Acrokeratosis verruciformis

Specialty: Skin.

• Hopf's acrokeratosis
• Hopf's acral warty papules

Why it occurs

• Inherited autosomal dominant genetic disorder characterized by a mutation in the ATP2A2 gene (closely associated and sharing a locus with Darier disease) that alters epidermal calcium homeostasis
• Idiopathic familial predisposition without generalized skin disease
• Repeated physical microtrauma on the backs of the hands and feet that stimulate local benign epidermal proliferation
• Cumulative solar actinic exposure in exposed acral areas.

Initial workup

Skin biopsy of a papule on the back of the hands to confirm the classic histopathological diagnosis: massive hyperkeratosis, regular acanthosis, very marked papillomatosis ("church needle") with a prominent granular layer, and characteristically, absence of the cellular vacuolization typical of warts of viral etiology (HPV).

Standard management

• It does not require mandatory pharmacological treatment if it is asymptomatic. 6% or 10% salicylic acid ointment — Topical keratolytic of choice applied daily to reduce the thickness of the papules and soften the surface of the skin
• Tretinoin cream 0.05% or 0.1% — topical retinoid applied at night to modulate local keratinocytic differentiation
• Cryosurgery with liquid nitrogen — selective physical destruction for individual bothersome or unsightly lesions
• Oral acitretin (systemic retinoid reserved only for very extensive or disabling forms, doses of 10 to 25 mg daily under metabolic control).