Adult-Onset Still’s Disease with an Atypical Cutaneous Manifestation

Main Article Content

Shae Margulies
Nicole Nagrani
Paul Rodriguez-Waitkus
Lilia Correa-Selm

Keywords

Adult Onset Still's Disease, flagellate rash, Yamaguchi Criteria

Abstract

Adult-onset Still’s disease (AOSD) is a rare systemic inflammatory disorder, characterized by daily fevers, arthralgias, and rash without evidence of infection. Recently there has been a growing number of cases reported with atypical cutaneous manifestations. We present a 37-year-old type VI Fitzpatrick skin type female who was seen in the hospital for a pruritic rash on her chest, back, and legs. Skin examination was significant for hyperpigmented papules coalescing into plaques on the chest, and hyperpigmented linear patches and excoriated plaques on the back and legs. Punch biopsy showed acanthosis and numerous apoptotic keratinocytes in the upper layers of the epidermis, which was characteristic of AOSD. As of recently, there is a growing body of evidence describing an atypical eruption of persistent, pruritic papules and plaques with typical histologic findings of AOSD. Further, these atypical features may be associated with more severe symptoms, higher incidence of macrophage activation syndrome, and possible delayed malignancy. Due to the scarcity of atypical cutaneous manifestations reported in the literature, these eruptions have not been included in the diagnostic criteria and can therefore be overlooked and misdiagnosed. Prompt recognition of these atypical eruptions is imperative to prevent a worsening prognosis and serious adverse effects.

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