Atypical Presentation of Pemphigus Vulgaris: Nail Involvement in a 20-Year-Old Male

Abstract

Pemphigus vulgaris (PV) mainly causes blistering of the skin and mucous membranes, with nail unit involvement being rare. Nail involvement may serve as an indicator of disease severity. We present a case of a 20-year-old male with PV who had both cutaneous and nail findings, with nail changes corresponding with disease severity. The patient with biopsy-confirmed PV, on prednisone and mycophenolate, presented to the emergency department with an acute flare of PV and severe mandibular pain and lymphadenopathy. At follow-up in our outpatient department, the physical examination was significant for onychomadesis and onycholysis of the fingernails. Prednisone and mycophenolate dosages were increased, and rituximab infusions were initiated. Bullae and mucosal lesions resolved on the follow-up, and nail changes improved. This case appends an unusual perspective to the limited literature on PV-associated nail changes, especially in younger patients. It advocates for meticulous history taking and physical examination and supports a correlation between nail symptoms and PV disease severity.

Keywords: nail changes; nail dystrophy; nails; onychodystrophy; onychomadesis; pemphigus vulgaris.

Figure 1. Clinical presentation of bilateral index fingernails at initial visit showing onychomadesis and brown discoloration of the nail plate proximally

The black arrows indicate the presence of onychomadesis, while the blue arrow indicates brown discoloration of the nail plate.

Figure 2. Clinical presentation of fingernails showing more distal onychomadesis

The black arrows indicate the presence of onychomadesis.