Dyskeratosis, intertriginous, acantholytic

Synonym(s)

Papular acantholytic dyskeratosis

Definition
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Rare, acquired chronic dermatosis which clinically and also histologically (suprabasal cleft formation with acantholysis and dyskeratotic cells) largely corresponds to the classic picture of Darier's disease, but without its genetic basis.

Clinical features
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Especially in the Intertrigines, there are multiple, whitish to brownish, papules, which can confluent to larger macerated areas with unpleasant foetor. Occasionally strong itching.

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Dermatology

Mallorca acne

Genetically predisposed, photoreactive, acneiform (papular) variant of polymorphic light dermatosis.

Histology
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Corresponds to dyskeratosis follicularis with moderate to distinct acanthosis, hyperkeratosis and focal, mostly columnar parakeratosis. Characteristic is the usually clearly prominent suprabasal cleft formation. In the stratum spinosum and granulosum dyskeratotic elements.

Case report(s)
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A 41-year-old female patient diagnosed with "recurrent condyloma acuminata" for 10 years showed rapid recurrence of the lesions despite adequate previous therapies (including two surgical procedures under general anesthesia) and thus a severe reduction in quality of life. A further surgical intervention under intubation anaesthesia was planned shortly.
Findings: Vaginal, perineal and perianal multiple whitish to brownish macerated papules.
Histology: Suprabasal acantholysis with prominent intraepidermal cleft and dyskeratosis. By immunohistological p16 staining and molecular genetic analysis a papilloma virus infection could be excluded.
Local therapy with zinc paste and low-potent glucocorticoids led to a significant improvement of symptoms, especially itching.