Linear IgA bullous disease of childhood: an experience from Kuwait.
Linear IgA bullous disease of childhood is a rare autoimmune blistering disease. We report eight patients with this disease seen in our autoimmune bullous diseases clinic over a span of 12 years. They constituted 89% of the total number of those with linear IgA bullous disease of childhood seen during this period, with an age-adjusted minimum estimated incidence of 2.3 cases/million population/year. Males outnumbered females by a 1.7:1 ratio. The age at onset ranged between 10.5 months and 13 years, with a mean of 6.8 +/- 4.17 years. The majority of patients (62.5%) had moderately severe disease. Fifty percent of patients were observed to have an association with either an autoimmune disease (Crohn disease and post-streptococcal glomerulonephritis in one each) or an infection (beta-hemolytic streptococcal and hepatitis A virus infection in one each). The treatment of choice was dapsone alone or in combination with systemic steroids. Seventy-one percent of patients achieved complete remission by the end of 2 years. The study highlights the significance of systematic clinicoepidemiologic surveys from different regions.
Nanda
Arti
A
As'ad Al-Hamad Dermatology Center, Al-Sabah Hospital, Salmiya, Kuwait. artinanda@hotmail.com
Dvorak
Richard
R
Al-Sabah
Humoud
H
Alsaleh
Qasem A
QA
D000293:Adolescent; D000554:Ambulatory Care Facilities; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D001327:Autoimmune Diseases / Q000150:complications* / Q000453:epidemiology* / Q000628:therapy; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007070:Immunoglobulin A; D015994:Incidence; D007223:Infant; D007730:Kuwait / Q000453:epidemiology; D008297:Male; D015995:Prevalence; D012872:Skin Diseases, Vesiculobullous / Q000150:complications* / Q000453:epidemiology* / Q000628:therapy
D000893:Anti-Inflammatory Agents; D007070:Immunoglobulin A