Definition
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Probable adult autoinflammatory syndrome.
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No gene known to date.
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This is a new clinical entity, with a recurrent macular eruption predominantly on the trunk, associated with systemic inflammation; thus, the acronym "SITRAME" stands for "Systemic Inflammatory Trunk Recurrent Acute Macular Eruption".
Epidemiology
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Disease described in Caucasians.
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The average age of onset of seizures is 35 years.
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Usually the cases described are sporadic.
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There are around forty cases identified in France in 2022.
Genetic
No specific mutations of known genes involved in innate immunity have been identified to date by conventional genetic analyses.
Clinical
These are patients presenting with a macular (flat, featureless) erythematous (red) recurrent non-pruritic (non-itchy) stereotyped eruption, always located on the same areas of the trunk, and sometimes the root of the limbs with in the vast majority cases a periumbilical respect. There is inflammation in the blood (raised C-reactive protein) during flare-ups and sometimes fever and great fatigue.
The duration of the eruption is on average 3 days with fever in more than half of the cases. Other symptoms: headache, abdominal pain, muscle pain, sore throat and a flu-like syndrome may be associated with the rash.
Diagnostic
Diagnosis is based on questioning, observation of the same type of recurrent lesions on the same areas evolving in flare-ups (on photos if necessary) and CRP assay in crisis. It is a diagnosis of elimination after having eliminated an infection, possibly a drug allergy or another known inflammatory dermatological disease.
Seizures can be triggered by concomitant medication and/or a viral or bacterial infection and even sometimes by certain vaccinations.
Evolution
The patients do not present any severe complication, no inflammatory amyloidosis, no excess mortality.
Treatment
It is not yet codified because the entity has just been described.
Colchicine can shorten the duration of symptoms and lengthen the interval between episodes in some patients.
Publications on the disease
Acute recurrent macular eruption of the systemic inflammatory trunk (SITRAME): A new autoinflammatory syndrome in adults?
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Angèle Soria, Emmanuelle Amsler, Bethsabée Garel, Philippe Moguelet, Nathalie Tieulié, Florence Cordoliani, Isabelle Guichard, Antoine Mahé, Gilles Grateau, Guilaine Boursier, Sophie Georgin-Lavialle
J Eur Acad Dermatol Venereol. 2022 Nov 25. doi: 10.1111/jdv.18771.
https://pubmed.ncbi.nlm.nih.gov/36426626/
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We have identified within the systemic autoinflammatory diseases unclassified to date an entity that we have called the SITRAME syndrome for "Systemic Inflammatory Trunk Recurrent Acute Macular Eruption". Indeed, we report 16 adult patients who presented with a recurrent nonpruritic macular eruption of the trunk and systemic inflammation. The median age at diagnosis was 55 years and affected both men and women. All had an elevation of C-reactive protein (CRP) and 56% fever during flare-ups. The disease began on average at 35 years of age. During the recurrences, the cutaneous involvement was superimposable in each patient. The median duration of the eruption was 3 days. Seven of the 16 patients reported more than 20 episodes, occurring over several decades, with no worsening of manifestations over time. The skin biopsy performed in thrust in 8 patients was not informative, showing non-specific results. No biological anomaly, apart from the elevation of CRP during flare-ups, was noted. Genetic exploration by next-generation sequencing of the autoinflammatory disease gene panel performed in 10 of the 16 patients failed to identify a specific mutation in the autoinflammatory disease genes already known in 2022. Colchicine administered continuously was effective in the 6 patients treated, with a reduction in the number and duration of relapses.
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Following this publication, we want to collect patients internationally and try to determine the cause of this syndrome and its treatment. You can contact us for an opinion: angele.soria@aphp.fr or sophie.georgin-lavialle@aphp or make an appointment on our site via our secretariat. Dedicated appointment slots are available for this syndrome.