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A Case of Eosinophilic Fasciitis and Generalized Morphea Overlap

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A Case of Eosinophilic Fasciitis and Generalized Morphea Overlap Volume 26 Number 2| February 2020| Dermatology Online Journal || Photo Vignette 26(2):15 A case of eosinophilic fasciitis and generalized morphea overlap Yukina Watanabe, Miyuki Yamamoto, Toshiyuki Yamamoto Affiliations: Department of Dermatology, Fukushima Medical University, Fukushima, Japan Corresponding Author: Yukina Watanabe, Fukushima Medical University, Fukushima 960-1295, Japan, Tel: 81-24 547 1309, Fax: 81-24 548 5412, Email: [email protected] presentation. One week following the pruning, he Abstract experienced pain in his left shoulder and edema in A 60-year old man developed skin hardening and the extremities. He noted limited range of edema on his extremities. Although he had been movement in the joints. At initial presentation in his treated with oral prednisolone at another hospital, local hospital, laboratory examination showed an skin stiffness relapsed during tapering of elevated eosinophil subset, and he was diagnosed as prednisolone. At the initial visit to our department, having eosinophilic fasciitis. He was treated with oral physical examination showed skin hardening of the prednisolone (20mg/day). However, skin hardening extremities and also symmetric erythematous macules on the back. Histological examination and peripheral eosinophilia relapsed when the revealed fasciitis on the forearm and morphea on the prednisolone was gradually decreased by a dose of back. Eosinophilic fasciitis is occasionally associated 2.5mg/day. Physical examination showed skin with morphea. However, cases of concurrent hardening of the upper and lower extremities eosinophilic fasciitis and generalized morphea are (Figure 1A, B). The groove sign was observed in the rare. In the present case, CD34 was differentially expressed in both lesions, suggesting eosinophilic fasciitis and morphea are separate diseases with different origin of mesenchymal cells. Keywords: eosinophilic fasciitis, generalized morphea, CD34 A B D Introduction Eosinophilic fasciitis is occasionally associated with morphea. However, cases of concurrent eosinophilic fasciitis and generalized morphea are rare. We herein describe a concurrent case of eosinophilic fasciitis and generalized morphea. C Case Synopsis Figures 1. Clinical features showing induration of the skin of the A 60-year-old man was referred to our hospital A) upper, and B) lower limbs. C) Groove sign was observed on the complaining of skin hardening and edema of his forearm. D) Brownish erythema was present on the bilateral sides extremities. He had pruned plants 10 months prior to of the back. - 1 - Volume 26 Number 2| February 2020| Dermatology Online Journal || Photo Vignette 26(2):15 forearm (Figure 1C). Neither sclerodactyly nor fasciitis/generalized morphea overlap was made Raynaud phenomenon was recognized. Further based on the clinical, analytical, and examination revealed symmetric erythematous histopathological findings. Immunohistochemistry macules on the back, but without hardening of the results showed a focal loss of CD34 expression in the skin (Figure 1D). fascia of the area of eosinophilic fasciitis, whereas abundant expression of CD34 was observed in the Laboratory examination showed a slightly elevated morphea lesion (Figure 2E, F). The dose of eosinophil subset (7%) and increased C-reactive prednisolone was escalated up to 40mg/day and protein levels (1.14mg/dl). However, antinuclear thereafter gradually tapered every one or two antibody, anti-dsDNA and creatine kinase levels months. Currently, the patient is controlled with were normal. A biopsy specimen taken from the left 15mg prednisolone daily, which is still being forearm revealed thickened collagen bundles in the gradually tapered. dermis and subcutaneous tissues with focal lymphocyte infiltrates in the subcutis and thickened fascia involvement (Figure 2A). There were Case Discussion perivascular and interstitial infiltrates of Eosinophilic fasciitis is occasionally induced by lymphocytes, plasma cells, and a few eosinophils in vigorous exercise as well as trauma, leading to the the thickened fascia (Figures 2B, C). The underlying hardening of the skin and limited range of skeletal muscle was not involved. Another specimen movement in the joints. Eosinophilic fasciitis is often taken from the patient’s back showed that the regarded to belong to the severe end of the morphea collagen bundles of the reticular dermis were spectrum [1] and an association with morphea has thickened (Figure 2D). A diagnosis of eosinophilic been reported in 19-41% of eosinophilic fasciitis A B C D E F Figures 2. A) A biopsy specimen from the forearm shows fibrosis of deep dermis and subcutis, and thickened muscle fascia with inflammatory cell infiltrates. H&E, 40×. B, C) Higher magnification shows that cellular infiltrates in the thickened fascia are composed of lymphocytes, plasma cells, and eosinophils (arrow). H&E, 200×. 400×. D) A second biopsy from the back shows thickened collagen bundle with deposition of homogeneous materials in the whole dermis. H&E, 40×. E) CD34 expression is absent in the fascia of eosinophilic fasciitis (forearm), 200×. F) whereas it is abundant in the morphea (back). 400×. - 2 - Volume 26 Number 2| February 2020| Dermatology Online Journal || Photo Vignette 26(2):15 Table 1. Seven cases of concurrent eosinophilic fasciitis and generalized morphea. Involved site of Age Involved site of generalized Preceding Case Sex eosinophilic fascilitis morphea onset Duration* Therapy 1 [5] 25M Extremities, trunk, scalp Left thigh Unknown — Prednisolone Chest, abdomen, breast, Generalized Prednisolone, etanercept 2 [6] 50F proximal portions of the Upper extremities 12Mo morphea methotrexate arm and legs Back, chest, Eosinophilic 3 [7] 44F Forearms, legs abdomen, proximal 12Mo Prednisolone fasciitis extremities 4 [8] 66M Upper limbs, chest Tibialis anterior Unknown — Prednisolone 5 [9] 61M extremities Abdomen, trunk Unknown — Prednisolone Prednisolone, azathioprine, Eosinophilic mycophenolate mofetil, 6 [10] 24M Arms, legs, trunk Chest, upper back 6Yr fasciitis cyclophosphamide, cyclosporine, rituximab Present 60M Extremities Back Unknown — Prednisolone case * Mo-months; Yr-years. patients [2-4]. In contrast, generalized morphea is circulation. Fibrocytes are induced by transforming rarely seen, and to our knowledge only seven cases growth factor-β to express α-smooth muscle actin, a of concurrent eosinophilic fasciitis and generalized myofibroblastic property. A recent paper morphea have been reported in the English demonstrated a further decreased CD34 expression literature [5-10]. The patient characteristics of these in the fascia of eosinophilic fasciitis, compared with reported cases are shown in Table 1. The average morphea profunda [13], suggesting that the two age at occurrence was 47.1 years old and men were diseases have different etiologies. We compared the predominant (5:2 male to female ratio). In all cases, local CD34 expression of biopsied tissue samples extremities, a common site of eosinophilic fasciitis, from different sites, which revealed that CD34 were involved. Generalized morphea occurred in the expression disappeared in the fascia of eosinophilic trunk in four cases. Eosinophilic fasciitis was fasciitis, whereas it was observed in the morphea diagnosed prior to generalized morphea in two lesion, consistent with previous reports. cases. The onset period was between twelve months and six years. In all cases, treatment was started with prednisolone from small to moderate amounts. Two Conclusion cases underwent additional treatment with Our case showed different expression of CD34 between eosinophilic fasciitis and morphea lesions, immunosuppressive drugs. suggests that both disorders possess separate Although generalized morphea and eosinophilic phenotypes with different origin of mesenchymal fasciitis are believed to be different diseases, they are cells. Alternatively, the possibility that CD 34 similar in presentation and are sometimes difficult to expression simply reflects the intensity of skin differentiate from each other. Previous studies fibrosis cannot be excluded. Further studies are showed that CD34 expression decreased or needed to clarify the causative mechanisms of disappeared in the lesional skin of scleroderma, eosinophilic fasciitis and deep morphea. including morphea [11], which is inversely related with the extent of morphea [12]. CD34 is a marker of dermal dendritic cells and expression of CD34 and Potential conflicts of interest type I collagen suggest fibrocytes in the peripheral The authors declare no conflicts of interests. - 3 - Volume 26 Number 2| February 2020| Dermatology Online Journal || Photo Vignette 26(2):15 References 1. Mertens JS, Seyger MMB, Thurlings RM, Radstake TRDJ, de Jong patient previously affected with eosinophilic fasciitis. Indian J EMGJ. Morphea and eosinophilic fasciitis: an update. Am J Clin Dermatol Venereol Leprol. 2012;78:654-656. [PMID: 22960833]. Dermatol. 2017;18:491-512. [PMID: 28303481]. 8. Alonso-Castro L, de las Heras E, Moreno C, Fleta-Asín B, Muñoz- 2. Lakhanpal S, Ginsburg WW, Michet CJ, Doyle JA, Moore SB. Zato E, Carrillo R, Jaén P. Eosinophilic fasciitis/generalized Eosinophilic fasciitis: clinical spectrum and therapeutic response morphea overlap successfully treated with azathioprine. Int J in 52 cases. Semin Arthritis Rheum. 1988;17:221-231. [PMID: Dermatol. 2014;53:1386-1388. [PMID: 24697582]. 3232080]. 9. Hanami Y, Ohtsuka M, Yamamoto T. Paraneoplastic eosinophilic 3. Endo Y, Tamura A, Matsushima Y, Iwasaki T, Hasegawa M, Nagai
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