Abstract
Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a type III hypersensitivity reaction characterized by urticaria with persistent acquired hypocomplementemia. Although HUVS is uncommon, it is important for physicians to be familiar with this disease, as the initial presentation is often life-threatening angioedema. The author reports the case of a 47-year-old white woman with a history of HUVS. She presented to an outpatient clinic complaining of a rash and difficulty swallowing. Urticaria and angioedema were diagnosed. The patient was given epinephrine in the clinic and then transferred to the hospital. Laboratory testing confirmed urticaria, and the patient was given intravenous methylprednisolone sodium succinate and an additional dose of epinephrine. After 1 week, the patient's angioedema improved.
First described in 1973, hypocomplementemic urticarial vasculitis syndrome (HUVS) is a form of severe vasculitis associated with several systemic findings including leukocytoclastic vasculitis, angioedema, pulmonary manifestations, arthritis, arthralgia, glomerulonephritis, and uveitis.
1 Laboratory findings include low complement levels of the classical complement, principally C1q, C2, C4, and the presence of anti-C1q antibodies. Treatment, which varies with disease severity, involves corticosteroids and other immunosuppressive agents. This disease is associated with high rates of morbidity and mortality, most commonly caused by chronic obstructive pulmonary disease and acute laryngeal edema.
2
A 47-year-old woman with a known diagnosis of HUVS presented to an outpatient rheumatology clinic with a chief complaint of “my throat feels like it is closing.” The patient's initial symptoms included urticaria on her arms and trunk and angioedema of the face, lips, and throat. In addition, she complained of a headache with blurry right-sided vision. The patient was presumed to have angioedema secondary to HUVS. She was given a 0.3-mg epinephrine injection at the outpatient rheumatology clinic and then transported to the intensive care unit at the local hospital because of airway compromise.
This patient was first diagnosed as having HUVS 1 year before presentation by her rheumatologist after presenting with muscle pains in her hands and shoulders, general fatigue, dizziness, and hair loss. She reported developing redness and a rash on her cheeks and nose with sun exposure as well as recurrent, painful wheals that left hyperpigmentation of her skin when resolved. She also experienced swelling in her tongue and lips on a monthly basis, for which she was treated with methotrexate and prednisone with good response. The patient also met the diagnostic criteria for systemic lupus erythematosus (SLE) and was started on hydroxychloroquine sulfate and prednisone. Hydroxychloroquine was discontinued because of adverse effects, but the patient was receiving 15 mg of prednisone daily when she presented to the clinic
Findings from a biopsy of skin wheals and a hematoxylin and eosin stain confirmed urticaria, and the direct immunoflourescence was consistent with HUVS. Results of laboratory testing are presented in the
Table.
Table.
Laboratory Findings for a 47-Year-Old Woman With Hypocomplementemic Urticarial Vasculitis Syndrome
Laboratory Testa | Finding | Reference Range |
Erythrocyte Sedimentation Rate, mm/h | 6 | ⩽ 20 |
C-reactive Protein, mg/L | 0.40 | <0.80 |
Creatinine Kinase, U/L | 18 | 40-150 |
Antibody Panel | | |
Antinuclear antibody, titer | Positive | Negative |
Dual pattern | | |
Homogeneous, titer | 1:80 | NA |
Speckled, titer | 1:160 | NA |
Centromere antibody | Negative | Negative |
Double-stranded DNA antibody | Negative | Negative |
SS-A antibody | Negative | Negative |
SS-B antibody | Negative | Negative |
SM antibody | Negative | Negative |
SM/RNP antibody | Negative | Negative |
Rheumatoid factor, IU/mL | 6 | <14 |
Cyclic citrullinated peptide antibody, U | <16 | <16 |
C3 complement, mg/dL | 50 | 90-180 |
C4 complement, mg/dL | 3 | 16-47 |
CH50, U/mL | <13 | 31-66 |
Antineutrophil cytoplasmic antibody | Negative | Negative |
Angioedema Panel | | |
C1 esterase inhibitor, mg/dL | 10 | 11-26 |
C1q complement, mg/dL | Undetected | 5.0-8.6 |
Table.
Laboratory Findings for a 47-Year-Old Woman With Hypocomplementemic Urticarial Vasculitis Syndrome
Laboratory Testa | Finding | Reference Range |
Erythrocyte Sedimentation Rate, mm/h | 6 | ⩽ 20 |
C-reactive Protein, mg/L | 0.40 | <0.80 |
Creatinine Kinase, U/L | 18 | 40-150 |
Antibody Panel | | |
Antinuclear antibody, titer | Positive | Negative |
Dual pattern | | |
Homogeneous, titer | 1:80 | NA |
Speckled, titer | 1:160 | NA |
Centromere antibody | Negative | Negative |
Double-stranded DNA antibody | Negative | Negative |
SS-A antibody | Negative | Negative |
SS-B antibody | Negative | Negative |
SM antibody | Negative | Negative |
SM/RNP antibody | Negative | Negative |
Rheumatoid factor, IU/mL | 6 | <14 |
Cyclic citrullinated peptide antibody, U | <16 | <16 |
C3 complement, mg/dL | 50 | 90-180 |
C4 complement, mg/dL | 3 | 16-47 |
CH50, U/mL | <13 | 31-66 |
Antineutrophil cytoplasmic antibody | Negative | Negative |
Angioedema Panel | | |
C1 esterase inhibitor, mg/dL | 10 | 11-26 |
C1q complement, mg/dL | Undetected | 5.0-8.6 |
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The patient received methylprednisolone sodium succinate (125 mg intravenously) and diphenhydramine hydrochloride (50 mg intravenously) as well as another single dose of 0.3 mg epinephrine. As the angioedema symptoms improved, oral prednisone was started (60 mg daily) and methylprednisolone sodium succinate was discontinued. After test results confirmed the patient did not have a glucose-6-phosphate dehydrogenase (G6PD) deficiency, dapsone (50 mg twice daily) was started. The patient was discharged after a 1-week hospital course and received a prescription for 0.3 mg subcutaneous epinephrine for emergency purposes. The patient had no return of angioedema symptoms at 2-week follow-up.