Definition
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This is an acquired autoinflammatory syndrome in adults, meaning it is not inherited from parents. Patients experience episodes that are:
• Systemic (with fever);
• Inflammatory (elevated CRP in the blood);
• Recurrent;
• Acute;
• erythematous macular (red) rash predominantly on the trunk (chest, back) and the root of the limbs.
The acronym "SITRAME" has been proposed in English and stands for:
S Systemic
I Inflammatory
T Truncular
R Recurrent
A Acute
M Macular
E Eruption
Epidemiology
The disease has so far been described in adult subjects of Caucasian origin The
average age of onset of attacks is between 25 and 50 years old.
The cases described are sporadic the patients are the only ones affected in their
families To date, there are approximately 50 identified cases in France.
Genetic
To date, no mutation in a gene already implicated in an autoinflammatory disease has
been identified by next-generation sequencing (panel) in the blood of patients.
Clinical
Patients present with recurrent acute episodes of fever followed by a stereotypical erythematous (red) macular (flat, without relief) rash that does not itch, always located in the same areas of the trunk and sometimes the root of the limbs, with the vast majority of cases sparing the periumbilical area.
Inflammation is found in the blood (elevated C-reactive protein) during flare-ups, and patients often experience severe fatigue after an episode.
The rash lasts an average of three days and is accompanied by fever in more than half of cases.
Other symptoms: headaches, abdominal pain, muscle pain, sore throat, and flu-like symptoms may be associated with the rash.
Diagnostic
The diagnosis of SITRAME is clinical, based on combination of major and minor criteria Major criteria (mandatory):
1. Systemic inflammation: at least one documented episode of CRP > 5 mg/ml during a skin flare up
2. Non pruritic macular rash of the trunk
3. Recurrence: at least 3 different episodes
4.Acute: duration of attacks less than 8 days
At least one minor criterion among:
1. Fever during flare
2. Flare ups triggered by infections, vaccinations or intense physical exercise
3. Asthenia during and/or after flare
4.Associated papular rash
It remains a diagnosis of exclusion : other causes such as infection, drug allergy, or known dermatologic or autoinflammatory conditions must be ruled out.
Episodes may be triggered by medications, infections (viral or bacterial), or occasionally vaccination
Referral to an expert center (Tenon Hospital) is recommended.
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.
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Publications on the disease
Acute recurrent macular eruption of the systemic inflammatory trunk (SITRAME): A new autoinflammatory syndrome in adults?
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/
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.
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.