Immunobullous disease and ulcerative colitis: a case series of six patients

Abstract

Cases of immunobullous skin disease associated with ulcerative colitis (UC) have been previously reported in the literature. There is no clear explanation for this association. In this series, we report six cases of immunobullous disease in patients with UC and discuss potential mechanisms of pathogenesis proposed to explain these concomitant diseases. The clinical presentation, immunopathology and treatment of six new cases are described and analysed. We report six patients, two with linear IgA bullous dermatosis (LABD), one with bullous pemphigoid (BP), one with mucous membrane pemphigoid (MMP) and two with IgA pemphigus. The patients' ages ranged from 33 to 66 years at the onset of their skin disease, and all but one case had a documented age of UC onset, confirmed with colonoscopy, prior to the development of skin disease. Direct immunofluorescence results in these patients demonstrated IgA basement membrane zone (BMZ) antibodies in the LABD cases, IgG antibodies at the BMZ in the BP and MMP cases, and IgA cell surface antibodies in the patients with IgA pemphigus. Additionally, indirect immunofluorescence was positive in one of the patients with LABD, the patient with BP and both of the patients with IgA pemphigus. The temporal association of UC and skin disease, in addition to the resolution of skin disease with total colectomy in one case, suggests colonic mucosal antigenic stimulation driving immune activation and leading to immunobullous skin disease.

Idiopathic linear IgA bullous dermatosis: prognostic factors based on a case series of 72 adults

“…Two of the patients in our series also had inflammatory bowel disease. It was suggested that chronic digestive antigenic inflammation could trigger immune activation and induce blistering disease . Furthermore, five other patients had solid cancers and three patients had haematological malignancies.…”

Linear immunoglobulin A/G bullous dermatosis associated with ulcerative colitis

“…Some reports have shown total resolution of LABD after proctocolectomy . Cases of bullous pemphigoid with UC have been reported, in which a diagnosis of UC was made before development of skin lesions . In our patient, UC occurred 2 months before exacerbations of skin rash, and combination therapy of prednisolone and DDS was effective for both bowel and skin conditions.…”

“…12 Cases of bullous pemphigoid with UC have been reported, in which a diagnosis of UC was made before development of skin lesions. 13 In our patient, UC occurred 2 months before exacerbations of skin rash, and combination therapy of prednisolone and DDS was effective for both bowel and skin conditions. This evidence suggests that the bowel inflammation observed in UC may have a causative effect on initiation of the immune response to cutaneous tissues and development of immunobullous skin disease.…”

Adalimumab-associated bullous pemphigoid in a patient with ulcerative colitis

“…The salt split technique may be considered to better differentiate subtypes of subepidermal immune bullous disease and represents an integral component of a dermatologist's armamentarium for diagnosis; however, in our patient's case this was noncontributory and anti-BP 180 antibody titers proved more revealing. The exact incidence of BP in patients with inflammatory bowel disease is unknown, although one case series does report a patient with an ulcerative colitis flare and concomitant cutaneous bullous eruption 10 . Our patient's ulcerative colitis was stable before his symptoms.…”