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

Purpura fulminans (PF) is a life-threatening disorder characterized by acute onset of progressive cutaneous hemorrhage, necrosis, and disseminated intravascular coagulation. Acute infectious PF occurs most commonly in the setting of meningococcal sepsis. When PF occurs in the setting of systemic lupus erythematosus (SLE), the catastrophic antiphospholipid antibody syndrome (CAPS) must be ruled out because urgent therapy is required. Plasmapheresis is effective in both cases, but immunosuppression (high-dose corticosteroids plus cyclophosphamide), although beneficial in patients with CAPS, could be harmful in patients with meningococcal PF. The authors report here a patient with SLE who presented to the intensive care unit with meningococcal PF, acute renal failure, and acute respiratory distress syndrome and discuss clinical similarities and laboratory differences from CAPS.
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PMID:Meningococcal purpura fulminans in a patient with systemic lupus erythematosus: a mimic for catastrophic antiphospholipid antibody syndrome? 1520 56

We report the case of a 25-year-old female patient who presented with purpura fulminans as a manifestation of primary antiphospholipid syndrome. Purpura fulminans is considered a rare cutaneous manifestation of antiphospholipid syndrome. Most frequently, it occurs in the context of catastrophic antiphospholipid syndrome and is associated with significant morbidity and mortality, either due to loss of affected extremities or thromboembolic damage to internal organs. After insufficient efficacy of parenteral anticoagulation and oral glucocorticosteroid treatment, we escalated treatment to high-dose intravenous glucocorticosteroid and five consecutive sessions of plasma exchange with good and sustained clinical response. At follow-up six months after admission, skin manifestations had healed with scarring, and no additional thrombotic events had occurred. Plasma exchange may hold promise as a therapeutic option in refractory or severe cases of antiphospholipid syndrome-related purpura fulminans with extensive cutaneous necrosis, although evidence is limited.
Lupus 2018 Nov
PMID:Therapeutic response to glucocorticoids, anticoagulation and plasma exchange in a patient with primary antiphospholipid syndrome presenting with purpura fulminans. 3029 Jul 9

Although the majority of neonatal skin rashes can be safely monitored without intervention, there are a significant few that are dermatologic emergencies. When called to assess a neonate, it is important to distinguish what requires immediate diagnosis and treatment from those that represent benign etiologies. The skin may be the first clue to certain infections such as herpes simplex virus, syphilis, varicella, cytomegalovirus, fungal infections, and staphylococcal scalded skin syndrome, all of which require immediate testing and some of which may lead to severe sequelae. Cutaneous findings in neonates may also indicate the need for further evaluation. Purpura fulminans, sclerema neonatorum, neonatal lupus, and blueberry muffin rash can be indications of other underlying disorders and are reviewed as well. This article outlines these potential neonatal dermatologic emergencies and highlights the important clinical clues to each. [Pediatr Ann. 2019;48(1):e36-e42.].
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PMID:Neonatal Skin Emergencies. 3065 41