Groove sign

Abstract

•Eosinophilic fasciitis is a rare disorder characterized by induration of the trunk and extremities.•Groove sign is a characteristic finding of eosinophilic fasciitis.•Corticosteroids treatment is the standard therapy for eosinophilic fasciitis. A 72-year-old man with a history of arterial hypertension, diabetes mellitus and hypercholesterolemia presented to our outpatient clinic with a 6-month history of induration in both forearms and proximal muscles of both legs. He also referred weight loss, paresthesias and numbness in both hands, and he had been recently diagnosed with bilateral carpal tunnel syndrome. Physical examination revealed a marked induration of the skin affecting extremities and abdominal wall. Elevation of the arms revealed the presence of grooves in the inner forearms (Fig. 1, white arrows). Levels of eosinophils were increased (1980 cells/mcL). Inflammatory markers (C reactive protein and erythrocyte sedimentation rate) were also elevated. A full-thickness wedge biopsy of the skin was performed. The muscular fascia was thicker, showing accumulation of eosinophils, lymphocytes and plasma cells. A diagnosis was made, and the patient was started on intravenous steroids. What is the diagnosis? Eosinophilic fasciitis, also called diffuse fasciitis with eosinophilia or Shulman disease is a scleroderma-like syndrome first described in 1974. It is characterized in its early stage by erythema and edema of the trunk or limbs, and subsequently by collagenous thickening of the subcutaneous fascia. The etiology is unclear and the pathogenesis is poorly understood. Numerous factors (hematological, infectious, autoimmune, exercise, chemicals drugs, solid tumors and physical factors) have been proposed as potential triggers. The “orange peel” (peau d'orange) and groove sign are characteristic findings. Other common symptoms are weight loss, fatigue, morning stiffness and carpal tunnel syndrome. Peripheral blood eosinophilia, hypergammaglobulinemia and elevation of erythrocyte sedimentation rate and C-reactive protein are the main laboratory findings [[1]Pinal-Fernandez I. Selva-O'Callaghan A. Grau J.M. Diagnosis and classification of eosinophilic fasciitis.Autoimmun Rev. 2014; 13: 379-382Crossref PubMed Scopus (107) Google Scholar]. Magnetic resonance is the best imaging test for diagnosing eosinophilic fasciitis, showing a marked increase in signal intensity around the muscle fascia [[2]Desvignes-Engelbert A. Saulière N. Loeuille D. Blum A. Chary-Valckenaere I. From diagnosis to remission: place of MRI in eosinophilic fasciitis.Clin Rheumatol. 2010; 29: 1461-1464Crossref PubMed Scopus (22) Google Scholar]. Biopsy of affected skin usually shows characteristic findings, such as thickening of the fascia, which is adhered to skeletal muscle, and accumulation of lymphocytes, eosinophils or plasma cells. Corticosteroid treatment with methylprednisolone pulses remains the standard therapy for eosinophilic fasciitis. Immunosuppressive drugs, mainly methotrexate might be useful as a second line therapy [[3]Lebeaux D. Francès C. Barete S. Wechsler B. Dubourg O. Renoux J. et al.Eosinophilic fasciitis (Shulman disease): new insights into the therapeutic management from a series of 34 patients.Rheumatology (Oxford). 2012; 51: 557-561Crossref PubMed Scopus (103) Google Scholar]. In short, we present a typical case of eosinophilic fasciitis, a rare disorder. Groove sign is a characteristic finding that may be helpful in the diagnosis. The authors state that they have no conflicts of interest.