CHRONIC BULLOUS DISEASE OF CHILDHOOD (CBDC): (CASE REPORT)

Linear IgA bullous disease is an acquired, autoimmune, vesiculobullous disease. Its name comes from linear deposition of immunoglobulin class (A)along the dermoepidermal junction when detected by direct immunofluorescence .Both children and adults are affected. Both sexes are equally affected with slight female predominance.In children it is referred as chronic bullous disease of childhood (CBDC). It affects children mainly between 1 and 11 years with peak between 6 and 8year. All races are affected but more common in developing countries.Its onset is acute and usually severe while recurrences become less severe. Diaminodiphenylsulfone (Dapsone) is the treatment of choice, in addition to systemic corticosteroids. We reported an 8-year-old boy with a vesiculobullous eruption which developed suddenly without any preceding infection or drugs in the previous two weeks. He complained of moderate itching diagnosed as scabies and received topical anti scabitic medications,with worsening of symptoms and appearance of new lesions.He was diagnosed as having contact dermatitis. The patient had many vesiculobullous and erosive lesions in the face, trunk, gentalia and back with sparing of palms and soles. We diagnosed him clinically as having (CBDC) and admitted in our hospital. Biopsy was taken from newly erupted intact vesicles for histopathology and prelesinal skin for immunofluorescence and soon we started Dapsone 2 mg/kg /day. After immunohistological verification, the treatment with oral prednisone 1mg/kg/day hadbeen started. Refrences 1)Wilson BD, Beutner EH, Kumar V, Chorzelski TP, Jablonska S. Linear IgA bullous dermatosis. An immunologically defined disease. Int J Dermatol. 1985;24:569–74. 2)Chan LS, Traczyk T, Taylor TB, Eramo LR, Woodley DT, Zone JJ. Linear IgA bullous dermatosis. Characterization of a subset of patients with concurrent IgA and IgG anti-basement membrane autoantibodies. Arch Dermatol 1995;131:1432-7. 3) Polat M, Lenk N, Kürekçi E et al. Chronic bullous disease of childhood in a patient with acute lymphoblastic leukemia: possible induction by a drug. Am J ClinDermatol. 2007; 8: 389-391 4)Varo, M., Fernández SL , Sitoe, A., Bassat Q.,(20170 Suspected case of chronic bullous disease of childhood in a rural area of Southern Mozambique .BMJ Case Rep 2017. doi:10.1136/bcr-2016-218315 5) Ho JCC, Ng PLP, Tan SH et al. Childhood linear IgA bullous disease triggered by amoxicillin-clavulanic acid. PediatrDermatol. 2007; 24: E40-43. 6)Haneef NS, Ramachandra S, Metta AK, Srujana L (2012) Chronic bullous disease of childhood with IgG predominance: what is the locus standi? Indian J Dermatol 57: 285-287. 7)Liu Z, Li N, Diaz LA, Shipley M, Senior RM, et al. (2005) Synergy between a plasminogen cascade and MMP-9 in autoimmune disease. J Clin Invest 115: 879-887. 8)Hofmann SC, Voith U, Schönau V, Sorokin L, Bruckner-Tuderman L, et al. (2009) Plasmin plays a role in the in vitro generation of the linear IgA dermatosis antigen LADB97. J Invest Dermatol 129: 1730-1739. 9)Horiguchi Y, Ikoma A, Sakai R, Masatsugu A, Ohta M, Hashimoto T. Linear IgA dermatosis: report of an infantile case and analysis of 213 cases in Japan. J Dermatol. 2008;35:737–743 10)Lin MS, Fu CL, Olague-Marchan M, Hacker MK, Zillikens D, et al. (2002) Autoimmune responses in patients with linear IgA bullous dermatosis: both autoantibodies and T lymphocytes recognize the NC16A domain of the BP180 molecule. ClinImmunol 102: 310-319. 11) Patsatsi A. Chronic bullous disease or linear IgA dermatosis of childhood—revisited. J Genet Syndr Gene Ther 2013;4:151.