Key features ▪ Dermatitis herpetiformis (DH) is differentiated from linear IgA bullous dermatosis (LABD) on the basis of granular IgA versus linear IgA deposition at the basement membrane zone, as observed by direct immunofluorescence ▪ DH is a cutaneous manifestation…
Evidence Levels: A Double-blind study B Clinical trial ≥ 20 subjects C Clinical trial < 20 subjects D Series ≥ 5 subjects E Anecdotal case reports Open full size image Dermatitis herpetiformis (DH) is a cutaneous manifestation of celiac disease…
Key Points Neurofibromatosis is an autosomal dominant disorder characterized by cutaneous neurofibromas, café-au-lait macules, and myriad systemic features with marked variability of expression. Patients with neurofibromatosis are at risk for the development of a malignant peripheral nerve sheath tumor. Increasing…
Key Points Gastrointestinal hemorrhage may be associated with pseudoxanthoma elasticum or hereditary hemorrhagic telangiectasia. Adenomatous polyposis may be associated with epidermoid tumors, fibromas, and dermoid tumors. Peutz–Jeghers syndrome and Cowden’s syndrome are associated with hamartomatous polyps. Malabsorption may be associated…
Key Points Immunobullous diseases are a complicated multisystem management challenge requiring advanced immunosuppressive regimens. Pathogenesis has been linked to disease-specific autoantibodies to structural antigens within the epidermis and basement membrane zone. The location of blistering, risk of subsequent scarring, and…