An Immunoglobulin A Vasculitis Case Without Skin Symptoms Complicated With Severe Abdominal Symptoms

Abstract

Immunoglobulin A vasculitis (IgAV) primarily affects childhood and can be categorized as immune complex vasculitis. It typically presents with purpura, abdominal pain, arthritis, and nephritis. IgAV can be diagnosed without hesitation when the characteristic skin lesions appear at onset; however, in cases where the abdominal symptoms precede the skin rash or there is no purpura at all, diagnosis can be challenging. Delayed diagnosis of IgAV may be associated with serious abdominal complications, such as gastrointestinal perforation. Here, we describe a girl with IgAV complicated with severe abdominal symptoms and lacking purpura. Despite this lack, the patient's elevated levels of D-dimer and C-reactive protein (CRP), suggestive of vasculitis, and localized small bowel intestinal wall thickening suggested IgAV. After administration of steroids relieved the abdominal symptoms and hypoalbuminemia, treatment was discontinued. Given the limited reports of patients with IgAV complicated with severe abdominal symptoms and no skin symptoms, the diagnosis and management process remains unclear. Therefore, it is imperative to consider IgAV as a differential diagnosis in patients with severe abdominal symptoms. Furthermore, we suggest checking D-dimer, CRP, and coagulation factor XIII activity levels in these patients.

Keywords: Abdominal symptoms; Child; Immunoglobulin A vasculitis.

Figure 1

Findings on an abdominal roentgenography, an ultrasonography, and a contrast computed tomography at disease onset. At disease onset, roentgenography revealed a gasless abdomen and an image of a partially enlarged colon (a). Abdominal ultrasonography and enhanced computed tomography showed images of the thickened wall of the small intestine (b, c, white arrow).

Figure 2

Clinical course in relation to laboratory findings. The horizontal axis shows days after the patient’s admission, and the left vertical axis shows the patient’s serum levels of CRP and Alb. The right vertical axis shows the levels of D-dimer and the quantitative value of stool OB. The upper row shows the transition of FXIII activity and treatment content. The polygonal lines show the transition of the inspection values. CRP: C-reactive protein; Alb: serum albumin; %FXIII: coagulation factor XIII activity; stool OB: stool occult blood; FOM: fosfomycin; PSL: prednisolone; mPSL: methylprednisolone; DDS: diaminodiphenyl sulfone; HD: hospital days.