Anti-C1q antibodies in systemic lupus erythematosus.

OBJECTIVE: Anti-C1q has been associated with systemic lupus erythematosus (SLE) and lupus nephritis in previous studies. We studied anti-C1q specificity for SLE (vs rheumatic disease controls) and the association with SLE manifestations in an international multicenter study. METHODS: Information and blood samples were obtained in a cross-sectional study from patients with SLE (n = 308) and other rheumatologic diseases (n = 389) from 25 clinical sites (84% female, 68% Caucasian, 17% African descent, 8% Asian, 7% other). IgG anti-C1q against the collagen-like region was measured by ELISA. RESULTS: Prevalence of anti-C1q was 28% (86/308) in patients with SLE and 13% (49/389) in controls (OR = 2.7, 95% CI: 1.8-4, p < 0.001). Anti-C1q was associated with proteinuria (OR = 3.0, 95% CI: 1.7-5.1, p < 0.001), red cell casts (OR = 2.6, 95% CI: 1.2-5.4, p = 0.015), anti-dsDNA (OR = 3.4, 95% CI: 1.9-6.1, p < 0.001) and anti-Smith (OR = 2.8, 95% CI: 1.5-5.0, p = 0.01). Anti-C1q was independently associated with renal involvement after adjustment for demographics, ANA, anti-dsDNA and low complement (OR = 2.3, 95% CI: 1.3-4.2, p < 0.01). Simultaneously positive anti-C1q, anti-dsDNA and low complement was strongly associated with renal involvement (OR = 14.9, 95% CI: 5.8-38.4, p < 0.01). CONCLUSIONS: Anti-C1q was more common in patients with SLE and those of Asian race/ethnicity. We confirmed a significant association of anti-C1q with renal involvement, independent of demographics and other serologies. Anti-C1q in combination with anti-dsDNA and low complement was the strongest serological association with renal involvement. These data support the usefulness of anti-C1q in SLE, especially in lupus nephritis.

Drug-induced subacute cutaneous lupus erythematosus.

Subacute cutaneous lupus erythematosus (SCLE) is a subset of cutaneous lupus erythematosus with unique immunologic and clinical features. The first description dates back to 1985 when a series of five patients were found to have hydrochlorothiazide-induced SCLE. Since that time, at least 40 other drugs have been implicated in the induction of SCLE.

Dermatology position paper on the revision of the 1982 ACR criteria for systemic lupus erythematosus.

The 1982 ACR classification criteria have become de facto diagnostic criteria for systemic lupus erythematosus (SLE), but a review of the criteria is necessary to include recent diagnostic tests. The criteria were not developed with the help of dermatologists, and assign too much weight to the skin as one expression of a multiorgan disease. Consequently, patients with skin diseases are classified as SLE based mostly on skin symptoms. We discuss specific problems with each dermatologic criterion, but changes must await a new study. We suggest the following guidelines for such a study, aimed at revision of the criteria. 1) The SLE patient group should be recruited in part by dermatologists. 2) The study should evaluate an appropriate international ethnic/racial mix, including late onset SLE as well as pediatric patients. 3) All patients should have current laboratory and clinical evaluations, as suggested in the paper, to assure the criteria can be up-to-date. This includes anti-SS-A and anti-SS-B antibodies and skin biopsies for suspected cutaneous lupus erythematosus except for nonscarring alopecia and oral ulcers. 4) The study should be based on a series of transparent power calculations. 5) The control groups should represent relevant differential diagnoses in numbers large enough to assess diagnostic problems that might be specific to these differential diagnoses. In order to demonstrate specificity of the criteria with a 95% confidence interval between 90 and 100%, each control group of the above should have at least 73 patients.

Vancomycin-induced linear IgA bullous disease presenting as toxic epidermal necrolysis.

Linear IgA bullous dermatosis (LABD) is a rare autoimmune vesiculobullous disorder characterized by variable clinical presentations that may mimic bullous pemphigoid, dermatitis herpetiformis, cicatricial pemphigoid and erythema multiforme. A few cases of drug-induced LABD that clinically resembled toxic epidermal necrolysis (TEN) have been reported. A subset of patients with LABD have been found to be drug-induced; the most common drug being vancomycin. The diagnosis of LABD is confirmed by the presence of a linear band of IgA along the basement membrane zone on direct immunofluorescence microscopy. We report a case of a 77-year-old man who presented to us with vancomycin-induced LABD that presented clinically as TEN. He had a complete recovery over a 3-week period following discontinuation of the vancomycin and the addition of oral dapsone therapy. It is important to be aware that drug-induced LABD can mimic TEN.

Update on the management of cutaneous lupus erythematosus.

This paper reviews the latest treatments for cutaneous lupus erythematosus. It focuses on evidence-based guidance for the management of cutaneous lupus erythematosus, with identification of the strength of evidence available at this time. In addition, I have briefly reviewed the epidemiological aspects, diagnosis and evaluation of patients with cutaneous lupus erythematosus. This review reflects data available from the Cochrane Library, Medline, literature searches, and the experience of the author managing patients with cutaneous lupus erythematosus for over 25 years.

Dermatomyositis: diagnosis, evaluation and management.

Dermatomyositis (DM) is a condition primarily of the skin and muscles, but other systemic features may occur. DM in adults is associated with malignancy and thus a careful evaluation of each patient should be part of their initial and follow-up assessments. Patients should also be evaluated for the presence of esophageal, pulmonary and cardiac disease. Corticosteroids, immunosuppressives, biologic agents and/or immune globulin are effective therapies for the myopathy of DM, whereas the skin disease is best managed with sunprotection, topical corticosteroids, antimalarials, methotrexate and/or immune globulin. The prognosis is good except for patients with malignancy, those with severe weakness and those with cardiopulmonary dysfunction. However, many patients are left with residual weakness even after control of the disease.

Sweet's syndrome in acute myelogenous leukemia presenting as periorbital cellulitis with an infiltrate of leukemic cells.

Sweet's syndrome is characterized by the abrupt onset of fever, neutrophilic leukocytosis, and erythematous, tender pseudovesiculated plaques or nodules that respond readily to corticosteroid therapy. It is usually distinguished by the presence of mature neutrophils on histopathologic examination. We describe a 38-year-old man with acute myelogenous leukemia who had an erythematous vesicular eruption of the left eye develop that resembled cellulitis. A biopsy specimen revealed a dermal infiltrate of mature neutrophils and immature myeloblastic precursors. He later had hemorrhagic pseudovesiculated plaques develop bilaterally on his hands. A biopsy specimen again revealed abundant neutrophils with immature forms. A similar eruption developed at the site of a Hickman catheter placement 4 months later. His skin lesions responded rapidly to oral corticosteroids. This case is unique in that his initial presentation of Sweet's syndrome resembled orbital cellulitis that was characterized by immature myeloid precursors on histopathology.

Rheumatoid neutrophilic dermatitis in a woman with seronegative rheumatoid arthritis.

Rheumatoid neutrophilic dermatitis (RND) is an unusual cutaneous reaction in patients with rheumatoid arthritis (RA). RND is characterized by symmetric, erythematous papules, plaques, nodules, and urticarial lesions often located over the joints, extensor surfaces of the extremities, or the trunk. This entity demonstrates an intense neutrophilic dermal infiltrate without vasculitis. All patients previously reported with RND had severe RA with relatively high titers of rheumatoid factor when tested. A 67-year-old woman had a 2-month history of multiple, tender, 4 to 8 mm erythematous, crusted papules and nodules that occurred in clusters on her anterior thighs, knees, and legs. She suffered from severe disabling seronegative RA. RND may complicate seronegative RA.

Calcifying lupus panniculitis in a patient with subacute cutaneous lupus erythematosus: response to diltiazem and chloroquine.

Lupus panniculitis has been reported to occur with a frequency of 2-3% in patients with lupus erythematosus (LE). It is most often reported in association with lesions of discoid LE. We describe a patient with subacute cutaneous LE who developed calcified nodules that were histopathologically consistent with lupus panniculitis. She was treated with a combination of chloroquine and diltiazem with a good therapeutic response. The addition of diltiazem may be beneficial in patients with calcified nodules of lupus panniculitis.

Subacute cutaneous lupus erythematosus induced or exacerbated by terbinafine: a report of 5 cases.

BACKGROUND: Subacute cutaneous lupus erythematosus is a nonscarring, non-atrophy-producing photosensitive cutaneous disorder. Half of the patients have 4 or more of the criteria for classification as systemic lupus erythematosus. In some patients, drugs induce or exacerbate the cutaneous disease. OBSERVATION: We describe 5 patients who had either an exacerbation or a new onset of subacute cutaneous lupus erythematosus while taking terbinafine for presumed onychomycosis. CONCLUSION: In general, terbinafine is a safe drug, but perhaps patients with known lupus erythematosus or photosensitivity are predisposed to drug-induced or drug-exacerbated disease.

Immunosuppressive and cytotoxic drugs in the treatment of rheumatic skin disorders.

Cytotoxic and immunosuppressive drugs are regularly used to treat proliferative, immunologically mediated inflammatory disorders and some neoplastic diseases of the skin. Methotrexate, azathioprine, mycophenolate mofetil, cyclosporin cyclophosphamide, chlorambucil, and other related drugs have potential benefits in the treatment of severe and/or recalcitrant rheumatic skin diseases. The therapeutic window for these agents is narrow. The major uses of these drugs are for life-threatening cutaneous disorders or as steroid-sparing agents.

Epidermolysis bullosa acquisita responsive to dapsone therapy.

BACKGROUND: Epidermolysis bullosa acquisita (EBA) is a chronic subepidermal blistering disease that is frequently resistant to therapy. OBJECTIVE: A 58-year-old man who had a one-year history of a bullous eruption involving the hands, forearms, trunk, scalp, and oral mucosa. Histopathology revealed a subepidermal bulla, and direct and indirect immunofluorescence studies were consistent with EBA. The patient failed respond to niacinamide and tetracycline and oral prednisone 40 mg per day. METHODS: Complete control of his blistering was achieved within two months of initiating oral dapsone, 150 mg per day. CONCLUSION: Dapsone may be an effective agent for some patients with EBA.