An Atypical Skin Presentation of Adult-Onset Still's Disease With Histological Description: A Case Report.
M Nordmann, T Mazza, S Menzinger et al.
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Case in brief
A 30-year-old man presented with high spiking fever, odynophagia, diffuse arthralgia, and generalized asthenia, alongside a slightly pruritic erythematous maculopapular and urticarial rash that spread from his face and limbs to involve the whole body. The lower-limb lesions had purpuric features, and nonfollicular pustules overlaid the rash. Skin biopsy showed subcorneal neutrophil collections, mild epidermal spongiosis, numerous suprabasal necrotic keratinocytes, and a moderate mixed superficial dermal infiltrate. After investigations excluded other diagnoses, he was diagnosed with adult-onset Still's disease and had a good initial response to corticosteroids.
What made this case unusual
The atypical eruption combined purpuric features and overlying nonfollicular pustules; biopsy also showed numerous suprabasal necrotic keratinocytes.
Diagnostic / clinical pearl
Adult-onset Still's disease can present with skin findings beyond the typical evanescent salmon-pink rash. In this case, the atypical eruption required histological assessment and investigation to exclude other diagnoses.