Complex extra-intestinal complications of ulcerative colitis in a patient with alpha1-antitrypsin deficiency.
Ulcerative colitis (UC) can manifest with a variety of extra-intestinal disorders frequently affecting the skin, joints, and liver. An aetiologic role of alpha1-antitrypsin deficiency in chronic inflammatory bowel disease has recently been suggested. We report a patient with UC and alpha1-antitrypsin deficiency who presented with disseminated cutaneous leucocytoclastic vasculitis clinically appearing with target-like purpuric patches and haemorrhagic oedemas. In addition, he displayed acute haemorrhage of the eyes and the respiratory tract consistent with a systemic vasculitic process. Moreover, he had autoimmune haemolytic anaemia. Systemic vasculitides, such as Wegener's granulomatosis, Churg-Strauss syndrome, and microscopic polyangiitis, could widely be excluded. Systemically administered glucocorticosteroids and azathioprine led to dramatic improvement of extra-intestinal symptoms. On the basis of alpha1-antitrypsin deficiency and UC, the present patient likely developed severe systemic vasculitis with multi-organ involvement. UC should at times be viewed within the context of a more generalized immune imbalance affecting multiple organs, and not as an isolated pathological entity. Testing for alpha1-antitrypsin deficiency in UC patients may detect individuals at higher risk of severe extra-intestinal involvement.
Gambichler
T
T
Department of Dermatology, Ruhr-University Bochum, Germany. t.gambichler@klinikum-bochum.de
Reich
S
S
Banasch
M
M
Altmeyer
P
P
D003093:Colitis, Ulcerative / Q000150:complications*; D003232:Conjunctivitis, Acute Hemorrhagic / Q000209:etiology; D006801:Humans; D011695:IgA Vasculitis / Q000209:etiology; D008297:Male; D008875:Middle Aged; D012140:Respiratory Tract Diseases / Q000209:etiology; D019896:alpha 1-Antitrypsin Deficiency / Q000150:complications*