Background/Purpose: There is no data about adult-onset Still’s disease (AOSD) skin eruption in individuals of sub-Saharan ancestry, from basic description to potential clinical implications.

Methods: We conducted a population-based retrospective study in tertiary center Martinique between 2004 and 2022. Patients were included if they were 16 years of age or older, of sub-Saharan ancestry origin and followed up for AOSD. Typical eruption was defined as salmon-pink, non-pruritic, evanescent macules and/or papules. Atypical eruption was defined as any skin symptomatology not meeting the definition of typical eruption, with the exception of the color criterion.

Systemic pattern of AOSD was defined as monocyclic with a unique flare followed by remission or polycyclic with first flare followed by one or more relapses, whereas the chronic articular pattern of AOSD was defined as a disease with predominant joint involvement. Localisation, time of appearance, skin histopathology, clinical and biological data, complications and treatment were collected and compared between groups.

Results: Thirty-three patients were identified and 29 patients (76% women) with a median age of 35.6 years [27.3; 59.4] had an eruption and were included.

21 (72.4%) patients had an atypical eruption with the following characteristics: persistent (n=8; 38%), pruritic (n=17; 80.9%), urticarial (n=8; 38%), eczematous (n=1; 4.8%), plaques (n=8; 38%), patches (n=6; 28.6%), bullae (n=1; 4.8%), and erythroderma, erosion, scales, purpura and necrosis (n=2; 9.5% each). 

Typical and atypical eruptions were mainly located on the chest (43% and 58%, respectively) and lower limbs (43% and 53%, respectively). Histopathology was not specific with oedema and inflammatory infiltrate of lymphocytic cells or neutrophils.

Patients with an atypical rash had predominantly a systemic polycyclic pattern (47.6%), and patients with a typical rash only predominantly had a monocyclic pattern (75%) (p=0.08). Ten (47.6%) and 6 (28.6%) patients with atypical skin eruption and 6 (75%) and 1 (12.5%) patients with typical skin eruption developed, pulmonary and cardiac involvement, respectively, with no statistical difference.

However, patient with atypical rash significantly had higher SGOT (p=0.0143) and SGPT (p=0.003) and received more methotrexate (p=0.007) and anti-IL1 therapy (p=0.038) than those with typical eruption. Finally, 3 (14.3%) macrophage activating syndrome and 2 deaths (9.5%) occurred, exclusively in patients with atypical skin eruption.

Conclusion: Atypical skin eruption is predominant in our Caribbean population of sub-Saharan ancestry with Still’s disease. It might be associated with a more severe systemic phenotype and worse outcomes but more data are needed.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/characteristics-of-adult-onset-stills-disease-skin-eruption-in-individuals-of-sub-saharan-ancestry/