The American Society of Dermatopathology

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Interactive Case Study - February 2021

54-year-old man with arthralgia, weight loss, fever and rash

Tayler D. Parker, BA; John R. Griffin, MD

Question 1:

A 54-year-old man presents with a 2-year history of episodic, migratory arthralgia, fever, pruritic rash, 60-pound weight loss, headaches, and joint stiffness. Episodes consist of 2-3 days of joint pain that is accompanied by a pruritic rash lasting for about 24 hours.  Physical examination demonstrates numerous, scattered, 1-2 cm urticarial pink papules on the trunk and upper extremities. There is no evidence of swollen joints, but bilateral inguinal adenopathy is present. Laboratory evaluation is significant for an elevated CRP (81 mg/L, normal 0-4.9 mg/L) and ESR (73 mm/hr, normal 0-30mm/hr), a mild leukocytosis (12x10^3/uL, normal 3.4-10.8x10^3/uL), and an IgA gammopathy. Rheumatoid factor, ANA, and complement levels are within normal limits. A lymph node biopsy, bone marrow biopsy, and bone survey yielded normal results. Treatment with oral corticosteroids provided significant symptomatic relief for the joint pain and rash without complete resolution.

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Which of the following is the best diagnosis?