Epidermolytic Acanthomas Research Articles

Multiple epidermolytic acanthomas mimicking condyloma: a retrospective study of 8 cases.

Epidermolytic acanthoma (EA) is an uncommon benign cutaneous lesion. Multiple epidermolytic acanthomas (multiple EAs) are rarely reported. We retrospectively identified patients diagnosed with multiple EAs between 2005 and 2017 from our dermatopathology database and analyzed their clinical, pathological, and immunohistochemical features. We also evaluated the association of multiple EAs with human papillomavirus (HPV) infection. In total, eight patients (average age 51years; 3:1 male predominance) with multiple EAs were found. All patients had lesions on the genitocrural area. The two most common clinical diagnoses were condyloma (5/8) and soft fibroma (3/8), which were predominantly skin-colored (5/8) or whitish (2/8). The lesions were less than 1cm in diameter, and most (6/8) appeared to have a smooth surface. No molecular evidence suggesting HPV infection was found. Immunohistochemical staining showed low mitotic activity. The lesions were removed in one of two patients via cryotherapy, and one patient was treated with electrocauterization. The other five patients were followed without treatment. The genital area was the most common location for multiple EAs, which was commonly misdiagnosed clinically as condyloma. Patients presenting with uniformly small-sized, skin-colored to whitish, smooth papular lesions in the genitalia should be carefully evaluated. The specific pathological features of epidermolytic hyperkeratosis are diagnostic, and the lesions can be observed without aggressive treatment after confirmed diagnosis.

Hyperkératose épidermolytique génitale (acanthomes épidermolytiques génitaux multiples)

Epidermolytic hyperkeratosis presents a particular histological image common to several clinical pictures, including that of keratinopathic ichthyoses. It may also occur fortuitously in various tumoral and inflammatory lesions. It is the elementary histopathological lesion of epidermolytic acanthoma, which may either be single or multiple, and when it occurs in the genital area, is known as epidermolytic hyperkeratosis of the genitalia or multiple epidermolytic acanthoma of the genitalia. Herein, we report two characteristic cases of epidermolytic hyperkeratosis of the genitalia. The first patient was a 50-year-old woman consulting for vulvar pain in whom clinical examination revealed the presence of multiple papules on the labia majora and minora. The second patient was a 44-year-old man consulting for verrucous lesions of the scrotum. In both cases, biopsy revealed an histopathological aspect identical with acanthosis, hyperkeratosis, changes in the keratinocytes, in which the cytoplasm contained clear vacuoles, numerous keratohyalin granules and eosinophilic bodies, resulting in a diagnosis of epidermolytic hyperkeratosis of the genitalia. Epidermolytic hyperkeratosis of the genitalia is a rare disease, occurring in middle-aged men and women, but chiefly men. The lesions found on the genital organs may be either single, or, more frequently, multiple, and are described as hyperkeratotic papules, which are also reported under the term multiple epidermolytic acanthomas of the genitalia. The aetiology is unknown; certain authors incriminate a traumatic factor; the role of human papillomavirus (HPV) has been suggested but immunohistochemical studies and molecular biology studies generally reveal no viral DNA. Where lesions cause discomfort to the patient, treatment consists of emollients combined with destruction by cryotherapy or CO2 laser. Imiquimod, calcipotriol, tacrolimus and pimecrolimus have all resulted in regression of lesions.

Presentation of Epidermolytic Acanthomas as Multiple Tan Papules on the Vulva

Epidermolytic hyperkeratosis is a histological reaction pattern seen in a variety of disease processes, including epidermolytic ichthyosis, Vorner's epidermolytic palmoplantar keratoderma, epidermal nevus, and solitary epidermolytic acanthoma. Here we present the case of a 59-year-old woman with multiple asymptomatic papules on her vulva. Clinical differential diagnoses included condyloma acuminata, seborrheic keratoses, bowenoid papulosis, adnexal tumors, and papular acantholytic dyskeratosis. Shave biopsy revealed findings consistent with epidermolytic hyperkeratosis. This case represents an interesting presentation of focally disseminated vulvar epidermolytic acanthomas and highlights the importance of a biopsy in establishing this diagnosis.

A Case of Multiple Epidermolytic Acanthoma of the Scrotum: Is the Human Papillomavirus a Culprit?

Dear Editor: Epidermolytic acanthoma is a rare benign tumor that was first described in 1970 by Shaprio and Baraf. It usually presents in adulthood as an asymptomatic tumor <1 cm in diameter with a verrucous surface1. Herein, we report a very uncommon case of multiple epidermolytic acanthoma localized on the scrotum. A 60-year-old man visited our clinic with asymptomatic scrotal dermatosis that was discovered several months ago. He had no significant medical history and denied any triggering factors such as trauma, immunosuppressive therapy, or radiation exposure. Physical examination revealed multiple well-demarcated gray to whitish round papules on the scrotum (Fig. 1). Laboratory tests, including complete blood count, chemical battery, and screening tests for HIV and treponemal disease such as syphilis, were performed. No abnormal finding was found. Skin biopsy showed hyperkeratosis without parakeratosis and a hypertrophic granular layer with numerous keratohyalin granules. Separation and edematous changes in the spinous layer were also observed (Fig. 2). The histopathological findings were consistent with epidermolytic acanthoma. The differential diagnosis included epidermolytic verrucous nevus, bullous congenital ichthyosiform erythroderma, and Vorner's palmoplantar keratoderma with a rather distinct clinical aspect. Seborrhoreic keratoses, solar keratoses, or Bowen's disease could also show some focal changes of epidermolytic hyperkeratosis. We also tested for the presence of the human papillomavirus (HPV) genome by using a novel HPV DNA chip (GG HPVCHIP; Goodgene Inc., Seoul, Korea). The lesion was HPV 16 positive2. Fig. 1 Multiple well-demarcated <5 mm gray to whitish round papules on the scrotum. Fig. 2 Compact hyperkeratosis with acanthosis. Perinuclear vacuolization, of variable size, of the keratinocytes in the stratum spinosum and stratum granulosum. The keratinocytes exhibited non-well-defined limits that gave the epidermis a reticulate appearance. ... Epidermolytic acanthoma is a benign, asymptomatic tumor that usually presents as a solitary papule in adulthood. The multiple or disseminated form was also reported as a rare variant1. The pathogenesis of epidermolytic acanthoma is not clear. Various contributing factors have been reported, such as HPV, other viral infections, ultraviolet radiation, immunosuppression, trauma, and mutations of genes encoding for keratin 1 and 103. Although some authors have suggested the role of HPV in the pathogenesis, immunohistochemical and molecular studies had failed to detect a viral genome in the lesions4. However, the authors used a conventional method: polymerase chain reaction followed by dot-blot analysis with specific radiolabeled detection probes, which have limitations in detecting and typing HPV. The HPV DNA chip arrayed by using multiple oligonucleotide probes for 42 types of HPV and human β-globin gene, which we used in our case, was recently developed and has been found to be a highly sensitive tool for identifying HPV2. In our case, doubt exists about whether the presence of HPV 16 was only a coincidence, because HPV can be detected in otherwise normal genital skin. Although we could not clarify the possible pathogenic role of HPV in epidermolytic acanthoma, our case is important because it is the first reported case of HPV detected on skin affected by epidermolytic acanthoma. Although larger studies including several cases are required to prove the possibility, several facts imply the role of HPV in the pathogenesis of epidermolytic acanthoma: its site of predilection is the genital area; it usually has a verrucous surface; and, in a recent report, epidermolytic acanthomas were successfully treated with topical imiquimod, which is also used to treat HPV-induced lesions5.

Anogenital epidermolytic acanthomas: effective treatment of pruritus with 0.1% tacrolimus ointment

Epidermolytic acanthoma is an uncommon benign tumor with a predilection for the anogenital region, that is characterized histologically by epidermolytic hyperkeratosis. Most reported cases are asymptomatic while some are pruritic. Treatment of this condition is challenging and not well studied. We report a 55-year-old man with pruritic anogenital epidermolytic acanthomas that persisted despite various topical and ablative therapies, and whose pruritus was not relieved with emollients and antihistamines. He was given 0.1% tacrolimus ointment twice daily and experienced a marked improvement in his pruritus within 2 weeks. At 1 month, there was marked improvement in itch score from 8 to 3.5 (maximum of 10), itch frequency from 20 times a day to once every 4 days, sleep score from 5 to 1 (maximum of 5), and Dermatology Life Quality Index score from 15 to 5 (maximum of 30). Apart from a transient burning sensation, no other adverse effects were noted. To our knowledge, this is the first report of the effective use of a topical calcineurin inhibitor in relieving the intractable itch associated with epidermolytic acanthoma.

Solitary epidermolytic acanthoma

Solitary epidermolytic acanthoma is thought to be an uncommon lesion. It can present as a solitary, localized or disseminated process that is unrelated to the genetic form of icthyosis. A retrospective review of solitary epidermolytic acanthomas was performed at the Ackerman Academy of Dermatopathology, NY, over a 2 year period. The clinical and histopathological features of solitary epidermolytic acanthomas were described in 64 biopsies from 60 individuals. In situ hybridization for human papillomavirus (HPV) was performed on all genital lesions. The incidence of epidermolytic acanthomas in our series was higher than previously reported (27.8/100,000). Genital location was the most common and the incidence of genital lesions was 8/100,000. In situ hybridization showed no evidence of genital HPV types within the lesions. The histological features of solitary epidermolytic acanthoma were re-evaluated. Genital skin was the most common location for solitary epidermolytic acanthoma, but we found no evidence to suggest a role for genital HPV types. A strong male predominance was noted, and the lesions demonstrate a wide range of clinical and pathological findings.

Multiple Epidermolytic Acanthomas Must Not be Confused with Genital Human Papillomavirus Infection

We describe two patients with multiple epidermolytic acanthomas on the scrotum initially referred to our clinic as genital warts. Both patients presented with multiple tan-colored warty papules scattered on the scrotal skin. Biopsy revealed hyperkeratosis, hypergranulosis, and clear cell changes consistent with epidermolytic acanthoma. Human papillomavirus was not detected and the lesions cleared rapidly with a liquid nitrogen cryoprobe. Despite only a few reports of multiple epidermolytic acanthomas of the scrotum, it may not be as rare as previously thought. In addition, genital localization of the wart-like lesions may lead to confusion with other conditions such as condylomata acuminata, molluscum contagiosum, or bowenoid papulosis.

Multiple scrotal epidermolytic acanthomas successfully treated with topical imiquimod

Multiple scrotal epidermolytic acanthomas successfully treated with topical imiquimod

Epidermolytic acanthomas: clinical characteristics and immunohistochemical features.

Epidermolytic hyperkeratosis in bullous congenital ichthyosiform erythroderma results from mutations in the K1 and K10 genes. Epidermolytic acanthomas are solitary or multiple lesions with microscopic features that are identical to those in bullous congenital ichthyosiform erythroderma. In this study, the clinical and epidemiologic characteristics of epidermolytic acanthomas were summarized, and the expression of keratins (using antibodies to K1, K6, K10, K14, K16, and K19) in five solitary epidermolytic acanthomas was determined using immunohistochemistry techniques. The intensity of staining for K1 and K10 was (a) less in the altered granular layer, as compared to the adjacent nonaltered granular layer of the lesional skin, and (b) less in the lesional skin as compared to the perilesional, histologically normal-appearing skin. Expression of K6 and K16 was noted not only in the basal layer and suprabasal layers of the lesions, but also in the corresponding layers of the adjacent normal skin. Staining for K14 was also observed in the basal layers and suprabasal layers of the lesional and adjacent normal epidermis; within the lesional and perilesional normal skin, the intensity of positive staining for K14 was greater in the basal layers than in the suprabasal layers of the epidermis. The specimens did not stain for K19. In conclusion, using immunohistochemistry techniques on solitary epidermolytic acanthomas, we were able to demonstrate (1) an abnormality in K1 and K10 expression in the lesional skin as compared to the adjacent, histologically normal-appearing skin and (b) the expression of hyperproliferative keratins not only with the lesional skin, but also in the perilesional normal skin. We hypothesize that the pathogenesis of epidermolytic hyperkeratosis in lesions of solitary epidermolytic acanthomas results from mutations in the K1 and K10 genes.

Isolated Dyskeratotic Acanthoma A Variant of Isolated Epidermolytic Acanthoma

Disorders affecting the maturation of the epidermis are regarded as incidental findings in normal skin and otherwise unrelated benign lesions. These include epidermolytic hyperkeratosis, focal acantholytic dyskeratosis, and pagetoid dyskeratosis. The former two entities also occur as primary pathologic lesions. We report a hyperkeratotic lesion with a specific histologic pattern consisting of dyskeratotic cells throughout the epidermis and a parakeratotic horn, with large, rounded cells at all levels of the stratum corneum. Because we believe it to be a variant of the solitary, acquired lesions characterized by abnormal epidermal maturation, such as epidermolytic acanthomas, we suggest the term "dyskeratotic acanthoma" for this lesion.