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Key Clinical Updates in Pigmentary Disorders

Topical or systemic JAK inhibitors (tofacitinib, ruxolitinib) may be effective in some patients with recalcitrant vitiligo, although response to therapy is not the rule.

Although the color of skin may be altered by many diseases and agents, the vast majority of patients have either an increase or decrease in pigment secondary to an inflammatory disease, such as acne or atopic dermatitis.

Other pigmentary disorders include those resulting from exposure to exogenous pigments, such as carotenemia, argyria, and tattooing. Other endogenous pigmentary disorders are attributable to metabolic substances (eg, hemosiderin [iron]) in purpuric processes, to homogentisic acid in ochronosis, and bile pigments.

CLASSIFICATION

First, determine whether the disorder is hyperpigmentation or hypopigmentation, ie, an increase or decrease in skin pigment from the patient’s baseline. Each may be considered to be primary or to be secondary to other disorders. Depigmentation, the absence of all pigment, should also be differentiated from hypopigmentation, in which the affected skin is lighter than baseline skin color, but not completely devoid of pigment.

The evaluation of pigmentary disorders is helped by Wood light, which accentuates epidermal pigmentation in hyperpigmented disorders and highlights complete loss of pigment in depigmentating disorders. Depigmentation, as seen in vitiligo, enhances with Wood light examination, whereas postinflammatory hypopigmentation does not.

A. Primary Pigmentary Disorders

1. Hyperpigmentation

The disorders in this category are nevoid, congenital, or acquired. Nevoid and congenital disorders include pigmented nevi, mosaic hyperpigmentation, ephelides (juvenile freckles), and lentigines (senile freckles). Hyperpigmentation due to systemic diseases may be seen in in association with Addison disease, vitamin B12 deficiency, hemochromatosis, and Wilson disease. Melasma (chloasma) occurs as patterned hyperpigmentation of the face, most commonly as a direct effect of estrogens. It may occur during pregnancy, exposure to oral contraceptives, or be idiopathic. Although more common in women, melasma affects both sexes and all races.

2. Hypopigmentation and depigmentation

Depigmenting disorders in this category are vitiligo, albinism, and piebaldism. In vitiligo, pigment cells (melanocytes) are destroyed (Figure 6–39) (eFigure 6–38). Vitiligo, present in approximately 1% of the population, may be associated with other autoimmune disorders, such as autoimmune thyroid disease, pernicious anemia, diabetes mellitus, and Addison disease.

Figure 6–39.

Depigmented—vitiligo. (Used, with permission, from Richard P. Usatine, MD, in Usatine RP, Smith MA, Mayeaux EJ Jr, Chumley H. The Color Atlas of Family Medicine, 3rd ed. McGraw-Hill, 2019.)

B. Secondary Pigmentary Disorders

Any damage to the skin (irritation, allergy, infection, excoriation, burns, or dermatologic therapy, such as chemical peels and freezing with liquid nitrogen) may result in hyperpigmentation or hypopigmentation. Several disorders of clinical importance are described below.

1. Hyperpigmentation

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