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.
