Acantholytic dermatosis localized to the vulvocrural area

Abstract

This report describes 6 women with acantholytic lesions localized to the vulvocrural area. Clinically, multiple papules or solitary papules or plaques were situated on the labia majora or adjacent skin. None of the patients had similar abnormalities elsewhere on the body or family histories of similar eruptions. Two patients had concurrent candidal vaginitis. Microscopic findings consisted of acantholytic dyskeratosis resembling that observed in Darier's disease or acantholysis with minimal dyskeratosis similar to that seen in Hailey-Hailey disease. In one patient, acantholytic dyskeratosis with a cup-like configuration resembled the microscopic appearance of warty dyskeratoma. Immunofluorescence microscopy was negative in one patient. Solitary lesions were cured by local excision, but the papular eruption of one patient was recalcitrant to therapy. The literature contains descriptions of eight additional women with comparable lesions. Acantholytic reactions localized to the vulvocrural area are not apparently rare and need to be distinguished from other acantholytic dermatoses that can affect this anatomic site.

Mimickers of classic acantholytic diseases

“…Also known as papular acantholytic dermatosis of the vulvocrural area, this entity has been described in males . Clinically, it presents as white to tan papules (and occasionally as a solitary lesion) on the labia majora, scrotum, penis, perianal area and upper medial thighs . Biopsies demonstrate acantholytic dyskeratosis of the Darier or Hailey–Hailey type, with some lesions demonstrating the cup‐shaped epidermal architecture of a warty dyskeratoma (Fig.…”

Acantholytic dyskeratotic acanthoma: case report and review of the literature

“…[a]Abbreviations: AK, actinic keratosis; BCC, basal cell carcinoma; CR, current report; F, female; GC, granular and corneal layers; M, male; SCC, squamous cell carcinoma; SK, seborrheic keratosis[b]Two patients with solitary papules with focal acantholytic dyskeratosis described by Ackerman [1] and one patient described by Barnette and Cobb [21] with a solitary acantholytic acanthoma with mild dyskeratosis reported in the literature prior to the establishment of acantholytic dyskeratotic acanthoma yet showing some of the features observed in this lesion, have not been included in the table [12][c]One patient with focal acantholytic dyskeratosis of the nail unit described by Isonokami and Higashi [19], reported in the literature prior to the establishment of acantholytic dyskeratotic acanthoma yet sharing some of the features observed in this lesion has not been included in the table[d]Papule: the lesion is less than 5 mm[e]Plaque: the lesion is greater than or equal to 5 mm[f]Confluent: epidermis with acanthosis and confluent acantholytic dyskeratosis involving multiple levels of epidermis[g]Epidermal acanthosis with subungual hyperkeratosis lifting nail plate (parakeratosis with neutrophils); many suprabasal acantholytic clefts and dyskeratotic cells (corps ronds and grains)[h]GC: epidermis with acanthosis and confluent acantholytic dyskeratosis present mostly in the granular and corneal layers[i]Focal, discrete foci of acantholytic dyskeratosis[j]Total number may exceed number of patients due to multiple clinical differential diagnoses per each individual…”

“…[b]Two patients with solitary papules with focal acantholytic dyskeratosis described by Ackerman [1] and one patient described by Barnette and Cobb [21] with a solitary acantholytic acanthoma with mild dyskeratosis reported in the literature prior to the establishment of acantholytic dyskeratotic acanthoma yet showing some of the features observed in this lesion, have not been included in the table [12]…”

Dermatosis acantolítica papular del área genitocrural: ¿forma localizada de Darier o Hailey-Hailey?

“…En general es asintomática, aunque puede asociarse a prurito intenso. Histopatológicamente presenta acantólisis con grados variables de disqueratosis y la inmunofluorescencia directa e indirecta es negativa en casi todos los casos estudiados 5 . Presentamos el caso de una paciente de 58 años, sin antecedentes personales ni familiares de interés, que consultó por lesiones pruriginosas de 2 meses de evolución en la región genital, por lo que fue remitida al Servicio de Dermatología.…”