J Korean Med Sci.  2017 Dec;32(12):2064-2068. 10.3346/jkms.2017.32.12.2064.

Hypocomplementemic Urticarial Vasculitis Syndrome with Membranous Nephropathy: Case Report

• Jung SW
• Choi YY
• Choi IS
• Kim S
• Jeong KH
• Song R
• Lee SH
• Yang HI
• Hong SJ
• Lee YA

• KMID: 2396387
• DOI: http://doi.org/10.3346/jkms.2017.32.12.2064

Abstract

Urticarial vasculitis is a rare disorder that principally manifests with recurrent urticarial, sometimes hemorrhagic, skin lesions and/or angioedema. Its clinical presentation is not always limited to cutaneous lesions and it can potentially affect other organs, such as the joints, lungs, kidneys, and eyes. Systemic involvement can either be present at the onset of disease or develop over time. In cases with systemic manifestations, urticarial vasculitis is more likely to be associated with a low complement level. We present the case of a teenage boy with hypocomplementemic urticarial vasculitis syndrome (HUVS) that occurred shortly following swine-origin influenza A virus infection in 2009. Afterwards, HUVS was systemically complicated with myositis and membranous nephropathy that developed several months and about 2 years after its onset, respectively. A combination of glucocorticoid and immunosuppressive agents has been used to effectively control disease activity.

Keyword

Hypocomplementemic Urticarial Vasculitis Syndrome; Membranous Nephropathy; Myositis

MeSH Terms

Angioedema
Complement System Proteins
Glomerulonephritis, Membranous*
Humans
Immunosuppressive Agents
Influenza A virus
Joints
Kidney
Lung
Male
Myositis
Skin
Vasculitis*
Complement System Proteins
Immunosuppressive Agents

Figure

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  Fig. 1 Skin changes in the patient. (A) Bruise on the left hand. (B) Subsequent erythematous change with diffuse swelling on the left neck.

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  Fig. 2 Skin punch biopsy from a petechial area of right arm. (A) Moderate inflammatory cell infiltrates composed of neutrophils and lymphocytes involving the superficial dermis (H & E stained; original magnification × 100). (B) High-power view highlights leukocytoclastic vasculitis in the dermal vasculature (H & E stained; original magnification × 200). H & E = hematoxylin and eosin.

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  Fig. 3 Renal biopsy findings. (A) Mild glomerular capillary wall thickening with normal cellularity and patent lumen (PAS stain; original magnification × 400). (B) Immunofluorescence staining for IgG along the glomerular capillary wall (original magnification × 200). (C) Immunofluorescence staining for C3 along the glomerular capillary wall (original magnification × 200). (D) A number of subepithelial deposits are observed with foot process effacement and basement membrane thickening (electron microscopy; one length of black or gray scale bar represents 1 µm). PAS = periodic acid-Schiff, IgG = immunoglobulin G.

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