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November 15, 2017

Vegetating Darier Disease Treated With Botulinum Toxin

Author Affiliations
  • 1Department of Dermatology, Hospital Universitario Puerta del Mar, Cádiz, Spain
JAMA Dermatol. Published online November 15, 2017. doi:10.1001/jamadermatol.2017.4299

Darier disease (DD) is a genodermatosis related to a mutation in the ATP2A2 gene, a keratinization disorder of autosomal dominant inheritance. The treatment is symptomatic and in some cases can be a challenge for the dermatologist. We present a case of vegetating DD with persistent and refractory lesions in the intergluteal fold and auricles that was successfully treated with infiltrations of botulinum toxin type A (hereinafter “Botox”; Allergan Inc).

A man in his 40s was seen for pruritic and painful lesions in the intergluteal fold of more than 10 years’ evolution. Examination showed brown papules 2 to 3 mm in diameter grouped predominantly in the lumbosacral area along with macerated and malodorous vegetative plaques in the intergluteal region (Figure 1). In addition, he presented with hyperkeratosis and serosanguinous scabs in auricles and longitudinal bands in some nail plates with V-shaped notches. Skin biopsy showed acantholytic dyskeratosis without deposits of immunocomplexes under direct immunofluorescence. Laboratory findings were normal. A diagnosis of DD was determined.

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