Bullous Pemphigoid in a Young Female: A Case Report
- Department of Dermatology and Venereology, Faculty of Medicine, Universitas Sumatera Utara, Medan, Indonesia
- Resident of Dermatology and Venereology, Faculty of Medicine, Universitas Sumatera Utara, Medan, Indonesia
Abstract
Introduction: Bullous pemphigoid (BP) is the most common autoimmune subepidermal blistering disease, mediated by autoantibodies against hemidesmosomal proteins BP180 and BP230, which activate complement, recruit eosinophils and neutrophils, and produce subepidermal bullae. Although BP typically affects older adults, it can occasionally arise in young patients, and prompt recognition and objective disease activity monitoring are important.
Case Description: A 22-year-old woman presented with a two-week history of pruritic, fluid-filled tense bullae over the trunk, axillae, forearms, palms, and thighs, which were unresponsive to topical betamethasone and cetirizine. Examination revealed tense, clear, and haemorrhagic bullae on erythematous macules with a negative Nikolsky’s sign, and the initial Bullous Pemphigoid Disease Area Index (BPDAI) was 23 (moderate). Histopathological examination revealed subepidermal bullae with eosinophil- and neutrophil-rich infiltrates, confirming BP diagnosis. Laboratory testing revealed thrombocytopenia underlying the haemorrhagic lesions, and oral mucosal involvement was observed during follow-up. She was treated with systemic and topical corticosteroids, an antihistamine, and supportive care. The BPDAI progressively decreased to 17 at two weeks and 7 at three weeks, with no new lesions.
Conclusion: Oral and topical corticosteroids were effective in treating moderate bullous pemphigoid in this patient. Serial BPDAI scoring provides a practical, objective measure of treatment response and supports timely dose adjustment.
Keywords: Autoimmune Blistering Disease, BPDAI, Bullous Pemphigoid, Corticosteroid, Young Female
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- Miyamoto D, Santi CG, Aoki V, Maruta CW. Bullous pemphigoid. A Bras Dermatol. 2019;94(2):133-146.PubMedGoogle Scholar
- Kang S, Amagai M, Bruckner AL, Enk AH, Margolis DJ, McMichael AJ, et al., editors. Fitzpatrick’s Dermatology. 9th ed. New York: McGraw-Hill Education; 2019. p. 3021-3034.PubMedGoogle Scholar
- Pratasava V, Sahni VN, Suresh A, Huang S, Are A, Hsu S, et al. Bullous pemphigoid and other pemphigoid dermatoses. Medicina (Kaunas). 2021;57(10):1061.PubMedGoogle Scholar
- Karakioulaki M, Eyerich K, Patsatsi A. Advancements in bullous pemphigoid treatment: a comprehensive pipeline update. Am J Clin Dermatol. 2024;25(2):195-212.PubMedGoogle Scholar
- Fuertes de Vega I, Iranzo-Fernández P, Mascaró-Galy JM. Bullous pemphigoid: clinical practice guidelines. Actas Dermosifiliogr. 2014;105(4):328-346.PubMedGoogle Scholar
- Deotto M, Spiller A, Sernicola A, Alaibac M. Bullous pemphigoid: an immune disorder related to aging (Review). Exp Ther Med. 2022;23(1):50.PubMedGoogle Scholar
- Gu A, Zhang L, Ma F, Kong X. Induction of localized bullous pemphigoid on a young woman following a chemical peel. Indian J Dermatol Venereol Leprol. 2021;87(5):706-708.PubMedGoogle Scholar
- Moro F, Fania L, Sinagra JLM, Salemme A, Di Zenzo G. Bullous pemphigoid: trigger and predisposing factors. Biomolecules. 2020;10(10):1432.PubMedGoogle Scholar
- Jordon RE, et al. Bullous pemphigoid: an overview. J Am Acad Dermatol. 2020;83(4):1237-1248.PubMedGoogle Scholar
- Zhang Y, Liu Z, Zhou X. Pathogenesis of bullous pemphigoid. Autoimmun Rev. 2015;14(6):482-489.PubMedGoogle Scholar
- Baigrie D, Nookala V. Bullous pemphigoid. In: StatPearls. Treasure Island (FL): StatPearls Publishing; 2023.PubMedGoogle Scholar
- Bernard P, Antonicelli F. Bullous pemphigoid: a review of its diagnosis, associations and treatment. Am J Clin Dermatol. 2017;18(4):513-528.PubMedGoogle Scholar
- Santos L, Queirós J, Carlos J, Figueiredo A. Hemorrhagic bullae—an alarm signal. Eur J Intern Med. 2013;24(8):e177-e178.PubMedGoogle Scholar
- Masmoudi W, Vaillant M, Vassileva S, Patsatsi A, Quereux G, Moltrasio C, et al. international validation of the Bullous Pemphigoid Disease Area Index severity score and calculation of cut-off values for defining mild, moderate and severe bullous pemphigoid. Br J Dermatol. 2021;184(6):1106-1112.PubMedGoogle Scholar
- Feliciani C, Joly P, Jonkman MF, Zambruno G, Zillikens D, Ioannides D, et al. Management of bullous pemphigoid: the European Dermatology Forum consensus in collaboration with the European Academy of Dermatology and Venereology. Br J Dermatol. 2015;172(4):867-877.PubMedGoogle Scholar
- Babu S, Sunil S, Pratap A, Mathew A. Mucous membrane pemphigoid. In: Treatment of Skin Disease: Comprehensive Therapeutic Strategies. 4th ed. London: Elsevier; 2017. p. 517-521.PubMedGoogle Scholar