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Adult-Onset Still’s Disease: A Classic Presentation of a Rare Illness

On admission, skin examination revealed a diffuse maculopapular rash involving the back (A) and bilateral lower extremities (B).

Adult-onset Still’s disease is a rare inflammatory disorder of unclear etiology. Here, we highlight a classic presentation of this disorder.

Case Description

A 30-year-old woman presented with a 1-month history of daily fevers, myalgias, 7.5-kg unintentional weight loss, and a 2-week history of diffuse skin rash. Her medical history was notable for Hashimoto’s thyroiditis, and she was on levothyroxine replacement. She had traveled to Turkey and Colorado within the preceding 2 months. On admission, she was febrile to 38.9°C. Physical examination revealed a 2/6 systolic ejection murmur loudest at the apex, right upper quadrant tenderness to deep palpation without peritoneal signs, and a scattered maculopapular rash on all extremities (Figure, A), back (Figure, B), and abdomen.

Initial laboratory testing (reference range in parentheses) showed hemoglobin 9 g/ dL (12-15.5), leukocytes 14.9 × 109/L (3.5-10.5) with neutrophilic predominance, alkaline phosphatase 110 U/ L (37-98), aspartate transaminase 91 U/ L (8-43), and alanine transaminase 84 U/ L (7-45). A chest radiograph revealed patchy opacification in the left lower lobe. Abdominal ultrasound illustrated mild hepatosplenomegaly and a mildly thickened gallbladder wall with a negative Murphy’s sign. Transthoracic echocardiogram demonstrated normal left ventricular size, function, and wall motion. She was treated with a 5-day course of ceftriaxone and azithromycin for presumed community-acquired pneumonia with no change in symptoms.

During the first 4 days of hospitalization, she had daily fevers to 39.3°C. Additional laboratory testing revealed iron 33 mcg/ dL (35-145), total iron binding capacity 125 mcg/ dL (250-400), and ferritin 11,933 mcg/ L (11-307). An exhaustive infectious workup that included testing for Legionella, Mycoplasma, syphilis, human immunodeficiency virus, tuberculosis, blastomycosis, histoplasmosis, parvovirus B19, Coxiella burnetii (Q fever), and Leishmaniainfection was unremarkable. Computed tomography of the chest, abdomen, and pelvis did not demonstrate evidence of adenopathy or malignancy. Punch biopsies of the rash from the right forearm and thigh showed mild mixed perivascular inflammation, including a few eosinophils. A bone marrow biopsy revealed a normocellular marrow with normal trilineage hematopoiesis and without an increase in phagocytic histiocytes. A diagnosis of adult-onset Still’s disease was made. The patient was treated with prednisone 60 mg daily followed by a prolonged taper. The fevers, myalgias, abdominal pain, and rash resolved completely after 2 days of steroid therapy.

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-Amrit K. Kamboj, MD, Samantha M. Ryan, MD, Mark J. Enzler, MD

This article originally appeared in the October issue of The American Journal of Medicine.

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