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.
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.
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.
