We report the case of an infant who presented with isolated cutaneous
manifestations of Langerhans cell histiocytosis before the evolution o
f systemic features. In the transition period, at 9 months of age, nai
l unit changes became prominent, and persisted throughout the duration
of systemic treatment. A change in clinical features coincided with a
course of systemic gamma-interferon, which was given because immune p
aresis was suspected. Nail unit changes are rare in Langerhans cell hi
stiocytosis, and this case illustrates the range of findings, includin
g paronychia, nail fold destruction, onycholysis with subungual expans
ion, and nail plate loss. The significance of these changes as a progn
ostic indicator is controversial.