A Case of Successful Management of Adult-Onset Linear IgA Bullous Disease With Sulfasalazine During the COVID-19 Pandemic

December 2022 | Volume 21 | Issue 12 | 1355 | Copyright © December 2022


Published online November 14, 2022

Sana Ashraf MB BCh BAO MSc, Shahd Elamin MB BCh BAO MSc, Julia Tolland BSc MB BAO BCh MD FRCP

Department of Dermatology, Ulster Hospital Dundonald, Belfast, UK

Abstract
Linear IgA bullous disease (LABD) is a rare, acquired, autoimmune, pruritic, and blistering skin condition. Dapsone is a first line treatment option, however, there are limited options if this fails, or if contraindicated. We present a case of successful management of LABD with sulfasalazine.

A 46-year-old Caucasian female with LABD was commenced on high dose corticosteroids. She failed to wean, and dapsone was contraindicated due to a history of primary sclerosing cholangitis and risk of hepatitis. Following the failure of mycophenolate mofetil, sulfasalazine was trialed and successfully controlled both this patient’s LABD and ulcerative colitis. There is little literature on the use of sulfasalazine in dermatological conditions. We present sulfasalazine as an option for patients who are unable to use classically used treatments for LABD, or in those who have a dual diagnosis, as in this case, allowing for one agent to manage both conditions. Furthermore, The National Institute for Health and Care Excellence guidance mentions sulfasalazine as one of the few drugs that can be continued during the COVID-19 pandemic, and its use spared this patient from the significant immunosuppression associated with other treatment modalities.

J Drugs Dermatol. 2022;21(12): doi:10.36849/JDD.6717

INTRODUCTION

Linear IgA bullous disease (LABD) is a rare, acquired, autoimmune skin condition that is associated with urticarial plaques and subepidermal blistering and mainly affects young children and older adults.1 In children, this can present as blistering at the periphery of urticarial plaques (which themselves can assume an annular or polycyclic pattern), known as the 'string-of-pearls' sign, and often affects the face, genitals, and buttocks.1 In adults, such bullae can again present on urticarial plaques, but also on normal skin, and is more commonly distributed over the trunk, head, and limbs, as well as mucosal involvement.1 Direct immunofluorescence (IMF) is essential for diagnosis, although histology can also be supportive. Direct IMF shows linear deposition of immunoglobulin A (IgA) along the dermal-epidermal junction.2 Whilst most cases are idiopathic, LABD can also be drug-induced, or associated with systemic autoimmune diseases such as ulcerative colitis,3 as in the case we present here.

Dapsone, corticosteroids, and sulphapyridine are commonly used options in the management of LABD,4 with dapsone being first line, however, there are limited options if this were to fail, or be an inappropriate option. We present a case of successful management of LABD with sulfasalazine, where use of dapsone was deemed unsafe. There is minimal literature regarding the use of sulfasalazine as a treatment option, and therefore we find this to be an interesting case suggesting an alternative therapeutic option.

CASE PRESENTATION

A 46-year-old North European, Caucasian female was referred to the dermatology department during the COVID-19 pandemic, with an acute 1-month history of a severely pruritic and blistering rash affecting mainly the trunk, with limb and head involvement. She had no prior dermatological history. Her background included a long-standing history of ulcerative colitis (UC), which was stable on mesalazine, primary sclerosing cholangitis (PSC), and fatty liver disease. Her medication history also included ursodeoxycholic acid and atorvastatin. No new medications were commenced prior to onset of the rash.

Clinical examination revealed widespread vesicles and bullae, arising from urticated patches and distributed in an annular fashion (Figures 1, 2). There was no mucosal involvement.