Premier auteur : Angèle Soria
Revue: JEADV
Lien vers L’article: https://doi.org/10.1111/jdv.18771
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.