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.

Chlorambucil is an effective corticosteroid-sparing agent for recalcitrant pyoderma gangrenosum.

BACKGROUND: Pyoderma gangrenosum (PG) may fail to respond to corticosteroids. Immunosuppressive and cytotoxic agents are useful in patients with recalcitrant disease. We describe our experiences with chlorambucil for PG. OBJECTIVE: Our purpose was to evaluate the effectiveness of oral chlorambucil in patients with PG recalcitrant to treatment with prednisone, immunosuppressive therapy, or both. METHODS: Six patients with recalcitrant PG were given oral chlorambucil 2 to 4 mg/day. Four patients were treated with a combination of prednisone and chlorambucil, and two received chlorambucil alone. Response was based on (1) a diminution in the size of the ulcers, or their complete healing, or (2) a decrease in the dose of corticosteroid therapy. RESULTS: Beneficial effects were noted within 6 to 8 weeks in all six patients, and corticosteroids were eventually discontinued in all patients. Currently only two patients are still receiving chlorambucil; the other four stopped chlorambucil after 6 to 24 months of treatment. Their disease has remained in remission for 4 to 9 years. Relapse of disease occurred within 1 to 4 months after stopping therapy in one of the two remaining patients or reducing the dose in the other. In both patients, the disease is again responding to treatment. Minimal chlorambucil toxicity has been noted, consisting of leukopenia in one patient. CONCLUSION: Our findings suggest that chlorambucil is an effective corticosteroid-sparing agent for the control of PG.

Unilateral emboli in a patient with thrombotic thrombocytopenic purpura.

Thrombotic thrombocytopenic purpura is a rare disease most commonly associated with microangiopathic hemolytic anemia, thrombocytopenia, fever, neurologic disorders, and renal dysfunction. We describe a patient with a history of thrombotic thrombocytopenic purpura that had been quiescent for 4 months; he had a 3-week history of painful purpuric lesions on the left hand only. He also had mottling and a livedoid purpura of the distal fingertips, splinter hemorrhages of the left fingernails, and a decreased radial pulse. Findings of a biopsy specimen revealed multiple capillary and small vessel thromboses. Contrast aortography demonstrated a pseudoaneurysm of the proximal descending thoracic aorta with stenosis of the left subclavian artery at its origin and an associated thrombus.

Azathioprine. An effective, corticosteroid-sparing therapy for patients with recalcitrant cutaneous lupus erythematosus or with recalcitrant cutaneous leukocytoclastic vasculitis.

Azathioprine sodium has been reported to be effective therapy for chronic cutaneous lupus erythematosus (LE) but rarely for chronic cutaneous leukocytoclastic vasculitis (LV). We used azathioprine in the treatment of six patients with cutaneous LE, four of whom had subacute cutaneous (nonscarring) LE and two of whom had chronic cutaneous (scarring, discoid) LE, and six patients with chronic cutaneous LV. The conditions of all patients had been resistant to conventional therapy, and they required long-term oral corticosteroid therapy for control of their disease. Two of the patients with LE had prominent palmar and/or plantar involvement. Three patients with LE had an excellent response to azathioprine, with near complete clearing of the skin lesions, allowing a decrease in prednisone dosage. One patient with LE initially demonstrated significant improvement, but azathioprine therapy had to be discontinued because of pancreatitis. The treatment failed in two patients with LE; one had nausea and the other repeatedly developed a drug-induced fever. Five of the six patients with LV had improved conditions, with complete control of the disease occurring in two patients and partial control in three patients. Azathioprine is effective for some patients with cutaneous LE and chronic cutaneous LV, but it should be reserved for patients with severe disease in whom more conventional treatment fails.