Search

Verrucous papulonodules are an uncommon manifestation of chronic discoid lupus erythematosus. The differential diagnosis and pathogenesis of this rare entity are discussed. This is the first report of successful surgical excision of trauma-induced lesions of verrucous lupus erythematosus.

We describe four new patients with a unique syndrome of persistent urticaria, with leukoclastic angiitis, severe angioedema, occasional life-threatening laryngeal edema, arthritis, arthralgia, neurologic abnormalities and pronounced persistent hypocomplementemia. The complement abnormalities involved markedly reduced levels of the Clq subunit of the first component of complement (Cl) in the presence of near normal levels of Clr and Cls subunits of Cl; modest to marked depletion of the fourth component of complement (C4), the second component of complement (C2) and the third component of complement (C3); and normal levels of the fifth through ninth components of complement (C5 through C9) and properdin factors B and D. A striking serologic abnormality found in all patients was the presence of low molecular weight (7S) proteins which precipitated with Clq in agarose gels; these previously were shown to be comprised at least in part of immunoglobulin G. The present experience is offered to help to define the clinical, histopathologic and serologic characteristics of this entity, designated hypocomplementemic vasculitic urticarial syndrome, and to emphasize its distinctiveness and prevalence.

Clinical and pathological observations are presented on five patients with scleroderma who developed thrombosis of a major vessel. Three died following intestinal infarction or limb gangrene and two had digit or extremity amputation. Severe intimal thickening of major arteries with thrombus formation was noted but no significant abnormality was found in the clotting activity of the blood. Histochemical studies in three cases suggested a reduction of fibrinolytic activity at the site of thrombosis in the diseased vessels. These studies support the view that the vascular system is profoundly altered in scleroderma and may play an important role in some manifestations of the disease.

The infant of a mother with systemic lupus erythematosus (SLE) developed an extensive cutaneous eruption at 5 weeks of age. Biopsy findings were consistent with cutaneous lupus erythematosus (LE). Splenomegaly, anemia, neutropenia, and depressed total hemolytic complemtnt levels were additional findings. The course was benign, and all manifestations disappeared by 4 months of age. Fifty-two previously reported infants with cutaneous lesions, congenital atrioventricular heart block, or hematologic manifestations of neonatal LE are reviewed.

The infant described in this report had been generalized, subepidermal bullous eruption and concurrent glomerulonephritis. Immunofluorescence studies demonstrating deposition of immunoglobulin and complement in the skin and kidney suggest a common pathogenesis for the two disorders. The elevation of this infant's serum immunoglobulins and depression of serum complement support an immunologic process as the likely mechanism. It is important for clinicians caring for children with bullous skin diseases to search for evidence of renal disease and complement abnormalities.

The effects of several chemotherapeutic agents on the chemotaxis of human leukocytes were studied in an in vitro system using a Sykes-Moore chamber and a double-filter technique. Chemotactic factor was generated by the interaction of normal human serum and zymosan. At concentrations comparable to and below therapeutic blood levels, tetracycline HCl, erythromycin base and clindamycin HCl were all inhibitory, causing marked suppression of leukocyte chemotaxis and slight reduction of random migration. Penicillin G-Na, dapsone, and sulfapyridine did not alter white cell motility at the concentrations of drug tested. It is postulated that the capacity of some of these agents to inhibit leukocyte chemotaxis may account, in part, for their efficacy in inflammatory skin diseases such as acne vulgaris.

The effect of zymosan-activated serum samples, obtained from patients with acne who were receiving oral tetracycline hydrochloride therapy, on the chemotaxis and random migration of autologous polymorphonuclear leukocytes (PMNLs) and PMNLs from control donors was assayed in a Sykes-Moore chamber by a double-filter technique. Cells incubated with autologous serum specimens from these patients demonstrated significant suppression of both random migration and chemotaxis, with the greatest effect seen at a dosage of 1 g daily and a significant effect found at all dosage levels. When zymosan-activated serum samples from these patients were assayed with control donor PMNLs, similar inhibitory effects were shown. Assays of control sera and PMNLs from tetracycline-treated patients with acne showed the least degree of inhibition of chemotaxis. Although the mechanism of action is unclear, tetracycline appears to have a direct effect on neutrophil movement.

A 65-year-old black female with Sezary syndrome had generalized intractable pruritus, erythroderma, alopecia, onychogryphosis, lumphadenopathy and hepatomegaly. Abnormal lymphocytes with large, convoluted and grooved nuclei (Sezary cells) were identified in the skin and peripheral blood. A striking feature of her disease was severe, deforming arthropathy of the hands and knees, a clinical finding which has been described previously in only one patient with Sezary syndrome. At necropsy no associated lymphoma was found.

The clinical features, laboratory studies, and therapeutic responses of two boys with chronic bullous dermatosis of childhood are described. Direct immunofluorescent preparations of sections from a lesion, skin adjacent to a lesion, and uninvolved skin demonstrated linear deposition of IgA at the dermoepidermal junction in all three biopsy specimens from one patient. Similar preparations from the second child were negative for staining. No circulating antibodies to skin components were detected in either child by means of multiple substrates. Neither child had clinical manifestations nor laboratory findings suggestive of an associated gastrointestinal lesion; therefore, small bowel biopsies were not performed. Immunologic studies failed to demonstrate any of the abnormalities frequently described in dermatitis herpetiformis. Both boys responded dramatically to sulfapyridine therapy.