An In-Depth Understanding of The Pathophysiology and Therapeutic Approaches to Chronic Bullous Disease of Childhood (CBDC)

Tassa Nasirah; Cut Putri Hazlianda

doi:10.59141/-.v8i2.580

Authors

Tassa Nasirah Universitas Sumatera Utara
Cut Putri Hazlianda Universitas Sumatera Utara

DOI:

https://doi.org/10.59141/-.v8i2.580

Keywords:

Chronic Bullous Disease of Childhood, Linear IgA Bullous Dermatosis, Autoimmune Disease, Immunoglobulin A, Therapeutic Management

Abstract

Chronic bullous disease of childhood (CBDC), also known as childhood linear IgA bullous dermatosis, is a rare autoimmune blistering disorder characterized by subepidermal blister formation and linear immunoglobulin A (IgA) deposition along the basement membrane zone. Its heterogeneous clinical presentation and similarities to other autoimmune blistering disorders pose diagnostic and therapeutic challenges. This study aimed to synthesize current evidence regarding the pathophysiology, clinical manifestations, diagnostic approaches, and therapeutic management of CBDC. A descriptive literature review was conducted using relevant original research articles, case reports, review articles, and clinical guidelines. The literature was organized thematically and comparatively evaluated to identify consistent findings, variations in clinical presentation, and limitations in the available evidence. The reviewed studies identified IgA autoantibodies targeting components of the dermal–epidermal junction, particularly BP180 (type XVII collagen), as important contributors to inflammatory responses and subepidermal blister formation. Clinical manifestations included tense vesicles and bullae, frequently exhibiting a characteristic "string of pearls" arrangement, with variable mucosal involvement. Direct immunofluorescence (DIF) examination of perilesional skin remained essential for diagnostic confirmation because clinical and histopathological features could overlap with those of other blistering disorders. Dapsone was identified as a first-line treatment, while systemic corticosteroids and alternative therapeutic agents were considered according to disease severity, treatment response, and tolerability. However, the predominance of observational evidence limited definitive comparisons of treatment effectiveness and safety. In conclusion, CBDC requires a comprehensive diagnostic approach and individualized therapeutic management. Further prospective pediatric studies are needed to strengthen the evidence regarding treatment efficacy, safety, recurrence, and long-term clinical outcomes in affected children.

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References

Bernett, C. N., Fong, M., Yadlapati, S., & Rosario-Collazo, J. A. (2023). Linear IGA Dermatosis. In StatPearls.

Caux, F., Patsatsi, A., Karakioulaki, M., Antiga, E., Baselga, E., & Borradori, L. (2024). S2k guidelines on diagnosis and treatment of linear IgA dermatosis initiated by the European Academy of Dermatology and Venereology. Journal of the European Academy of Dermatology and Venereology, 38, 1006–1023.

Damayanti, N., Irmawati, Y. E., Radiono, S., Widodo, Y., & Dwianingsih, E. K. (2020). Chronic Bullous Disease of Childhood: Tinjauan Klinis, Histopatologi, dan Direct Immunoflourescence Pada Penegakan Diagnosis. Media Dermato-Venereologica Indonesiana, 49(3).

do Vale, E. C. S., Dimatos, O. C., Porro, A. M., & Santi, C. G. (2019). Consensus on the treatment of autoimmune bullous dermatoses: dermatitis herpetiformis and linear IgA bullous dermatosis – Brazilian Society of Dermatology. Anais Brasileiros de Dermatologia, 94, 48–55.

Feng, X., Zheng, H., Wang, M., Wang, Y., Zhou, X., & Zhang, X. (2025). Autoimmune bullous diseases: pathogenesis and clinical management. Molecular Biomedicine, 6. https://doi.org/10.1186/s43556-025-00272-9

Khan, M., Park, L., & Skopit, S. (2023). Management Options for Linear Immunoglobulin A (IgA) Bullous Dermatosis: A Literature Review. Cureus, 15(3).

Kowalewski, C., & Wozniak, K. (2025). Linear IgA bullous dermatosis – a fifty year experience of Warsaw Center of bullous diseases. Frontiers in Immunology, 15, 1–14.

Mellyanawati. (2021). Laporan Kasus: Seorang anak dengan penyakit bula yang diduga Chronic Bullous Disease of Childhood A Child with Bullous Disease Suspected Chronic Bullous Disease in Childhood. Jurnal Ilmiah Kedokteran Wijaya Kusuma, 10(2), 176–186.

Mori, F., Saretta, F., Liotti, L., Giovannini, M., Castagnoli, R., & Arasi, S. (2022). Linear Immunoglobulin A Bullous Dermatosis in Children. Frontiers in Pediatrics, 10.

Nasri, J., Jungo, P., Blickenstorfer, M., Mühleisen, B., Navarini, A. A., & Juratli, H. A. (2022). Linear IgA bullous dermatosis of childhood. Dermatology Online Journal, 28(5).

Onalaja-underwood, A. A., Hurley, M. Y., & Sokumbi, O. (2024). Diagnosis and Management of Bullous Disease. Clinics in Geriatric Medicine, 40, 37–74.

Pradhan, S., Jha, K., Kumari, P., Shrestha, S., & Jha, A. K. (2022). Chronic Bullous Disease of Childhood: A Case Report and Review of Literature of Bullous Diseases in Children. Nepal Journal of Dermatology, Venereology & Leprology, 20(2).

Shin, L., Ii, J. T. G., & Dao, H. (2021). Updates in the Diagnosis and Management of Linear IgA Disease: A Systematic Review. Medicina, 57.

Shin, S., Bai, L., & Burnett, R. T. (2021). Air pollution as a risk factor for incident chronic obstructive pulmonary disease and asthma: A 15-year population-based cohort study. American Journal of Respiratory and Critical Care Medicine, 203(9), 1138–1148. https://doi.org/10.1164/rccm.201909-1744OC

Stoj, V., & Lu, J. (2022). Nutrition and bullous diseases. Clinics in Dermatology, 40(2), 156–165. https://doi.org/10.1016/j.clindermatol.2021.10.009

Wilson, A., & Murrell, D. F. (2022). Clinical features of chronic bullous dermatosis of childhood. Clinical and Experimental Dermatology, 47, 873–881.

Yadav, R., Poudel, I., Poudyal, Y., & Chaudhary, N. (2026). A Rare Type of Bullous Disorder in a Child. Can It Be Chronic Bullous Disease of Childhood? Clinical Case Reports, 14.