Dr. Francisco Gonzalez Otero
Hospital de Clínicas Caracas
Libro Online de Dermatología Pediátrica.
Una contribución de dermatologiapediatrica.net.
Le responderemos tan pronto sea posible, leemos todos los correos recibidos.
Papular Acantholytic Dyskeratosis of the Vulva
Francisco González, M.D.* and José R. Sardi, M.D.*
* Department of Dermatology, Hospital Universitario de Caracas, “Luis Razetti” School of Medicine, and
† Department of Dermatology, Hospital Universitario de Caracas, Central University of Venezuela, Caracas, Venezuela
We describe an 11-year-old girl with a persistent pruritic papular eruption on the vulva. Clinically, the lesions consisted of whitish papules and erosions located on the inner aspect of the labia majora. There was no familial history of skin diseases. Histologically, a biopsy specimen showed difuse hyperkeratosis, parakeratosis, acantholysis throughout the thickness of the epidermis, and the presence of corps ronds. Those findings were consistent with a diagnosis of acantholytic dyskeratosis. At 3 years followup, only isolated hyperkeratotic, asymptomatic papules on the same location remained. The occurrence of this focal and sporadic, localized form of acantholytic dyskeratosis seems to be rare in the pediatric population, as we could find no other child with this entity reported in the literature.
Acantholytic dyskeratosis has a distinctive histologic pattern characterized by suprabasilar clefting, acantholysis, dyskeratosis with formation of corps ronds, and parakeratosis with grain formation (1). It is a regular histologic finding in Darier disease (2), transient acantholytic dermatosis (3), and warty dyskeratoma (4). It is also occasionally observed as an incidental histologic feature in a variety of conditions, described as an entity in genitoanal areas (5,6) and is an occasional finding in linear epidermal nevus (7).
We describe a patient with a persistent papular acantholytic eruption that involved the vulva.
An 11-year-old girl complained of an itchy papular eruption located on the vulvar area, present for 8 months. An earlier diagnosis of candidiasis had been made by her pediatrician and her pediatric gynecologist because of severe pruritus; she received various therapies for that condition without any improvement. There was no family history of skin disease.
Examination found scattered, skin-colored, and whitish, slightly keratotic papules, and multiple grouped superficial erosions located on the inner aspect of the labia majora of the vulva. There were no lesions on other locations (Fig. 1).
A provisional diagnosis of bullous disease was made and a biopsy was taken.
Light microscopic study showed diffuse hyperkeratosis, acatholysis throughout the thickness of the epidermis, and dyskeratotic cells resembling corps ronds and grains, as well as irregular proliferation of basaloid cells with villi (Figs. 2, 3).
Direct immunofluorescence was negative with no deposition of immunoglobulins or complement in the skin.
A diagnosis of acantholytic dyskeratosis of the vulva was made. The patient did not receive any specific treatment.
Three years later, at age 14 years, she is asymptomatic, and on examination, had only sparse, slightly hyperkeratotic papular lesions without erosions, located at the vulva.
In 1972 Ackerman (7) introduced the term “focal acantholytic dyskeratosis” to refer, solely and specifically, to a distinctive histologic pattern characterized by focal changes consisting of suprabasilar clefts (lacunae) around preserved papillae (villi), acantholytic and dyskeratotic cells at all levels of the epidermis (corps ronds in the granular zone and grains in the parakeratotic zone), and hyperkeratosis and parakeratosis. These histologic characteristics were observed in a variety of conditions such as Darier disease, warty dyskeratoma, Hailey–Hailey disease, transient acantholytic dermatosis, and linear epidermal nevus (1). He proposed a classification, based upon the size and number of lesions and their duration, attempting to encompass the broad clinical range of these histologic changes (Table 1).
Several different papular or papulo-vesicular conditions with acantholysis, and accompanied by various degrees of dyskeratosis have been described (1). Except for transient acantholytic dermatosis, all of them usually followed a more or less chronic course (8). There are also reports of multiple papular lesions on the genital region, which histologically showed acantholysis and varying degrees of dyskeratosis (8). In 1984 an occurrence of papular acantholytic and dyskeratotic dermatosis of the vulva was reported; it was questioned as to whether this might be a distinct entity (10). Coppola et al (11) described a patient with vulval and perineal papular lesions that coalesced to form plaques, diagnosed as papular acantholytic dyskeratosis. Six patients were later reported with a vulvoperineal location of the dermatosis (11). None of the patients had a family history and none had similar lesions elsewhere on the body, as was observed in our patient. Her condition seems to be self-limited, although whether the sesions could be chronic has not been established yet. After 3 years of follow-up, the number of her lesions has considerably declined, and she has become asymptomatic.
We concluded from the clinical and histopathologic findings that our patient is best classified as having a benign persistent papular acantholytic and dyskeratotic eruption of the vulva.
This is the first report, to the best of our knowledge, of this entity in a pediatric patient.
TABLE 1. Classification of Acantholytic Dykeratosis (7)
Clinically inapparent, histologically incidental
Nodular (isolated Darier disease, isolated dyskeratosis follicularis,
Systematized (uniltateral, linear, zosteriform)
Evanescent: transient (transient acantholytic dermatosis)
1. Cottoni F, Masala MV, Cossu S. Acantholytic dyskeratotic epidermal naevus localized unilaterally in the cutaneous and genital areas. Br J Dermatol 1998;138:875–878.
2. Gottlieb SK, Lutzner MA. Darier’s disease. Arch Dermatol 1973:225–230.
3. Grover RW, Duffy JL. Transient acantholytic dyskeratosis. J Cutan Path 1975;2:111–127.
4. Tanay A, Mehregan AH. Warty dyskeratoma (review). Dermatologica 1969;138:155–164.
5. Grossin M, Belaich S. Another case of focal acantholytic dyskeratosis of the anal canal (letter). Am J Dermatopathol 1993;15:194–195.
6. Zanca A, Danese P, Bertazzoni MG. A case of acantholytic dyskeratotic epidermal naevus. G Ital Dermatol Venereol 1993;128:265–268.
7. Ackerman B. Focal acantholytic dyskeratosis. Arch Dermatol 1972;106:702–706.
8. Van Joost TH, Vuzevski VD, Tank B, et al. Benign persistent papular acantholytic and dyskeratotic eruption: a case report and review of the literature. Br J Dermatol 1991;124:92–95.
9. Cooper PH. Acantholytic dermatosis localized to the vulvocrural area. J Cutan Path 1989;16:81–84.
10. Chorzelsky TP, Kudejko J, Jablonska S. Is papular acantholytic dyskeratosis of the vulva a new entity? Am J Dermatopathol 1984;6:557–559.
11. Coppola G, Muscardin LM, Piazza P. Papular acantholytic dyskeratosis. Am J Dermatopathol 1986;8:364–366.