Purpura Fulminans From Meningococcemia Mimicking Stevens-Johnson Syndrome in an Adult Patient Taking Etanercept

Gunawardane, Nilanthi D.; Menon, Kavita; Guitart, Joan; Cotliar, Jonathan

doi:10.1001/2013.jamadermatol.31

Cited by 9 publications

(5 citation statements)

References 5 publications

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“…Systemic corticosteroids (22), intravenous immunoglobulin (IVIG) (23), and cyclosporine (24) are often used to treat CTL-mediated SCARs; however, the clinical outcomes of these therapies remain controversial (25,26). Recently, several studies revealed that treatment with anti-TNF-α biologic agents appeared beneficial in patients with TEN and TEN-like acute cutaneous lupus erythematosus (17,(27)(28)(29)(30)(31)(32)(33)(34)(35)(36). However, all of these studies were based on case reports and lacked a randomized, systematic trial design compared with traditional therapy studies.…”

Section: Preclinical Testing Of Etanercept: Ex Vivo Testing For Potenmentioning

confidence: 99%

Randomized, controlled trial of TNF-α antagonist in CTL-mediated severe cutaneous adverse reactions

Wang¹,

Yang²,

Chen³

et al. 2018

Journal of Clinical Investigation

204

156

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Section: Preclinical Testing Of Etanercept: Ex Vivo Testing For Potenmentioning

confidence: 99%

Randomized, controlled trial of TNF-α antagonist in CTL-mediated severe cutaneous adverse reactions

Wang¹,

Yang²,

Chen³

et al. 2018

Journal of Clinical Investigation

204

156

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“…The underlying thrombosis in PF can lead to cutaneous hemorrhage, together with a broad epidermolysis this can mimic clinical features of TEN. A case report from Cotliar et al 10 (2012) describes a meningococcal PF resembling SJS features, and this case describes the challenge of differentiating SJS/TEN from a patient with PF presenting with bullae. The histopathological analysis can be helpful, and an early clinical recognition could help prevent the progression.…”

Section: Discussionmentioning

confidence: 92%

Purpura fulminans, TEN, and disseminated herpes simplex: An unexpected combination

Holm

Thurfjell

Lara-Valencia

et al. 2022

Clinical Case Reports

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Stevens–Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), and purpura fulminans (PF) are all rare conditions. A combination of these 3 conditions together with a viral infection is very rare. A 52‐year‐old, previously healthy woman which developed SJS, potentially due to a reaction to CT contrast, although this is still unknown. This developed into TEN on day 10 of the initial admission, the patient scored 3 points on SCORTEN. On day 12 from initial admission, she developed unexpected multiorgan failure and PF. The patient passed away 2 days later, the autopsy demonstrates herpes simplex virus in the bladder and lungs on immunohistological staining. Our clinical case encountered the challenge of differentiating TEN and PF. The microscopic and immunochemical examination confirmed the clinical suspicion of PF but also a disseminated herpes simplex infection. We speculate the clinical route of this case started SJS and TEN, leading to superimposed infection with three different types of bacteria, confirmed in blood cultures, and a disseminated viral infection. The combination of all these diagnoses are very rare, no similar case has been described in adults to the authors’ knowledge. We recommend a prompt diagnosis and early recognition of both bacterial and viral infections to prevent the development of PF.

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“…[14][15][16] In addition, PF may mimic Stevens-Johnson syndrome/toxic epidermal necrolysis. 17,18 In most situations, these differentials may be distinguished from acute PF through clinical and laboratory studies, in which biopsy remains an indispensable diagnostic tool. Histopathologically, dermal vascular thrombosis with epidermal necrosis as in PF may also be seen with Warfarininduced skin necrosis and rarely in thrombotic thrombocytopenic purpura and cryoglobulinemia.…”

Section: Resultsmentioning

confidence: 99%

“…The major differential diagnoses to various stages of acute PF include, among others, immune or thrombotic thrombocytopenic purpura, Warfarin‐induced skin necrosis, cocaine/levamisole toxicity, cutaneous vasculitis, and toxic shock syndrome 14‐16 . In addition, PF may mimic Stevens‐Johnson syndrome/toxic epidermal necrolysis 17,18 . In most situations, these differentials may be distinguished from acute PF through clinical and laboratory studies, in which biopsy remains an indispensable diagnostic tool.…”

Section: Discussionmentioning

confidence: 99%

Acute purpura fulminans—a rare cause of skin necrosis: A single‐institution clinicopathological experience

et al. 2020

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Background: Purpura fulminans, an uncommon syndrome of intravascular thrombosis with hemorrhagic infarction of the skin, is often accompanied by disseminated intravascular coagulation (DIC) and multi-organ failure, and may ultimately lead to death. Methods: Herein, we document 13 skin biopsies from 11 adult patients with the clinical diagnosis of sepsis and confirmed histopathologic diagnosis of intravascular thrombosis and/or DIC, compatible with acute infectious purpura fulminans (AIPF). Detailed history and clinical examination were performed, and the lesions were correlated with histopathologic findings. Any underlying medical disease was taken into consideration. Results: There were 5 males and 6 females with lower extremity or peri-incisional purpuric skin lesions. The most important comorbidities identified were a history of surgical procedure or neoplasm, although 4 patients had no relevant underlying history. Most skin biopsies revealed focal epidermal ischemia or necrosis and 3 showed full-thickness epidermal necrosis. In the underlying dermis, there were fibrin thrombi in superficial and deep blood vessels with acute inflammation. Changes of an inflammatory destructive vasculitis were identified in 5 cases. No bacteria or fungi were identified on histopathology. Conclusions: AIPF is a rapidly-progressing medical emergency which may be identified by histopathology in culture-negative cases. Biopsies may show neutrophilic infiltrate without infective organisms.

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Purpura Fulminans From Meningococcemia Mimicking Stevens-Johnson Syndrome in an Adult Patient Taking Etanercept

Cited by 9 publications

References 5 publications

Randomized, controlled trial of TNF-α antagonist in CTL-mediated severe cutaneous adverse reactions

Randomized, controlled trial of TNF-α antagonist in CTL-mediated severe cutaneous adverse reactions

Purpura fulminans, TEN, and disseminated herpes simplex: An unexpected combination

Acute purpura fulminans—a rare cause of skin necrosis: A single‐institution clinicopathological experience

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