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Acantholytic Dermatosis of the Vulvocrural Area Ravi S

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Acantholytic Dermatosis of the Vulvocrural Area Ravi S continuing medical education Acantholytic Dermatosis of the Vulvocrural Area Ravi S. Krishnan, Houston, Texas Leslie S. Ledbetter, MD, Houston, Texas Jon A. Reed, MD, Houston, Texas Sylvia Hsu, MD, Houston, Texas GOAL To discuss a case of acantholytic dermatosis of the vulvocrural area OBJECTIVES Upon completion of this activity, dermatologists and general practitioners should be able to: 1. Describe the clinical presentation of acantholytic dermatosis of the vulvocrural area. 2. Outline etiological possibilities and the differential diagnosis of acantholytic dermatosis. 3. Delineate treatment for acantholytic dermatosis. CME Test on page 220. This article has been peer reviewed and ACCME to provide continuing medical ed- approved by Michael Fisher, MD, Professor ucation for physicians. of Medicine, Albert Einstein College of Albert Einstein College of Medicine Medicine. Review date: February 2001. designates this educational activity for a This activity has been planned and im- maximum of 1.0 hour in category 1 credit plemented in accordance with the Essen- toward the AMA Physician’s Recognition tials and Standards of the Accreditation Award. Each physician should claim only Council for Continuing Medical Education those hours of credit that he/she actually through the joint sponsorship of Albert spent in the educational activity. Einstein College of Medicine and Quadrant This activity has been planned and HealthCom, Inc. The Albert Einstein produced in accordance with ACCME College of Medicine is accredited by the Essentials. Acantholytic dermatosis of the vulvocrural area is perkeratosis and focal parakeratosis. We report a a rare skin disorder characterized by solitary or case of this rare disease and discuss its differen- multiple skin-colored to white, smooth papules or tial diagnosis and treatment. plaques. Histopathological features of both Hailey- Hailey disease and Darier’s disease are present. cantholytic dermatosis of the vulvocrural area There is acantholysis, which may involve the full is an uncommon clinical and histopathological thickness of the epidermis, and dyskeratosis with entity that belongs to the spectrum of focal A 1 corps ronds and grains. There may be marked hy- acantholytic dyskeratoses described by Ackerman. In 1984, Chorzelski et al2 proposed the term papular Mr. Krishnan and Drs. Ledbetter, Reed, and Hsu are from Baylor acantholytic dyskeratosis of the vulva to describe the College of Medicine, Houston, Texas. Mr. Krishnan is a medical disorder and suggested that this dermatosis may rep- student. Dr. Ledbetter is a Resident in the Department of resent a unique entity. Since the initial description by Dermatology. Dr. Reed is Associate Professor in the Departments Chorzelski et al, there have been sporadic reports of of Pathology and Dermatology. Dr. Hsu is Associate Professor in 3-7 the Department of Dermatology. acantholytic dermatoses of the vulvocrural area, as Reprints: Sylvia Hsu, MD, Department of Dermatology, Baylor well as a report of clinically and histologically similar College of Medicine, One Baylor Plaza, Houston, TX 77030. lesions in males.8 VOLUME 67, MARCH 2001 217 ACANTHOLYTIC DERMATOSIS Figure 2. Acantholysis involving the suprabasal layers of the epidermis and dyskeratosis with some cells resembling corps ronds and grains (H&E, original mag- nification ϫ250). disease and Darier’s disease. There is prominent acantholysis, which may involve the full thickness of the epidermis, and dyskeratosis with corps ronds Figure 1. Confluent skin-colored papules on the medial and grains.5 There may be pronounced hyperker- thighs, inguinal folds, and labia majora. atosis and focal parakeratosis.4,6 Direct immunoflu- orescence studies are consistently negative, with no Case Report deposition of immunoglobulin or complement in A 27-year-old Hispanic woman presented with a the skin.2-6 Indirect immunofluorescence studies are 9-month history of a slightly pruritic eruption in her also negative.2,3,6 groin. There was no family history of similar lesions. The differential diagnosis of acantholytic der- On physical examination, the patient showed con- matosis of the vulvocrural area consists of acan- fluent skin-colored, smooth, 2- to 4-mm papules on tholytic disorders that can produce lesions localized her medial thighs, inguinal folds, and labia majora to the genital region,7,9-11 including Darier’s disease, that coalesced into plaques (Figure 1). Hailey-Hailey disease, pemphigus vegetans, warty A 4-mm punch biopsy revealed acantholysis in- dyskeratoma, and squamous cell carcinoma. These volving the suprabasal layers of the epidermis and disorders are usually distinguished from each other on dyskeratosis with some cells resembling corps ronds the basis of clinical and histopathological features. and grains (Figure 2). Direct immunofluorescence was When performing a diagnostic evaluation, it is cru- negative, and treatment with topical tretinoin 0.05% cial to establish a negative family history. This helps cream produced no significant improvement. to distinguish acantholytic dermatosis of the vul- vocrural area from Darier’s disease and Hailey-Hailey Comment disease,9,10 which are autosomal dominant acan- Acantholytic dermatosis of the vulvocrural area pre- tholytic disorders. Immunofluorescence studies also sents as solitary or multiple skin-colored to white, are significant because negative results will rule out smooth papules or plaques.2-6 The lesions are most pemphigus vegetans.7 commonly located on the labia majora but also may Grover’s disease (transient acantholytic dermato- be located elsewhere in the perineum, in the inguinal sis) also produces histologically similar lesions. How- folds, or on the superior medial aspects of the thighs.4 ever, it is excluded from the differential diagnosis Two cases have been reported in which the sub- because, unlike acantholytic dermatosis of the vul- mammary region also was involved.5,6 The papules vocrural area, it consistently involves the trunk and may be painful or pruritic.3,5 Concurrent Candida often resolves within weeks to months.12 vaginitis was observed in a small minority of cases.4 The etiology and pathogenesis of acantholytic Acantholytic dermatosis of the vulvocrural area dermatosis of the vulvocrural area are not fully elu- displays histological features of both Hailey-Hailey cidated. Electron microscopy shows a disappearance 218 CUTIS® ACANTHOLYTIC DERMATOSIS of intercellular connections and a reduced number REFERENCES of desmosomes.5 In addition, there is detachment 1. Ackerman AB. Focal acantholytic dyskeratosis. Arch Der- of tonofilaments from the desmosomal plate and matol. 1972;106:702-706. perinuclear aggregations of tonofilaments.5 The un- 2. Chorzelski TP, Kudejko J, Jablonska S. Is papular acan- derlying cause of these changes is unknown. It has tholytic dyskeratosis of the vulva a new entity? Am J Der- been hypothesized that the warm, moist environ- matopathol. 1984;6:557-560. ment of the genital region triggers this disorder and 3. Coppola G, Muscardin LM, Piazza P. Papular acantholytic accounts for the localization of lesions to the vul- dyskeratosis [letter]. Am J Dermatopathol. 1986;8:364-365. vocrural area.4,8 Furthermore, Coppola et al3 suggest 4. Cooper PH. Acantholytic dermatosis localized to the vul- a possible connection between ovarian hormones vocrural area. J Cutan Pathol. 1989;16:81-84. and the pathogenesis of this disease, based on their 5. Van Joost T, Vuzevski VD, Tank B, et al. Benign persistent patient’s premenstrual exacerbations of itching papular acantholytic and dyskeratotic eruption: a case report and burning. and review of the literature. Br J Dermatol. 1991;124:92-95. Effective treatment of acantholytic dermatosis of 6. Leverkus M, Rose C, Brocker EB, et al. Intertrigenous acan- the vulvocrural area is difficult. The lesions tend to tholytic dermatosis: abortive form of Darier’s disease or a persist for years. With the exception of topical specific entity? Hautzart. 1999;50:733-738. tretinoin cream, which has been reported to give 7. Wong KT, Wong KK. A case of acantholytic dermatosis of only a transient improvement in a single patient,4 the vulva with features of pemphigus vegetans. J Cutan there is no evidence that any topical or systemic Pathol. 1994;21:453-456. medication can ameliorate or cure this dermatosis. 8. Wong TY, Mihm MC Jr. Acantholytic dermatosis localized Chorzelski et al2 described the failure of cortico- to genitalia and crural areas of male patients: a report of steroids (local and systemic) and sulfones to im- three cases. J Cutan Pathol. 1994;21:27-32. prove the eruption. Coppola et al3 also reported the 9. Wieselthier JS, Pincus SH. Hailey-Hailey disease of the failure of multiple treatments. vulva [letter]. Arch Dermatol. 1993;129:1344-1345. Surgical treatments, although impractical for 10. Salopek TG, Krol A, Jimbow K. Case report of Darier dis- multiple lesions, have proven more effective than ease localized to the vulva in a 5-year-old girl. Pediatr Der- medications. Cooper4 reports that excision of lo- matol. 1993;10:146-148. calized lesions is curative, based on follow-up peri- 11. Duray PH, Merino MJ, Axiotis C. Warty dyskeratoma of ods ranging from 3 to 30 months. Furthermore, the vulva. Int J Gynecol Pathol. 1983;2:286-293. some papules have been successfully removed 12. Heenan PJ, Quirk CJ. Transient acantholytic dermatosis. by electrocautery.2 Br J Dermatol. 1980;102:515-520. DISCLAIMER The opinions expressed herein are those of the authors and do not necessarily
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