Internal disorders associated with bullous disease of the skin. A critical review.

Acquired bullous dermatoses, including pemphigus, bullous pemphigoid (BP), dermatitis herpetiformis (DH), and porphyria cutanea tarda (PCT), have been reported in association with multiple internal disorders. These associations, as well as those cases of bullous lesions in specific systemic disorders, may prove to be important markers of internal disease. Patients with acquired bullous disorders may require specialized evaluation or follow-up. Pemphigus is associated with thymoma and/or myasthenia gravis; however, the course of disease is rarely affected. Pemphigus, pemphigoid, and DH are associated with other autoimmune disorders. Particularly important are the associations of pemphigoid and rheumatoid arthritis (RA) and DH and thyroid disorders. PCT may occur with cutaneous lupus erythematosus (LE). Malignancy is rarely associated with bullous dermatoses except coincidentally, with the exception of porphyria and hepatic tumors, and DH and lymphoma of the gastrointestinal tract.