Juvenile pemphigus vulgaris

A Current Review of Juvenile Pemphigus Vulgaris

“…[6,7,9,11,18] A combination of oral corticosteroids and cyclophosphamide was given to one patient, [12] and a combination of oral azathioprine and intramuscular (IM) gold and oral corticosteroids was given to one patient. [2] Two patients received dapsone with oral corticosteroids.…”

“…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.…”

“…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.…”

“…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.…”