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

Papular Acantholytic Dyskeratosis of the Inguinal Area in a 49-Year-Old Man

“…Lesions have been reported on the genitalia, perineum, inguinal area, and upper thigh1. PAD shows very similar histopathologic findings with that of both Hailey-Hailey and Darier diseases (HHD and DD, respectively), although HHD tends to show more diffuse and DD shows more prominent dyskeratosis than PAD2. HHD and DD are autosomal dominant diseases caused by loss of function mutation of the ATP2C1 and ATP2A2 calcium pump genes, respectively.…”

“…Recently, heterozygous mutations in the ATP2C1 and ATP2A2 genes were found in three cases and one case of PAD, respectively12. It is speculated that segmental mosaic mutations in ATP2C1 or ATP2A2 gene, whether somatic or germinal mutation, can result in the distinct clinical phenotype referred to as PAD1.…”

“…It is speculated that segmental mosaic mutations in ATP2C1 or ATP2A2 gene, whether somatic or germinal mutation, can result in the distinct clinical phenotype referred to as PAD1. Since cases of localized HHD or DD to genitocrural area have been rarely reported, genetic study might be helpful to rule-out HHD or DD2.…”

“…Cryotherapy, topical steroids, topical tretinoin, topical tacrolimus, oral isotretinoin, and laser ablation have been reported with variable results2.…”

Hailey-Hailey disease with lichenoid lesions around the anus

“…Surgical therapy, CO 2 or Er:YAG laser ablation, dermabrasion, and argon plasma coagulation are reportedly useful for extensive perianal lesions of HHD. [ 7 , 8 ] This case has proven that tacrolimus ointment may also be helpful for chronic perianal HHD.…”