Epidermolytic Palmoplantar Keratoderma With Constriction Bands on Bilateral Fifth Toes

Case of epidermolytic palmoplantar keratoderma with knuckle pads

“…Recently, Funakushi et al. 15 reported an EPPK case with pseudoainhum‐like constriction bands and keratotic erythema similar to our case. The border of K9 expression may vary depending on the individual and lead to distinctive clinical features.…”

Vörner type palmoplantar keratoderma: novel KRT9 mutation associated with knuckle pad-like lesions and recurrent mutation causing digital mutilation

“…In the previous patients with EPPK, however, no digital mutilation has been reported. It should be noted that a Japanese EPPK patient with slight constriction of the fifth toes has been recently reported and the proband of that study had the same mutation as that of our patient 7 . The distinct phenotypic heterogeneity in family 2 may be explained by additional genetic and/or epigenetic factors in the proband.…”

Hereditary palmoplantar keratodermas. Part I. Non‐syndromic palmoplantar keratodermas: classification, clinical and genetic features

“…11,13 Pseudoainhum and digit mutilations have been rarely described in patients showing EPPK due to KRT9 mutations. 14,15 More recently, mutations in KRT1 have also been reported in EPPK cases [16][17][18][19][20] (Table 2). EPPK due to KRT1 mutations may extend over wrists (transgrediens) and present distant hyperkeratosis, 15,16 or may spread onto the dorsal aspects of hands, feet and skin over the Achilles' tendons (progrediens and transgrediens) in the Greither's disease.…”

“…Histopathological features are typical of EPPK . Pseudoainhum and digit mutilations have been rarely described in patients showing EPPK due to KRT9 mutations . More recently, mutations in KRT1 have also been reported in EPPK cases (Table ).…”