Nail involvement in pemphigus vulgaris is relatively rare. We describe a ca
se of severe pemphigus involving both the skin and oral mucosa in which an
acute exacerbation was preceded by the onset of nail involvement of all 4 e
xtremities. Nail involvement occurred in the form of hemorrhagic paronychia
of multiple digits. Oral, cutaneous, and nail manifestations of the diseas
e were all well controlled by systemic therapy. A review of the literature
on nail involvement in pemphigus reveals that this involvement may be manif
ested in multiple ways, with chronic paronychia and onychomadesis being the
most common. Involvement of the nail occurs most frequently either as part
of the initial presentation, or just before or concurrent with a Rare of p
re-existing disease. Nail involvement, when it occurs, is usually present w
hen the disease is severe. Topical therapy is insufficient, and systemic th
erapy is warranted. In the majority of cases, nail recovery is complete, wi
th no residual damage.