Pemphigus vulgaris and complications of systemic corticosteroid therapy: a case report.

Abstract

Pemphigus refers to a group of diseases characterized by painful lesions caused by intraepidermal acantholytic structures in the skin and mucous membrane. The exact nature of the disease remains unknown. Pemphigus is a rare chronic mucocutaneous disease characterized by intra-epithelial bulla formation, due to autoantibodies directed against proteins of the desmosome-tonofilament complex between keratinocytes. The bullous lesions are painful, slow to heal and with a tendency to become invasive. Any part of the oral cavity may be affected, with the soft palate, buccal mucosa and lips being the most common sites. The high doses and prolonged administration of corticosteroids often required to control the disease result in several side effects, many of which are serious or life-threatening. In the present case, steroid treatment was begun at 180 mg/day and subsequently increased to 250 mg/day and 350 mg/day. Oral lesions were treated locally with 0.2% chlorhexidine gluconate in addition to systemic corticosteroids containing an immunosuppressive. Oral lesions were observed to recur without healing completely. Respiratory problems occured, necessitating direct lung radiographs and computerised tomography. Pulmonary embolism was diagnosed and the patient was transferred to the vascular surgery department. Unfortunately the patient died due to pulmonary embolism on the seventh day. Pemphigus vulgaris (PV) is a chronic autoimmune mucocutaneous disease that often primarily involves the oral cavity. Therefore, early diagnosis of oral symptoms is crucial for the successful treatment of PV. Although there is no consensus regarding the initial steroid dosage needed to induce remission, it is suggested that high doses of corticosteriods may cause fatal complications.