Linear IgA bullous dermatosis: report of an exuberant clinical case and literature review
Introduction: Linear IgA dermatosis (LIGAD) is a rare acquired disease, with a probable autoimmune origin. Its differential diagnosis involves other bullous dermatosis.
Case Report: A previously healthy 12-month-old male was observed at the Emergency Department due to a 4-day itchy vesiculobullous rash in the perineal region, lower abdomen, hands, and feet. Analytical study was normal. Flucloxacillin and hydroxyzine were initiated without improvement. New (some of which confluent) lesions, erosions, and serohematic crusts developed on the periphery of previous lesions. A skin biopsy was performed at this time, revealing a subepidermal blister with neutrophilic infiltrate at histological examination. Direct immunofluorescence uncovered linear IgA deposits along the basement membrane. Lesion remission occurred without further therapeutic measures.
Discussion: Although clinically exuberant, LIGAD is usually a self-limited disease. A high degree of suspicion is important, since immunofluorescence is diagnostic and pathognomonic, avoiding late diagnosis, unnecessary treatments, and parental anxiety.
- Rocha F, Silva A, Fonseca P, Teixeira P, Vieira AP, Oliveira JMG. Dermatose Bolhosa Crónica Linear por IgA: Apresentação de um Caso Clínico e Revisão da Literatura. Acta Pediatr Port. 2004; 35: 521-524.
- Ferreira O; Mota A; Morais P, Duarte AF, Bettencourt H, et al. Dermatose IgA linear da Infância – Apresentação em cacho de uva escrotal. Revista SPDV 2011; 69(4).
- Souza BC, Fregonesi NC, Tebcherani AJ, Sanchez AP, Aoki V, e tal. Linear IgA bullous dermatosis: report of an exuberant case. An Bras Dermatol 2013. Nov-Dec 88(6 Suppl 1):67-70.
- Magalhães JC, Oliveira AC, Machado S, Reis MG. Dermatose bolhosa IgA linear. Acta Pediatr Port. 2013; 44: 46-47.
- Alajlan A, Al-Khawajah M, Al-Sheikh O, Al-Saif F, Al-Rasheed S, Al-Hoqail I, et al. Treatment of linear IgA bullous dermatosis of childhood with flucloxacillin. J Am Acad Dermatol. 2006; 54(4):652-6.
- Romani L, Diociaiuti A, D'Argenio P, El Hachem M, Gargiullo L, Boldrini R, et al. A Case of Neonatal Linear IgA Bullous Dermatosis with Severe Eye Involvement. Acta Derm Venereol. 2015 4; 95(8):1015-1017.
- Lings K, Bygum A. Linear IgA bullous dermatosis: a retrospective study of 23 patients in Denmark. Acta Derm Venereol. 2015; 95(4):466-71.
- Reyes-Baraona F, Andino R, Carrasco JE, Arriagada C, Guerrero S. Linear IgA bullous dermatosis of childhood: case report. Arch Argent Pediatr. 2014; 112(2): e57-60
- Chanal J, Ingen-Housz-Oro S, Ortonne N, Duong TA, Thomas M, Valeyrie-Allanore L, et al. Linear IgA bullous dermatosis: comparison between the drug-induced and spontaneous forms. Br J Dermatol. 2013; 169(5):1041-8.
- Venning VA. Linear IgA disease: clinical presentation, diagnosis, and pathogenesis. Immunol Allergy Clin North Am. 2012; 32(2): 245-53.
- Kharfi M, Khaled A, Karaa A, Zaraa I, Fazaa B, Kamoun MR. Linear IgA bullous dermatosis: the more frequent bullous dermatosis of children. Dermatol Online J. 2010 15; 16(1): 2.
- Nantel-Battista M, Al Dhaybi R, Hatami A, Marcoux D, Desroches A, Kokta V. Childhood linear IgA bullous disease induced by trimethoprim-sulfamethoxazole. J Dermatol Case Rep. 2010 19; 4(3):33-5.
- Patsatsi A. Chronic Bullous Disease or Linear IgA Dermatosis of Childhood –Revisited. J Genet Syndr Gene Ther 2013; 4(6): 151.
- Moleiro S, Santos V, Calha M, Pessoa G. Atypical response to treatment in linear IgA bullous dermatosis of childhood: Revision of literature. Dermatol Online J. 2011; 17(6):5.
- 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(11): 737-43.
Copyright (c) 2019 NASCER E CRESCER - BIRTH AND GROWTH MEDICAL JOURNAL
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
Copyright and access
This journal offers immediate free access to its content, following the principle that providing free scientific knowledge to the public provides greater global democratization of knowledge.
The works are licensed under a Creative Commons Attribution Non-commercial 4.0 International license.
Nascer e Crescer – Birth and Growth Medical Journal do not charge any submission or processing fee to the articles submitted.