Linear IgA disease associated with lymphocytic colitis

Abstract

A 66-year-old woman presented with a bullous skin eruption and chronic diarrhoea. Lesional skin showed subepidermal blistering, and direct immunofluorescence of perilesional skin revealed linear deposits of IgA at the dermoepidermal junction, establishing a diagnosis of linear IgA disease (LAD). Chronic watery diarrhoea complicated by substantial loss of body weight preceded the skin eruption for several months. On endoscopy, the colon appeared macroscopically normal. On histology, the colon mucosa showed increased numbers of intraepithelial lymphocytes and infiltrates of mononuclear cells in the lamina propria, indicative of lymphocytic colitis. Treatment with methylprednisolone and dapsone led to complete clearing of the bullous skin eruption and marked improvement of the patient's diarrhoea. Gastrointestinal disorders such as lymphocytic colitis have rarely been reported in patients with LAD. Whether the simultaneous occurrence of these two diseases is coincidental or due to related pathogenetic mechanisms remains to be seen.

Bullous Pemphigoid with Lymphocytic Colitis: A Case Report and Short Literature Review

“…In a recent case series, only one patient with UC and concomitant BP was detected, while two had LAD or IgA pemphigus, respectively, and one had mucous membrane pemphigoid (MMP) [ 6 ]. To date, only one case of LAD in a 66-year-old female with lymphocytic colitis has been reported [ 3 ]. In our case, the subsequent occurrence of BP in preexisting enteropathy could certainly be coincidental.…”

“…As a subset of microscopic colitis the macroscopic aspects of the colon mucosa on endoscopy remain unsuspicious, while histology reveals increased infiltrates of lymphocytes, plasma cells and eosinophils in the epithelium and lamina propria, respectively. The etiology is unknown, but auto-immunity is suggested [ 2 , 3 ].…”

Association of linear IgA bullous disease of childhood with Crohn's disease

“…The exact pathogenesis is not clear and has been related to lgA autoantibodies directed against various antigens including bullous pemphigoid (BP) antigen II (NC‐16 A domain of BP 180), 2,3 BP antigen I (BP 230), 2 and 285‐kDa 1 . Linear lgA bullous disease has been reported in association with inflammatory bowel disease (IBD), 4–10 drugs, 11 malignancy, 12 connective tissue diseases, 13 and infections 14 . It seems to represent a reaction pattern to various stimuli where IgA autoantibodies are directed against various antigenic structures at the basement membrane zone (BMZ).…”

Bullous colon lesions in a patient with bullous pemphigoid

“…18 BP, and more frequently linear IgA bullous dermatosis, are known to occur concomitantly with inflammatory bowel diseases, especially ulcerative colitis. [5][6][7][8][9] In patients with linear IgA bullous dermatosis, a disorder commonly associated with IgA auto-antibodies targeting a 97/120 kd protein, deposits of IgA within the colon mucosa were occasionally detected. Although the specificity of these immunoreactants was not assessed, it is again unlikely that the GI manifestations in these patients resulted from autoantibodies to the same 97/120 kd protein expressed in the skin, because the latter appears to correspond to the shedded extracellular domain of BP180.…”

“…4 BP and linear immunoglobulin (Ig) A bullous dermatosis, a condition that might overlap with BP, have been associated with GI disorders such as ulcerative colitis. [5][6][7][8][9] However, the exact pathophysiologic relationship remains unclear because small intestine and colon mucosa do not possess bona fide hemidesmosomes with BP180 and BP230 components. This is a description of a patient with typical clinical and immunopathologic features of BP who developed severe bullous lesions of the colon reminiscent of BP, and a discussion of the potential pathogenic link between the immune-mediated subepidermal disorder and the bowel disease.…”