DITRA syndrome in a Vietnamese patient: efficacy of etanercept

Babic, Valérie; Moawad, S.; Bursztejn, Anne‐Claire; Schmutz, J.‐L.

doi:10.1684/ejd.2018.3219

Cited by 2 publications

(4 citation statements)

References 10 publications

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“…30 However, there have been some more recent reports of success in managing disease symptoms with various biologics targeted at specific elements of the autoinflammatory cascade. 5,[11][12][13][16][17][18][19][20]27,28,[30][31][32] Here, we review the cutaneous manifestations reported in DITRA to improve our understanding and management of this rare autoinflammatory disorder. In addition, we summarize the existing literature on the presentation, genetics and treatment of skin inflammation in individuals with DITRA.…”

Section: Backg Rou N Dmentioning

confidence: 99%

“…Since the initial description of DITRA, which included 16 patients from nine families, 2,7,[10][11][12] only occasional cases of the disease in various age groups have been reported. 1,8,[10][11][12][13][14][15][16][17][18][19][20][21][22][23][24][25][26][27][28][29] Unfortunately, treatment options for DITRA are limited, especially in children. 30 However, there have been some more recent reports of success in managing disease symptoms with various biologics targeted at specific elements of the autoinflammatory cascade.…”

Section: Backg Rou N Dmentioning

confidence: 99%

“…Unfortunately, treatment options for DITRA are limited, especially in children 30 . However, there have been some more recent reports of success in managing disease symptoms with various biologics targeted at specific elements of the autoinflammatory cascade 5,11–13,16–20,27,28,30–32 . Here, we review the cutaneous manifestations reported in DITRA to improve our understanding and management of this rare autoinflammatory disorder.…”

Section: Introductionmentioning

confidence: 99%

“…Since the initial description of DITRA, which included 16 patients from nine families, 2,7,10–12 only occasional cases of the disease in various age groups have been reported 1,8,10–29 . Unfortunately, treatment options for DITRA are limited, especially in children 30 .…”

Section: Introductionmentioning

confidence: 99%

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Cutaneous findings and treatments in deficiency of interleukin‐36 receptor antagonist (DITRA): A review of the literature

Okorie,

Nayudu,

Nambudiri

2023

Experimental Dermatology

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Deficiency of the interleukin‐36 receptor antagonist (DITRA) is a rare autoinflammatory disorder caused by mutations in the IL36RN gene. This mutation leads to a lack of functional interleukin‐36 receptor antagonists (IL‐36Ra), which results in an overactive immune system and chronic inflammation. Despite its rarity, numerous case series and individual reports in the literature emphasize the importance of recognizing and managing DITRA. Early identification of the cutaneous signs of DITRA is crucial for accurate diagnosis and timely administration of appropriate treatment. This review article provides a comprehensive overview of the current understanding of the cutaneous, non‐cutaneous and histopathological manifestations of DITRA, with a focus on reported treatments. The disease typically presents in early childhood, although the age of onset can vary. Patients with DITRA exhibit recurrent episodes of skin inflammation, often with a pustular or pustular psoriasis‐like appearance. Additionally, non‐cutaneous manifestations are common, with recurrent fevers and elevated acute‐phase reactants being the most prevalent. The exact prevalence of DITRA is unknown. Some cases of loss‐of‐function mutations in the IL36RN gene, considered a hallmark for diagnosis, have been identified in patients with familial generalized pustular psoriasis (GPP). Biological therapies with inhibition of IL‐12/23 and IL‐17 are promising treatment options; paediatric patients with DITRA have shown complete response with mild relapses. New and emerging biologic therapeutics targeting the IL‐36 pathway are also of interest in the management of this rare autoinflammatory disorder.

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