Nail disease in pemphigus vulgaris

Abstract

Patients affected by pemphigus vulgaris will occasionally present with associated and characteristic nail changes. This manuscript was prepared as a review of this unique presentation of pemphigus. Articles describing pemphigus vulgaris of the nail were compiled and reviewed and pertinent information was extracted to provide a concise analysis. The literature on this topic is still maturing. Publications to date suggest the incidence of nail and periungal involvement may correlate with the severity of mucocutaneous symptoms and the duration of the disease. The most common nail manifestations reported were acute or chronic paronychia and onychomadesis, and involvement of the fingernails. The nail changes may precede or develop concurrently with the mucocutaneous presentation. The diagnosis can be made by histologic identification and/or by the presence of IgG and C3 with direct immunoflourescence testing. Systemic treatment is required and highly effective; it includes a tailored combination of anti-inflammatory medications, immunosuppressive agents, and/or immunomodulatory treatments. This review aims to provide a better understanding of pemphigus vulgaris nail changes and guidance for clinical treatment. Unknown etiologies, predisposing factors, discrepancies evident in the literature, and limited research warrant further investigation of pemphigus vulgaris of the nail.