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A 6 year old male term child, born of consanguineous marriage presented with pruritic, erythematous, hyperkeratotic fixed scaly plaques along with erythematous patches showing bilaterally symmetrical distribution affecting lower abdomen, inguinal fossa, thighs, axilla and side of neck since his neonatal age (Figure 1). KOH mounting for fungus was negative. Systemic examination and developmental milestones were normal. Skin biopsy from the fixed hyperkeratotic plaque revealed orthohyperkeratosis, papillomatosis, normal granular layer and acanthosis. Based on the typical history, characteristic clinical features and histopathological findings, diagnosis of ‘Erythrokeratoderma Variabilis (EKV)’ was made and the child was put on topical keratolytics only with mild improvements.

Erythrokeratoderma Variabilis in a 6 Year Old Child: An Uncommon Genodermatosis with Typical Presentation