Childhood pemphigus vulgaris: five cases in 16 years

Abstract

Pemphigus vulgaris (PV) usually occurs in adults. There are only a few reports of large PV series concerning childhood cases. We report here five cases of PV in patients younger than 16 years. They were analyzed among 169 PV cases out of a total of 192 pemphigus patients diagnosed between 1988-2004. The ratio of childhood cases was 2.9% in our large PV series. This relatively high ratio of childhood patients suggests that PV should not be neglected in the differential diagnosis of bullous lesions in childhood. Four of the five cases were followed up between 2-4 years and all of these four cases showed at least one relapse. PV also seems to show a relapsing course in the pediatric age group like in adults.

A Current Review of Juvenile Pemphigus Vulgaris

“…Thus, 24 patients were treated with systemic corticosteroids only. [2,4,5,8,[13][14][15][16][17]20] Additionally, one patient was treated with topical corticosteroids only (table I). [10] …”

“…[2] Two patients received dapsone with oral corticosteroids. [2,20] One patient received mycophenolate mofetil with oral corticosteroids (table I). [20] …”

“…It was not always clear if the patients were still receiving systemic therapy at the time of reporting (table I). [2,[4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22] Elias et al [4] ( Lynde et al [11] ( Asarch et al [22] ( Hence, the analysis indicates that in approximately 50% of the patients, systemic corticosteroids were the mainstay of treatment and that the majority of patients were relatively clear of disease in less than 2 years. The use of immunosuppressive agents and other systemic therapies was used in approximately 50% of the patients.…”

“…The disease affects patients from a wide range of ethnicities and appears to affect males and females equally. [2,[4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22] In contrast, PV in adults has a slight female predominance. [24][25][26] In this analysis, the average time before diagnosis was 8 months.…”

Rituximab in Childhood Pemphigus Vulgaris: A Long-Term Follow-Up Case and Review of the Literature

“…However, high-dose and prolonged administration of these agents may result in numerous well-known side effects. Adjuvant steroid-sparing therapies, such as azathioprine [5,6,7,8], methotrexate [6,7], oral gold [9], mycophenolate mofetil [10], intravenous immunoglobulins (IVIg) [11] and dapsone [7,12], have been previously reported as effective in isolated cases of CPV. Therefore, evidence-based guidelines have not been reported for CPV yet.…”