We report the case of a 78-year-old Caucasian male examined in our dermatological unit for swelling of the right arm. He described an increase in right arm size, which had a slow and progressive growth over the last two years and a perception of skin hardening and difficulty in flexing and extending the joint. Moreover, he has been experiencing muscle pain during the previous eight months. The past medical history accounts for a non-Hodgkin B cell lymphoma marginal type treated ten years before with splenect

Baraldi C.

Bardazzi F.

Clarizio G.

Mussi M.

Pileri A.

Piraccini B. M.

Zengarini C.

English

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[page 94]                                                                 [Dermatology Reports 2024; 16:9807]                              Dermatology Reports 2023; volume 15:Dear Editor,We report the case of a 78-year-old Caucasian male examinedin our dermatological unit for swelling of the right arm. Hedescribed an increase in right arm size, which had a slow and pro-gressive growth over the last two years and a perception of skinhardening and difficulty in flexing and extending the joint.Moreover, he has been experiencing muscle pain during the previ-ous eight months.The past medical history accounts for a non-Hodgkin B celllymphoma marginal type treated ten years before with splenecto-my and cycles of rituximab, bendamustine, and six-year-oldhypertension managed with valsartan 10 mg tablet daily. The restof the medical history was unremarkable.During the physical examination, we observed a visible rightarm swelling, almost doubling in size the left one (58 cm circum-ference vs. 30 cm), erythema and palpable skin induration with atypical texture resembling an orange peel. However, no joint painwas triggered during the physical examination (Figure 1).Due to the typical clinical presentation, we suspectedeosinophilic fasciitis in the first instance (Shulman syndrome).1We performed a full-thickness biopsy deep down the fascia of thearm, which showed lymphocytic infiltrate, eosinophils and oede-ma in the dermis. Blood tests demonstrated moderate eosinophilia(1.65×109/L) and no other abnormalities, and the magnetic reso-nance image revealed an inflammatory process of the muscle fas-cia, confirming the diagnosis of unilateral eosinophilic fasciitis.1,It should be noted that it has not been considered any correlationwith the previous lymphoma history, given the elapsed time.We started glucocorticoid therapy with daily prednisone at thedose of 1 mg per kilogram.3,4 Still, achieving no results after twomonths of steroid therapy, we changed to the administration ofsubcutaneous methotrexate at a dosage of 15 mg per week, fol-lowed the day after with 2 mg of folic acid in tablets. At the endof three months of methotrexate therapy, the patient reported nomore muscle pain and a return into the normality range foreosinophil count, but arm swelling was unaffected.After performing an eco-color doppler of the arm vessels toexclude arterial affection at the end of the three months of the sys-temic therapy, we decided to add to methotrexate an elastic com-pression brace made by nonwoven-fabric with a pressure range of18-21 mmHg, which enveloped the limb from fingertips up to thearmpit to be used at least during daytime.1,5Two months later, we significantly reduced the circumference(34 cm vs. 30 cm of the left arm) and improved skin texture(Figure 2). In addition, the total mobility of the right arm wasrecovered.Eosinophilic fasciitis, also known as Shulman’s disease, is arare condition that initially can be misdiagnosed as lymphedemaor scleroderma manifested by a rapid onset of erythema, oedema,and induration of skin and soft tissues symmetrically, preferablyon the limbs, and occasionally on the trunk, associated withperipheral eosinophilia.1,3Muscle biopsy can be crucial during the diagnostical proce-dure, showing eosinophils located at the muscle-fascia interfacewith variable involvement of the underlying muscle, often associ-ated with typical peri-fascicular atrophy and inflammatory infil-trates in the epimysial region. Sometimes, when a skin sample isincluded, eosinophils are also seen in the dermis in variable num-bers.6 Moreover, it is known that the management of this diseasemust be timely; achieving an almost complete remission is possi-Correspondence: Giacomo Clarizio, Dermatology Unit, IRCCS ofAzienda Ospedaliero-Universitaria di Bologna, via Massarenti 9,40138, Bologna, Italy. Tel.: +39.0512144848.E-mail: giacomo.clarizio@studio.unibo.itKey words: Shulman; fasciitis; eosinophilia; oedema; brace.Contributions: AP, conceptualization, CZ, data curation, formalanalysis, software; BMP, funding acquisition; FB, AP, investigation;GC, MM, AP, methodology; FB, CB, BMP, AP, supervision, valida-tion, writing – original draft; CZ, GC, writing - review & editing. Allthe authors approved the final version to be published.Conflict of interest: the authors declare no potential conflict of interest.Data availability statement: data available on request from theauthors.Consent for publication: the patients in this manuscript have givenwritten informed consent to the publication of their case details.Received: 20 July 2023.Accepted: 23 July 2023.This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License (CC BY-NC 4.0).©Copyright: the Author(s), 2023Licensee PAGEPress, ItalyDermatology Reports 2024; 16:9807doi:10.4081/dr.2023.9807Publisher's note: all claims expressed in this article are solely thoseof the authors and do not necessarily represent those of their affiliat-ed organizations, or those of the publisher, the editors and thereviewers. Any product that may be evaluated in this article or claimthat may be made by its manufacturer is not guaranteed or endorsedby the publisher.Unilateral eosinophilic fasciitis (Shulman syndrome) with excellentresponse to combined treatment with methotrexate therapy and compression braceCorrado Zengarini,1,2 Giacomo Clarizio,1,2 Federico Bardazzi,1,2 Martina Mussi,1,2 Carlotta Baraldi,1,2Bianca Maria Piraccini,1,2 Alessandro Pileri1,21Dermatology Unit, IRCCS of Azienda Ospedaliero-Universitaria, Bologna; 2Department of Medical and Surgical Sciences, Universityof Bologna, Italy4 6 9807ble while the oedematous phase is still ongoing. In contrast, oncethe fibrotic second phase is reached, it is harder to treat this con-dition since tissue modification tends to be irreversible.1Although methotrexate has already been reported to be effec-tive in treating eosinophilic fasciitis, even when started late,7 thisdid not happen in our case.Since it is known that plasma exudate can lead to the increaseof cytokines that can aggravate or maintain the inflammatoryresponse,8-10 we assumed that that compression, known to favorthe reabsorption of liquids from the third space, could bring a syn-ergic benefit and accelerate the clinical improvement in the actualoedematous phase.After one year and a half, the patient maintains excellent clin-ical results and no haematological alterations with this treatment.Finally, we can state that using a compressive aid when theedematous phase is still present can be an additional therapy,added to the systemic one, which has almost no side effects butseems very effective in swelling control.Letter to the EditorFigure 1. A) Deep skin biopsy of the arm reaching the muscular tissue showing eosinophilic infiltrate and edema in the dermis and hypo-dermic tissue reaching the fascia E&H 5× stain 5×; B) magnetic resonance image axial projection showing oedema with inflammation andthickening of the muscular fasciae and hypodermic tissue.Figure 2. A) Patient’s right arm. Note the arm size, which was more than twice the contralateral, causing the patient difficulties in flexingthe arm and swelling, with the appearance of orange peel skin; B) patient after six months of treatment with methotrexate and the use ofthe elastic compression device. Note the significant reduction in the volume of the affected arm and the skin appearance, now much moresimilar than the unaffected one.                                                                            [Dermatology Reports 2024; 16:9807]                                                           [page 95][page 96]                                                             [Dermatology Reports 2024; 16:9807]References1. Lebeaux D, Sène D. Eosinophilic fasciitis (Shulman disease).Best Pract Res Clin Rheumatol 2012;26:449-58. 2. Long H, Zhang G, Wang L, Lu Q. Eosinophilic skin diseases:a comprehensive review. Clin Rev Allergy Immunol2016;50:189-213.3. Urzal J, Cimbron M, Mendonça T, Farinha F. Eosinophilicfasciitis (Shulman’s disease): review and comparative evalua-tion of seven patients. Reumatologia 2019;57:85-90.4. Primitivo A, Madeira N, Lopez D, Afonso D. Eosinophilicfasciitis (Shulman disease) with clinical, imaging and patho-logical correlation. BMJ Case Rep 2021;14(:e246151. 5. Lakhanpal S, Ginsburg WW, Michet CJ, et al. Eosinophilicfasciitis: clinical spectrum and therapeutic response in 52cases. Semin Arthritis Rheum 1988;17:221-31.6. Pehl D, Preuße C, Allenbach Y, et al. Eosinophilic fasciitis(Shulman syndrome)-recognition of the histological spectrumallows for new insights into possible pathomechanisms.Rheumatology (Oxford) 2023;62:2005-14.7. Shinozaki A, Hayashi S, Hongo T, et al. Efficacy ofmethotrexate for steroid-resistant eosinophilic fasciitis withdelayed start of treatment: a case report. Int J Dermatol2023;62:e67-8.8. Mikhal’chik EV, Piterskaya JA, Budkevich LY, et al.Comparative study of cytokine content in the plasma andwound exudate from children with severe burns. Bull ExpBiol Med 2009;148:771-5.9. Saito M, Shima C, Takagi M, et al. Enhanced exudation offibrinogen into the perivascular space in acute inflammationtriggered by neutrophil migration. Inflamm Res 2002;51:324-31. 10. Baum SJ, MacVittie TJ, Brandenburg RT, Levin SG.Stimulatory effects of products from inflammatory exudatesupon stem cell production. Exp Hematol 1978;6:405-9.Letter to the Editor

Unilateral eosinophilic fasciitis (Shulman syndrome) with excellent response to combined treatment with methotrexate therapy and compression brace

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Unilateral eosinophilic fasciitis (Shulman syndrome) with excellent response to combined treatment with methotrexate therapy and compression brace

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