Familial porokeratosis of Mibelli: A peerless entity
Keywords:
Ala nasi, cornoid lamella, finger, parakeratosis, porokeratosisAbstract
Porokeratosis are a group of hereditary or acquired disorders characterized by annular plaques with an atrophic centre and a hyperkeratotic peripheral ridge. Pathologically, porokeratosis is characterized by a column of parakeratotic cells, called the cornoid lamella. This report describes a 55-year-old male with his two sons affected by porokeratosis of Mibelli and it was confirmed histopathologically. The rarity of this disorder in family, its clinical exuberance and the destructive character of the lesions, as well as, nasal, finger and abdomen involvement were unusual in case of porokeratosis.References
Ruiz Villaverde R, Alonso Corral MJ, Sanchez Cano D et al. Linear porokeratosis of Mibelli. An Pediatr (Barc). 2005;63:376-7.
Bacharach-Buhles M, Weindorf N, Altmeyer P. Porokeratosis Mibelli gigantea. Hautarzt. 1990;41:633-5.
Lin JH, Hsu MM, Sheu HM, Lee JY. Coexistence of three variants of porokeratosis with multiple squamous cell carcinomas arising from lesions of giant hyperkeratotic porokeratosis. J Eur Acad Dermatol Venereol. 2006;20:621-3.
Hartman R, Rizzo C, Patel R et al. Porokeratosis palmaris et plantaris disseminata or a disseminated late-onset variant of porokeratotic eccrine ostial and dermal ductal nevus (PEODDN) with follicular involvement. Dermatol Online J. 2009.15 (8):8.
Jurecka W, Neumann RA, Knobler RM. Porokeratoses: immunohistochemical, light and electron microscopic evaluation. J Am Acad Dermatol. 1991;24:96-101.
Dongre A, Adhe V, Sanghavi S. Genital Porokeratosis: A rare entity. Indian J Dermatol. 2013;58:81-3.
Vasudevan B, Chatterjee M, Grewal R et al. A case of disseminated superficial porokeratosis associated with giant Porokeratosis in pregnancy. Indian J Dermatol. 2014;59:492-4.
Goerttler EA, Jung EG. Porokeratosis of Mibelli and skin carcinoma: a critical review. Humangenetik. 1975;26:291-6.
Bhutani LK, Kanwar AJ, Singh OP. Porokeratosis of Mibelli with unusual features. Dermatologica. 1977;155:296-300.
Commens CA, Shumack SP. Linear porokeratosis in two families with disseminated superficial actinic porokeratosis. Pediatr Dermatol. 1987;4:209-14.