Unna-Thost keratoderma is an autosomal dominant inherited disorder of keratinization, with mutation in Keratin 1 gene. The eponym refers specifically to nonepidermolytic keratoderma, although the original Thost family is now known to have had epidermolytic keratoderma. The condition usually presents in the first few months of life and is usually obvious by the age of 4 years. It rarely appears ...

P rimary biliary cirrhosis (PBC) is an autoimmune disease of the liver characterized by the presence of highly specific antimitochondrial antibodies with resulting immune-mediated injury of small intrahepatic bile ducts. Palmoplantar keratodermas (PPKs) are characterized by hyperkeratosis of the skin on the palms and soles. Multiple cases have been reported associating PPK with autoimmune thyro...

The association of herpetoid corneal changes with circumscribed palmo-plantar keratoderma was first described by Richner (I938). Hanhart (1947) suggested that this represented a recessively inherited ectodermal syndrome, and the condition is sometimes called the Richner-Hanhart syndrome. Subsequent reports have been relatively few: Cremona (I 957); Kuske (I959); Franceschetti and Thier (I96I); ...

administration of multiple cycles of topical and systemic corticosteroid therapy, topical tazarotene, ciclosporin at a dose of 3 mg/kg/d (whose efficacy cannot be adequately evaluated due to treatment interruption after a month because of failure to attend follow-up), and methotrexate at a maximum dose of 20 mg/wk, which the patient continues to receive. Interestingly, despite hypertrophic LP b...