A 23-year-old Hispanic woman presented with a 1-month history of rash, arthritis, and fever. The patient was in excellent health until 1 month prior to presentation, when she noted the spontaneous onset of a pruritic rash that began on her face and then progressed to involve her trunk and extremities. Shortly afterward, she developed pain and swelling of both hands, particularly of her wrists and metacarpophalangeal (MCP) joints. One week prior to presentation, she noted a sore throat and diffuse body aches together with subjective fevers and chills. She denied drenching sweats during febrile episodes. On evaluation in an emergency department, she was afebrile and had a diffuse erythematous rash that spared her palms and soles and mucous membranes. Laboratory evaluation showed a normal complete blood cell count, normal chemistries, including liver tests, and a normal urinalysis. The erythrocyte sedimentation rate (ESR) was 35 mm/hour (normal value ≤20) and the C-reactive protein (CRP) level was 53 mg/liter (normal value <3.1). A rapid test on a pharyngeal swab for group A Streptococcus was negative, as were urine tests for Neisseria gonorrhoeae and Chlamydia trachomatis. Her chest radiograph revealed a calcified granuloma in the right mid-lung, but was otherwise normal. She was discharged with a diagnosis of “polyarthritis and rash” with a recommendation to take prednisone 40 mg orally daily and ibuprofen for 5 days.
Despite compliance with the prednisone and ibuprofen, her symptoms persisted without improvement. She continued to have fevers and noted that her rash worsened during her febrile episodes. She was admitted to this institution for further evaluation.
Past Medical History
Her past medical history was unremarkable. She had had 4 pregnancies (3 live births, 1 induced abortion). There was no history of spontaneous abortions or of thromboembolic disease. In addition to the prednisone and ibuprofen prescribed in the emergency department, she was taking medroxyprogesterone for contraception. She took no other medications, including over the counter medications.
Social and Family History
The patient was born in El Salvador and immigrated to the San Francisco Bay area at age 14 years. She denied recent travel. She was married and lived with her husband and 3 children ages 1–6 years. She worked as a cashier at a mall. She did not use tobacco, alcohol, or any other illicit drugs. Her family was well, and there was no family history of prolonged febrile illnesses, arthritis, autoimmune diseases, or malignancy.
Review of Systems
She experienced increased fatigue for several weeks. Her weight was stable. She had no oral or nasal ulcers, alopecia, photosensitivity, or symptoms of Raynaud's phenomenon. She had no shortness of breath, chest pain, or abdominal pain. She denied any changes in her urine.
At the time of admission, the patient appeared fatigued but nontoxic and in no acute distress. She was febrile with an oral temperature of 38.4°C. Her blood pressure was 112/59 mm Hg, her pulse was 60 per minute, her respiratory rate was 18 per minute, and her room air oxygen saturation was 100%. She had no alopecia or nasal or oral ulcers. There was shotty cervical lymphadenopathy and bilateral axillary lymphadenopathy, right greater than left, with mobile, tender lymph nodes up to 1 cm in size. Her lungs were clear to percussion and auscultation. She had a nonradiating 2/4 systolic murmur along the left sternal border. There was no pericardial rub. Her abdomen was soft and without organomegaly. She had no peripheral edema; her peripheral pulses were 2+ and symmetric. There was synovitis and tenderness of both wrists and of the MCP joints bilaterally. Her left elbow was tender to palpation and had an effusion. She had pain on active and passive range of motion of her shoulders, hips, and right ankle. There was a faint, blanching erythematous rash of the face (particularly malar areas and eyebrows) and anterior chest. Her extremities had erythematous papules and thin 5-mm plaques with linear excoriation. The rash demonstrated Koebnerization. She had normal nails and nail folds. Her neurologic examination was normal.
Initial Laboratory Evaluation
Results of the initial laboratory evaluation are shown in Table 1. Her blood smear showed anisocytosis of the red blood cells and a few atypical lymphocytes. Her urinalysis was unremarkable. Radiographs of her hands and feet were normal.
|Variable||Normal value||Inpatient result|
|White blood cell count, cells/mm3||4,000–11,000||11,600*|
|Differential cell count, %|
|Absolute lymphocyte count, cells/mm3||1,000–5,100||800*|
|Platelet count, cells/mm3||150–400||307|
|Mean corpuscular volume, femtoliters||80–100||81.4|
|International normalized ratio||≤1.2||1.2|
|Partial thromboplastin time, seconds||≤37.6||32.5|
|Urea nitrogen, mg/dl||6–20||10|
|Serum ferritin, ng/ml||10–291||5,998*|
|Creatine kinase, units/liter||26–140||54|
|Total bilirubin, mg/dl||0.1–1.2||0.4|
|Total protein, gm/dl||6.4–8.3||6.5|
|Alkaline phosphatase, units/liter||42–98||99*|
|Aspartate aminotransferase, units/liter||10–41||77*|
|Alanine aminotransferase, units/liter||7–35||94*|
|Glutamic oxaloacetic transaminase, units/liter||12–43||59*|
|Thyroid-stimulating hormone, μU/ml||0.37–4.42||0.67|
|Erythrocyte sedimentation rate, mm/hour||0–20||46*|
|C-reactive protein level, mg/liter||<3.1||136*|
|Antinuclear antibodies||<1:40||1:40, speckled*|
|Russell's viper venom time||29.6–43.6||46.7*|
|Anticardiolipin IgG, IgG phospholipid units||Negative||Negative|
|Anticardiolipin IgM, IgM phospholipid units||<20||20*|