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Dlo130062.Pdf Letters entine dihydrochloride in this interdisciplinary case. For the re- tissue. To our knowledge, as of 2006, there were a total of 86 maining lesions, we offered liquid nitrogen cryotherapy or ab- reported cases of cutaneous Rosai-Dorfman disease (CRDD) in lative laser resurfacing to the patient. Unfortunately, the patient the literature,2,3 with several additional cases reported since was subsequently lost to follow-up. then. Simin Hellriegel, MD Report of a Case | An African American woman in her 50s re- Hans P. Bertsch, MD ported a sudden eruption of dozens of facial papules and nod- Steffen Emmert, MD ules 3 months previously and a 7-month history of a groin Michael P. Schön, MD plaque. She had been treated with cefadroxil, topical clobeta- Holger A. Haenssle, MD sol, oral prednisone, and a short course of oral isotretinoin with- out significant benefit. Author Affiliations: Clinic of Dermatology, Venereology and Allergology, Physical examination revealed approximately 75 pink- University Medical Center, Georg-August-University, Göttingen, Germany domed papules on the cheeks, upper lip, and chin (Figure 1) (Hellriegel, Bertsch, Emmert, Schön, Haenssle). and dozens of red-brown papules becoming confluent on the Corresponding Author: Holger A. Haenssle, MD, Clinic of Dermatology, Venereology and Allergology, University Medical Center, right inguinal and suprapubic skin. Laboratory examination Georg-August-University, Göttingen, Robert-Koch Strasse 40, 37075 revealed a mildly elevated erythrocyte sedimentation rate (32 Göttingen, Germany ([email protected]). mm/h) and total triglyceride levels (145 mg/dL). Findings of Published Online: April 16, 2014. complete and differential blood cell counts, a comprehensive doi:10.1001/jamadermatol.2013.8635. metabolic panel, serum protein electrophoresis, and a light Conflict of Interest Disclosures: None reported. chains assay were within normal limits. Complete computed 1. Bull PC, Thomas GR, Rommens JM, Forbes JR, Cox DW. The Wilson disease tomography of the chest, abdomen, and pelvis revealed no sig- gene is a putative copper transporting P-type ATPase similar to the Menkes gene. Nat Genet. 1993;5(4):327-337. nificant retroperitoneal, mesenteric, or pelvic lymphadenop- 2. Roberts EA, Schilsky ML; American Association for Study of Liver Diseases athy. Biopsies from the groin revealed a dense infiltrate of lym- (AASLD). Diagnosis and treatment of Wilson disease: an update. Hepatology. phocytes and large histiocytes with abundant pale cytoplasm 2008;47(6):2089-2111. (Figure 2). The histiocytes showed emperipolesis of lympho- 3. Iozumi K, Nakagawa H, Tamaki K. Penicillamine-induced degenerative cytes and occasionally red blood cells. The histiocytes seen in dermatoses: report of a case and brief review of such dermatoses. J Dermatol. CRDD stain positively for macrophage marker CD68. The his- 1997;24(7):458-465. topathologic differential diagnosis also includes Langerhans 4. Rosen LB, Muellenhoff M, Tran TT, Muhart M. Elastosis perforans serpiginosa secondary to D-penicillamine therapy with coexisting cutis laxa. Cutis. 2005;76 cell histiocytosis. While S-100 may stain positively in both (1):49-53. CRDD and Langerhans cell histiocytosis, findings of CD1a stain- 5. Bardach H, Gebhart W, Niebauer G. “Lumpy-bumpy” elastic fibers in the skin ing are characteristically negative in CRDD. In our case, cells and lungs of a patient with a penicillamine-induced elastosis perforans did not stain with melanocyte markers Melan-A or HMB45 or serpiginosa. J Cutan Pathol. 1979;6(4):243-252. with cytokeratin AE1/AE or CD34 as in epithelioid sarcoma. Findings of acid-fast bacilli and Grocott methenamine silver Cutaneous Rosai-Dorfman Disease Successfully stainings were negative. Treated With Low-Dose Methotrexate The patient’s prednisone dose was tapered, and she be- In 1969, Rosai and Dorfman1 first described a series of pa- gan treatment with oral methotrexate, 15 mg once weekly, and tients with sinus histiocytosis with massive lymphadenopa- significant improvement was noted over the next 11 months. thy, characterized by histiocytic infiltration of lymph nodes and Subsequently, the methotrexate dose was tapered to 5 mg Figure 1. Clinical Images Showing Gradual Improvement in Facial Papules A B C Patient observed at 1 month (A), 8 months (B), and 14 months (C) after initiation of treatment with methotrexate. jamadermatology.com JAMA Dermatology July 2014 Volume 150, Number 7 787 Copyright 2014 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/29/2021 Letters Corresponding Author: Natalie Z. Sun, MD, Department of Dermatology, Figure 2. Biopsy Specimen Taken From the Patient’s Groin University Hospitals Case Medical Center, 11100 Euclid Ave Lakeside 3500, Cleveland, OH 44106 ([email protected]). Published Online: April 30, 2014. doi:10.1001/jamadermatol.2013.8679. Conflict of Interest Disclosures: None reported. 1. Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy. A newly recognized benign clinicopathological entity. Arch Pathol. 1969;87(1): 63-70. 2. Foucar E, Rosai J, Dorfman R. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): review of the entity. Semin Diagn Pathol. 1990;7(1):19-73. 3. Frater JL, Maddox JS, Obadiah JM, Hurley MY. Cutaneous Rosai-Dorfman disease: comprehensive review of cases reported in the medical literature since 1990 and presentation of an illustrative case. J Cutan Med Surg. 2006;10(6): 281-290. 4. Nasseri E, Belisle A, Funaro D. Rosai-Dorfman disease treated with methotrexate and low-dose prednisone: case report and review of the literature. J Cutan Med Surg. 2012;16(4):281-285. A dense infiltrate of lymphocytes, neutrophils, and large histocytes with 5. Scheel MM, Rady PL, Tyring SK, Pandya AG. Sinus histiocytosis with massive abundant cytoplasm; histiocytes show emperipolesis of lymphocytes lymphadenopathy: presentation as giant granuloma annulare and detection of (hematoxylin-eosin, original magnification ×40). human herpesvirus 6. J Am Acad Dermatol. 1997;37(4):643-646. 6. Gebhardt C, Averbeck M, Paasch U, et al. A case of cutaneous Rosai-Dorfman disease refractory to imatinib therapy. Arch Dermatol. 2009;145(5):571-574. weekly for the next 4 months, and patient showed a com- 7. Bassis AV, Fairley JA, Ameln RT, Swick BL. Cutaneous Rosai-Dorfman disease plete clinical response. During this time, she also received sev- following pneumococcal vaccination. J Am Acad Dermatol. 2011;65(4):890-892. eral intralesional Kenalog injections to the larger nodules of the face and groin. Paraproteinemia-Associated Scleredema Treated Successfully With Intravenous Immunoglobulin Discussion | There have been a variety of treatment techniques Scleredema is a fibromucinous connective tissue disease used for CRDD, including cryotherapy, surgical excision, irra- characterized by symmetric, nonpitting edema and indura- diation, oral corticosteroids, dapsone, thalidomide, and tion of the face, neck, and trunk. Although the pathogenesis isotretinoin. To our knowledge, the use of methotrexate alone remains elusive, associations with infection, diabetes or in combination with other agents has been reported in 9 mellitus, and paraproteinemia have been established.1 cases of systemic Rosai-Dorfman, and a complete to partial re- Paraproteinemia-associated scleredema is typically charac- sponse was reported in most cases.4 By contrast, methotrex- terized by a progressive course, for which no standard ate therapy has been reported in only 3 cases of CRDD, and par- therapeutic protocol exists.2 To our knowledge, the patient tial or no improvement was reported.3,5,6 However, in all of described herein represents the first reported case of scler- these cases, the eruption had already been present for well over edema with paraproteinemia successfully treated with a year. In our patient, a lack of response to prednisone, preex- intravenous immunoglobulin (IVIG). isting diabetes, and significant disease burden prompted the choice of low-dose methotrexate, to which a complete clini- Report of a Case | A woman in her 40s presented with a 2-year cal response was seen over 11 months. history of progressive erythema and induration of the face, Though the exact cause of CRDD remains unknown, the neck, and upper trunk. She denied a history of preceding in- presence of Epstein-Barr virus, human herpesvirus 6 by poly- fection or diabetes mellitus. Physical examination revealed ery- 7 merase chain reaction, and reported eruption after vaccination thema, brawny edema, and induration of the face, neck, up- with spontaneous remission over months to years suggests that per trunk, and upper arms. There was significant limitation of CRDD is a benign reactive process involving a particular pat- neck flexion, extension, and lateral rotation as well as shoul- tern of immune dysregulation. Early diagnosis remains a chal- der abduction and internal rotation (Figure, A). The patient de- lenge in CRDD owing to its nonspecific clinical manifesta- nied difficulty swallowing or restricted breathing. tions, including variable numbers of papules, nodules, plaques, A skin biopsy from the upper back revealed dermal thick- or tumors. Timely diagnosis and initiation of methotrexate ening with separation of enlarged collagen bundles by Alcian therapy may be key to effecting a rapid clinical remission while blue–positive mucin deposition, consistent with a diagnosis this disease remains in its active phase. of scleredema. Findings
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