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Provisional diagnostic criteria for systemic inflammatory trunk recurrent acute macular eruption diagnosis (SITRAME)

Updated: Mar 5

First author: Angèle Soria

SITRAME

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.




Systemic inflammatory trunk recurrent acute macular eruption (SITRAME): A new auto-inflammatory syndrome in adult?

Premier auteur : Angèle Soria

Revue: JEADV

Lien vers L’article: https://doi.org/10.1111/jdv.18771


Systemic inflammatory trunk recurrent acute macular eruption (SITRAME)

Summary:


Undifferentiated systemic autoinflammatory disorders (SAIDs) are diseases related to innate immunity that do not correspond to any known autoinflammatory disease and lack a confirmed molecular diagnosis.


These syndromes are characterized by recurrent, generalized inflammation in the absence of infection or autoimmune disease, with most patients presenting skin manifestations as the primary clinical feature.


We report here a series of 16 adult patients (Table 1), equally distributed between males and females (50%), who exhibited recurrent, non-pruritic, stereotyped macular eruptions, sometimes papular, consistently affecting the trunk. Certain areas of the skin were spared, with sharp demarcation. These cutaneous manifestations were associated with systemic inflammation in 100% of cases.


The median age at diagnosis was 53 years (range: 28–68). All cases were sporadic and involved adults from diverse backgrounds. The first disease episodes occurred at a mean age of 35 years (range: 18–52), with no cases reported during childhood.


In conclusion, we describe a new clinical entity characterized by systemic inflammation and a recurrent, stereotyped macular eruption of the trunk, always occurring with the same topography. This clinical presentation meets the criteria for an undetermined SAID, and we propose the acronym SITRAME (Systemic Inflammatory Trunk Recurrent Acute Macular Eruption). Further studies are needed to determine its etiology and pathophysiology to optimize therapeutic management.




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