Abstract

Eosinophilia-myalgia syndrome (EMS) is a rare multi-system disorder that affects muscles, skin and lungs. It was first described in 1989 when several patients developed symptoms after consumption of contaminated L-tryptophan. Eosinophilic fasciitis (EF) is a related condition where inflammation leads to debilitating thickening of the fascia. Here we present a case with overlapping feature of both EMS and EF with the exceptional histology of granulomatosis fasciitis. 44-year-old previously healthy male developed an asymptomatic macular eruption on his bilateral arms, axillae, and back that was followed by dyspnea and myalgias. Significant edema of the hands and feet prevented flexion leading to limited range of motion. Symptoms developed three weeks after starting a supplement composed primarily of Griffonia simplicifonia seed extract containing 99% 5-hydroxytryptophan. Initial workup was positive for eosinophilia (10.9%, 1.19 k/microL), aldolase 11.6 (ref 1.5 -8.1 U/L), and mild elevation in ACE (68 U/L). Negative work-up included ANA, ANCA, SPEP, UPEP, TSH, Thyroglobulin antibodies, quantiferon gold, and an unremarkable chest CT. Fascial biopsy revealed a non-caseating sarcoidal granulomatous infiltrate in the deep fascial planes. No epidermal, dermal, or superficial subcutaneous involvement was noted. The infiltrate was primarily histiocytic with few scattered lymphocytes and very rare eosinophils. Tissue cultures for mycobacterium and fungal organisms were negative. The patient was placed on 60mg prednisone for one month, then tapered to 40mg and placed on 20mg methotrexate weekly with improvement although significant induration and nodularity persisted. This case highlights the unique spectrum of EMS and related conditions, helping clinicians and dermatopathologist further elucidate these disorders.