Hereditary Epidermolytic Palmoplantar Keratoderma

Hereditary Painful Callosities: Case Report and Review of the Literature

“…Palmoplantar keratodermas (PPK) are a nonhomogenous group of keratinization disorders that predominately involve the hands and feet. 1 This diffuse group of conditions all involve disorders of keratinization which present as gross thickening of the palmoplantar skin. Keratosis palmoplanataris nummularis is a form of PPK described by Wachters et al 13 They described 13 patients in two families with both palmar and plantar lesions and four patients in one family with exclusively plantar lesions.…”

“…Gait evaluation was pertinent for a bilateral antalgic gait, with the patient walking as though he were 1 on 'hot coals.' Examination of his skin revealed no other areas of callous formation aside from that on the soles of his feet.…”

Inflammatory Familial Palmoplantar Keratoderma: Greither’s Disease?

“…Ours show anomalies of tonofilaments resembling those found in hereditary epidermolytic palmoplantar keratoderma of the Vorner type. This rare form of PPK [10][11][12][13] is inherited as an autosomal dominant trait like Greithcr's disease and Unna-Thost's keratoderma. Because of a similar mode of inheritance and clinical aspect between Unna-Thost's keratoderma and epidermolytic keratoderma of the Vorner type, its real frequency is prob ably underestimated [ 14] since histology is not routinely performed in all cases of PPK.…”

Hereditary Palmoplantar (Epidermolytic) Keratoderma: Illustration Through a Familial Report

“…The clear areas of cytoplasm are filled with a fibrillar material and cellular organelles, abnormal clumps of tonofilaments, and keratohyalin granules. [10][11][12][13] Electron microscopy is a useful adjunct to define the details of the features and might assist in differentiating clinical variants of HPPK. Histopathologic examination performed on 91 biopsies taken from the dominant form of HPPK revealed no case of epidermolytic PPK.…”

“…Large clear spaces in the cells of granular and upper spinous layers are other associated components. The clear areas of cytoplasm are filled with a fibrillar material and cellular organelles, abnormal clumps of tonofilaments, and keratohyalin granules 10–13 . Electron microscopy is a useful adjunct to define the details of the features and might assist in differentiating clinical variants of HPPK.…”