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Atypical Adult-onset Still’s disease with flagellate morphology in a patient with skin of color

Summary

A 28-year-old African American woman was diagnosed with Adult-onset Still’s disease, a rare inflammatory condition causing fever, joint pain, and skin rashes. Her rash had an unusual linear, stripe-like pattern with dark pigmentation that doesn’t typically appear in standard descriptions of this disease. She was successfully treated with anakinra and later tocilizumab, medications that reduce inflammation, leading to complete symptom resolution and sustained improvement over nine months.

Background

Adult-onset Still’s disease (AOSD) is an autoinflammatory disorder presenting with arthralgias, dermatitis, organomegaly, fever, and lymphadenopathy. Although nearly 30% of AOSD cases occur in ethnic minorities, there is limited published documentation of AOSD presentation in patients with skin of color, particularly with atypical cutaneous manifestations.

Objective

To describe a case of atypical AOSD with flagellate morphology and hyperpigmented plaques in a 28-year-old African American woman, highlighting the importance of recognizing atypical presentations and inflammatory features in patients with skin of color.

Results

Patient presented with polyarthralgia, fever, and pruritic hyperpigmented linear and flagellate plaques with symmetric distribution. Lab findings showed leukocytosis with eosinophilia, elevated inflammatory markers (ESR > 130 mm/hr, CRP 23.7 mg/L), and hyperferritinemia (13,660 mcg/L). Skin biopsy confirmed atypical AOSD with dyskeratotic keratinocytes, acanthosis, hyperkeratosis, and eosinophil-rich dermal infiltrate.

Conclusion

Atypical AOSD can present with persistent flagellate morphology and hyperpigmentation in patients with skin of color. Recognition of inflammatory features in darker skin types requires optimized examination techniques. Treatment with IL-1 inhibitors (anakinra) followed by IL-6 inhibitors (tocilizumab) resulted in symptom resolution and sustained remission.
  • Published in:JAAD Case Reports,
  • Study Type:Case Report,
  • Source: PMID: 37492429, DOI: 10.1016/j.jdcr.2023.04.026
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