report, some patients can keep the same treatment, without relapse/recurrence of the skin necrosis.  Disclosure. Financial support: none. Conflict of interest: none. 1

Neurology Department, Dermatology Department, Centro Hospitalar do Porto Hospital Santo António, Porto, Portugal 2

˜ 1 Raquel SAMOES Sandrina CARVALHO2 Rosário ALVES2 Tiago TORRES2 Ana Martins SILVA1

1. Hartung HP, Montalban X, Sorensen PS, Vermersch P, Olsson T. Principles of a new treatment algorithm in multiple sclerosis. Expert Rev Neurother 2011; 11: 351-62. 2. Balak DM, Hengstman GJ, C ¸ akmak A, Thio HB. Cutaneous adverse events associated with disease-modifying treatment in multiple sclerosis: a systematic review. Mult Scler 2012; 18: 1705-17. 3. Bosca I, Bosca M, Belenguer A, et al. Necrotising cutaneous lesions as a side effect of glatiramer acetate. J Neurol 2006; 253: 1370-1. 4. Feldmann R, Schierl M, Rauschka H, Sator PG, Breier F, Steiner A. Necrotizing skin lesions with involvement of muscle tissue after subcutaneous injection of glatiramer acetate. Eur J Dermatol 2009; 19: 385. 5. Harde V, Schwarz T. Embolia cutis medicamentosa following subcutaneous injection of glatiramer acetate. J Dtsch Dermatol Ges 2007; 5: 1122-3. 6. Gaudez C, Regnier S, Aractingi S, Heinzlef O. Livedo-like dermatitis (Nicolau’s syndrome) after injection of Copolymer-1 (Glatiramer acetate). Rev Neurol (Paris) 2003; 159: 571-3. 7. Martínez-Morán C, Espinosa-Lara P, Nájera L, et al. Embolia cútis medicamentosa (síndrome de Nicolau) trasinyección de acetado de glatirámero. Actas Dermosifiliogr 2011; 102: 742-4. 8. Kurtzke JF. Rating neurologic impairment in multiple sclerosis: an expanded disability status scale (EDSS). Neurology 1983; 33: 1444-52. 9. Kluger N, Thouvenot E, Camu W, Guillot B. Cutaneous adverse events related to glatiramer acetate injection (copolymer-1, Copaxone). J Eur Acad Dermatol Venereol 2009; 23: 1332-3. 10. Frohman EM, Brannon K, Alexander S, et al. Disease modifying agent related skin reactions in multiple sclerosis: prevention, assessment, and management. MultScler 2004; 10: 302-7.

neurofibroma on the right forehead. He was an NF-1 patient meeting the criteria of the National Institutes of Health, including plexiform neurofibromas, many café-aulait macules, mild scoliosis and positive family history. On physical examination, the subcutaneous nodule was 4 cm and had a rubber-like consistency, adhering to the surrounding neurofibroma (figure 1A). He complained of spontaneous pain in it. We suspected that the nodule was a malignant peripheral nerve sheath tumor (MPNST) and performed an excisional biopsy. Histopathologically, the nodule showed a biphasic composition, namely, neurofibromatous and perineuriomatous areas. The neurofibromatous areas were composed of cells with an oval or spindle-shaped nucleus and scant, indefinite cytoplasm. The matrix showed pale staining with delicate wavy collagen. The perineuriomatous areas were composed of slender spindle cells with elongated bipolar cytoplasmic processes in a prominent myxoid stroma, displaying fascicular and whorled patterns (figures 1B-D). There were no nuclear atypia or aberrant mitoses. Immunohistochemically, the neurofibromatous areas were positive for S-100 protein and CD34, but negative for epithelial membrane antigen (EMA), human erythrocyte glucose transporter-1 (GLUT-1) and claudin-1. In contrast, the perineuriomatous areas showed the reverse staining pattern: GLUT-1 (figures 1E-F), EMA and claudin1 were positive but S-100 protein and CD34 were negative. These two areas overlapped in the transitional area. On the basis of these findings, a diagnosis of hybrid perineuriomaneurofibroma was made. His postoperative course was uneventful and the pain was completely relieved. No recurrence was noted three months after the surgery. Perineurioma is a rare BPNST, composed of perineurial cells, which develops in the dermis, subcutis or deep soft tissue. Soft-tissue perineurioma occurs as a painless mass, most commonly in the subcutis of the extremities and trunk of young to middle-aged adults, without favoring either gender. It behaves in a benign fashion and rarely recurs [4]. Most of the tumor cells are positive for perineurial

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Hybrid perineurioma-neurofibroma in a patient with neurofibromatosis type 1, clinically mimicking malignant peripheral nerve sheath tumor Perineurioma is a rare benign peripheral nerve sheath tumor (BPNST) featuring perineurial differentiation. Sporadic case reports of hybrid tumors of BPNST have been reported; hybrid perineurioma-neurofibroma in neurofibromatosis type 1 (NF-1) is extremely rare [1-3]. Here, we present a hybrid tumor arising in a diffuse plexiform neurofibroma on the face of an NF-1 patient. A 30-year-old Japanese man presented with a two-month history of subcutaneous nodules in a diffuse plexiform

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Figure 1. A) A subcutaneous nodule in a diffuse plexiform neurofibroma (circle). B) A perineuriomatous area (circle) adjacent to a neurofibromatous area (H&E staining ×40). C) High power view of neurofibromatous lesion (H&E staining ×200). D) High power view of perineuriomatous lesion (H&E staining ×200). E) Immunostaining for GLUT-1 highlighting perineurial areas (×40). F) Perineurial tumor cells displaying fascicular and whorled pattern (immunostaining for GLUT-1, ×100). EJD, vol. 24, n◦ 3, May-June 2014

cell markers, including EMA, GLUT-1 and claudin-1, but usually negative for S-100 protein [4, 5]. BPNST generally consists of Schwann cells, fibroblasts and perineurial cells in various proportions. There have been several recent reports describing hybrid BPNSTs, such as neurofibroma and schwannoma, neurofibroma and perineurioma, perineurioma and granular cell tumor, and schwannoma and perineurioma [6]. To our knowledge, only a few cases of composite perineurioma have been documented in association with NF-1 [1-3, 7, 8]. Zamecnik and Michal analyzed the immunohistochemical features of 99 neurofibromas [8, 9]. Eight tumors contained EMA-positive perineurial cells inside the neurofibromatous lesions, four of which showed perceivable perineurial cell differentiation by routine hematoxylin and eosin (H&E) staining. Given the similar histopathology between perineurioma and neurofibroma, perineurial differentiation in neurofibroma is easily overlooked. Therefore, hybrid tumors of perineurioma-neurofibroma may be more common than recognized. Plexiform neurofibromas are characterized by a nodular growth pattern that may be attributed to the presence of a perineurial component circumscribing the Schwann cell proliferation. In our case, the perineurial GLUT-1 component was mainly located at the edge of the nodule, and this perineurial component might be more developed than in the majority of NF-1 plexiform tumors. The current patient’s hybrid tumor had developed in the plexiform neurofibroma as a painful nodule. In such cases, the primary differential diagnosis should be MPNST, but a hybrid tumor can occur. MPNST typically arises as a rapidly growing, painful mass in a pre-existing plexiform neurofibroma, with dismal prognosis. Using H&E staining alone, it is sometimes difficult to distinguish perineurioma from other spindle cell neoplasms [4, 10]. In the current case, the perineuriomatous areas showed definite expression of EMA, GLUT-1 and claudin-1 but lacked S-100 protein and CD34 expression, whereas the neurofibromatous areas showed the opposite characteristics, with the staining pattern clearly highlighted these two lesions. We should be aware that hybrid perineurioma-neurofibroma can, in NF-1 patients, mimic MPNST.  Disclosure. Financial support: none. Conflict of interest: none. 1

Department of Dermatology Department of Anatomic Pathology, Kyushu University, 3-1-1 Maidashi, Higashiku, Fukuoka 812-8582, Japan 2

Yusuke INATOMI1 Takamichi ITO1 Konosuke NAGAE1 Yuichi YAMADA2 Mari KIYOMATSU1 Misa NAKANONAKAMURA1 Hiroshi UCHI1 Yoshinao ODA2 Masutaka FURUE1

1. Schaefer I, Strobel P, Thiha A, et al. Soft tissue perineurioma and other unusual tumors in a patient with neurofibromatosis type 1. Int J Clin Exp Pathol 2013; 6: 3003-8. 2. Lassmann H, Jurecka W, Lassmann G, Matras H, Watzek G. Different types of benign nerve sheath tumors. Light microscopy, electron microscopy and autoradiography. Virchows Arch A Path Anat And Histol 1977; 375: 197-210. EJD, vol. 24, n◦ 3, May-June 2014

3. Theaker JM, Gatter KC, Puddle J. Epithelial membrane antigen expression by the perineurium of peripheral nerve and in peripheral nerve tumours. Histopathology 1988; 13: 171-9. 4. Hornick JL, Fletcher CDM. Soft tissue perineurioma. Clinicopathologic analysis of 81 cases including those with atypical histologic features. Am J Surg Pathol 2005; 29: 845-58. 5. William A, Robert V, Bolette L, Dylan V, Andrew L. GLUT-1 expression in mesenchymal tumors: an immunohistochemical study of 247 soft tissue and bone neoplasms. Human Pathology 2008; 39: 1519-26. 6. Requena L, Sitthinamsuwan P, Fried I, et al. A benign cutaneous plexiform hybrid tumor of perineurioma and cellular neurothekeoma. Am J Surg Pathol 2013; 37: 845-52. 7. Ausmus GG, Piliang MP, Bergfeld WF, Goldblum JR. Soft-tissue perineurioma in a 20-year-old patient with neurofibromatosis type 1 (NF1): report of a case and review of the literature. J Cutan Pathol 2007; 34: 726-30. 8. Kacerovska D, Michal M, Kuroda N, et al. Hybrid peripheral nerve sheath tumors, including a malignant variant in type 1 neurofibromatosis. Am J Dermatopathol 2013; 35: 641-9. 9. Zamecnik M, Michal M. Perineurial cell differentiation in neurofibromas. Report of eight cases including a case with composite perineurioma-neurofibroma features. Pathol Res Pract 2001; 197: 53744. 10. Folpe AL, Billings SD, McKenney JK, Walsh SV, Nusrat A, Weiss SW. Expression of claudin-1, a recently described tight junctionassociated protein, distinguishes soft tissue perineurioma from potential mimics. Am J Surg Pathol 2002; 26: 1620-6. doi:10.1684/ejd.2014.2353

Omeprazole-induced drug reaction with eosinophilia and systemic symptoms (DRESS) We report here a case of Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) following treatment with omeprazole, which, to our knowledge has not been reported before. An 88-year-old woman with a history of arterial hypertension, chronic renal failure (creatinine clearance estimated by MDRD: 34.8 ml/min) and chronic lymphocytic leukemia (CLL) diagnosed in 2005, presented with a cutaneous rash and pruritus. Despite treatment with hydroxyzine, the rash became widespread and pruritus persisted. The patient was hospitalized 8 days later with facial and cervicobrachial oedema, difficulty in swallowing and dysphonia. She had begun treatment with omeprazole 53 days prior to, and discontinued it 7 days before hospitalization. Her current treatment included celiprolol, furosemide, losartan, lysine acetylsalicylate and acetaminophen. Physical examination showed a maculopapular exanthema on the trunk and the face converging with wide patches with purpuric lesions (including the hard palate) and unremitting pruritus over the whole body. Maculopapular lesions were scattered on the limbs. Examination also revealed right axillary lymphadenopathy and fever (38 ◦ C) after stopping acetaminophen. At admission, blood tests showed leukocytosis, lymphocytosis (probably related to untreated CLL), inflammatory syndrome and hepatic and renal dysfunction. Between days 3 and 9 after admission, the patient’s blood tests deteriorated (table 1). Given these features, the diagnosis of DRESS was made. A skin biopsy showed a lichenoid

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Hybrid perineurioma-neurofibroma in a patient with neurofibromatosis type 1, clinically mimicking malignant peripheral nerve sheath tumor.

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