Familial pityriasis rubra pilaris

(Juvenile) Pityriasis rubra pilaris

“…Genetic factors have been considered to be important. 63 The pattern of inheritance by and large is autosomal dominant with variable expressivity. 1,2 Autosomal recessive inheritance has also been recorded.…”

“…Keratin 6 and 16 are also expressed, indicating keratinocyte activation. 63 A recent addition to the histopathology of classical PRP is the observation of acantholysis and focal acantholytic dyskeratosis. 78,[83][84][85] Magro and Crowson 78 recorded acantholytic dyskeratosis in 23 of 32 biopsies from PRP patients, whereas it was absent in 23 psoriatic controls.…”

Pityriasis Rubra Pilaris (PRP): Report of Four Cases

“…The current de-scription of clinical findings of our two patients from one family, may once again focus attention on a rare familial pityriasis rubra pilaris which expresses itself as an autosomal dominant disorder. Essentially, the clinical features and microscopic findings in sporadic and familial PRP agree (13). The aforementioned clinical description leads readily to the diagnosis of pityriasis rubra pilaris.…”

Atypical Pityriasis Rubra Pilaris Associated with Arthropathy and Osteoporosis: A Case Report with 15‐Year Follow‐Up

“…Most cases are acquired, but a familial form does exist. This is generally seen with an autosomal dominant inheritance with variable expressivity (8). Autosomal recessive inheritance has also been described (9).…”