Summary

Pemphigus vulgaris (PV) is a rare but potentially life-threatening autoimmune disease affecting the mucosa and the skin. The disease is caused by circulating antibodies to desmosomes (important adhesion proteins linking cells together). Disruption of these intercellular connections results in a loss of cohesion between cells (acantholysis). The clinical result of this process is the development of multiple blisters that easily rupture, leaving behind painful sloughing eroded areas of mucosa and/or skin. We report a case of severe PV in a 56-year-old man presenting with widespread, painful, eroded mucocutaneous lesions. The severity of the disease demanded a range of medical and surgical specialties to successfully manage the problem. This paper highlights the importance of an early multidisciplinary team approach to improve the outcome of patients suffering with this disease.