Pigmentation Disorders: Diagnosis and Management
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Pigmentation Disorders: Diagnosis and Management SCOTT PLENSDORF, MD, Lancaster General Health Family Medicine Residency Program, Lancaster, Pennsylvania MARIA LIVIERATOS, MD, Mayville Family HealthCare, Mayville, Michigan NABIL DADA, MD, Norton Audobon Hospital, Louisville, Kentucky Pigmentation disorders are commonly diagnosed, evaluated, and treated in primary care practices. Typical hyperpig- mentation disorders include postinflammatory hyperpigmentation, melasma, solar lentigines, ephelides (freckles), and café au lait macules. These conditions are generally benign but can be distressing to patients. Appropriate derma- tologic history, skin examination, and skin biopsy, when appropriate, can help exclude melanoma and its precursors. In addition to addressing the underlying condition, hyperpigmentation is treated with topical agents, chemical peels, cryotherapy, light or laser therapy, or a combination of these methods. Café au lait macules are treated with surgical excision or laser therapy if treatment is desired. Hypopigmentation disorders include vitiligo, pityriasis alba, tinea versicolor, and postinflammatory hypopigmentation. Treatment of vitiligo depends on the distribution and extent of skin involvement, and includes topical corticosteroids and calcineurin inhibitors, ultraviolet A therapy (with or with- out psoralens), narrowband ultraviolet B therapy, and cosmetic coverage. Patients with stable, self-limited vitiligo may be candidates for surgical grafting techniques, whereas those with extensive disease may be candidates for depig- mentation therapy to make skin tone appear more even. Other hypopigmentation disorders may improve or resolve with treatment of the underlying condition. (Am Fam Physician. 2017;96(12):797-804. Copyright © 2017 American Academy of Family Physicians.) CME This clinical content igmentation disorders of the skin novel treatments, and current evidence sup- conforms to AAFP criteria are commonly encountered in pri- porting their use. Table 1 summarizes cur- for continuing medical 1 education (CME). See mary care practice. Although they rent treatments. CME Quiz on page 772. are often benign and easily distin- Author disclosure: No rel- Pguishable based on appearance and location, Skin Types evant financial affiliations. it may be necessary to perform a skin biopsy The Fitzpatrick scale, which ranges from ▲ Patient information: to exclude melanoma and its precursors. skin types I to VI, is used to define and A handout on this topic is Some disorders result in cosmetic or psycho- estimate skin response to ultraviolet expo- available at https://family 1 doctor.org/condition/ logical concerns to the patient, necessitating sure (Table 2 ). Certain skin pigmentation melasma/. evaluation and treatment. Proper diagnosis disorders are more common in particu- allows the physician to facilitate appropriate lar skin phenotypes (e.g., solar lentigines skin treatment, provide reassurance, or ini- or ephelides in lighter skin [types I to III], tiate appropriate referral. melasma in darker skin [types IV to VI]).1 Hyperpigmentation is the result of excess Skin types can be classified using online melanin production, distribution, or trans- tools, such as the one at http://www. port. Common etiologies include postin- skincancer.org/prevention/are-you-at-risk/ flammatory hyperpigmentation, melasma, fitzpatrick-skin-quiz. solar lentigines, ephelides, and café au lait macules. Hypopigmentation results from Hyperpigmentation Disorders a reduction of melanocytes or an inability POSTINFLAMMATORY HYPERPIGMENTATION of the melanocytes to produce melanin or Postinflammatory hyperpigmentation is a properly transport melanosomes. Vitiligo, common consequence of trauma or inflam- pityriasis alba, tinea versicolor, and postin- mation primarily in darker skin types (III to flammatory effect are common causes of VI). Lesions can persist for months or years, pigment loss. This article discusses clini- and can be psychologically devastating to cal features of each condition, updated and some patients. DecemberDownloaded 15, from 2017 the American◆ Volume Family 96, Number Physician 12website at www.aafp.org/afp.www.aafp.org/afp Copyright © 2017 American Academy of FamilyAmerican Physicians. Family For the Physician private, noncom 797- mercial use of one individual user of the website. All other rights reserved. Contact [email protected] for copyright questions and/or permission requests. Pigmentation Disorders Table 1. Management of Pigmentation Disorders Disorder Description Location Etiology Treatment* Hyperpigmentation disorders Postinflammatory Irregular, darkly Previous sites Trauma, inflammation Triple combination therapy,† hyperpigmentation pigmented macules/ of injury or hydroquinone, retinoids, patches inflammation azelaic acid (Finacea), chemical peels, laser therapy Melasma Pigmented, well-defined Face (centrofacial Pregnancy, oral Triple combination therapy,† macules; light brown, 63%, malar 21%, contraceptives, chemical peels, light or laser brown, or gray mandibular 16%) phenytoin (Dilantin), therapy, sunscreen or forearms idiopathic Solar lentigines 1- to 3-cm well- Face, hands, Acute or chronic Triple combination therapy† circumscribed macules; forearms, chest, ultraviolet exposure with or without cryotherapy, light yellow to dark back, shins hydroquinone, retinoids, brown, variegated chemical peels, cryotherapy, intense pulsed light, laser therapy Ephelides 1- to 2-mm sharply Face, neck, chest, Childhood onset after Fades in winter months defined macules, red arms, legs sun exposure in so treatment may be or tan to light brown susceptible individuals unnecessary; cryotherapy, (skin types I or II) hydroquinone, azelaic acid, chemical peels, laser therapy Café au lait macules 1- to 20-cm tan to Most commonly Increased melanin in Laser therapy, surgical excision brown macules, trunk but may melanocytes/basal epidermal, present at appear anywhere keratinocytes birth or early childhood Hypopigmentation disorders Vitiligo Hypopigmented macules Face, hands, Unknown, possibly High-potency topical and patches; sharply forearms, neck, immune-mediated corticosteroids (class II and III), defined; 5 to 50 mm; genitalia, body topical calcineurin inhibitors, coalescent folds, periorificial; narrowband ultraviolet B, lip-tip pattern psoralen and ultraviolet A, systemic corticosteroids Pityriasis alba Hypopigmented, Face, head, neck, Possible association Sunscreen, topical irregular patches; fine forearms with atopic dermatitis corticosteroids, tacrolimus scale; itchy aggravated by sunlight (Protopic) ointment exposure Tinea versicolor Hypopigmented or Neck, chest, Malassezia spp. infection Topical antifungals, topical pink plaques; may be back, abdomen, adapalene (Differin) gel hyperpigmented; fine proximal scale extremities Postinflammatory Loss of pigment Varies Tinea versicolor, atopic Treatment of underlying hypopigmentation (variable), macules, dermatitis, pityriasis condition when applicable patches alba, psoriasis, guttate parapsoriasis, dermabrasion, chemical peels, intralesional corticosteroid use *—Some treatments may not be covered by insurance. †—Fluocinolone 0.01%/hydroquinone 4%/tretinoin 0.05% (Tri-luma). Adapted with permission from Plensdorf S, Martinez J. Common pigmentation disorders. Am Fam Physician. 2009;79(2):114. Postinflammatory hyperpigmentation presents as Combination therapy usually produces the best irregular, darkly pigmented skin at sites of previous results. Treatment of the underlying condition (e.g., injury or inflammation, as a consequence of laser or light acne, eczema) can help speed recovery, although weeks therapy, or following cryotherapy. Inflammation stimu- or months of therapy may be required, and resolution of lates prostaglandin, leukotriene, and thromboxane pro- hyperpigmentation is slow. Kligman’s formula (hydro- duction, resulting in epidermal melanocyte hypertrophy quinone 2%, tretinoin [Retin-A] 0.025%, and mometa- and increased melanin synthesis (Figures 1 and 2). sone [Elocon] 0.1%) and Tri-luma (fluocinolone 0.01%/ 798 American Family Physician www.aafp.org/afp Volume 96, Number 12 ◆ December 15, 2017 Pigmentation Disorders Table 2. Fitzpatrick Skin Type Classification Skin type Skin/hair/eye color Characteristics I White; very fair; red or blond Always burns, never hair; blue eyes; freckles tans II White; fair; red or blond hair; Usually burns, tans blue, hazel, or green eyes with difficulty III Cream white; fair with any eye Sometimes mild burn, or hair color; very common gradually tans IV Brown; typical Rarely burns, tans Mediterranean/Caucasian easily skin V Dark brown; Middle-Eastern Very rarely burns, skin tans very easily VI Black Never burns, tans Figure 1. Postinflammatory hyperpigmentation of acne very easily lesions. Image used with permission from VisualDx. Adapted with permission from Plensdorf S, Martinez J. Common pigmentation disorders. Am Fam Physician. 2009;79(2):110. hydroquinone 4%/tretinoin 0.05%) are among the com- mercially available combination products. Azelaic acid (Finacea), dexamethasone, salicylic acid, glycolic acid peels, retinoids, and laser treatments are sometimes helpful as monotherapy. Initial treatment with hydro- quinone followed by serial chemical peels may be use- ful in chronic hyperpigmentation. Risk of ochronosis (blotchy hyperpigmentation) with typical hydroquinone formulations (2% to 4%) is rare. Topical retinoids (e.g., tretinoin, 0.05% to 0.1%; tazarotene [Tazorac], 0.1%) are somewhat effective at reducing hyperpigmenta-