Juvenile pemphigus.
Seven cases of juvenile pemphigus vulgaris and one case of juvenile pemphigus foliaceus are presented. A detailed review of the literature is also presented. The majority of patients with juvenile pemphigus develop pemphigus vulgaris. The patients present with a wide clinical spectrum. The oral cavity is frequently involved. The mean duration is 4 years. Clinical follow-up is similar to the adult variety. Several patients warrant high doses of systemic corticosteroids and develop serious side effects, most notably growth retardation and opportunistic infections. Short-term judicious use of immunosuppressive agents is advocated. Dapsone is a helpful adjuvant to therapy. It is proposed that relevant immunofluorescent studies be done very early. Early diagnosis and therapy are associated with a better prognosis.
D000293:Adolescent; D000324:Adrenocorticotropic Hormone / Q000627:therapeutic use; D000367:Age Factors; D001379:Azathioprine / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D003348:Cortisone / Q000627:therapeutic use; D003622:Dapsone / Q000627:therapeutic use; D005260:Female; D005455:Fluorescent Antibody Technique; D006801:Humans; D006854:Hydrocortisone / Q000627:therapeutic use; D008297:Male; D010392:Pemphigus / Q000175:diagnosis* / Q000188:drug therapy; D011241:Prednisone / Q000627:therapeutic use; D011379:Prognosis; D013997:Time Factors
D003622:Dapsone; D000324:Adrenocorticotropic Hormone; D001379:Azathioprine; D003348:Cortisone; D011241:Prednisone; D006854:Hydrocortisone