Hypocomplementemic urticarial vasculitis arising in a patient with immunoglobulin G4-related disease.
BACKGROUND
Hypocomplementemic urticarial vasculitis (HUV) has been defined as a syndrome associated with urticarial lesions caused by leukocytoclastic vasculitis. It has been observed in patients with systemic lupus erythematosus and related conditions. Immunoglobulin G4 (IgG4)-related disease is a lymphoproliferative disorder characterized by sclerosing pancreatitis, retroperitoneal fibrosis, sclerotic cholangitis, acute interstitial nephritis, and Mikulicz's disease, and associated with elevated levels of IgG4 and hypocomplementemia. Various cutaneous lesions may occur in IgG4-related disease.


METHODS
We report a 74-year-old woman with HUV, followed by systemic organ involvement suggestive of IgG4-related disease. Symptoms included submandibular gland swelling, lymphadenopathy, interstitial nephritis, and a thyroid nodule. Laboratory examinations revealed an elevated serum IgG4 level of 637 mg/dl in a total IgG level of 2176 mg/dl, and the production of autoantibodies such as rheumatoid factor and antiphospholipid antibodies. In the swollen lymph node, neither clonal proliferation of T or B cells nor Epstein-Barr virus infection was detected, although the structure had been destroyed by the infiltration of lymphocytes and plasma cells. Among total IgG-producing cells, approximately 40% were positive for IgG4.


RESULTS
All symptoms and abnormal laboratory findings improved in response to oral prednisolone.


CONCLUSIONS
Hypocomplementemic urticarial vasculitis may represent a clinical symptom of IgG4-related disease.
Department of Dermatology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan.
Mariko
M
Takao
Department of Dermatology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan.
Toshihisa
T
Hamada
Department of Dermatology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan.
Tatsuya
T
Kaji
Department of Dermatology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan.
Kazuko
K
Ikeda-Mizuno
Department of Dermatology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan.
Chinatsu
C
Takehara-Yasuhara
Department of Pathology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan.
Kouichi
K
Ichimura
Department of Pathology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan.
Hiroyuki
H
Yanai
Department of Pathology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan.
Tadashi
T
Yshino
Department of Dermatology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan.
Keiji
K
Iwatsuki
D000368:Aged; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D003165:Complement System Proteins / Q000172:deficiency*; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood*; D011239:Prednisolone / Q000627:therapeutic use; D014581:Urticaria / Q000097:blood / Q000150:complications* / Q000188:drug therapy; D018366:Vasculitis, Leukocytoclastic, Cutaneous / Q000150:complications* / Q000188:drug therapy / Q000473:pathology
D000893:Anti-Inflammatory Agents; D007074:Immunoglobulin G; D003165:Complement System Proteins; D011239:Prednisolone