Juvenile pityriasis rubra pilaris with isolated IgA deficiency

(Juvenile) Pityriasis rubra pilaris

“…The efficiency of retinoids in the treatment of PRP points to the probable role of vitamin A metabolism. Hypogammaglobulinemia 54 and isolated IgA deficiency 55 have also been recorded in juvenile PRP. The widely reported association of PRP with autoimmune disorders may point to a probable underlying immunologic abnormality.…”

“…53 recorded enhanced spontaneous activity of T-suppressor cells, with an impairment of T-helper functions, in a 6-year-old child with PRP. Hypogammaglobulinemia 54 and isolated IgA deficiency 55 have also been recorded in juvenile PRP. Circumscribed juvenile-onset PRP was recorded in two patients with Down's syndrome, one of whom also had vitiligo.…”

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

“…The exact mechanism remains unclear, although some researchers state that the development of PRP may be related to an abnormal immune response to antigenic triggers (1). PRP has been reported in patients with hypogammaglobulinemia (22,23) and furunculosis (22), T-lymphocyte abnormalities (24), lymphocyte hypersensitivity to superantigens (25), and HIV infection (26,27). An immunologic examination on our patient has not been done at this time.…”