- Angèle S. et al
- Oct 22, 2024
- 2 min read
Updated: Mar 5, 2025
First author: Angèle Soria
Link to article: https://pubmed.ncbi.nlm.nih.gov/36426626/
Summary:
The Systemic Inflammatory Trunk Recurrent Acute Macular Eruption (SITRAME) syndrome is a recently described sporadic autoinflammatory syndrome in adults. It is characterized by recurrent non-pruritic macular eruptions on the trunk, accompanied by at least one documented episode of systemic inflammation with elevated C-reactive protein (CRP) levels during flares. The eruptions have a fixed topography and appear rapidly with confluent exanthem. They always involve at least the trunk, sparing certain areas of the skin with clear demarcation. The median duration of the eruptions was 3 days (range: 2–7 days).
No specific genetic mutation has been associated with the SITRAME syndrome. The objective of this study was to define diagnostic criteria to improve its identification. Four major criteria were defined as being present in all the patients studied: systemic inflammation with CRP > 5 mg/mL during a skin flare, non-pruritic macular trunk eruption, recurrence with at least three different episodes, and acute flares lasting less than 8 days. All four major criteria are mandatory for the diagnosis of SITRAME. Four minor criteria were present in at least half of the patients. At least one minor criterion is required for the diagnosis. Additionally, exclusion criteria include another monogenic autoinflammatory disease, an evolving neoplasm, or an autoimmune disease.
Histopathological evaluation showed limited specificity, characterized by a markedly poor dermal infiltrate, mainly with lymphocytes and minimal spongiosis. This shows that skin biopsy is not a discriminating diagnostic criterion. When comparing the major criteria of SITRAME with those of other adult-onset autoinflammatory syndromes, such as Schnitzler's syndrome and adult-onset Still's disease, none of the CAPS or adult-onset Still's disease patients met the major criteria for SITRAME.
The diagnosis of SITRAME syndrome requires the presence of four mandatory major criteria, at least one minor criterion, and the exclusion criteria.
