Fever and Rash, Not always Viral Exanthem

Stacey Ulrich, Peter Chiraseveenuprapund
Department of Pediatrics, Ready Children’s Hospital, San Diego, USA

Address for Correspondence: Dr Stacey Ulrich, Department of Pediatrics, Ready Children’s Hospital, San Diego 3020 Children’s Way, MC 5075, San Diego, CA 92123. Email: slueker7@hotmail.com

Clinical Problem:
A 7 years old previously healthy female presented to the emergency department for facial and truncal rash, facial swelling, and fever to 102°F which started 2 weeks prior. Patient also reported sore throat, pain in her back, knee, abdomen, decreased appetite, and vomiting. Mother denies any new food, lotions, or medications. Patient lives in the United States but does travel to Tijuana, Mexico. She was seen at a clinic in Mexico during first week of symptoms and given 2 doses of dexamethasone injection intramuscular 48 hours apart and ceftriaxone injection intramuscular for 3 consecutive days for a presumed throat infection. No throat cultures were obtained. Throat pain improved after the medications but her rash worsened. There were no upper respiratory tract symptoms, chest pain, diarrhea, dysuria, eye or tongue redness, or swelling to extremities. On presentation to us, temperature was 37.3°C, heart rate 128 beats/minute, respiratory rate 22 breaths/minute, blood pressure 121/77mm Hg, oxygen 100% on room air. On examination, she had mild diffuse facial edema, hyperemic and edematous gums, posterior palate erythematous ulcers (Fig. 1A), generalized abdominal tenderness, diffuse paraspinal and midline lumbar tenderness and a confluent erythematous macular rash to face and chest (Fig. 1B, C). Investigations revealed negative rapid streptococcal antigen test, negative monospot test, normal C-reactive protein (CRP) 0.8mg/dl and ESR 6mm at end of 1 hour. Serum sodium was 132mmol/L, potassium 4.6mmol/L, chloride 98mmol/L, pCO2 22mmol/L, BUN 11mg/dL, creatinine 0.37mg/dL, glucose 106mg/dL, calcium 8.7mg/dL, alkaline phosphatase 282 IU/L, albumin 3.3g/dL, total protein 6.8g/dL, total bilirubin 1.1 mg/dL, AST 650 IU/L, and ALT 227 IU/L. Complete blood count revealed white blood cells (WBC) 9900 cells/cumm, hemoglobin 15.4 g/dL, hematocrit 46.2%, MCV 89.7fL, MCH 29.8pg, MCHC 33.3%, RDW 14.5 Unit, platelets 63,000 cells/cumm. Consultations from dermatology, infectious disease, ophthalmology, and rheumatology were obtained. Additional investigations revealed elevated creatine kinase (CK) 2011U/L, lactate dehydrogenase (LDH) 1567U/L, ferritin 605ng/mL (Normal 6-70ng/mL), and C3 complement 125mg/dL (Normal 70-124mg/dL). C4 was decreased at 14mg/dL (Normal 20-42mg/dL). Infectious work up was negative for serum enterovirus PCR, herpes simplex virus 1,2 PCR, hepatitis A, B, C and E Elisa and antistreptolysin O < 25IU/mL. Blood culture did not grow any organism. Mycoplasma IgM, Epstein-Barr virus VCA IgM, cytomegalovirus IgM, herpes simplex virus 6 IgM, and dengue IgM were also negative. Rheumatology work up was negative for ANA , p and c-antineutrophilic cytoplasmic antibody (ANCA), smith antibody, antiphospholipid IgG and IgM, ds DNA, but positive for ribonucleoprotein (RNP) antibody [5.8AI (Normal <1 AI0] and aldolase [28.4U/L (Normal 3.4-8.6U/L)]. She had progression of edema evolving into extremity and truncal edema associated with myalgias. She was started on intravenous (IV) methyl prednisolone (15mg every 6 hours) with improvement in her symptoms and was discharged home on oral prednisolone (15mg every 8 hours). On follow up with rheumatology, she subsequently re-developed weakness with myalgias, diffuse tissue edema, persistent hard palate ulcers, and a more classic malar type rash upon slow wean of steroids. RNP remained persistently elevated. Patient is followed by rheumatology with additional flares requiring higher dose steroids in addition to mycophenolate mofetil and hydroxychloroquine.

What is the likely diagnosis?