Erythrokeratodermia variabilis

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Synonym(s)

Erythrokeratoderma; keratosis rubra figurata; erythrokeratodermia figurata variabilis

Erythromelalgia 225

Definition

Disorder of cornification associated with transient noninflammatory erythema and persistent, but changing, scaliness

Pathogenesis

Mutations identified in the connexin gene GJB3; possibly caused by impaired gap junctional intercellular communication due to a defect in gap junctions

Clinical manifestation

Transient, circumscribed, highly variable, figurate erythematous patches, sometimes surrounded by a hypomelanotic halo, involving any part of the skin; lesions most prevalent during childhood and sometimes becoming less frequent as the patient ages; burning sensation sometimes preceding or accompanying erythema; variably changing, brownish, hyperkeratotic plaques with geographic borders, symmetrically distributed over the limbs, buttocks, and trunk; flexures, face, and scalp usually spared

Differential diagnosis

Progressive symmetric erythrokeratoder-mia; Giroux-Barbeau erythrokeratodermia with ataxia; Greither disease; erythrokera-tolysis hiemalis; ichthyosis linearis circum-flexa; psoriasis; mycosis fungoides; lupus erythematosus; lamellar ichthyosis; gyrate erythema; atopic dermatitis

Therapy

Acitretin*; emollients and/or keratolytics, such as alpha hydroxy acids

References

Hendrix JD Jr, Greer KE (1995) Erythrokeratoder-mia variabilis present at birth: case report and review of the literature. Pediatric Dermatology

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