Dirty hyperkeratotic papular eruption over the face, trunk, and extremities

A woman in her fifties presented with diffuse pruritic papules since puberty. The lesions first appeared on the face and neck, gradually extending over 5 years to involve the chest, abdomen, back, and extremities. She reported exacerbation with heat, sweating, and sunlight, along with malodor. Clinical examination revealed yellowish-brown hyperkeratotic papules coalescing into plaques on the face [Figure 1a], trunk [Figure 1b], and extremities [Figure 1c], with additional findings of nail V-nicking, palmoplantar pits [Figure 1d], and palatal cobble stoning [Figure 1e]. Histopathology demonstrated suprabasal clefting, corps ronds, and corps grains, consistent with acantholytic dyskeratosis [Figure 2]. A diagnosis of Darier disease was established, and treatment with oral acitretin and topical keratolytics was initiated.

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Figure 1:

A 55-year-old female with Darier disease presented with yellowish-brown hyperkeratotic papules coalescing into plaques on the (a) face, (b) trunk, (c) lower legs, (d) along with palmar pits, and (e) palatal cobble stoning.

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Figure 2:

Histopathology image shows basket weave orthokeratosis, hyperkeratosis, focal parakeratosis, mild spongiosis, suprabasal split (black arrow) along with corps ronds (red arrow) and grains(yellow arrow), Hematoxylin and Eosin ×100.

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Darier disease is an autosomal dominant genodermatosis caused by ATP2A2 mutations, characterized by dirty hyperkeratotic pruritic papules, nail and mucosal changes, and malodorous plaques.^[1] This case illustrates the full spectrum of its classic clinical features, including dirty hyperkeratotic papules coalescing into plaques over seborrheic sites, palmoplantar pitting, palatal cobblestoning, and characteristic acantholytic dyskeratosis on histopathology.