Clinical and Histopathological Findings in a Patient of Darier-White Disease with Acrokertasosis Verruciformis of Hopf

Abstract

Darier disease (DD) is a rare genodermatoses characterized by greasy hyperkeratotic papules in seborrheic regions and nail and oral changes. Histologically, it presents as suprabasal clefts with acantholytic and dyskeratotic cells. Acrokertasosis verruciformis of Hopf (AKVH) is considered an allelic variant with clinical overlap where Church spires are seen histologically without dyskeratoses. Patients are susceptible to various viral and bacterial skin infections requiring prevention and treatment of infection. Nonspecific treatment includes patient counseling on exacerbating factors. Although there are no curative treatments for DD, topical corticosteroids and systemic retinoids may be used to control inflammation and hyperkeratosis. We are reporting a rare case with clinical and histological findings of DD with AKVH in a 17-year-old boy with keratotic papules, presented on the hands and feet, nose, and ears without mucosal involvement.

Figure 1

(a, b) Multiple flat-topped brownish verrucous plaques over the forehead, neck, and the periauricular area.

Figure 2

Multiple keratotic papules over the dorsum of bilateral feet.

Figure 3

Multiple keratotic papules over the dorsum of the hand.

Figure 4

H&E stain with 20×: acantholysis and dyskeratosis represented by corps ronds and corps grains in DD.

Figure 5

H&E stain with 10×: focal areas of hyperkeratosis, papillomatosis, and acanthosis with church spire appearance in AKVH.