Korean J Dermatol.  2016 May;54(4):288-292.

A Case of Atypical Adult-onset Still's Disease That Presented with Unique Histopathologic Findings

• Kim DJ
• Lee DY
• Lee SK
• Kim MS
• Lee UH
• Hahm JH

Abstract

Adult-onset Still's disease (AOSD) is an uncommon disorder characterized by fever, polyarthralgia, elevated white blood cell count, and maculopapular rash, the histologic features of which have not been well known. A 54-year-old woman presented with fever, arthritis, and persistent pruritic lichenified rash. These cutaneous lesions showed unique histological features such as dyskeratosis with a peculiar, distinctive distribution in the upper epidermis and cornified layers with hyperkeratosis. In addition, dermal mucin deposition was increased, with inflammation and minimal fibroblast proliferation. The patient was diagnosed with AOSD according to the diagnostic criteria of Yamaguchi. Herein, we present an atypical form of AOSD showing distinctive clinical and pathological manifestations.

Keyword

Adult-onset Still's disease; Dyskeratotic cell; Persistent pruritic papules and plaques

MeSH Terms

Arthralgia
Arthritis
Epidermis
Exanthema
Female
Fever
Fibroblasts
Humans
Inflammation
Leukocyte Count
Middle Aged
Mucins
Still's Disease, Adult-Onset*
Mucins