Juvenile Pemphigus Vulgaris

Abstract

Pemphigus vulgaris is extremely rare in childhood. Only 35 cases of juvenile pemphigus vulgaris have been reported in the literature up until to now. During the pre-steroid era, the outcome of the disease was usually regarded as fatal. Today, the prognosis has improved because of the possibility of early diagnosis by immunofluorescent techniques, determination of antibodies against epidermal intercellular substance and early therapy with systemic steroids. A case of juvenile pemphigus vulgaris with a favourable outcome in a 13-year old girl is described.

A Current Review of Juvenile Pemphigus Vulgaris

“…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.…”

“…Although it is not uniformly positive in all patients with JPV, 91% of patients had positive autoantibody titers on indirect immunofluorescence when these were reported in this analysis. [2,[4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22] Similarly, approximately 80% of adult patients have detectable levels of circulating antibodies. Furthermore, titers of autoantibodies on indirect immunofluorescence appear to correlate with disease severity in both juvenile and adult patients.…”

“…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] …”

“…This lack of detailed information on follow-up is a serious limitation of the literature (table I). [2,[4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22] 3. Discussion JPV is a rare autoimmune blistering disease affecting adolescents between 13 and 18 years of age.…”

“…Although rare, JPV is an important disease to consider in the differential diagnosis of blistering disease in patients in this age group. [2,[4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22] For an adequate diagnosis of JPV, histology of lesional skin and direct immunofluorescence of perilesional skin are both essential. These observations are identical to the adult age group.…”