British Journal of Dermatologff i\ 991) 124, 498-502. ADONIS 0OO7O96391(M)119P A case report of a patient with features of systemic lupus erythematosus and linear IgA disease M.LAU. I.KAUFMANN-GRCNZINGHK* AND M.RAGHUNATH Division of tmmutioikithologn. Institute of Immunalogif and 'Department of DermatolosH. Vnivenitfi of Heidelberg. Germany Accepted for publication 14 Novt-mbor 1990 Summary A case is reported of a 5S-yeur-()ld woniiin with a 22-year history of systemic lupus erythemutosus (Sli:) who later developed a blistering eruption compatible with the diagnosis of linear IgA disease. Since 1979 linear IgA disease has heen differentiated as a distinct entity trom that of dermatitis herpetiformis hy the homogeneous linear deposition of IgA at the base- ment membrane zone (BMZ) of the epidermis and with a lack ofan associated gluten-dependent enteropathy.'"* hospital in Heidelberg. She was pyrexial and there were urticated areas of erythema and large tense blisters arranged in a herpetiform manner on her abdomen and left thigh (Fig. 1). There was no involvement of the mucous membranes. Case report A 3 5-year-oId woman had from the age of 15 a history of intermittent arthralgia. fever and anaemia. She was diagnosed as having systemic lupus erythematosus and in April 19S5 she was seen at the Mannheim Clinic with fever and having had a haemoptysis. A peri myocarditis and pericardial effusion with an alveolitis and hilateral pieural effusions were present, and 5 days after admis- sion she developed an erythematous rash on the trunk and thighs with several blisters on the right thigh. Vesicles later appeared on the biiccal mucous membrane and on the tongue. Oedema of both legs and purpura was associated with a vascuiitis and a thrombotytcjpenia. Anti-ONA antihodies and antinuclear antihodies in a titre of 1 :640 were present. The serological findings were compatible with a diagnosis of systemic lupus erythematosus (SIJi). The patietit was initially treated with 100 mg predni- sone daily and with an improvement of the symptoms the dose was reduced to 60 mg daily and she was then discharged from hospital. She remained well over the next 4 years until March 1989. when the dose of prednisone was reduced further to 2- 5 mg daily, hi mid- July 1989 she developed blisters in itchy areas of erythema on the abdomen and thighs. She later had an arthralgia with fever and diarrhoea and was admitted to Corrcspondemc: l> M.Uu. llnivcrsitHtskHnikum Stcgiilz. Mautklinlk WF 07. HindcnbiirKdamni JU. U-IOOO Berlin 45. Uemiany. 498 Utboratorii investigalious Haemoglobin was 11-7 g/dl. WBC 24-9 x 10^1. with 50% neutrophils. ?6% lymphocytes. 6% monocytes, 2% atypical lymphocytes. The platelet count was 4S1.000/ ml and the liSK was 37 mm/h. The serum immuno- globulins were IgG 20-S g/l, IgA 2-14 g/l and IgM la'i g/i. Kchocardiography revealed a small pericardial effu- sion and there was also a small left-sided pieural diffusion. A skin biopsy from the medial aspect of the thigh showed a subepidermal hulla. the lumen of which was filled with fibrinoid material and inflammatory cells that consisted mainly of lymphocytes and eosinophils (Fig. 2). There was a marked lymphohistiocytic inliliralion of the upper dermis with numerous eosinophils und a perivascular- mlxed infiltrate in the dermis. hnmunopathologii I'our separate skin biopsies were examined by direct immunotluorescence (I)ll") and the findings are shown in Table 1. In all four biopsies there was a homogeneous linear deposition of IgA at the dermo-epidermal junction zone (Fig. 3). This was most intense in the biopsy taken of a fresh lesion from non-sun-exposed skin and showed only subclass IgAl. In the lesional non-sun-exposed skin there was also an additional faint homogeneous deposi-