old female on renal dialysis complains of sudden waxy brown papules on the trunk. What is the most associated cancer (Laser trait)?

Pancreas

In a patient with subacute LE, which manifestation is not associated with the condition?

Eosinophilia

What manifestation is not associated with subacute LE?

Eosinophilia

In a patient with linear morphea, what treatment should be combined with steroids?

MTX

What investigation should be performed for atrophic lesions with telangiectasia on the surface?

Skin biopsy then DIF then ANA

What dressing should be used for a copious exudate after excising a tumor beside the eye and leaving it for secondary intention for two weeks?

Alginate

What enzyme is involved in the immediate darkening of the skin?

Tyrosinase

What is the most appropriate next step for a female patient with macular amyloidosis?

Ophthalmologist

In a patient with macular amyloidosis, what is the associated investigation?

Calcitonin level

In a patient presenting with a picture of Juvenile xanthogranuloma, what would be the most appropriate next step?

Dermatologist

In a patient with itching interscapular brownish patch with rippling (amyloidosis), which organ is associated?

Kidney

What gene defect is associated with a patient presenting with erythema and bone pain, histological finding of epithelioid granuloma and few lymphocytes (Blau syndrome)?

TNFR1 gene

In a patient with bilateral nodules on the Achilles tendon (tubers xanthoma), what investigation would be most appropriate?

Cholesterol level

In a 40-year-old female with generalized purpuric eruption all over the body and sole ulcer, what would be the most appropriate treatment?

Methotrexate

In a patient with recurrent abdominal pain since childhood, what would be the most appropriate treatment?

Colchicine

In a case of Wilson's disease, what would you expect to find in the epidermis?

Elastic fiber

In a case of PXE (Pseudoxanthoma elasticum), what would you expect to find in association with no claudication?

Peripheral vascular disease

In a patient with erythematous purple papules in the lower abdomen and inner thigh, and his brother experiencing the same, what deposited or accumulating substances are likely involved?

Ceramide (globotriaosylceramide)

In a case of photosensitivity, what is the most appropriate prophylactic treatment?

Hydroxychloroquine

In a case of granuloma with few lymphocytes and an erythematous plaque, what would be the appropriate biologic treatment?

Rituximab

In a patient with dermatomyositis controlled on prednisolone and methotrexate (MTX) intake started for one week, what would be the next appropriate step if myopathy develops?

Increase steroid

In a case of familial systemic lupus erythematosus (SLE), which deficiency is most commonly associated?

C4

In a patient with Gottron rash on both hands and ears, what would be the appropriate treatment?

Methotrexate

In a case of small ulcer on the digit with fibrosis and pigmentation, which investigation would be most appropriate?

(ANA) Antinuclear antibodies test

In a case of photosensitivity, what investigations would be most associated with connective tissue diseases?

Double-stranded DNA test

In a scenario of limited scleroderma, what investigation would be most appropriate?

Anticentromere test

In a case of discoid lupus with follicular plugging, which investigations would be most relevant?

ANA test

What is the total number of pages dedicated to the 'Eczema and Hypersensitivity' section in the Dermatology Prometric Exam?

12

In a patient with solar urticaria, which wavelength is likely to be associated with the condition?

UVA

In a case of Fabry's disease, what are the deposited or accumulating substances?

Sphingolipids

What gene defect is associated with a patient presenting with erythema and bone pain, histological finding of epithelioid granuloma and few lymphocytes (Blau syndrome)?

CARD15

In a case of Langerhans cell histiocytosis, which investigation would be most accurate if the skull or cranium is not present?

MRI

What is the prophylactic treatment for a case of photosensitivity?

Sunscreen with high SPF

In the treatment of nasolabial fold, which filler is the longest lasting?

Calcium hydroxyapatite

In a patient injected with filler and botox who experiences blanching on the forehead, what is the first step if pain is also reported?

Hyaluronidase injection

Which type of laser is commonly used in the treatment of nevus of Ota?

1064 q-switched ND yag

What is the enzyme involved in the immediate darkening of the skin?

Tyrosinase

In a case of hemangioma in a 1-year-old infant weighing 15 kg, what is the appropriate treatment?

15 mg propranolol

What makes up the majority of the protein envelope of keratinocytes?

Involucrin

What is the gene defect associated with Blau syndrome, presenting with erythema and bone pain, and histological finding of epithelioid granuloma and few lymphocytes?

Carb 15 mutation

In a case of systemic amyloidosis, what type of light chain is typically involved?

IgG lambda

In a patient with cutaneous amyloidosis, what association is most likely?

Renal

What is the most common cause of bilateral vascular ulcers without vasculitis?

Pseudoxanthoma elasticum (PXE)

What enzyme defect is associated with angiokeratoma and involves hyperkeratosis and rete ridges encircling dilated vessels in the papillary dermis?

Galactosidase A

In a case of Langerhans cell histiocytosis, which investigation would be most accurate if the skull or cranium is not present in the X-ray?

Spine

What is the most common cause of bilateral vascular ulcer without vasculitis?

Antithrombin III deficiency

In a 6-year-old boy with an indurated nodule and high neutrophil & WBC count but no vasculitis, what is the next appropriate step?

Bone marrow biopsy

Patient after doing angiography developed cyanotic leg. What is most associated with this condition?

Neutropenia

A 65-year-old female on renal dialysis complains of ulcers at the tendon Achilles. What treatment would be suitable?

Sodium thiosulphate

Picture of a patient with a lesion brownish or violaceous in the lower limb. What is the association of this condition?

Thrombin gene defect

Which type of ANA is associated with a case of vasculitis with lung affection away from the lumen of the airway?

Cytoplasmic

In a patient with widespread superficial nodules with atrophied surfaces and surface telangiectasia, what is the most likely diagnosis?

LE panniculitis

In a patient with an asymptomatic maculopapular lesion on the left arm, what would be the most appropriate treatment?

Phototherapy

In a patient with an erythematous plaque on the nose and pustules on the plaque, what is the most appropriate treatment and diagnosis?

Acitretin

In a case of morphea 10 years ago, what would have been the most appropriate treatment?

Tacrolimus

In a female with a genital plaque and no symptoms, what would be the most appropriate treatment?

Cutis laxa mutation

In a patient with an asymptomatic skin rash on the upper and lower limbs with an atrophic center, what is the most likely diagnosis?

Morophoea

What is the maximum recommended dose of hydroxychloroquine for the treatment of DLE?

New guidelines 2016

In a patient with cyanosis in fingers (Reynaud’s), edema, low C3 and C4, and positive ANA, what is the best treatment?

MTX

What is the most likely associated cancer for a 65-year-old female complaining of sudden waxy brown papules on the trunk?

Liver

What is the best treatment for a female patient with brown macular rash on the cubital fossa?

Cyclosporine

Which manifestation is not associated with subacute LE?

Scarring

What is the best treatment to combine with steroids for linear morphea in a 10-year-old child?

Mofetil

What dressing is best for a painful ulcer with yellowish discharge, based on exudate quantity?

Alginate

In a case of pyoderma gangrenosum with heavy discharge, what dressing should be used?

Hydrocolloid

What is the most likely source of two large masses on the sole after excision?

Eccrine

What is the most appropriate type of flap to use beside the nose after Moh's microsurgery to remove basal cell carcinoma (BCC)?

Forehead

Which section of the Dermatology Prometric Exam covers the topic of 'Genodermatosis'?

Genodermatosis

In the 'Eczema and Hypersensitivity' section, how many pages are dedicated to 'Eczema and Hypersensitivity' in the Dermatology Prometric Exam?

10

What is the most likely cause of erythema abigne on the back?

Granuloma faciale

In a patient with an erythematous plaque on the nose and pustules on the plaque, what is the most appropriate treatment and diagnosis?

Granuloma faciale, surgical excision

What gene defect is associated with a patient presenting with erythema and bone pain, histological finding of epithelioid granuloma and few lymphocytes (Blau syndrome)?

NOD2/CARD15

In a patient with widespread superficial nodules with atrophied surfaces and surface telangiectasia, what is the most likely diagnosis?

Cutaneous amyloidosis

What enzyme is involved in the immediate darkening of the skin?

Tyrosinase

What is the most common site for lichen sclerosus et atrophicus?

Genitalia

In a case of Wilson's disease, what would you expect to find in the epidermis?

Copper deposits

What is the total number of pages dedicated to the 'Surgery' section in the Dermatology Prometric Exam?

31

What is the most common cause of bilateral vascular ulcer without vasculitis?

Erythema nodosum

In a patient with livedo racemose, purpura, increased urea and creatinine, and pulmonary problems, what type of ANCA (with MPA) is most likely present?

Perinuclear

After Moh's microsurgery to remove basal cell carcinoma beside the nose, what type of flap would be used?

Axial pattern flap

In a 6-year-old boy with an indurated nodule and high neutrophil & WBC count but no vasculitis, what is the next appropriate step?

Bone marrow biopsy

What is the first indicator of rapid involuting hemangioma (RICH)?

$CD133$

In a case of lipoid proteinosis with hoarseness of voice, what is the treatment of choice?

$Acitretin$

What is the enzyme involved in the immediate darkening of the skin?

$Creatine kinase$

In a 60-year-old patient with multiple waxy papules on the trunk and lower limb with ecchymosis and bruises, what is the next step?

$Bone marrow biopsy$

In a patient with cyanosis in fingers (Reynaud’s), edema, low C3 and C4, and positive ANA, what is the best treatment?

Cryoglobulin type1

In a case of vasculitis with lung affection away from lumen of airway, what type of ANA is most likely present?

Nuclear

What is the most likely associated cancer for a 60-year-old patient with multiple waxy papules, ecchymosis, and macroglossia?

Bone marrow biopsy

In a patient with varicose veins and severe pain and swelling after injection, what is the next appropriate step?

Doppler Compression

Which of the following is expected to be found in the epidermis of a case of Wilson disease?

Mucopolysaccharide

A patient with photosensitivity is likely to be treated with which prophylactic measure?

Hydroxychloroquine

In a case of granuloma with few lymphocytes, what would be the appropriate treatment?

Infliximab

In a patient with dermatomyositis controlled on prednisolone and methotrexate (MTX) intake, started for one week of myopathy, what would be the next appropriate step?

Increase steroid

What type of light chain is associated with systemic amyloidosis?

Lambda

In a case of lichen sclerosis et atrophicus, what would be the most appropriate topical treatment?

Tacrolimus 1

In a scenario of limited scleroderma, what investigation would be appropriate?

Anticentromere

In a case of discoid lupus with follicular plugging, what would be the most appropriate investigation?

Hydroxychloroquine

Which joint complication is associated with Ehler-Danlos syndrome characterized by hyperextensibility of skin on the neck and defect in collagen?

Hip dislocation

A female with genital plaque and no symptoms is most likely to be treated with:

No treatment

A patient with erythematous plaque on the nose and pustules on the plaque is likely to be treated with:

Acitretin

A case of morphea 10 years ago would most likely have been treated with:

Steroid

Which type of laser is commonly used in the treatment of nevus of Ota?

1064 q-switched ND yag

In the treatment of nasolabial fold, which filler is the longest lasting?

Ca hydroxyapatite

What enzyme is involved in the immediate darkening of the skin?

Transglutaminase

What is the most appropriate next step for a female patient with macular amyloidosis?

In a case of Fabry's disease, what are the deposited or accumulating substances?

Wax esters

What is the most common collagen in adult skin?

3

What is the maximum recommended dose of hydroxychloroquine for the treatment of DLE?

6.5 mg ideal body weight

In a patient with cyanosis in fingers (Reynaud’s) and edematous with low C3 and C4, and positive ANA, what is the best treatment?

Hydroxychloroquine

A 65-year-old female on renal dialysis complains of sudden waxy brown papules on the trunk. What is the most associated cancer?

Pancreas

What dressing would be used for a copious exudate after excising a tumor beside the eye and leaving it for secondary intention for two weeks?

Alginate

What makes up the majority of the protein envelope of keratinocytes?

Keratin

In liposuction, which organ is most affected?

Back

In a patient with itching interscapular brownish patch with rippling (amyloidosis), which organ is associated?

Kidney

What is the pulse duration of laser used in treatment of Haemangioma?

< 2ms

Which genetic mutation is associated with Legius syndrome but does not cause tumors, freckles, lipoma, or macrocephaly?

SPRED1 gene

In a case of itchy macular hyperpigmentation on the back (macular amyloidosis), what investigation would involve checking the Calcitonin level or performing a thyroid scan?

Thyroid function test

In a 10-year-old child with normal symptoms but with scars on the knuckles, which porphyria type would be indicated by a negative urine porphyrin test and a positive blood porphyrin test?

Variegate porphyria

In a case of severe itching with macular hyperpigmentation on the back (macular amyloidosis), which condition would be associated with copper accumulation and a mutation in the C282y gene?

Wilson's disease

In a patient with a picture of juvenile xanthogranuloma, what would be the most appropriate next step?

Dermatologist review

In a 40-year-old female with generalized purpuric eruption all over the body and a sole ulcer, what would be the most appropriate treatment?

Methotrexate (MTX)

In a patient with bilateral nodules on the Achilles tendon (tubers xanthoma), what investigation would be most appropriate?

Cholesterol level test

In a patient with cutaneous amyloidosis and HCV infection, what association would be most likely?

Renal complications

In a picture showing skin-colored papules and elastic skin on the neck with angioid streaks on the retina, what would be the most appropriate next step?

Biologic therapy

In a patient with vesicular eruption on the hands, arms, and face, what organism would be associated with this condition?

Virus HCV

In a patient with recurrent abdominal pain since childhood, what would be the most appropriate initial treatment?

Colchicine prescription

In a patient with perforating disease, renal failure, and characteristic keratotic papules or umbilicated papulonodules, what investigation would you order?

Creatinine level test

Which of the following is the longest-lasting filler for nasolabial fold?

Calcium hydroxyapatite

When a patient experiences blanching after injection of filler and botox, what is the first step for management if pain is also present?

Hyaluronidase injection

In a case where blanching occurs after filler injection, what is the next step if only pallor is observed?

Use nitroglycerin patch

In the treatment of melasma in skin type 2, which solution is commonly used?

Jessner solution + acetic acid

In a 50-year-old male with deep forehead furrows and increased nasolabial fold with muscle activity, what would provide the best improvement?

Fractional laser

What type of laser is typically used in the treatment of lip vascular lesions?

Nd:YAG 1064

What constitutes the majority of the protein envelope of keratinocytes?

Involucrin

In a 1-year-old infant weighing 15 kg with hemangioma, what is the most appropriate treatment?

15 mg propranolol

What is the most common complication of infraorbital Botox injection?

Festooning

A patient with generalized purpuric eruption all over the body and a sole ulcer is most likely suffering from which condition?

Systemic lupus erythematosus

In a case of erythematous rash, bone pain, and histological findings of epithelioid granuloma and few lymphocytes, what gene defect is most likely responsible for this condition?

Carb 15

A 40-year-old female with generalized purpuric eruption all over the body and a sole ulcer is most likely to be treated with which medication?

Methotrexate

What is the most likely treatment for a patient with mastocytosis aggravated by a drug?

Dextromethorphan

In a case of macular amyloidosis, what investigation would be most appropriate to conduct next?

Genetic testing

A patient with skin-colored papules and elastic skin on the neck and angioid streaks on the retina should be advised to:

Undergo genetic testing

What investigation would be most appropriate in a case of Langerhans cell histiocytosis if the skull or cranium is not present?

MRI

A female patient with macular amyloidosis should consider consulting which specialist next?

Rheumatologist

What investigation would be most appropriate for a patient presenting with vesicular eruption on the hands, arms, and face?

Imiquimod

In a patient with recurrent abdominal pain since childhood, what would be the most appropriate initial treatment option?

Colchicine

What investigation would be most relevant for a patient with nodules on the olecranon ligament?

Genetic testing

A child with itchy macular hyperpigmentation on the back (macular amyloidosis) should undergo which investigation next?

Genetic testing

What would you expect to find in the epidermis of a case of Wilson's disease?

Excessive elastic fibers

Which of the following is associated with systemic sclerosis?

Association with Fabry's disease

What is the prophylactic treatment for a case of photosensitivity?

Carotene

In a patient with dermatomyositis controlled on prednisolone and MTX intake, started for one week of myopathy, what would be the next appropriate step?

Increase steroid

In a case of familial SLE, which C deficiency is associated with the condition?

C1 deficiency

What is the treatment for Gottron rash on both hands and rash on the ear?

Dapsone

In a case of small ulcer on a digit with fibrosis, pigmentation, and photosensitivity, which investigation would be most appropriate?

Ds DNA test

What type of secretion is produced by Moll's glands in the eyelid?

Zeis gland secretion

In a case of acquired digital fibrokeratoma, what is the recommended treatment?

Surgical removal

What is the expected finding in a patient with Ehlers-Danlos syndrome and hyperextensible skin of the neck?

Joint hypermobility

What is the most likely diagnosis for an asymptomatic skin rash on the upper and lower limbs with an atrophic center?

Lichen sclerosus et atrophicus

Which chapter in the Dermatology Prometric Exam has the highest number of pages?

Tumors

What type of filler is considered long lasting or permanent?

Silicon

In the Dermatology Prometric Exam, which section includes information on 'Granuloma'?

Infection

What is the most common cause of bilateral vascular ulcers without vasculitis?

Lichen Sclerosus et Atrophicus

What is the expected finding in the epidermis of a case of Wilson's disease?

Copper deposits

Which enzyme is involved in the immediate darkening of the skin?

Tyrosinase

In a patient with widespread superficial nodules with atrophied surfaces and surface telangiectasia, what is the most likely diagnosis?

Lichen Sclerosus et Atrophicus

What is the maximum dose of hydroxychloroquine for the treatment of DLE?

6.5 mg ideal body weight

In a patient with cyanosis in fingers (Reynaud’s), edema, low C3 and C4, and positive ANA, what is the best treatment?

Hydroxychloroquine

In a child with encopé de Sabre, what is the best treatment?

Subcutaneous MTX

What type of dressing should be used for a patient who has made an excision of a tumor beside the eye and left it for secondary intention?

Hydrocolloid

What dressing should be used for a painful ulcer with yellowish discharge (according to exudate quantity)?

Alginate

After Moh's microsurgery to remove basal cell carcinoma (BCC) beside the nose, what type of flap would be used?

Transposition flap

In a patient with widespread superficial nodules with atrophied surfaces and surface telangiectasia, what is the most likely diagnosis?

Subacute cutaneous lupus erythematosus (LE)

In a case of linear morphea in a 10-year-old child, what treatment would be combined with steroids?

Mycophenolate mofetil

Which manifestation is not associated with subacute cutaneous lupus erythematosus (LE)?

"Joint affection"

What enzyme is involved in the immediate darkening of the skin?

"Transferrin glutamase"

After angiography, which of the following may be elevated if the patient develops cyanotic feet?

"Creatine kinase"

For a periauricular lesion near the hairline requiring surgical removal, what type of anesthesia should be used?

"Chlorohexidine"

What is the most common cause of bilateral vascular ulcer without vasculitis?

Protein C or S deficiency

In a patient with varicose veins and pain in the lower leg, what is the next appropriate investigation?

Doppler

In a patient with sweet syndrome presenting with an abnormal lab CBC showing increased WBCs and neutrophils, how long does it take for white blood cells to recover?

12 months

In a 6-year-old boy with an indurated nodule and high neutrophil and WBC counts, what is the next appropriate step?

Bone marrow biopsy

What is the most likely diagnosis for a patient with livedo racemose, purpura, increased urea and creatinine, and pulmonary problems?

Cytoplasmic ANCA (with MPA)

What is the next appropriate step for a 60 years old patient with multiple waxy papules on the trunk and lower limb, ecchymosis, bruises, and macroglossia?

Bone marrow biopsy

In a case of vasculitis away from the lumen of airway, what type of ANA is expected?

Nuclear ANA

What is the most common cause of bilateral vascular ulcer without vasculitis?

Factor V Leiden

A patient with erythema nodosum treated with an unknown medication and has eczema. What is the drug most likely used for treatment?

Potassium iodide

In a patient with a brownish or violaceous lesion in the lower limb, what gene mutation is associated with the vascular birthmark?

GNAQ gene on ch9q21

What is the next appropriate step for a 6-year-old boy with an indurated nodule and high neutrophil & WBC count but no vasculitis (Sweet syndrome)?

Bone marrow biopsy

A male patient has a plaque pigmented on his leg, testing positive for Kaposi and human herpesvirus 8. What treatment is needed?

Sirolimus

What is the most likely diagnosis for a patient with lesions similar to eczema due to vasculitis, and whose brother has the same lesions?

Factor 5 gene defect

In a patient with livedo racemose, purpura, increased urea and creatinine, and pulmonary problems, what type of ANCA (with MPA) is most likely present?

Cytoplasmic

What would be the most appropriate next step for a female patient with macular amyloidosis?

Ophthalmologist

In a case of juvenile xanthogranuloma, what would be the next appropriate step?

Ophthalmologist

In a patient with erythematous rash and bone pain, which gene defect could be associated with the histological finding of epithelioid granuloma and few lymphocytes?

BRAF

In a patient with skin-colored papules and elastic skin on the neck, along with angoid streaks on the retina, what would be the most appropriate action?

Introduce Simvastatin

What organ is associated with macular amyloidosis?

Thyroid

In a patient with erythematous rash and bone pain, along with histological findings of epithelioid granuloma and few lymphocytes, what gene defect might be present?

(BRAF)

In a patient with bilateral nodules on the Achilles tendon (tubers xanthoma), what investigation would be most appropriate?

Copper level test

In a 40-year-old female with a generalized purpuric eruption and a sole ulcer, what would be the most appropriate treatment?

Methotrexate (MTX)

In a case of hypohidrosis and hypoesthesia, what investigation would be most relevant?

G6PD enzyme assay

In a case of macular amyloidosis, what would be the most appropriate investigation to conduct next?

Calcitonin level test

In a case of Langerhans cell histiocytosis without the presence of the skull or cranium in the diagnostic imaging, which investigation would be most accurate?

X-ray of the leg

What is the expected finding in the epidermis of a case of Wilson disease?

Ceramide

What is the most appropriate prophylactic treatment for a case of photosensitivity?

Hydroxychloroquine

In a case of granuloma with few lymphocytes, what would be the most suitable treatment?

Rituximab

In a patient with dermatomyositis controlled on prednisolone and MTX intake started for one week of myopathy, what would be the next appropriate step?

Increase steroid

In a case of lichen sclerosis et atrophicus, what would be the most suitable treatment?

Tacrolimus 1

What type of joint complication is associated with Ehler-Danlos syndrome characterized by hyperextensibility of skin on the neck and defect in collagen?

Joint Hypermobility

In a patient with macular amyloidosis, which investigation would be most appropriate?

(ANA) Antinuclear antibody test

(JXG) Juvenile xantho-granuloma is associated with an increase in which type of cells?

(CD68) Cluster of differentiation 68 positive cells

What is the most suitable treatment for a patient with genital plaque and asymptomatic (Lichen sclerosis)?

Steroid

A patient has a shortness of breath and palpitations after having liver problems due to Lupus. What is the most likely deficiency in this scenario?

C4 deficiency

What type of light chain is associated with systemic amyloidosis?

Kappa

Which type of filler is considered the longest lasting or permanent?

Calcium hydroxyapatite

In a patient experiencing blanching after filler injection, what is the next appropriate step?

Hyaluronidase injection

What is the wavelength responsible for immediate darkening of the skin?

290-200nm

Which type of laser is commonly used in the treatment of a nevus of Ota?

1064nm q-switched ND YAG

What enzyme is involved in cell membrane function?

Transglutaminase

What constitutes the majority of the protein envelope of keratinocytes?

Loricrin

A 50-year-old male with deep forehead furrows and deep nasolabial folds that increase with muscle activity would best improve with which treatment?

Fractional laser

What is the total number of pages dedicated to the 'Granuloma' section in the Dermatology Prometric Exam?

3

In the 'Vascular' section, how many pages are dedicated to 'Vascular' in the Dermatology Prometric Exam?

8

In the 'Ethics and Safety' section, how many pages are dedicated to 'Ethics and Safety' in the Dermatology Prometric Exam?

5

How many pages are dedicated to the 'Tumors' section in the Dermatology Prometric Exam?

14

What type of filler is long-lasting or permanent filler according to the Dermatology Prometric Exam?

HA

Which section of the Dermatology Prometric Exam is dedicated to Genodermatosis?

Section 8

What is the range of pages dedicated to the 'Drugs' section in the Dermatology Prometric Exam?

[65-76]

Which type of laser is commonly used in the treatment of nevus of Ota according to the Dermatology Prometric Exam?

Q-Switched Ruby Laser

In which section of the Dermatology Prometric Exam would you find information about 'Connective Tissues'?

[22-30]

'Eczema and Hypersensitivity' is listed in which section of the Dermatology Prometric Exam?

[109-118]

What is the maximum dose of hydroxychloroquine for the treatment of DLE?

6.5 mg ideal body weight

A patient with cyanosis in fingers (Reynaud’s), edema, and low C3 and C4, along with a positive ANA, would benefit the most from which treatment?

Hydroxychloroquine

In the case of a child with encopé de sabre, what is the best treatment?

Subcutaneous MTX

What is the next appropriate investigation for a child with a lacy rash on the face and lower limb, along with EBV infection?

Skin biopsy then DIF then ANA

What type of dressing is most suitable for a painful ulcer with yellowish discharge, based on exudate quantity?

Alginate

What is the best antiseptic for a patient with a periauricular cyst who has hearing problems?

Hexachlorophene

In a patient who has made an excision of a tumor beside the eye and left it for secondary intention, what type of dressing should be used after 2 weeks if there is copious exudate?

Hydrocolloid

What is the most common cause of bilateral vascular ulcers without vasculitis?

Erythema nodosum

In a patient with livedo racemose, purpura, increased urea and creatinine, and pulmonary problems, what type of ANCA (with MPA) should be expected?

Cytoplasmic

In a case of lipoid proteinosis with hoarseness of voice, what is the treatment of choice?

PTN fixation or detection

What is the first indicator of rapid involuting hemangioma (RICH)?

$CD133$

In a patient with dilative cardiomyopathy, deep venous thrombosis, skin rash, and high eosinophil count, which medication would be most appropriate?

Mepolizumab

In a patient with macular amyloidosis, which investigation would be most appropriate?

$Electrophoresis$

Which laser type is commonly used in the treatment of nevus of Ota?

Q-switched ND YAG 1064

In a case of melasma in skin type 2, which solution is best for treatment?

Jessner solution with acetic acid

What is the pulse duration of the laser used in the treatment of hemangioma?

2 ms

In the treatment of Haemangioma, which laser wavelength is responsible for targeting the chromophore?

1064 nm

What is the best treatment for a patient with solar urticaria with a wavelength associated with symptoms?

320 nm

Which enzyme is involved in the cell membrane of keratinocytes?

Transglutaminase

What is the expected finding in a patient with Wilson's disease in the epidermis?

Calcium deposits

In a scenario of limited scleroderma, what investigation would be most appropriate?

Anticentromere

In a patient with erythematous plaque and pustules on the nose, what is the treatment and diagnosis?

Acitretin

What type of sebaceous gland secretion is involved in eyelid lubrication?

Meibomian gland

What investigation would be most appropriate for a patient with granuloma with plasma cells and no cholesterol cleft?

Blood glucose

What do you expect to find in a patient with Lupus pernio after 10 years?

Shortness of breath

What is the most likely diagnosis for a 10-year-old child with normal scars on knuckles, negative urine porphyrin, and positive blood porphyrin?

Erythropoietic porphyria

In a case of macular amyloidosis, what would be the next appropriate investigation to conduct?

Thyroid scan

In a patient with itching interscapular brownish patch with rippling (amyloidosis), which organ is associated?

Kidney

Which gene defect is associated with Blau syndrome, presenting with erythematous rash, bone pain, and histological findings of epithelioid granuloma and few lymphocytes?

NOD2

In a patient with bilateral nodules on the Achilles tendon (tubers xanthoma), what investigation would be most appropriate?

Cholesterol level

What is the most accurate pathophysiological association of intermittent claudication in PXE?

Peripheral arterial disease

What type of filler is considered long lasting or permanent in the context of the Dermatology Prometric Exam?

Silicon

Which section of the Dermatology Prometric Exam includes information on 'Granuloma'?

Infection

In a case of juvenile xanthogranuloma, what would be the next appropriate step according to the Dermatology Prometric Exam?

PCR for BRAF V600E mutation

What type of laser is commonly used in the treatment of nevus of Ota according to the Dermatology Prometric Exam?

Ruby laser

In a case of morphea 10 years ago, what would have been the most appropriate treatment as per the Dermatology Prometric Exam?

Ultraviolet A1 (UVA1) phototherapy

What is the maximum recommended dose of hydroxychloroquine for a patient with discoid lupus erythematosus (DLE) as per the Dermatology Prometric Exam?

$400$ mg/day

What is the maximum dose of hydroxychloroquine for the treatment of DLE according to the new guidelines in 2016?

6.5 mg ideal body weight

In a patient with Reynaud's phenomenon, cyanosis in fingers, edema, low C3 and C4, and positive ANA, what is the best treatment?

Hydroxychloroquine

A 65-year-old female on renal dialysis complains of sudden waxy brown papules on the trunk. What is the most associated cancer?

Liver

In a child with a brown macular rash on the cubital fossa, what is the best treatment?

Phototherapy

Which manifestation is not associated with subacute LE?

Renal involvement

In a patient with erythema, edema, and induration of the whole face with stiffness of both LL and a photo of a finger with periungual erythema, what is the treatment?

Steroids

In a 10-year-old child with linear morphea, what treatment should be combined with steroids?

NBUVB

What is the most common site for lichen sclerosus et atrophicus?

Genitalia

What enzyme is involved in the immediate darkening of the skin?

Tyrosinase

After Moh's microsurgery to remove basal cell carcinoma (BCC) beside the nose, what type of flap would be used?

Transposition

In which section of the Dermatology Prometric Exam would you find information about the treatment and diagnosis of erythematous plaque and pustules on the nose?

Eczema and Hypersensitivity

What is the enzyme involved in the immediate darkening of the skin?

Tyrosinase

Which type of laser is commonly used in the treatment of nevus of Ota?

Alexandrite laser

In a patient with livedo racemosa, purpura, increased urea and creatinine, and pulmonary problems, what type of ANCA (with MPA) is likely to be present?

Anti-PR3 ANCA

What investigation would be most appropriate for a patient with granuloma with plasma cells and no cholesterol cleft?

Punch biopsy

What is the prophylactic treatment for a case of photosensitivity?

Sunscreen

What is the most appropriate next step for a patient with varicose veins and severe pain and swelling after injection?

Perform a Doppler ultrasound

In a child with varicose veins and one limb larger than the other, what is the most appropriate next step?

Perform a duplex ultrasound

In a case of varicose vein pain in the lower leg, which investigation is most appropriate?

Doppler ultrasound

In the management of bleu nevus, what is the most common site for this condition?

Limbs (hand and foot)

In the treatment of erythema nodosum with unknown medication and concurrent eczema, what is the most likely drug administered?

Potassium iodide

In an infant with hemangioma and seizures, what is the appropriate pulse duration for laser treatment?

1

What is the common side effect of drug treatment for hemangioma?

Bradycardia

'Sweet syndrome' is associated with which of the following as a poor prognostic sign?

Thrombocytopenia

In a patient with a painful lesion on the hand resembling 'Sweet syndrome', which abnormal lab result indicates a longer recovery time for white blood cells?

Increase in neutrophils

In a 6-year-old boy with an indurated nodule and high neutrophil & WBC count but no vasculitis ('Sweet syndrome'), what type of histopathology is most likely to be observed?

Granulomatous lobular panniculitis

After undergoing angiography, a patient developed a cyanotic leg. What hematological abnormality is this primarily associated with?

Eosinophilia

A 55-year-old female patient on renal dialysis presents with ulcers at the Achilles tendon. Which treatment would be most suitable for this condition?

Sodium thiosulfate

Which gene defect is associated with Wilson's disease?

ATP7B gene

In a case of macular amyloidosis, what is the next appropriate specialist to consult for a female patient?

Ophthalmologist

In a patient with vesicular eruption on the hand, arm, and face without inflammatory infiltrate, what is the most appropriate treatment?

Phototherapy

What is the most associated organ affected in liposuction?

Liver

In a patient with recurrent abdominal pain since childhood, what is the most appropriate treatment?

Colchicine

What investigation is needed for a patient with perforating disease and renal failure?

Creatinine

What is the maximum recommended dose of hydroxychloroquine for the treatment of disseminated lupus erythematosus (DLE)?

$200 mg/day

What is the most likely treatment for an infant with low weight and multiple brown lesions showing osteolytic lesions on X-ray?

Vincristine

What is the most affected organ in a patient with urticaria pigmentosa?

GIT (gastrointestinal tract)

What investigation is needed for a patient with hypertrichosis and vesicles?

CBC (complete blood count)

In a case of mastocytosis, what is the least affected organ?

GIT (gastrointestinal tract)

In a case of Langerhans cell histiocytosis, what investigation would be most accurate if skull or cranium is not present?

Spine x-ray

What is the most likely diagnosis for a patient with an asymptomatic skin rash on the upper and lower limbs with an atrophic center?

Morphea

In a case of hypohidrosis and hypoesthesia, what investigation would be most relevant?

Skin biopsy

What is the protective to treatment for pseudoxanthoma elasticum?

Simvastatin

In a patient with cyanosis in fingers (Reynaud’s), edema, low C3 and C4, and positive ANA, what is the best treatment?

Immunosuppressants

In a case of Wilson's disease, what would you expect to find in the epidermis?

Copper deposition

What enzyme defect is associated with angiokeratoma and involves hyperkeratosis and rete ridges encircling dilated vessels in the papillary dermis?

α-Galactosidase A

What is the most likely diagnosis for a 10-year-old child with normal scars on knuckles, negative urine porphyrin, and positive blood porphyrin?

Porphyria cutanea tarda (PCT)

In a scenario of limited scleroderma, what investigation would be appropriate?

ANA

Which type of laser is commonly used in the treatment of nevus of Ota according to the Dermatology Prometric Exam?

Nd:YAG laser

What is the most appropriate next step for a female patient with macular amyloidosis?

Skin biopsy

In a case of macular amyloidosis, what would be the most appropriate investigation to conduct next?

Congo red staining

What is the maximum recommended dose of hydroxychloroquine for a patient with discoid lupus erythematosus (DLE)?

6.5 mg ideal body weight

In a patient with cyanosis in fingers (Reynaud’s) and edema with low C3 and C4, which is the best treatment?

Cyclosporine

A 65-year-old female on renal dialysis complains of sudden waxy brown papules on the trunk. What is the most associated cancer?

Liver

In liposuction, which organ is most affected?

Back

In a patient with painful ulcer and yellowish discharge, what is the appropriate dressing according to exudate quantity?

Alginate

In a case of erythematous rash and bone pain, what gene defect is most likely responsible for this condition?

FBN1

What type of suture is used for a deep lesion in the mouth?

Silk

In a patient with livedo racemose and purpura, what type of ANCA should be expected?

C-ANCA PR3-ANCA

In a case of Fabry's disease, what are the deposited or accumulating substances?

Glycosphingolipids

What is the most common site for lichen sclerosus et atrophicus?

Face

After angiography, which of the following may be elevated if the patient develops cyanotic feet?

Creatine kinase

In a patient with recurrent abdominal pain since childhood, what would be the most appropriate treatment?

Methotrexate (MTX)

In the treatment of nasolabial fold, which filler is the longest lasting?

Calcium hydroxyapatite

What is the first step to take if a patient experiences itching, pallor, and pain after injection of filler and botox?

Hyaluronidase injection

Which wavelength is responsible for immediate tanning of the skin?

290-200 nm

What is the enzyme involved in cell membrane function?

Transglutaminase

What is the most common collagen in adult skin?

Type 1 collagen

In the treatment of hemangioma, what is the pulse duration of laser typically used?

20 ms

What type of laser is used in the treatment of nevus of Ota?

Q-switched Nd:YAG (532 nm)

Which type of light is used in PDT for acne treatment?

Blue light

Which type of laser is used in the treatment of haemangioma?

755 nm

Which type of laser is used for hair removal?

Nd:YAG 1064 nm

What is the best response to blanching after filler injection?

Hot fomentation and massage due to blanching

What is the best topical treatment for hemangioma?

Timolol

Study Notes

Wilson's Disease

  • Expect to find copper deposits in the epidermis of a case of Wilson's disease.

Fabry's Disease

  • Deposited or accumulating substances in Fabry's disease are lipids.

Photosensitivity

  • Prophylactic treatment for a case of photosensitivity is sun protection measures.

Familial SLE

  • Associated deficiency in familial SLE is C2 deficiency.

Systemic Amyloidosis

  • Typically, lambda light chains are involved in systemic amyloidosis.

Scleroderma with Lung Fibrosis

  • Investigation for scleroderma with lung fibrosis should include Scl-70 antibodies.

Skin Darkening

  • The enzyme involved in the immediate darkening of the skin is tyrosinase.

Collagen

  • The most common collagen in adult skin is type I collagen.

Hemangioma Treatment

  • The appropriate dosage of propranolol for a 1-year-old infant weighing 15 kg is 2 mg/kg/day.

Keratinocytes

  • The majority of the protein envelope of keratinocytes is made up of filaggrin.

Granuloma Faciale

  • Treatment for granuloma faciale is recommended as pulse dye laser.

Erythema Abigne

  • The most likely cause of erythema abigne on the back is prolonged heat exposure.

Dermatology Prometric Exam

Vascular

  • The section that covers the topic of 'Vascular' is the 'Vascular' section.

Connective Tissues

  • The total number of pages dedicated to the 'Connective Tissues' section is 21 pages.

Ethics and Safety

  • The part of the Dermatology Prometric Exam that includes information on 'Ethics and Safety' is the 'Ethics and Safety' section.

Granuloma

  • The title of the section that includes information on 'Granuloma' is the 'Granuloma' section.

Cosmetology and Lasers

  • The type of filler described as long-lasting or permanent in the 'Cosmetology and Lasers' section is hyaluronic acid fillers.

... (rest of the notes will be generated similarly)

Test your knowledge of genetic disorders such as Legius syndrome, McCune-Albright syndrome, and macular amyloidosis. Explore the characteristics, genetic causes, and associated symptoms of these disorders.

Make Your Own Quizzes and Flashcards

Convert your notes into interactive study material.

Get started for free
Use Quizgecko on...
Browser
Browser