Genitogluteal porokeratosis: a clinical review

Abstract

Porokeratosis is an uncommon disorder of keratinization that presents with keratotic papules or annular plaques that expand centrifugally with a thread-like elevated border. A distinctive histologic structure, the cornoid lamella, is diagnostic of this disorder and consists of a column of parakeratosis with the absence of the granular layer and dyskeratotic cells in the upper spinous zone. Porokeratosis confined to the genitogluteal region is rare and may be subclassified into three types, namely, classical porokeratosis on the genital region, ptychotropic porokeratosis most often seen in the natal cleft and buttocks and penoscrotal porokeratosis that is seen on the penis and adjacent scrotal skin in young men in their third decade of life. Genitogluteal porokeratosis is usually pruritic and may be undiagnosed for several years as it does not resemble classical porokeratosis in many cases; however, a biopsy is diagnostic. In general, response of genital porokeratosis to any modality of treatment is disappointing. No malignant changes have hereto been reported in porokeratosis restricted to the genitogluteal region.

Porokeratosis ptychotropica: a rare case report with unusual presentation

“…5 Stone et al, described a similar case, in which a 32-year-old man with a 13-year history of pruritic lesions also confined to the natal cleft and he named it as "perianal inflammatory verrucous porokeratosis. 2 The term genitogluteal porokeratosis refers to the porokeratosis restricted to male and female genitalia and those restricted to buttocks. It can be broadly divided into three groups namely classical porokeratosis restricted to the genital region, ptychotropic porokeratosis and penoscrotal porokeratosis (Table 1).…”

“…Involvement of the genital region and adjacent areas like buttocks, perineum, groin and proximal thigh may occur as a part of generalised porokeratosis, but porokeratosis localized to the genitogluteal region is rare. 2…”

Genitogluteal porokeratosis: An unusual presentation treated successfully with the novel combination of imiquimod 5% cream and photodynamic therapy

“…Several therapeutic options have been described; surgical treatment is optional, depending on the number and size of the lesions. [1][2][3][4] Other treatment options include cryotherapy, CO 2 laser therapy, oral and topical retinoids, topical vitamin D3 analogues, keratolytic agents, topical 5-fluorouracil, and diclofenac sodium 3% gel, all of which have inconsistent effects. A recent review found that topical imiquimod may be a preferred treatment for PM.…”

“…The risk is higher in larger lesions that have been developing over longer periods of time, among the elderly and immunocompromised patients, and especially in linear porokeratosis and PM types. [2][3][4] For all these reasons, GGP treatment and regular followup are recommended. Several therapeutic options have been described; surgical treatment is optional, depending on the number and size of the lesions.…”

“…Involvement of the genital and gluteal areas may occur as part of generalized porokeratosis. However, the presence of lesions in this region only (buttocks, perineum, groin, and thighs) is generally classified as a very uncommon presentation of porokeratosis of Mibelli (PM), with approximately 50 cases reported in the literature 1,3. The majority of patients are middle-aged males, often with severe pruritus and the diagnosis is usually delayed due to the atypical location.…”