1/37. Diversity in presenting manifestations of systemic lupus erythematosus in children.OBJECTIVE: To describe the diversity in presenting manifestations of systemic lupus erythematosus (SLE) in children. STUDY DESIGN: Initial clinical and laboratory manifestations of 39 children, who fulfilled >/=4 American College of rheumatology criteria for SLE, were retrospectively analyzed. RESULTS: Median age at onset was 12 years. The male to female ratio was 1:18.5, and racial/ethnic backgrounds were white 41%, black 33%, and Hispanic 26%. Initial manifestations included musculoskeletal 74%, cutaneous 72%, constitutional 67%, neurologic 28%, renal 28%, lymphadenopathy 15%, and Raynaud's phenomenon 10%. Laboratory abnormalities at presentation to our clinic included elevated erythrocyte sedimentation rate 87%, anemia 72%, lymphopenia 59%, leukopenia 31%, proteinuria or cellular casts 44%, low C(3) or C(4) level 77%, antinuclear antibodies 97%, and anti-double-stranded dna 95%. One third (33%) presented with features not initially suggestive of SLE. Six patients presented with unusual manifestations including parotitis, quadriplegia, chorea, severe abdominal pain, persistent cough, and dizziness. However, 85% of patients with atypical manifestations had abnormal complete blood count or urinalysis results at presentation. CONCLUSION: Presenting manifestations of SLE in children are diverse. A detailed history, thorough review of systems, complete physical examination, complete blood count, urinalysis, and a high index of suspicion help to make the correct diagnosis of SLE in patients with atypical presentations.- - - - - - - - - - ranking = 1keywords = black (Clic here for more details about this article) |
2/37. A case of black dot ringworm with a review of Japanese cases.Black dot ringworm (BDR), caused by trichophyton violaceum var. glabrum (T. glabrum), was observed in a 28-year-old Japanese female who had been treated with prednisolone (22.5 mg/day) for systemic lupus erythematosus. It was successfully treated with oral terbinafine (125 mg/day) for 12 weeks. The causative fungus was identified by molecular analysis as well as morphological and biochemical examination. The chitin synthase 1 (CHS1) gene cleavage pattern of the clinical isolate with restricted enzyme HinfI was identical to that of T. violaceum. We reviewed previous reports of BDR to determine the historical trend of this infection in japan. Since 1974, 93 Japanese cases have been reported. The age distribution was bi-modal: the higher peak consisted of children (aged 0-15 years), and the lower peak was composed of the elderly (aged 60-75 years). In the elderly group, females were predominant (M:F=1:22, p<0.001). T. violaceum, including T. glabrum, was identified as the most common causative fungus of BDR (75.3%). Sixty percent of cases showed slight erythema. In 8 families, 16 cases were found to be intrafamilial infections. A history of previous steroid treatment was described in about 40%.- - - - - - - - - - ranking = 4keywords = black (Clic here for more details about this article) |
3/37. Coexisting systemic lupus erythematosus and sickle cell disease: a diagnostic and therapeutic challenge.Systemic lupus erythematosus (SLE) and sickle cell disease (SCD) are relatively common disorders with comparable prevalence among blacks. The coexistence of these 2 conditions in the same individual appears to be rare. We report 4 cases of coexisting SLE and SCD. These patients displayed a broad spectrum of musculoskeletal, central nervous system, and renal complications that may be associated with either SCD or SLE. Because of a substantial overlap between the clinical manifestations of these 2 disorders, the diagnosis of SLE in patients with SCD may be difficult to establish and is often delayed. Up to 23% of patients with SCD may have antinuclear antibodies. All patients in this series had antecedent SCD but new important complications from SLE.- - - - - - - - - - ranking = 1keywords = black (Clic here for more details about this article) |
4/37. Systemic lupus erythematosus in a black South African child. First documented case report.Systemic lupus erythematosus (SLE) is poorly described among black children in africa despite being more frequent among some black adult populations than their white counterparts. The first black South African child with SLE is documented. The patient was a 10-year-old girl who had fever, facial rash (with complement (C4) deposited at the dermo-epidermal junction of normal skin), weight loss, central nervous system involvement (depression, withdrawal, retinal exudates), renal involvement (glomerular filtration rate 54 ml/min/1.73 m2; membranous nephropathy with mild mesangial proliferation; world health Organisation classification Vb), alopecia, lymphadenopathy, hepatomegaly, positive coombs test, hypeocomplementaemia, anti-dna antibodies and positive anti-nuclear factor.- - - - - - - - - - ranking = 7keywords = black (Clic here for more details about this article) |
5/37. Detection of anti-basement membrane zone antibodies in bullous systemic lupus erythematosus.We describe a 42-year-old black woman with long-standing systemic lupus erythematosus in whom vesiculobullous lesions developed. Routine histologic and immunologic studies fulfilled the criteria for the diagnosis of bullous systemic lupus erythematosus. Indirect immunofluorescence showed antinuclear antibodies without basement membrane zone fluorescence. We destroyed the nuclear antigens of the indirect immunofluorescence substrate with 2 mol/L sodium chloride, which unmasked basement membrane zone linear IgG staining. We also confirmed anti-basement membrane zone antibodies by employing a new technique of direct immunofluorescence on sodium chloride-split skin. Our finding prove that a thorough search for anti-basement membrane zone antibodies can be revealing. Our results support the idea that a subset of bullous systemic lupus erythematosus has the staining characteristics of epidermolysis acquisita, with the dermal side of the split skin showing linear immunoglobulin deposition.- - - - - - - - - - ranking = 1keywords = black (Clic here for more details about this article) |
6/37. epidermolysis bullosa acquisita preceding the development of systemic lupus erythematosus.In most cases of epidermolysis bullosa acquisita that occur in patients with systemic lupus erythematosus, the diagnosis of systemic lupus erythematosus is made before the development of blistering. We observed three patients with well-documented epidermolysis bullosa acquisita that developed several years before the onset of systemic lupus erythematosus. One patient was producing anti-U1RNP autoantibodies at the time epidermolysis bullosa acquisita was diagnosed, and all five produced this antibody during the systemic lupus erythematosus phase of their illness. In addition, in all five cases of epidermolysis bullosa acquisita with systemic lupus erythematosus antibodies to double-stranded dna ultimately developed, and severe systemic lupus erythematosus and lupus nephritis developed in four patients. Sera from 15 other patients with epidermolysis bullosa acquisita without overt systemic lupus erythematosus were analyzed for systemic lupus erythematosus-related autoantibodies. Four patients were found to have at least one such autoantibody. These findings further document an association between epidermolysis bullosa acquisita and systemic lupus erythematosus and suggest that patients with systemic lupus erythematosus who present with epidermolysis bullosa acquisita may represent a subset of lupus erythematosus that puts the patient at increased risk for the development of more severe systemic illness. patients presenting with epidermolysis bullosa acquisita, especially those who are black or Hispanic, should be monitored for the development of potentially life-threatening systemic lupus erythematosus.- - - - - - - - - - ranking = 1keywords = black (Clic here for more details about this article) |
7/37. Concomitant systemic lupus erythematosus and ankylosing spondylitis.The case is reported of a 42 year old white woman meeting currently used diagnostic criteria for both ankylosing spondylitis and systemic lupus erythematosus (SLE). As found in a previously described similar case of a black man, HLA typing showed antigens associated with both SLE and seronegative spondyloarthropathy. This case thus supports the hypothesis that the two diseases occur together only when this rare combination of hla antigens is present.- - - - - - - - - - ranking = 1keywords = black (Clic here for more details about this article) |
8/37. hypothermia and systemic lupus erythematosus.We describe an 18-year-old black woman with systemic lupus erythematosus (SLE) who presented with an exacerbation of polymyositis and neuropsychiatric symptoms. After starting prednisone she became hypothermic for 5 days. hypothermia associated with SLE has been described in only 3 patients.- - - - - - - - - - ranking = 1keywords = black (Clic here for more details about this article) |
9/37. Systemic lupus erythematosus with deficiency of the T4 epitope on T helper/inducer cells.Three black Jamaicans with systemic lupus erythematosus (SLE) were identified whose T helper/inducer cells lacked the T4 epitope (T4 epitope-deficient phenotype). All three patients had lymphadenopathy as part of their syndromes. The asymptomatic and otherwise healthy T4 epitope-deficient brother of one of these patients also had lymphadenopathy in a distribution identical to that of his sister with SLE. family studies pointed to an autosomal codominant mode of inheritance not linked to the HLA locus for the T4 epitope phenotype. Cultures of peripheral-blood mononuclear cells revealed impaired B-cell differentiation upon stimulation with pokeweed mitogen in cells originating from the T4 epitope-deficient family members as compared with those originating from their T4 epitope-intermediate relatives. Ratios of T helper/inducer cells to T suppressor/cytotoxic cells, the presence of various autoantibodies, and proliferation in response to mitogens and in the mixed lymphocyte reactions did not correlate with T4 epitope phenotype. We suggest that SLE in association with the T4 epitope-deficient phenotype may represent a unique subset of patients with SLE that has distinct clinical and immunologic properties.- - - - - - - - - - ranking = 1keywords = black (Clic here for more details about this article) |
10/37. autoantibodies to the insulin receptor as a cause of autoimmune hypoglycemia in systemic lupus erythematosus.A 52-year-old black woman presented with clinical features of systemic lupus erythematosus (SLE) and severe fasting hypoglycemia. hypoglycemia was secondary to autoantibodies to the insulin receptor that were detected in the patient's serum. There were no anti-insulin antibodies, and other causes of hypoglycemia were excluded. Treatment with high-dose glucocorticoids resulted in restoration of euglycemia associated with resolution of circulating anti-receptor antibodies and parallel improvement in clinical and laboratory features of SLE. This case is compared with other cases of autoimmune hypoglycemia due to anti-receptor antibodies.- - - - - - - - - - ranking = 1keywords = black (Clic here for more details about this article) |
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