Purpura rétiforme et pemphigoïde bulleuse : une association fatale ?

Doffoël-Hantz, V.; Longueville, C.; Durox, H.; Bédane, C.; Sparsa, A.; Bonnetblanc, J.‐M.

doi:10.1016/j.revmed.2011.03.208

Cited by 2 publications

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“…In our search of subepidermal AIBD complicated by NF, patients with BP were searched and 6 case reports were found with a total of 9 patients, presented in Table 1 [9][10][11][12][13][14]. The mean age of this case series, including our patient, was 71.5 years, ranging from 51-86 years old.…”

Section: Discussionmentioning

confidence: 99%

Necrotizing fasciitis – a complication of autoimmune skin blistering diseases?

Jurišić

Nikolić²,

Zivanović

et al. 2023

J Infect Dev Ctries

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Introduction: Autoimmune bullous diseases (AIBD) are organ-specific skin blistering diseases clinically manifesting as bullae and vesicles of the skin and mucous membranes. The loss of skin barrier integrity renders patients susceptible to infection. Necrotizing fasciitis (NF), a rare yet severe infectious complication of AIBD has been insufficiently documented in the literature. Case report: We present a case of a 51-year-old male patient with NF initially misdiagnosed as herpes zoster. Given the local status, CT imaging, and laboratory parameters, NF diagnosis was made and the patient was taken for an urgent surgical debridement. In a further development, new bullae in remote areas erupted and a perilesional biopsy, direct immunofluorescence as well as local status, the patient's age, and atypical presentation, imposed an initial diagnosis of epidermolysis bullosa acquisita. Differential diagnoses were bullous pemphigoid (BP) and bullous systemic lupus. In the literature, 9 other described cases were found and are reviewed. Conclusions: Due to its unspecific clinical picture, necrotizing fasciitis itself presents a frequently misdiagnosed soft tissue infection. Altered laboratory parameters in immunosuppressed patients often lead to misdiagnosing of NF and loss of precious time, which plays a major role in survival. Given the manifestation of AIBD as loss of skin integrity and immunosuppressive therapy, these patients could be more predisposed to NF than the general population.

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Section: Discussionmentioning

confidence: 99%

Necrotizing fasciitis – a complication of autoimmune skin blistering diseases?

Jurišić

Nikolić²,

Zivanović

et al. 2023

J Infect Dev Ctries

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Necrotizing Fasciitis—Severe Complication of Bullous Pemphigoid: A Systematic Review, Risk Factors, and Treatment Challenges

Stojičić

Jurišić²,

Marinković³

et al. 2023

Medicina

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Background and objectives: Bullous pemphigoid (BP), the most common subepidermal autoimmune skin blistering disease (AIBD) has an estimated annual incidence of 2.4 to 42.8 new cases per million in different populations, designating it an orphan disease. Characterized by disruption of the skin barrier combined with therapy-induced immunosuppression, BP could pose a risk for skin and soft tissue infections (SSTI). Necrotizing fasciitis (NF) is a rare necrotizing skin and soft tissue infection, with a prevalence of 0.40 cases per 100,000 to 15.5 cases per 100,000 population, often associated with immunosuppression. Low incidences of NF and BP classify them both as rare diseases, possibly contributing to the false inability of making a significant correlation between the two. Here, we present a systematic review of the existing literature related to the ways these two diseases correlate. Materials and methods: This systematic review was conducted according to the PRISMA guidelines. The literature review was conducted using PubMed (MEDLINE), Google Scholar, and SCOPUS databases. The primary outcome was prevalence of NF in BP patients, while the secondary outcome was prevalence and mortality of SSTI in BP patients. Due to the scarcity of data, case reports were also included. Results: A total of 13 studies were included, six case reports of BP complicated by NF with six retrospective studies and one randomized multicenter trial of SSTIs in BP patients. Conclusions: Loss of skin integrity, immunosuppressive therapy, and comorbidities commonly related to BP patients are risk factors for necrotizing fasciitis. Evidence of their significant correlation is emerging, and further studies are deemed necessary for the development of BP-specific diagnostic and treatment protocols.

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Purpura rétiforme et pemphigoïde bulleuse : une association fatale ?

Cited by 2 publications

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Necrotizing fasciitis – a complication of autoimmune skin blistering diseases?

Necrotizing fasciitis – a complication of autoimmune skin blistering diseases?

Necrotizing Fasciitis—Severe Complication of Bullous Pemphigoid: A Systematic Review, Risk Factors, and Treatment Challenges

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