Answer to Image of the Month February 2016

Submitted by Inchara YK


Darier's disease

The epidermis shows multiple foci of suprabasal clefting with acantholytic cells in the spinous and granular layer. Several dyskeratotic cells are noted. Also noted are columns of parakeratosis. Focal downward proliferation of the epidermis is noted with a mild perivascular lymphocytic infiltrate in the dermis.

Darier's disease is a autosomal dominant genodermatosis named after a French dermatologist Ferdinand-Jean Darier. It usually presents during the second decade as dirty brown, rough surfaced, keratotic papules affecting the seborrheic regions and extensor surfaces. Histologic features are focal acantholysis with dyskeratotic cells, parakeratosis and intraepidermal clefts. The differential diagnosis include Grover's disease and warty dyskeratoma.

Grover's disease is an inflammatory process ocurring in adults over 40 years and affecting the V of the chest, thighs, flanks and thighs. Histologically, it shows spongiosis and acantholysis in the same focus. Often, in the same biopsy specimen one sees features of pemphigus, Hailey -Hailey disease, spongiosis with acantholysis and acantholysis with dyskeratosis. Tiny intraepidermal vesicles and eosinophils in the papillary dermis are common. Evidence of excoriation is often present.

Warty dyskeratoma is a solitary papule /nodule with an umbilicated centre affecting the head and neck of middle aged and elderly people. Histology reveals a cup shaped well circumscribed invaginating lesion characterised by suprabasilar clefting,acantholysis, dyskeratosis and a keratinous plug. Protruding into the lacunae are dermal villi covered by a basal layer of cells.