A current review of juvenile pemphigus vulgaris: analysis of data on clinical outcomes.
Forty-seven cases of juvenile pemphigus vulgaris have been reported in the English literature. Histology of lesional skin and direct immunofluorescence of perilesional skin are both necessary for a complete diagnosis. The autoimmune bullous condition can affect the skin and mucous membranes individually, but typically affects both concurrently. Disease characteristics in juvenile patients are similar to those in adults; however, a disruption of biologic and social development is of particular concern during adolescence. Although systemic corticosteroids have been used to successfully treat the disease in most cases, long-term use is often necessary for adequate control. Adverse effects from therapy can have devastating effects during this critical period of hormonal changes, physical and mental growth, and social and cultural development that occurs during adolescence. Newer therapies must be designed to adequately treat juvenile patients while also limiting serious adverse effects.