Bullous Darier's disease

Abstract

A 50-year-old woman, with multiple, hyperkeratotic, brown papules on the so-called seborrheic areas of the body, had had vesicles and bullae on the inner aspects of the lower extremities and the flexor aspects of the upper extremities each summer since childhood. Her father, brother, and daughter had similar papular lesions. Histologically, suprabasilar lacunae and dyskeratosis of epidermal cells were observed in the hyperkeratotic papules as well as in the vesicular and bullous lesions. The major difference between the histologic features of the hyperkeratotic papules and those of the vesicles or bullae was the size of the lacunae.

DermO; an ontology for the description of dermatologic disease

“…These diseases are not usually included in the same group within standard terminologies, but clustering here reflects an underlying pathoetiological mechanism in some patients where disruption to epithelial junctions in the bullous diseases is often associated with hyperkeratosis and disruption of the dermal-epidermal junction. For example poro- and hyper- keratosis are seen together with bullous pathology in syndromic skin diseases such as the epidermolytic hyperkeratoses, and keratosis follicularis (Darier disease, (DERMO:0000064)) can present in bullous form [ 34 ]. Similarly palmoplantar keratoderma (palmoplantar keratosis), (DERMO:0000049) is also reported associated with blistering [ 35 ] reflecting an autoimmune origin of both phenotypes.…”

A Bullous Flare of a Hyperkeratotic Affair

“…The vesiculo-bullous variant of Darier’s disease is rare. 1-5 Several previously reported cases have demonstrated clinical and microscopic features resembling familial benign chronic pemphigus or Hailey-Hailey disease. 2,3 Here we describe a case of Darier’s disease with blister formation and clinical features suggestive not of Hailey-Hailey disease, but rather bullous pemphigoid.…”

Can Darrier disease be localized bullous??!!

“…Other clinical variants of Darier's disease documented so far are hypertrophic type,[10] vesiculobullous type,[1112] linear or zosteriform type, follicular/Comedonal type. Oral vitamin A can be effective in treating Darier's disease in high doses.…”