The majority of pemphigus vulgaris (PV) patients suffer from a live-threatening loss of intercellular adhesion between keratinocytes (acantholysis). The disease is caused by auto-antibodies that bind to desmosomal cadherins desmoglein (Dsg) 3 or Dsg3 and Dsg1 in mucous membranes and skin. A currently unresolved controversy in PV is whether apoptosis is involved in the pathogenic process. The ob...

A 52-year-old man who had had WaldenstroÈm's disease for 5 years presented to our department with clinical manifestations of hyperviscosity syndrome including headaches and paresthesias of the extremities. All treatments for the haemopathy had been withdrawn 6 months earlier because of drug resistance. Besides splenomegaly, physical examination showed 2 types of cutaneous lesions: nonfollicular...

Pemphigus vulgaris (PV) is an autoimmune blistering disorder of the skin and mucosal surfaces characterized by acantholysis (loss adhesion between epidermal cells). Esophageal involvement PV underdiagnosed entity as routine diagnostic endoscopy not recommended in asymptomatic patients. Dysphagia odynophagia are common presenting symptoms; however, upper gastrointestinal bleeding (UGIB) associat...

Ichthyosis hystrix of Curth-Macklin ( IHCM) is an extremely rare keratinization disorder, with verrucous to hystrixlike hyperkeratotic plaques of varying extent. The diagnosis is based on a distinctive pattern of epidermolytic hyperkeratosis and the presence of binucleate cells under light microscopy, as well as unique electron microscopic findings of continuous perinuclear tonofibril shells in...