BACKGROUND Pachyonychia congenita (PC) is a group of autosomal dominant keratinizing disorders caused by a mutation in one of 4 keratin genes. Previous classification schemes have relied on data from case series and case reports. Most patients in these reports were not genetically tested for PC. OBJECTIVE We sought to clarify the prevalence of clinical features associated with PC. METHODS W...

In the district of Srikakulam in Andhra Pradesh in South India the habit of reverse smoking is widespread. In a house to house survey of oral cancer and precancerous conditions, comprising 10,169 villagers, 43·8% were reverse smokers; the female: male ratio being 1·7: 1. Ten previously undiagnosed cases of oral cancer, 9 located to palate, were found. The prevalences for leukoplakia, preleukopl...

Pachyonychia congenital (PC) is a rare autosomal dominant genodermatosis characterized hyperkeratosis affecting the nails and palmoplantar areas, oral leukokeratosis, and cystic lesions. A 39-year-old woman with PC type 1 (Jadassohn-Lewandowsky syndrome) and B-cell lymphoma is described. No similar disorders or parental consanguinity were found in her family. Typical features of PC developed si...

PC is commonly described as a rare genodermatosis characteristically manifesting as massive subungual hyperkeratosis with nail thickening, focal palmoplantar keratoderma alongwith deep f issur ing and blistering, oral leukokeratosis and discrete follicular hyperkeratosis [1,2]. Other features including abnormalities of teeth, hairs and larynx can also be seen depending on the clinical types. On...

Pachyonychia Congenita (PC) is a rare autosomal dominant keratin disorder that affects a number of ectodermal structures including the nails and palmoplantar skin, and often involves the oral mucosa, tongue, larynx, teeth and hair. Clinical features are usually present at birth or early infancy. There are two main subtypes of PC. Fingernail thickening and oral keratosis are more common and seve...

Pachyonychia congenital (PC) is a rare genodermatosis affecting the nails, skin, oral mucosae, larynx, hair, and teeth. General clinical presentation includes thickening of finger and toenails, painful plantar keratoderma, hyperhidrosis, oral leukokeratosis, follicular keratosis, palmar keratoderma, cutaneous cysts, hoarseness, twisted hair and prenatal or natal teeth. Clinically, PC is d...

Erratum After publication of [1] the authors realised that the original publication had two descriptive errors when referring to diseases. “periodontal” was written rather than “mucous membrane” in the article on page 1, lines 12 and 25. “periodontal disease” was written rather than “mucous membrane hyperkeratosis” in the article on page 2, lines 72 and 73. The correct spelling of these is incl...

As reviewed by [Cooper, S. J. (2008). From Claude Bernard to Walter Cannon: emergence of the concept of homeostasis. Appetite 51, 419-27.] Claude Bernard's idea of stabilisation of bodily states, as realised in Walter B. Cannon's conception of homeostasis, took mathematical form during the 1940s in the principle that externally originating disturbance of a physiological parameter can feed an in...

pachyonychia congenital (pc) is a rare autosomal dominant genodermatosis characterized hyperkeratosis affecting the nails and palmoplantar areas, oral leukokeratosis, and cystic lesions. a 39-year-old woman with pc type 1 (jadassohn-lewandowsky syndrome) and b-cell lymphoma is described. no similar disorders or parental consanguinity were found in her family. typical features of pc developed si...

Dear Editor, A 10-year-old boy presented with progressive thickening of the nails over the previous 5 years. He did not have any other cutaneous and/or systemic symptoms. He was born to healthy non-consanguineous parents following an uneventful pregnancy. Upon examination, he was found to have symmetrical involvement of the finger and toenails with marked hard thickening of the distal portion o...