Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0024141 (systemic lupus erythematosus)
 44,322 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A review of the literature reveals that pyogenic gangrenosum (ecthyma gangrenosum) is fatal to most patients, especially infants. In this article, the authors review the differential diagnosis of facial necrosis, present two cases of infants who succumbed to the systemic manifestations of the disorder, and a third case of survival in an adult with concomitant systemic lupus erythematosus and pyogenic gangrenosum. Finally, a brief discussion of Pseudomonas gastroenteritis, Pseudomonas septicemia, and pseudomembranous enterocolitis is presented.
 ...
PMID:Infectious facial and nasal cutaneous necrosis: evaluation and diagnosis. 681 12

The use of ablative intravenous cyclophosphamide (50 mg/kg per day for 4 days) without stem cell rescue has been described in patients with refractory autoimmune diseases such as paraneoplastic pemphigus, systemic lupus erythematosus, and aplastic anemia. We describe a 33-year-old patient with pemphigus vulgaris recalcitrant to multiple therapies. The patient presented with numerous oral ulcerations, erosions, and hyperpigmented crusted plaques on his face, trunk, and arms. Findings of a skin biopsy and direct immunofluorescence were consistent with pemphigus vulgaris. The circulating pemphigus vulgaris autoantibodies were present at a titer of 1:640. The patient received immunoablative therapy (50 mg/kg of cyclophosphamide for a total of 4 days) and tolerated the regimen well. Complications such as thrombocytopenia and Pseudomonas septicemia were quickly treated. Four months after the 4-day therapy, his oral and skin lesions completely healed, and his pemphigus titers have decreased to zero. He is no longer receiving prednisone and no new lesions have developed. This provides further evidence that this regimen is relatively safe and provides a potential "cure" for refractory autoimmune diseases such as pemphigus vulgaris.
 ...
PMID:Immunoablative high-dose cyclophosphamide without stem cell rescue in a patient with pemphigus vulgaris. 1283 31

Thrombotic thrombocytopenic purpura is a rare but serious condition in childhood. It can be idiopathic or a complication of other diseases or drug therapy. We report on a 12-year-old Chinese girl who presented with fulminant systemic lupus erythematosus with progressive renal failure, pancytopenia, and cerebral dysfunction due to thrombotic thrombocytopenic purpura. The patient also had Pneumocystis carinii pneumonia, Pseudomonas septicaemia, and Herpes zoster infections as a result of immunosuppressive treatment. She responded to combined therapy with pulse methylprednisolone, cyclophosphamide, plasmapheresis, and intensive care support, and completely recovered renal and neurological function. A review of the English-language medical literature since 1968 identified 20 other paediatric cases of systemic lupus erythematosus and thrombotic thrombocytopenic purpura. Clinical features, treatment, and outcome of these cases are presented and discussed. Early recognition is important, and although plasmapheresis is not of proven benefit in severe cases of systemic lupus erythematosus, it is life-saving in lupus-related thrombotic thrombocytopenic purpura and must be instituted early to avoid a poor outcome.
 ...
PMID:Thrombotic thrombocytopenic purpura as a rare complication in childhood systemic lupus erythematosus: case report and literature review. 1453 May 32