Pigmentary Disorders Lecture Notes PDF

P I G M E N TA RY DISORDERS Sabrina Johnson PA-C, MPAS  AGENDA/LEARNING OBJECTIVES  Acanthosis Nigricans  Post Inflammatory Hyperpigmentation  Melasma  Lichen Sclerosis  Vitiligo 20XX...

P I G M E N TA RY DISORDERS Sabrina Johnson PA-C, MPAS  AGENDA/LEARNING OBJECTIVES  Acanthosis Nigricans  Post Inflammatory Hyperpigmentation  Melasma  Lichen Sclerosis  Vitiligo 20XX 2  LEARNING OBJECTIVES  Identify etiology, risk factors, and clinical presentation of the lesions or skin disease  Develop a treatment plan including diagnostics and pharmaceutical management  Identify preventive measures  Prognosis of the skin disorder 20XX 3  ACANTHOSIS NIGRICANS Velvety papillomatous brown or hyperpigmented patches or plaques Darkening and thickening (hyperkeratosis) or leathery appearance of the skin Mainly occurs on the neck, and the flexural areas of the axilla, groin, and inframammary region  ACANTHOSIS NIGRICANS 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) 20XX 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 Hyperpigmented Macular patch or plaques temporary pigmentation that follows injury or inflammatory disorder of the skin most common in darker skin types Fitz 4-6  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) 20XX 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, asymptomatic, light-to-dark brown macules 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.  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. 20XX 9  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 20XX 10  LICHEN SCLEROSIS Ivory white atrophic papule with a faint pink rim On the skin, flat roofed slightly raised papules coalesce into small oval plaques with a dull or glistening, smooth, white, atrophic wrinkled surface, and possible stenosis On mucosal surfaces papules are atrophic, white, and glistening with a wrinkled tissue paper surface  Etiology: not fully understood and may include genetic, hormonal, irritant, traumatic, and infectious components. LICHEN Extracellular matrix protein-1 (ECM-1) antibodies have been detected in 60–80% of SCLEROSIS women with vulval lichen sclerosis; considered to be autoimmune primarily involves the non-hair bearing, inner areas of the vulva. It can be localized to one small area or extensively involve perineum, labia minora and clitoral hood. It can spread onto the surrounding skin of the labia majora and inguinal fold and, in 50% of women, to the anal and perianal skin. 20XX 12  The female to male ratio is 10:1 The disease has predilection for the anogenital skin LICHEN Symptoms: SCLEROSIS 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 20XX P R E S E N TAT I O N T I T L E 13  LICHEN SCLEROSIS Diagnosis can start at any age, although it is most Biopsy is helpful in early cases often diagnosed in women over 50. Pre- anogenital sites is associated with an increased risk of vulval, penile pubertal children can also be affected. or anal cancer (squamous cell carcinoma, SCC). 15% of patients know of a family member Cancer is estimated to affect up to 5% of patients with vulval lichen sclerosus. with lichen sclerosis. Cancer is more likely if the inflammatory personal or family history of disease is uncontrolled. Invasive SCC presents as an enlarging lump or another autoimmune disease such as a sore that fails to heal. thyroid disease (about 20% of patients), High-grade squamous intraepithelial lesions in females (SIL) or males associated with lichen pernicious anemia, or alopecia areata sclerosus may be HPV-associated (usual type) or differentiated. Biopsy any unhealing lesion ! 20XX 14  LICHEN SCLEROSIS Treatment General measures for genital lichen sclerosus Wash gently once or twice daily, Use a non-soap cleanser, if any. Try to avoid tight clothing, rubbing and scratching. Apply emollients to relieve dryness and itching, and as a barrier Topical steroids high potency topical steroid (ex: clobetasol propionate 0.05%) or a medium strength topical steroid (eg, mometasone furoate 0.1% ointment or triamcinolone 0.1% ointment) may also be used in mild disease or when symptoms are controlled. An ointment base is less likely than cream to sting or to cause contact dermatitis. A thin smear should be precisely applied to the white plaques and rubbed in gently. Sig: apply the steroid ointment once a day. After one to three months (depending on the severity of the disease), the ointment can be used less often. One 30-g tube of topical steroid should last 3 to 6 months or longer. If severe, acute, and not responding to topical therapy, systemic treatment may rarely be prescribed. Options include: Intralesional or systemic corticosteroids Oral retinoids: acitretin, isotretinoin Methotrexate Other Cyclosporine Tx:Laser PRP Surgery 20XX 15  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.  VITILIGO Etiology Diagnosis Prognosis Slowly progresses over 1% of population affected and Wood’s lamp 50% begin before age 20 accentuates the years in a highly variable Both genders affected equally hypopigmentation course There is a positive family hx in 30% Skin biopsy shows Depigmented areas are at First onset usually in aftermath of absence of melanocytes increased risk for stress, illness or skin trauma and sparse lymphocytic sunburns and subsequent The strongest association is inflammation skin cancer with thyroid disease, which can affect up to 15% of adults and 5– Dermoscopy 10% of children with vitiligo 20XX P R E S E N TAT I O N T I T L E 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) Whole-body or localized UVB phototherapy 3x/wk x 6-12 months Excimer laser UVB (308 nm) or targeted UVB for small areas of vitiligo 20XX 18  VITILIGO Treatment Older systemic therapy Systemic steroids Short pulse therapy to slow rapid progression, or as mini-pulse oral steroids to stabilize active disease, eg, dexamethasone 2.5–4 mg, for two consecutive days per week, for 3–6 months. Methotrexate Cyclosporin Mycophenolate mofetil Oral minocycline 100 mg/day 20XX 19  DIFFERENTIAL DIAGNOSIS  Work up?  Dx? 20XX 20  T H A N K YO U Sabrina Johnson Dermatology of New Mexico 610 Broadway NE Albuquerque [email protected] 20XX P R E S E N TAT I O N T I T L E 21