Linear porokeratosis with nail dystrophy

Onychodystrophy due to porokeratosis of Mibelli: a rare association

“…They include splitting, longitudinal ridging, nail dystrophy, and pterygium unguis formation (2)(3)(4)(5)(6)(7)(8)(9)(10). Involvement of the nail bed and/or nail matrix by abnormal clonal keratinocytes causes onychodystrophy (2). After shedding of the nail plate, the nail bed shows warty debris, which was described as "hyperkeratosis eccentric atrophicans" by Respighi (11).…”

“…In a study of a patient with generalized PM with buccal lesions, toenail involvement resembled changes of onychomycosis (12). A few authors have observed shortening, distal digital narrowing, bone resorption, flexion contracture, and mutilation with loss of the digits involved above PM lesions and linear porokeratosis (2,6,13). Hence, patients with PM and linear porokeratosis, especially when involving the extremities, should undergo radiographic evaluation.…”

“…Topical therapies such as keratolytics, retinoids, vitamin D derivatives, 5-flurouracil ointment, imiquimod cream, diclofenac gel, dermabrasion, cryotherapy, photodynamic therapy, carbon dioxide laser, excision, and skin grafting have been used alone or in combination, whereas systemic retinoids are useful in generalized porokeratosis cases. However, none of them have shown promising efficacy and long-term remission (1,2,14). There is no clear consensus on treatment, especially when nail dystrophy is associated with porokeratosis.…”

Unilateral generalized linear porokeratosis with nail dystrophy

“…To date, there have been only a few case reports of onychodystrophy in linear porokeratosis. 2,3 In one case, there was bony narrowing of the digits. 3 Six main variants of porokeratosis have been described.…”

“…5 Though the exact pathogenesis of dystrophic nail is not yet understood, it is possible that nail changes occur through the involvement of the nail matrix and nail bed by atypical hyperproliferative keratinocytes, ultimately resulting in destruction of the whole nail. 2 Cases with nail involvement in linear porokeratosis are not as rare as those in the other porokeratosis variants. We speculate that this is because many cases of linear porokeratosis involve the distal portions of the extremities.…”