Aline Defaveri do Prado

Scleromyxedema with monoclonal gammopathy and neurological involvement: recovery from coma after plasmapheresis?

565–573. 3 Grant A, Gonzalez T, Montgomery MO, et al. Results of a double-blinded, placebo-controlled cross-over study evaluating longterm efficacy and safety (60 weeks) in patients with moderate to severe hidradenitis suppurativa treated with infliximab. Abstract 2213. American Academy of Dermatology Annual Meeting, March 6–10, 2009, San Francisco, CA. 4 Sotiriou E, Apalla Z, Vakirlis E, Ioannides D. Efficacy of adalimumab in recalcitrant hidradenitis suppurativa. Eur J Dermatol 2009; 19: 180–181. 5 Blanco R, Martinez-Taboada VM, Villa I, et al. Longterm successful adalimumab therapy in severe hidradenitis suppurativa. Arch Dermatol 2009; 145: 580–584. 6 Jurgensmeyer JC, Fleischer A. Clinical improvement of refractory hidradenitis suppurativa with etanercept. Poster 58. J Am Dermatol 2004; 50(Suppl 3): 15. 7 Henderson RL Jr. Treatment of atypical hidradenitis suppurativa with the tumor necrosis factor receptor-Fc fusions protein etanercept. J Drugs Dermatol 2006; 5: 1767–1770. 8 Sotiriou E, Apalla Z, Ioannidos D. Etanercept for the treatment of hidradenitis suppurativa. Acta Derm Venereol 2009; 89: 82–83.

Ultrasound and its clinical use in rheumatoid arthritis: where do we stand?

High-resolution musculoskeletal ultrasound (MSUS) has been increasingly employed in daily rheumatological practice and in clinical research. In rheumatoid arthritis (RA), MSUS can be now considered a complement to physical examination. This method evaluates synovitis through gray-scale and power Doppler and it is also able to identify bone erosions. The utilization of MSUS as a marker of RA activity has received attention in recent literature. Current data account for good correlation of MSUS with classical measures of clinical activity; in some instances, MSUS appears to perform even better. Diagnosis of subclinical synovitis by MSUS might help the physician in RA management. With some variation, interobserver MSUS agreement seems excellent for erosion and good for synovitis. However, lack of MSUS score standardization is still an unmet need. In this review, we describe several MSUS scores, as well as their correlation with clinical RA activity and response to therapy. Finally, we look at the relationship of MSUS with synovial tissue inflammation and discuss future perspectives for a better interpretation and integration of this imaging method into clinical practice.

Episcleris, arthrofasciitis and hypereosinophilia: primary hypereosinophilic syndrome or atypical Wegener’s granulomatosis?

Sir, The diVerential diagnosis of hypereosinophilic states can be complex. It includes a variety of conditions such as allergic diseases, infections, leukemias, eosinophilic fasciitis (EF), vasculitis, eosinophilia-myalgia syndrome, and the idiopathic hypereosinophilic syndrome (IHES) [1]. We herein describe a case of a patient with systemic disease whose diagnosis is a matter of debate. The patient, a 37-year-old Caucasian male, bank employee, had been complaining of cyclic arthralgias for the last 9 months. Severe episcleritis of the left eye was diagnosed 6 months before admission, with partial response to oral corticotherapy. Episcleritis had recently recurred. The patient was admitted to Hospital with arthritis of the second proximal interphalangeal joint of the right hand, right olecraneous bursitis, arthritis of the right wrist, arthrofasciitis of the left elbow, and fasciitis of the right thigh. Blood cell count revealed marked eosinophilia (from 3,000 up to 11,000 eosinophils) and erythrocyte sedimentation rate of 12 mm in the Wrst hour. Liver and renal function tests were normal. Testing for human immunodeWciency virus, as well as for B and C hepatitis virus, were negative. Antinuclear antibodies were absent and the rheumatoid factor (RF) test was weakly positive (34 UI/ml). The antineutrophil cytoplasmic antibodies (ANCA) test was positive (C-ANCA, titer 1/40). The chest X-ray and ecochardiogram were unremarkable. A fasciomuscular biopsy of the right thigh demonstrated an eosinophilic and plasmocytic inWltrate in fascia and muscle; vasculitis and an incomplete granuloma formation were also seen in muscle. The patient showed an excellent response to a combined intravenous “pulse” of cyclophosphamide/metilprednilone (1 gram each). The current therapy includes mycophenylate mofetil 2 g daily and prednisone 10 mg daily for control of a persistent episcleritis. At a Wrst sight, the patient might have EF [2]. Yet we found no description of ocular disease and C-ANCA in this circumstance. Of note, EF, myositis and arthritis were early manifestations of a T-cell lymphoma according to a recent report [3]. Our patient could potentially have IHES [4, 5] (hypereosinophilia, fasciomuscular and articular disease, eosinophilic inWltrate in fascia/muscle). Characteristic features of IHES, such as pulmonary and endomyocardial disease [4, 5] were lacking. Episcleritis, present in our patient, has been previously reported in IHES [6]. As regards to ANCA, a Japanese report dated from 1999 accounted for the presence of P, but not C-ANCA, in a man with IHES manifested by liver, lung and skin eosinophilic inWltration [7]. Thus, an incomplete subset of IHES might be a possible diagnosis for our patient. An atypical (by sparing kidney and lung) form of Wegener granulomatosis (WG) [8] is to be strongly considered in this case. A recognized characteristic of Churg– Strauss syndrome [9], hypereosinophilia is only eventually found in WG [10]. The musculoskeletal Wndings (fasciitis excluded) and episcleritis seen in our patient can be part of A. D. do Prado · H. L. Staub (&) Rheumatology Department, Faculty of Medicine of PUCRS, São Lucas Hospital, Av. Ipiranga 6690/220, 90610-000 Porto Alegre, Brazil e-mail: reumato@pucrs.br