Pigmentary Disorders Lecture Notes PDF

1/29/25 P I G M E N TA RY DISORDERS 1 AG E N DA / L E A R N I N G OBJECTIVES ž ž ž ž ž 2 LEARNING OBJECTIVES ž ž ž ž 3...

1/29/25 P I G M E N TA RY DISORDERS 1 AG E N DA / L E A R N I N G OBJECTIVES ž ž ž ž ž 2 LEARNING OBJECTIVES ž ž ž ž 3 1  1/29/25 AC A N T H O S I S N I G R I C A N S 4 AC A N T H O S I S N I G R I C A N S estimated prevalence is 13.3% of African Americans, 5.5% of Latinos, and 34.2% of Native Americans. It affects both males and females of all ages, with people < 40 years old typically being more affected. What causes acanthosis nigricans? exact cause is still unclear predominantly linked to states of insulin resistance - obesity, diabetes, metabolic disorders Insulin crosses the dermal-epidermal junction and can have growth stimulating effects by binding to type 1 insulin-like growth factor receptors (IGFRs) in the keratinocytes Rarely, there is a link with an internal malignancy. malignant acanthosis nigricans tend to be middle- aged, not obese, and lesions develop abruptly. Prevention: Treatment: topical retinoids, microdermabrasion, keratolytics (lactic, glycolic, salicylic or trichloroacetic acid) 5 P O S T I N F L A M M AT O RY H Y P E R P I G M E N TAT I O N temporary pigmentation that follows injury or inflammatory disorder of the skin most common in darker skin types Fitz 4-6 6 2  1/29/25 P O S T I N F L A M M AT O RY H Y P E R P I G M E N TAT I O N ( P I H ) Dx: usually clinical based on hx and skin type; biopsy if needed Tx: Identify the source of inflammation and sunscreen Lesions will typically resolve spontaneously within 6-12 months To help speed up the process: Skin lighteners such as hydroquinone (4-8% qhs x 1-2 months) Retinoids and topical steroids to increase skin cell turnover Microdermabrasion, Chemical peels (salicylic or glycolic acid) 7 MELASMA Hyperpigmented macular patches or plaques on the face or sun exposed areas of the neck and arms with no sign of inflammation with gradual onset bilateral, asym ptom atic, light-to-dark brown m acules or patches with irregular borders. Distinct patterns include: Centrofacial — forehead, cheeks, nose, upper lip (sparing the philtrum); 50-80% of presentations Malar — cheeks, nose Mandibular — jawline, chin Extrafacial — forearms, upper arms, shoulders in a sun-exposed distribution. 8 MELASMA more common in women than in men onset typically between the ages of 20 and 40 years common in (Fitzpatrick skin type III, IV). less common in people with fair skin (Fitzpatrick types I, II) or black skin (Fitzpatrick types V, VI). Gradual onset Risk factors: pregnancy, estrogen in contraceptives and HRT, sun exposure Prognosis: usually after pregnancy or with discontinuation of contraceptives fades gradually if in sun exposed areas and non hormonal, difficult to treat and can be frustrating to treat, both for the patient and the medical practitioner. It is slow to respond to treatment, especially if it has been present for a long time. Even in those who get a good result from treatment, pigmentation may reappear on exposure to summer sun. The chronicity and risk for relapse with the need for lifelong sun protection should be emphasized to set realistic goals and outcomes. 9 3  1/29/25 MELASMA Treatment Minimize sun exposure Broad spectrum UVA/UVB tinted sunscreen Oral anti-oxidants (omega 3 and 6, Vit E) Topical kojic acid, ascorbic acid, azelaic acid Hydroquinone d/c hormone Microdermabrasion Laser Chemical peels 10 LICHEN SCLEROSIS 11 Etiology: not fully understood and m ay include genetic, horm onal, irritant, traum atic, and infectious com ponents. LICHEN Extracellular m atrix protein-1 (ECM -1) antibodies have been detected in 60– 80% of w om en w ith vulval lichen sclerosis; considered to be autoim m une SCLEROSIS prim arily involves the non-hair bearing, inner areas of the vulva. It can be localized to one sm all area or extensively involve perineum , labia m inora and clitoral hood. It can spread onto the surrounding skin of the labia m ajora and inguinal fold and, in 50% of w om en, to the anal and perianal skin. 12 4  1/29/25 LICHEN SCLEROSIS Symptoms: Lichen sclerosus can be extremely itchy and sore (Sometimes bruises, blood blisters and ulcers appear after scratching, or from minimal friction (eg, tight clothing, sitting down). Dysuria or anuria Sexual intercourse can be very uncomfortable and lead to fissures. It may cause discomfort or bleeding when passing bowel movements adhesions and scarring The clitoris may be buried labia minora resorb/shrink introital stenosis 13 LICHEN SCLEROSIS D iagnosis can start at any age, although it is m ost Biopsy is helpful in early cases often diagnosed in wom en over 50. Pre- anogenital sites is associated with an increased risk of vulval, penile or anal cancer (squamous pubertal children can also be affected. cell carcinoma, SCC). Cancer is estimated to affect up to 5% of patients 15% of patients know of a fam ily m em ber with vulval lichen sclerosus. with lichen sclerosis. Cancer is more likely if the inflammatory disease is uncontrolled. personal or fam ily history of Invasive SCC presents as an enlarging lump or a sore that fails to heal. another autoim m une disease such as High-grade squamous intraepithelial lesions in thyroid disease (about 20% of patients), females (SIL) or males associated with lichen sclerosus may be HPV-associated (usual type) pernicious anem ia, or alopecia areata or differentiated. Biopsy any unhealing lesion ! 14 LICHEN SCLEROSIS Treatment General m easures for genital lichen sclerosus W ash gently once or twice daily, Use a non-soap cleanser, if any. Try to avoid tight clothing, rubbing and scratching. Apply em ollients to relieve dryness and itching, and as a barrier Topical steroids high potency topical steroid (ex: clobetasol propionate 0.05%) or a m edium strength topical steroid (eg, m om etasone furoate 0.1% ointm ent or triam cinolone 0.1% ointm ent) m ay also be used in m ild disease or when sym ptom s are controlled. One 30-g tube of topical steroid should last 3 to 6 m onths or longer. If s e v e r e , a c u te , a n d n o t r e s p o n d in g to to p ic a l th e r a p y , s y s te m ic tr e a tm e n t m a y r a r e ly b e p r e s c r ib e d. O p tio n s in c lu d e : In tr a le s io n a l o r s y s te m ic c o r tic o s te r o id s O r a l r e tin o id s : a c itr e tin , is o tr e tin o in M e th o tr e x a te C y c lo s p o r in e Other Tx: Laser PRP Surgery 15 5  1/29/25 VITILIGO depigmented macules and patches acquired depigmentation of the skin due to autoimmune destruction of melanocytes. It is characterized by well-circumscribed chalky- white macules and patches. Hairs in the involved skin may be normal or white. 16 VITILIGO Etiology Diagnosis Prognosis 1% of population affected and Wood’s lamp accentuates Slowly progresses over years 50% begin before age 20 the hypopigmentation in a highly variable course Both genders affected equally Skin biopsy shows absence Depigmented areas are at There is a positive family hx in 30% of melanocytes and sparse increased risk for sunburns First onset usually in aftermath of lymphocytic inflammation and subsequent skin cancer stress, illness or skin trauma The strongest association is Dermoscopy with thyroid disease, which can affect up to 15% of adults and 5– 10% of children with vitiligo 17 VITILIGO Treatment Topical corticosteroids Calcineurin inhibitors (pimecrolimus cream and tacrolimus ointment) Topical vitamin D derivatives (calcipotriol, tacalcitol) Second line Ruxolitinib cream Jak 1 and 2 inhibitors PD-1 inhibitors Phototherapy, oral psoralens and photochemotherapy (PUVA) W hole-body or localized UVB phototherapy 3x/wk x 6-12 months Excimer laser UVB (308 nm) or targeted UVB for small areas of vitiligo 18 6  1/29/25 VITILIGO Treatment Older system ic therapy System ic steroids Short pulse therapy to slow rapid progression, or as m ini-pulse oral steroids to stabilize active disease, eg, dexam ethasone 2.5–4 m g, for two consecutive days per week, for 3–6 m onths. M ethotrexate Cyclosporin M ycophenolate m ofetil Oral m inocycline 100 m g/day 19 DIFFERENTIAL D I AG N O S I S ž ž 20 T H A N K YO U 21 7