The hypocomplementemic urticarial-vasculitis syndrome: Therapeutic response to hydroxychloroquine

Lopez, Luis R.; Davis, Kathleen C.; Köhler, Peter F.; Schocket, Alan L.

doi:10.1016/0091-6749(84)90518-9

Cited by 96 publications

(31 citation statements)

References 8 publications

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“…25 In these cases, dapsone may be the drug of choice, either alone 51,[110][111][112] or in association with other agents 51,113,114 ; in particular, it is effective in patients with a SLE-like presentation of UV. 25 Colchicine 112,115-118 and hydroxychloroquine 119 have also been used with some success. Mycophenolate mofetil has also been effective in the treatment of UV and as maintenance therapy.…”

Section: Systemic Urticarial Syndromesmentioning

confidence: 98%

Urticarial lesions: If not urticaria, what else? The differential diagnosis of urticaria

Peroni

Colato

Zanoni

et al. 2010

Journal of the American Academy of Dermatology

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Section: Systemic Urticarial Syndromesmentioning

confidence: 98%

Urticarial lesions: If not urticaria, what else? The differential diagnosis of urticaria

Peroni

Colato

Zanoni

et al. 2010

Journal of the American Academy of Dermatology

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“…Skin lesions appear to be poorly responsive to antihistamines. Anecdotal success in treatment of urticarial vasculitis is reported with prednisone, hydroxychloroquine [12,13], or dapsone [14]. Joint lesions respond well to glucocorticoid or non-steroidal anti-inflammatory drug therapy.…”

Section: Discussionmentioning

confidence: 95%

Hypocomplementemic urticarial vasculitis: report of a pediatric case

Cadnapaphornchai

Saulsbury

Norwood

2000

Pediatric Nephrology

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Hypocomplementemic urticarial vasculitis syndrome (HUVS) is well described in adults but is quite rare in children. We report a pediatric case of HUVS initially diagnosed as juvenile rheumatoid arthritis and then as Henoch-Schönlein purpura. Beginning at 3 years of age, our patient developed polyarthritis with hypocomplementemia. She subsequently experienced an intermittent purpuric rash beginning at age 4 years, and she continued to have episodic arthritis and rash for years. Hematuria and proteinuria were noted at 12 years of age; renal biopsy revealed membranoproliferative glomerulonephritis with membranous features. Serum complement evaluation revealed activation of the classical pathway, consistent with HUVS. Therapy with oral dapsone led to improvement in proteinuria. HUVS should be considered in the differential diagnosis of pediatric patients with glomerulonephritis, urticarial rash, arthritis/arthralgias, and obstructive pulmonary disease.

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“…The role of its use in CSVV, as seen in the literature, is limited. Lopez et al [27] report a solitary case of a patient with HUV, previously refractory to antihistamines, nonsteroidal anti-inflammatory drugs, prednisone, and azathioprine, who successfully responded to 400 mg/d of hydroxychloroquine. The patient had significant disease recurrence upon numerous attempts at discontinuation of the medication with clearance upon reinstitution.…”

Section: Hydroxychloroquinementioning

confidence: 97%

Primary cutaneous small vessel vasculitis

Russell

Weenig

2004

Curr Treat Options Cardio Med

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Disorders associated with cutaneous vasculitis include numerous well-described etiologies. Primary cutaneous vasculitis limits discussion to primary leukocytoclastic vasculitis, essential mixed cryoglobulinemia, urticarial vasculitis, Henoch-Schönlein purpura, and erythema elevatum diutinum. Although the therapeutics for these disorders are based on limited data, we attempt to construct a consensus opinion on the management of primary cutaneous vasculitis. Therapy of primary cutaneous vasculitis is indicated for symptomatic or systemic involvement, because cutaneous small vessel vasculitis is frequently a self-limited, single episodic disease. Conservative, symptomatic treatment includes leg elevation, warming, antihistamines, and nonsteroidal anti-inflammatory drugs. For mild recurrent disease, colchicine, dapsone, and prednisone are first-choice agents. Systemic or severe cutaneous disease requires more potent immunosuppression (eg, prednisone, azathioprine, or mycophenolate mofetil). Plasmapheresis/plasma exchange and intravenous immunoglobulin are viable considerations for refractory disease, but are cumbersome and expensive modalities. There is insufficient evidence to advocate the use of new biological or monoclonal antibody therapies in primary cutaneous vasculitis.

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The hypocomplementemic urticarial-vasculitis syndrome: Therapeutic response to hydroxychloroquine

Cited by 96 publications

References 8 publications

Urticarial lesions: If not urticaria, what else? The differential diagnosis of urticaria

Urticarial lesions: If not urticaria, what else? The differential diagnosis of urticaria

Hypocomplementemic urticarial vasculitis: report of a pediatric case

Primary cutaneous small vessel vasculitis

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