Juvenile pemphigus

Abstract

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.

Juvenile Pemphigus vulgaris: A 4- to 19-Year Follow-Up of 4 Patients

“…Thus, we have the im pression that the prognosis quoad vitam in JP is better than that in adult pemphigus vulgaris. Our experience in the treatment of JP is in accordance with the approach em phasized by others that early diagnosis and therapy may influence the course of the dis ease toward better prognosis [2,3]. This certainly is true also in regard to adult pem phigus.…”

“…There are cases in which JP vulgaris is initially localized, and subsequently progressed to a generalized severe form. Sometimes it is rapidly progressive from the beginning, requiring high doses of systemic steroids [9] with or without adjuvant thera py such as dapsone [2], azathioprine and gold [10]. On the other hand, there are mild cases that respond to low doses of predni sone and, according to one report, the dis ease was controlled with topical steroids only [11].…”

“…We have been able to find only 3 cases of death from JP, secondary to sepsis, most probably as a consequence of the disease or its treatment [12][13][14], The mortality in adult pemphigus vulgaris, reported as a result of the disease or complication of its treatment is 8-24°7o [15,16], Exceptional is a recent report on zero fatality in pemphigus patients since 1976 with the use of a modified ther apeutic approach [17], Ahmed and Salem [2] pointed out that in their 8 cases of JP, a good prognosis was obtained even in severe cases, provided therapy was aggressive and was started early. The outcome in our cases also seems to be favorable: 2 cases apparent ly have been cured, 1 remained in remission under maintenance therapy and in 1, the dis ease presented only low-grade activity in spite of the fact that the patient had been off treatment for years.…”

“…Apart from the endemic form of pemphigus in South America, there have been, since 1950, 49 case reports of juvenil pemphigus vulgaris and foliaceous [2][3][4][5][6]. The largest series of 8 patients was published by Ahmed and Salem [2] in 1983. At the Beilinson Med ical Center a relatively large number of pem phigus patients has been seen in the last 26 years.…”

Pemphiguserkrankungen bei Kindern und Jugendlichen

“…Die juvenilen PV-Formen unterscheiden sich in der klinischen Ausprägung, im Verlauf und in der Ansprache auf immunsuppressive Therapien nicht von der Erwachsenenform. Wie beim erwachsenen Pemphigus vulgaris beginnt die Erkrankung im jugendlichen Alter in etwa 60% in der Mundhöhle [2], und auch bei Kindern können Medikamente, z. B. Enalapril oder Propylthiouracil, die Pemphiguserkrankung triggern oder exazerbieren [21,46].…”