Linear IgA bullous dermatosis: the more frequent bullous dermatosis of children.
BACKGROUND
Linear IgA bullous dermatosis (LAD) of children is relatively frequent in Africa. We undertook this study to evaluate the frequency of this disease among autoimmune bullous diseases (AIBD) in Tunisian children.


METHODS
We present a 32-year retrospective study (January 1976 to December 2007). Children with chronic acquired bullous diseases seen at the Charles Nicolle Hospital of Tunis and for whom direct immunofluorescence (DIF) of the perilesional skin demonstrated linear IgA immunoglobulin deposits were included in the study population.


RESULTS
Thirty-one children with LAD were collected representing 65.9 percent of all AIBD of children collected in the same period, with a mean age of 5.5 years and a sex ratio M/F of 2.4. Most of the children had a generalized eruption (28/31) but more profuse on the face, pelvic region, buttocks, and limbs. Mucosal lesions were present in only 4 children (12.9%). The mean duration of the disease was 14 months. Direct immunofluorescence demonstrated predominantly linear IgA deposits along the dermal-epidermal junction in all patients. Faint IgG, IgM, and complement were also seen (20/31). Indirect immunofluorescence was negative in 67 percent of cases. Eight patients responded to Dapsone, but prednisone had to be added in 7 children and erythromycin in 4 others to control the disease. A long term remission period (34 months) was achieved in 61.9 percent of patients.


CONCLUSIONS
This study confirms that LAD is the most common AIBD in children in Tunisia and it frequently occurs in preschool-aged males. Independently of the medication chosen for treatment, a long term remission is frequently observed.
Kharfi
Monia
M
Dermatology Department, Charles Nicolle Hospital, Tunis, Tunisia.
Khaled
Aida
A
Karaa
Amel
A
Zaraa
Ines
I
Fazaa
Becima
B
Kamoun
Mohamed Ridha
MR
D000293:Adolescent; D001327:Autoimmune Diseases / Q000145:classification / Q000188:drug therapy / Q000453:epidemiology* / Q000276:immunology / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D003167:Complement Activation; D003622:Dapsone / Q000008:administration & dosage / Q000627:therapeutic use; D004917:Erythromycin / Q000008:administration & dosage / Q000627:therapeutic use; D005260:Female; D019085:Fluorescent Antibody Technique, Direct; D006801:Humans; D007070:Immunoglobulin A / Q000032:analysis*; D007223:Infant; D008297:Male; D011241:Prednisone / Q000008:administration & dosage / Q000627:therapeutic use; D012074:Remission Induction; D012189:Retrospective Studies; D012872:Skin Diseases, Vesiculobullous / Q000145:classification / Q000188:drug therapy / Q000453:epidemiology* / Q000276:immunology / Q000473:pathology; D014416:Tunisia / Q000453:epidemiology
D007070:Immunoglobulin A; D004917:Erythromycin; D003622:Dapsone; D011241:Prednisone