Bullous Systemic Lupus Erythematosus Mimicking Bullous Pemphigoid: A Case Report

Vesri Yossy, Gardenia Akhyar, Alimuddin Tofrizal

Abstract


Introduction: Bullous systemic lupus erythematosus (BSLE) is an infrequent but distinct presentation of systemic lupus erythematosus (SLE) in less than 5% of lupus cases. It is characterized by vesicobullous skin eruption in SLE that can develop either before or after SLE diagnosis has been established. Distinguish between BSLE with other autoimmune blistering diseases such as bullous pemphigoid (BP), dermatitis herpetiformis, linear IgA, etc., is very important to prevent misdiagnosis. The physician must be able to combine clinical, histological and immunofluorescence finding for the diagnosis approach. We report a case of blistering skin eruption in SLE patient. Case Report: A 19-year-old female patient complained of tense blistering on her lip, face and wrists since one month ago. She was diagnosed with SLE two weeks ago. The dermatological state showed bullae and vesicle on erythematous/ normal base, erosions, excoriation and blackish red crust on the lip, face, armpit, neck, abdomen and wrists. Histopathological examination of the lesion showed sub-epidermal bullae containing PMN leukocytes consist of abundant neutrophils, only occasional eosinophils and the presence of keratotic plugs. Direct immunofluorescence (DIF) of the skin showed linear deposition of IgG, IgA, IgM and C1q at the dermo-epidermal junction. This patient exhibited similar features to both BSLE and BP with tensed clear blisters and subepidermal cleft. BSLE differ from BP by abundant neutrophils found on histopathological examination, whereas BP has abundant eosinophils. Conclusion: Immunofluorescence examination shows linear IgG in BP, whereas linear or granular IgG in BSLE. Establishing the correct diagnosis is important to prevent misdiagnosis and mistreatment.


Keywords


Dermatology; Allergy Immunology; Pathology

Full Text:

PDF

References


Miziara ID, Mahmoud A, Chagury AA, Alves RD. Bullous Systemic Lupus Erythematosus : Case report. Int Arch Otorhinolaryngol. 2013;17(3):344–6.

Duan L, Chen L, Zhong S, Wang Y, Huang Y, He Y, et al. Treatment of Bullous Systemic Lupus Erythematosus. J Immunol Res. 2015;1–6.

Sontheimer C, Costner M, Sontheimer R. Lupus Erythematosus. In: Kang S, Amagai M, Bruckner A, Enk A, Margolis D, McMichael A, editors. Fitzpatrick’s Dermatology 9th edition. New york: McGraw-Hill; 2019. p. 1037–60.

Chan L. Bullous systemic lupus erythematosus. In: Northcott J, editor. Blistering skin disease. Washington DC: Manson publishing; 2009. p. 68–70.

Sáez-de-ocariz M, Espinosa-rosales F, López-corella E, León-bojorge B De. Bullous lesions as a manifestation of systemic lupus erythematosus in two Mexican teenagers Report of cases. Pediatr Rheumatol. 2010;8(19):1–6.

Culton D, Liu Z, Diaz L. Bullous Pemphigoid. In: Kang S, Amagai M, Bruckner A, Enk A, Margolis D, McMichael A, et al., editors. Fitzpatrick’s Dermatology 9th edition. New York: McGraw-Hill; 2019. p. 944–59.

Chan L. Bullous pemphigoid. In: Northcott J, editor. Blistering skin disease. Washington DC: Manson publishing; 2009. p. 46–8.

Borradori L, Murrell D, Hall R. Bullous Pemphigoid: The Spectrum of Clinical Presentations. In: Murrell D, editor. Blistering Disease. Springer- Verlag Berlin Heidelberg; 2015. p. 335–42.

Tincopa M, Puttgen KB, Sule S, Cohen BA, Gerstenblith MR. Bullous Lupus : An Unusual Initial Presentation of Systemic Lupus Erythematosus in an Adolescent Girl. Pediatr Dermatol. 2010;27(4):373–6.

Sebaratnam DF, Woodley DT. Clinical Features of Bullous Systemic Lupus Erythematosus. In: Murrell D, editor. Blistering Diseases: Clinical Features, Pathogenesis, Treatment. Springer- Verlag Berlin Heidelberg; 2015. p. 413–8.

Ali C, Kamal S, Khan M. Bullous Systemic Lupus Erythematosus – A Case Report. J Dhaka Med Coll. 2013;22(1):3–5.

Fujimoto W, Hamada T, Yamada J. Bullous Systemic Lupus Erythematosus as an Initial Manifestation of SLE. J Dermatol. 2005;32:1021–7.

Elenitsas R, Chu E. Pathology of skin lesions. In: Fitzpatrick’s Dermatology 9th edition. New york: McGraw-Hill; 2019. p. 18–39.

Raman S, Grover A, Padhan P, Senapati U. A Case of Blistering Eruptions in Systemic Lupus Erythematosus. J Clin Diagnostic Res. 2019;13(9):5–6.

Maggio M, Corsello G, Prinzi E, Cimaz R. Systemic Lupus Erythematosus and Bullous Pemphigoid with Dramatic Response to Dapsone. Am J Case Rep. 2017;18:317–9.




DOI: https://doi.org/10.33854/heme.v3i2.667

Refbacks

  • There are currently no refbacks.


Creative Commons License   Health and Medical Journal This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.