Severe Sweet's Syndrome with Elevated Cutaneous Interleukin-1β after Azathioprine Exposure: Case Report and Review of the Literature

Imhof, Laurence; Meier, Barbara; Frei, Pascal; Kamarachev, Jivko; Rogler, Gerhard; Kolios, Antonios; Navarini, Alexander A.; Contassot, Emmanuel; French, Lars E.

doi:10.1159/000371879

Cited by 31 publications

(16 citation statements)

References 44 publications

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“…Systemic corticosteroid therapy remains the mainstay of treatment, but in cases of refractory and insufficient responses to conventional treatment, efficacy of IL‐1 inhibition has been reported, implying a significant contribution of IL‐1 to the pathogenesis of Sweet syndrome. A recent report of ours showing increased IL‐1β gene expression levels and immunostaining in the skin lesions of patients with Sweet syndrome further supports the involvement of IL‐1β in its pathogenesis …”

Section: Partially Interleukin‐1‐mediated Neutrophilic Dermatoses As supporting

confidence: 69%

“…A recent report of ours showing increased IL-1b gene expression levels and immunostaining in the skin lesions of patients with Sweet syndrome further supports the involvement of IL-1b in its pathogenesis. 79 Behc ßet disease Behc ßet disease (BD; OMIM #109650) is a chronic systemic inflammatory disorder characterized by recurrent oral aphthous ulcers, genital ulcers and uveitis. BD is histopathologically characterized by vasculitis accompanied by an inflammatory infiltrate of both T cells and neutrophils.…”

Section: Sweet Syndrome (Acute Febrile Neutrophilic Dermatosis)mentioning

confidence: 99%

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Are neutrophilic dermatoses autoinflammatory disorders?

et al. 2016

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Neutrophils constitute essential players in inflammatory responses and are the first line of defence against harmful stimuli. However, dysregulation of neutrophil homeostasis can result in excessive inflammation and subsequent tissue damage. Neutrophilic dermatoses are a spectrum of inflammatory disorders characterized by skin lesions resulting from a neutrophil-rich inflammatory infiltrate in the absence of infection. The exact molecular pathophysiology of neutrophilic dermatoses has long been poorly understood. Interestingly, neutrophil-rich cutaneous inflammation is also a cardinal feature of several autoinflammatory diseases with skin involvement, the latter being caused by aberrant innate immune responses. Overactivation of the innate immune system leading to increased production of interleukin-1 family members and 'sterile' neutrophil-rich cutaneous inflammation are features of both inherited autoinflammatory syndromes with skin involvement and an increasing number of neutrophilic dermatoses. Therefore, we propose that autoinflammation may be a cause of neutrophilic dermatoses.

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Section: Partially Interleukin‐1‐mediated Neutrophilic Dermatoses As supporting

confidence: 69%

Section: Sweet Syndrome (Acute Febrile Neutrophilic Dermatosis)mentioning

confidence: 99%

Are neutrophilic dermatoses autoinflammatory disorders?

et al. 2016

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“…SwS is frequently observed in patients with leukemia or connective tissue diseases. Overexpression of proinflammatory genes including IL-1β is reported in lesional skin of SwS patients (Marzano et al, 2014b; Imhof et al, 2015). …”

Section: Skin Diseases With Il-1 Involvementmentioning

confidence: 99%

Potential of IL-1, IL-18 and Inflammasome Inhibition for the Treatment of Inflammatory Skin Diseases

2017

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In 2002, intracellular protein complexes known as the inflammasomes were discovered and were shown to have a crucial role in the sensing of intracellular pathogen- and danger-associated molecular patterns (PAMPs and DAMPs). Activation of the inflammasomes results in the processing and subsequent secretion of the pro-inflammatory cytokines IL-1β and IL-18. Several autoinflammatory disorders such as cryopyrin-associated periodic syndromes and Familial Mediterranean Fever have been associated with mutations of genes encoding inflammasome components. Moreover, the importance of IL-1 has been reported for an increasing number of autoinflammatory skin diseases including but not limited to deficiency of IL-1 receptor antagonist, mevalonate kinase deficiency and PAPA syndrome. Recent findings have revealed that excessive IL-1 release induced by harmful stimuli likely contributes to the pathogenesis of common dermatological diseases such as acne vulgaris or seborrheic dermatitis. A key pathogenic feature of these diseases is IL-1β-induced neutrophil recruitment to the skin. IL-1β blockade may therefore represent a promising therapeutic approach. Several case reports and clinical trials have demonstrated the efficacy of IL-1 inhibition in the treatment of these skin disorders. Next to the recombinant IL-1 receptor antagonist (IL-1Ra) Anakinra and the soluble decoy Rilonacept, the anti-IL-1α monoclonal antibody MABp1 and anti-IL-1β Canakinumab but also Gevokizumab, LY2189102 and P2D7KK, offer valid alternatives to target IL-1. Although less thoroughly investigated, an involvement of IL-18 in the development of cutaneous inflammatory disorders is also suspected. The present review describes the role of IL-1 in diseases with skin involvement and gives an overview of the relevant studies discussing the therapeutic potential of modulating the secretion and activity of IL-1 and IL-18 in such diseases.

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“…Increasing evidence suggests that autoinflammation underlies these conditions. Indeed, IL‐1β is found in both conditions, and in pyoderma gangrenosum IL‐1β has even been shown to be a successful biological treatment target . Whether a genetic risk factor is at play, like in the classic autoinflammatory diseases CAPS (cryopyrin‐associated periodic syndrome), TRAPS (tumour necrosis factor receptor‐associated periodic syndrome) and others, remains to be demonstrated and is the focus of intense investigation in several laboratories.…”

mentioning

confidence: 99%