Erythrokeratoderma variabilis, characterized by migrating erythema and fixed keratotic plaques, is a rare congenital disorder which has recently been connected with connexin (Cx)30.3 or Cx31 gene mutations. We present a 9-month-old Japanese girl who exhibited the typical clinical features of the disease, but carried no Cx30.3 or Cx31 gene mutations. Histopathologically, regular acanthosis with ...

Linear porokeratosis is one of the infrequent variants of porokeratosis, a rare disorder of keratinization that may develop into several epidermal malignancies, among them squamous cell carcinoma. Clinical surveillance for malignancy is imperative, but in cases when large or many lesions are present, surgical removal of porokeratosis lesions would result in an unfavorable amount of scarring. A ...

BACKGROUND In addition to the well-defined hereditary primary ichthyoses, many sporadic or less well-defined keratinization disorders with or without systemic manifestations have been reported. Herein we describe ichthyosiform dermatosis associated with type 2 diabetes mellitus. OBSERVATIONS The patients were members of a large Arab family with heavy consanguinity. Eighteen members were affec...

Pityriasis rubra pilaris (PRP) is a chronic disorder of keratinization of unclear pathogenesis. PRP-like eruptions induced by drugs have rarely been described. A previously healthy 29-year-old man presented with a generalized, rapidly spreading, erythematosquamous dermatosis, that started three days after initiation of subcutaneous insulin therapy for diabetes mellitus type 1. Clinical and hist...

Circumscribed palmoplantar hypokeratosis is a recently recognized dermatosis and rarely reported. It was first described in 2002 and is characterized by localized loss of the horny layer in the palmoplantar area. This dermatosis is clinically presented with a sharply circumscribed, reddish and asymptomatic plaque with slightly depressed surface localized on the palms or the soles. The clinical ...

In the present study on recurrent pyoderma, dogs with a history of more than three episodes of skin infections in a period of one year were selected. The associated conditions and (or) underlying factors revealed upon thorough investigation were demodicosis, Malassezia dermatitis, flea infestation, hypothyroidism, keratinization disorder (seborrhea), combination of Malassezia dermatitis and tic...

Background. Darier's disease (DD), also known as Keratosis Follicularis or Darier-White disease, is a rare disorder of keratinization. DD can present as a generalized autosomal dominant condition as well as a localized or segmental postzygotic condition (Vázquez et al., 2002). Clinical features of DD include greasy, warty papules and plaques on seborrheic areas, dystrophic nails, palmo-plantar ...

Porokeratotic Eccrine Ostial and Dermal Duct Nevus (PEODDN) is a rare disorder of keratinization characterized by the presence of cornoid lamellae and association with eccrine sweat ducts. These lesions are usually benign and asymptomatic, so treatment is often for cosmetic purposes. Current therapies for PEODDN are either insufficient or impractical. We present a case of PEODDN treated with to...

Erythrosis pigmentosa peribuccalis (Brocq) (or erythrosis pigmentosa mediofacialis) and erythromelanosis follicularis faciei et colli, have been regarded as different disorders, mainly because the first occurs on the mediofacial area and is common in women and the second mostly occurs pre-auricularly in men. Both conditions show histological signs of abnormal follicular keratinization with tele...

Porokeratosis is a disorder of epidermal keratinization characterized clinically by a distinctive ridge-like border, and histologically by cornoid lamellae. The known clinical variants of porokeratosis are: classic porokeratosis of Mibelli, disseminated superficial (actinic) porokeratosis (DSAP), porokeratosis palmaris et plantaris disseminata, linear porokeratosis and punctate porokeratosis. I...