Papillon Lefevre syndrome is an extremely rare autosomal recessive disorder characterized by diffuse palmoplantar hyperkeratosis, rapidly progressive and devastating periodonitis, pyodermas. The etiopathogenesis of the multifactorial, with genetic immunological factors playing a major role. Consanguinity contributing factor. Genetic mutations gene 11q14- q21 encoding for cathepsin-c, lysosomal ...

An 8-year-old girl with the classic findings of keratoderma hereditaria mutilans (Vohwinkel's syndrome) was seen. Treatment with isotretinoin was instituted to decrease the hyperkeratosis and to prevent further autoamputation. After a 12-week course at 2 mg per kg per day, the patient had only minimal decrease in the amount of hyperkeratosis. Because of the well-known long-term risks of systema...

Epidermolytic hyperkeratosis (EHK) or bullous congenital ichthyosiform erythroderma is a rare autosomal dominant disorder characterized by an early onset, with erythroderma and bullous lesions, leading to severe generalized hyperkeratosis in adulthood. Mutations have been found in keratin 1 and keratin 10 genes. The clinical manifestations of EHK present striking heterogeneity and at least six ...

Mycosis fungoides is one of the great imitators in dermatology; it can mimic many dermatoses. Nevoid hyperkeratosis of the nipple and areola is a rare idiopathic disease with typical clinical features of verrucous thickening and brownish discoloration of the nipple, areola or both. Here, a 16-year-old male patient with mycosis fungoides mimicking nevoid hyperkeratosis of the nipple and areola h...

A 5-year-old caucasian boy, born of non-consanguineous parents, was referred to the dermatology department due palmar hiperlinearity and multiple 2-3 mm hyperkeratotic circular lesions in soles (Fig. 1). The remaining physical examination unremarkable. There a family history palmoplantar hyperkeratosis mother maternal grandfather. To spare child, punch skin biopsy taken from his mother. Patholo...

OBJECTIVES The objectives of this study were to determine the frequency of metatarsal pain and of hyperkeratosis on the plantar forefoot in female professional flamenco dancers, and to determine whether there is a relationship between the two disorders. METHOD Forty-four female professional flamenco dancers, with a minimum activity of 25 hrs/wk, participated in this cross-sectional study. The...

Hyperpigmentation, hyperkeratoses, and Bowen's disease are hallmarks of chronic arsenic exposure. The association between arsenic-induced skin lesions and subsequent internal cancers is examined by using a community-based prospective study. The cohort was enrolled from an arseniasis-endemic area in southwestern Taiwan, where 2,447 residents participated in skin examinations during the late 1980...