Rheumatology 2018;0:1 Clinical Vignettes doi:10.1093/rheumatology/key084 FIG.1 Bullous lesions in a patient with SLE: gross ap- pearance and histopathology A 15-year-old girl had a 4-month history of fever, arthritis of the elbows, left knee and left ankle, and maculopapular rashes with bullae on the neck, chest and abdomen (Fig. 1A and B). Anasarca, pallor, ulcers on the hard palate, matted cervical lymphadenopathy and mild hepa- tosplenomegaly were present. Haemoglobin was 6 g/dl; direct and indirect Coombs tests were positive; serum ANA and anti-dsDNA levels were elevated; 24-h urinary protein was 1.89 g. Renal biopsy showed diffuse [International Society of Nephrology (ISN)/Renal Pathology Society (RPS) grade 4] LN. Cervical lymph node biopsy revealed reactive Bullous lesions on the neck and abdomen (A and B); hyperplasia. Skin biopsy showed intra- and subepidermal photomicrograph of skin biopsy showing intra- and sub- cleft formation, pigmentary incontinence and mild inflam- epidermal blisters (C, haematoxylin and eosin); immuno- matory perivascular infiltrate (Fig. 1C). On DIF, linear de- fluorescence showing linear deposition of IgG in the base posits of IgG and IgA were seen at the dermoepidermal of the blisters (D, DIF). junction (Fig. 1D). She was given high-dose CS and 6-monthly pulsed doses of i.v. CYC, followed by AZA and HCQ whereupon there was improvement in symptoms. Bullous lupus may Disclosure statement: The authors have declared no or may not be associated with systemic activity; when conflicts of interest. present, it is usually in the form of nephritis and haemato- 1 1 2 Mohammad Ali , Ashish Sharma , Arvind Ahuja logical involvement. Dapsone is the treatment of choice and Vivek Arya for the bullous disease; however, where control of visceral 1 2 disease is simultaneously required, higher-than-usual Department of Medicine and Department of Pathology, Postgraduate Institute of Medical Education and Research, Dr doses of CS and other immunosuppressants can achieve Ram Manohar Lohia Hospital, New Delhi, India control of both. Funding: No specific funding was received from any Correspondence to: Mohammad Ali, C-322, Yojana Vihar, bodies in the public, commercial or not-for-profit sectors Delhi 110092, India. to carry out the work described in this manuscript. E-mail: firstname.lastname@example.org The Author(s) 2018. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: email@example.com Downloaded from https://academic.oup.com/rheumatology/advance-article-abstract/doi/10.1093/rheumatology/key084/4955853 by Ed 'DeepDyve' Gillespie user on 12 July 2018
Rheumatology – Oxford University Press
Published: Mar 28, 2018
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