ABSTRACT
Darier disease (DD) is an autosomal dominant acantholytic dermatosis with an estimated prevalence of 1 in 30 000-100 000. A localized form of DD was first described by Kreibich in 1906 and is thought to account for 10% of all cases. A number of clinical variants have been reported including: unilateral, linear, segmental or zosteriform DD. We present a case series of three patients with localized DD.
Subject(s)
Darier Disease/genetics , Darier Disease/pathology , Mosaicism , Adult , Age of Onset , Aged , Darier Disease/classification , Female , Humans , Male , Middle AgedSubject(s)
Acneiform Eruptions/pathology , Facial Dermatoses/pathology , Axilla/pathology , Female , Granuloma/pathology , Humans , Middle AgedSubject(s)
Lichen Sclerosus et Atrophicus/etiology , Urinary Diversion/adverse effects , Administration, Topical , Aged , Anti-Inflammatory Agents/therapeutic use , Fluocinonide/therapeutic use , Glucocorticoids , Humans , Lichen Sclerosus et Atrophicus/drug therapy , Male , Skin Ulcer/drug therapy , Skin Ulcer/etiologySubject(s)
Ear, External/pathology , Granuloma Annulare/pathology , Adult , Diagnosis, Differential , Ear Diseases/pathology , Humans , MaleABSTRACT
We describe two patients with unusual asymptomatic, papular lesions on the vulva, clinically resembling lichen planus, the histology of which revealed unexpected findings of suprabasilar clefting, acantholysis and dyskeratotic cells giving rise to corps ronds and grains together with hyperkeratosis and parakeratosis, features originally associated with a diagnosis of Darier's disease. Focal acantholytic dyskeratosis has been described in a wide variety of inflammatory and neoplastic processes including those involving mucous membranes and has been attributed various diagnostic labels. We feel that the findings in our patients are consistent with a diagnosis of papular acantholytic dyskeratosis of the vulva, a rare condition, which was first described in 1984.
