A Current Review of Juvenile Pemphigus Vulgaris

Abstract

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.

Successful management of pediatric oral pemphigus vulgaris with topical corticosteroid monotherapy

“…Although topical corticosteroid monotherapy has been previously reported in cases for confined mucosal pemphigus with success, clinicians typically use systemic treatment as first‐line therapy for oral PV . Especially in childhood, systemic corticosteroids are known to be associated with significant adverse effects including infection, weight gain, Cushingoid appearance, hypertension, acne, early cataracts, growth retardation, osteopenia, and avascular necrosis . Trials of mid‐strength or potent topical corticosteroids would therefore likely benefit children presenting with oral mucosal PV, as a good response to this treatment modality would spare children from the side effects of systemic corticosteroids.…”

Autoimmune bullous disease in childhood

“…Of note, most of the patients with juvenile pemphigus reported in the literature had a good prognosis; however, treatment-resistant cases, similar to our patients, have been reported. [58] As it is the case with pemphigus in adult age, rituximab represents a valuable treatment option for pediatric patients, as well. [9] In both of our patients, rituximab was required to obtain remission.…”

Bullous diseases: Kids are not just little people

“…As the condition often has a prolonged course with only rare spontaneous remission, numerous steroid sparing agents have been reported for use in pemphigus with varying success 70,71. One should note that the literature on treatment of pemphigus often includes the treatment of both patients with severe pemphigus foliaceus as well as pemphigus vulgaris.…”