ERYTHROKERATODERMIA VARIABILIS

By Gary M. White, MD


Erythrokeratodermia variabilis (EV) is a rare genetic disorder with two classic types of lesions:

    1.  Sharply marginated, transient figurate erythematous thin plaques that change over days to weeks.
    2.  Fixed, hyperkeratotic plaques.

Clinical

Circumscribed erythematous, brownish to bright red lesions which change shape over the course of several hours is characteristic. Exacerbation by cold, wind, heat or emotional stress has been reported. Erythema gyrated reopens lesions may rarely occur [Indian J Paediatr Dermatol 2016;17:202-5]. Additionally, hyperkeratotic yellowish-brown plaques are characteristic and may develop on normal or previously erythematous skin. The face, buttocks and limbs are favored sites. Palmoplantar keratoderma occurs in about half the patients.

EK from GJA1 Mutations

Treatment

The course is chronic and may persist throughout life. Some patients have partially regressed at puberty or improved during the summer. Topical retinoids may be tried, but response is variable. Oral retinoids are very effective (e.g. acitretin 25 mg/day) but cumulative side effects may limit their long-term use.

References

Migratory lesions. Indian Dermatol Online J 2013;4:340-3

Migratory lesions. Dermatology Online Journal

Fixed Plaques. Indian Dermatol Online J 2013;4:340-3

Erythema gyratum repens like lesions. Peds Derm 2002;19;285 and Indian J Paediatr Dermatol 2016;17:202-5

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