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); ...

Erythema gyratum reopens is a slowly expanding, mildly scaling dermatosis with a "wood-grain" pattern and is seen in patients with an underlying malignancy. To date only 49 cases have appeared in the literature, 41 of which (84%) were associated with a neoplasm, most commonly of the lung. Several patients also had pruritus, palmoplantar keratoderma, ichthyosis, vesiculobullous lesions, and/or e...

Pachyonychia congenita (PC) is a rare autosomal dominant genetic skin disorder due to a mutation in any one of the five keratin genes, KRT6A, KRT6B, KRT6C, KRT16, or KRT17. The main features are palmoplantar keratoderma, plantar pain, and nail dystrophy. Cysts of various types, follicular hyperkeratosis, oral leukokeratosis, hyperhidrosis, and natal teeth may also be present. Four unrelated Ind...

Helen Ollendorff Curth (1899-1982), is one of the pioneers in dermatology. In 1954, she and Madge Thurlow Macklin (1893–1962), an American medical geneticist, reported, a rare congenital genodermatosis that was later known as Curth-Macklin syndrome. This syndrome is a rare autosomal dominant skin disorder characterized by extensive hyperkeratosis and palmo-plantar keratoderma. This report shed ...

All member of family referred to our clinic complaining of white nails. Physical examination revealed clinical features of leukonychia totalis and also the presence of sensor ineural hearing loss (SNHL), palmoplantar keratoderma (PPK) and knuckle pads, the four essential criteria for the diagnosis of Bart Pumphrey syndrome. Three generations were affected with variable presentations in ma...