Linear IgA Disease and Celiac Disease: Are They Mutually Exclusive?

Abstract

Adult Linear IgA disease or linear IgA bullous dermatoses(LABD) is a idiopathic or drug-induced autoimmune blistering disease characterized by the linear deposition of IgA at the dermoepidermal junction. Only 0.5 to 2.3 cases of Linear IgA are estimated to be reported each year. Although the clinical features of this disorder can be difficult to distinguish from dermatitis herpetiformis, the distinct immunopathologic findings in LABD and the absence of an associated gluten-sensitive enteropathy has historically confirmed the status of LABD as a distinct disease. We present a case of a patient with LABD with a concomitant high-gluten sensitivity. A 42 year-old women with a history of recurrent gastric ulcers presented with skin blisters. The patient stated she also has noticed that she is very sensitive to wheat containing products including cleaning supplies and shampoos. Upon examination, a clinical diagnosis of dermatitis herpetiformis was made. An upper endoscopy was performed which was positive for gastric ulcers. On duodenal biopsies, histology showed increased intraepithelial lymphocytes with villous blunting. On biochemical testing the patient was found to have an iron deficient anemia with no elevated levels of IgA in her serum. Transglutaminase Antibody IgA Titers were > 250.0, gliadin deamidated Ab, IgG and IgA were both elevated. A skin biopsy was performed which showed linear deposits of IgA seen at the dermo-epidermal junction. Treatment was initiated with dapsone therapy in which the patient was responsive over the course of treatment. This case illustrates a rare condition of LABD in a patient with concomitant celiac disease. This shows the importance of recognizing that a patient can present with linear IgA disease and continue to display strong gluten sensitivity. A high index of suspicion must be mainitained in patients with presumed celiac disease and dermatological manifestations who are not responding adequately to gluten free therapy.