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varying doses of oral prednisone for a number ofmonths.

Three months after the patient’s original presen-tation, she was seen at the emergency departmentwith diffuse ST-T segment elevations on electrocar-diographic examination and elevated cardiac en-zymes. Her skin was clear; however, her laboratorytests revealed a marked leukocytosis with eosino-philia of 30%. Treatment with intravenous cortico-steroids was begun, but the patient rapidlydecompensated. An urgent cardiac catheterizationrevealed normal coronary arteries but a diffuselyhypokinetic left ventricle and a profoundly de-creased ejection fraction. At that time, an intra-aortic balloon pump was placed.

Approximately 24 hours after arrival, the patientsuffered cardiac arrest. She was successfully resusci-tated and was taken for open myocardial biopsy andplacement of a left-ventricular assist device. Thebiopsy specimen showed a lymphocytic and eosin-ophilic infiltrate, consistent with myocarditis. Shewas treated with high-dose intravenous methylpred-nisolone (1 g/d), IVIG (1 g/kg per day32 doses) andmycophenolate mofetil during her hospital course.Serial echocardiograms showed slow but progress-ive improvement in left ventricular ejection fraction,and the left-ventricular assist device was explantedafter 53 days. A mitral-valve replacement was alsoperformed at this time because of ruptured chordaetendinae.

The patient continued to improve and was dis-charged 71 days after admission. She currently isdoing well after 2 years but required mycophenolatemofetil and low-dose prednisone for 1 year asseveral attempts to taper off the immunosuppressiveagents resulted in elevation of the hepatic transam-inases and erythrocyte sedimentation rate.

Acute necrotizing eosinophilic myocarditis(ANEM) is a severe form of myocarditis that canoccur in DRESS, as demonstrated by these two cases.The presentation of ANEM is similar to an acutecoronary event with chest pain, ST-segment eleva-tion, elevated cardiac markers, but with normalcoronary arteries on angiography. The course pro-gresses rapidly into a fulminant heart failure.Mortality is greater than 50%, and median survivalis a few days. Definitive diagnosis is with an endo-myocardial biopsy that reveals an eosinophilic andlymphocytic infiltrate with associated myocyte ne-crosis, although the infiltrate may be patchy innature.1 The treatment is high-dose corticosteroidswith pharmacologic and/or mechanical ventricularsupport, as used in our second case.2,3

To our knowledge, this is the first case of DRESS-associated ANEM treated with a ventricular assist

device, and we recommend its use as a life-savingtreatment if rapidly deteriorating cardiac functiondevelops in these patients.

Greg P. Bourgeois, MD,a Jennifer A. Cafardi, MD,a

Vlada Groysman, MD,a Salpy V. Pamboukian,MD, MSPH,c James K. Kirklin, MD,d Aleodor A.Andea, MD,b and Lauren C. Hughey, MDa

Departments of Dermatology,a Pathology,b

Cardiology,c and Cardiothoracic Surgery,d Uni-versity of Alabama at Birmingham

Funding sources: None.

Conflicts of interest: None declared.

Reprint requests: Lauren C. Hughey, MD, Universityof Alabama at Birmingham, Department ofDermatology, 1530 3rd Ave South, EFH 414,Birmingham, AL 35294

E-mail: lchughey@uab.edu

REFERENCES

1. Burke AP, Saenger J, Mullick F, Virmani R. Hypersensitivity

myocarditis. Arch Pathol Lab Med 1991;115:764-9.

2. Sabatine MS, Poh KK, Mega JL, Shepard JA, Stone JR, Frosch MP.

Case records of the Massachusetts General Hospital. Case

36-2007. A 31-year-old woman with rash, fever, and hypoten-

sion. N Engl J Med 2007;357:2167-78.

3. Getz MA, Subramanian R, Logemann T, Ballantyne F. Acute

necrotizing eosinophilic myocarditis as a manifestation of

severe hypersensitivity myocarditis. Antemortem diagnosis

and successful treatment. Ann Intern Med 1991;115:201-2.

doi:10.1016/j.jaad.2010.12.013

Cutaneous Rosai-Dorfman disease followingpneumococcal vaccination

To the Editor: We present a novel association be-tween pneumococcal vaccination and the develop-ment of cutaneous Rosai-Dorfman disease (CRD).CRD is distinguished from classic Rosai-Dorfmandisease (RDD) (sinus histiocytosis with massivelymphadenopathy) by isolated skin involvementwithout lymphadenopathy or systemic symptoms.

A 68-year-old white woman was referred for a 2-year history of an enlarging lesion on the right armthat developed several days after administration ofthe 23-valent-pneumococcal-polysaccharide vac-cine. She was healthy with no fever, malaise, orweight loss. The lesion began as a tender erythem-atous papule at the site of vaccination and increasedin size with development of additional sites ofinvolvement. Bacterial and fungal cultures, tubercu-lin skin testing, blood interferon-gamma releaseassay for tuberculosis, blastomycosis and coccidio-mycosis serologies, cryptococcal antigen, and

Fig 1. Erythematous plaque studded with multiple hyper-keratotic papulonodules at site of vaccination.

Fig 2. A, Large polygonal pale staining histiocytes withfeathery cytoplasm containing numerous intracytoplas-mic inflammatory cells (emperipolesis). B, Large histio-cytes demonstrating strong S-100 protein expression.Inflammatory cells within cytoplasm remain unstainedand are surrounded by a clear halo. (A, Hematoxylin-eosin stain; B, S-100 stain; original magnification: A andB 3 400.)

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histoplasmosis urine antigen testing were all nega-tive. Treatment with rifampin, azithromycin, andmoxifloxacin was initiated for presumed atypicalmycobacterial infection without effect.

Physical examination revealed a large (20 3 20cm) erythematous plaque, with multiple hyperker-atotic papulonodules, involving the right arm(Fig 1). On the opposite arm, forearms, thighs,face, and ears, there were erythematous maculescoalescing into larger patches with overlying scale.Lymphadenopathy was absent. A biopsy specimenof the right arm revealed a mixed dermal infiltrate offoamy histiocytes, plasma cells, lymphocytes, neu-trophils, and large pale histiocytes with featheryborders demonstrating emperipolesis (Fig 2, A). Thelarge histiocytes were S-100 positive (Fig 2, B) andCD1a negative. There was no evidence of microor-ganisms on acid-fast bacilli, Gram, and periodicacideSchiffestained sections. Previous biopsy spec-imens from multiple smaller lesions demonstratedsimilar findings. No lymphadenopathy was notedon a computed tomographic (CT) scan of theabdomen, chest, and pelvis. Over the course of 3years under our care, the patient has shown partialimprovement with multiple therapies, includingtopical, intralesional, and systemic corticosteroids;dapsone; isotretinoin; and liquid nitrogencryotherapy.

Although the cause of RDD remains uncertain,clonality studies demonstrating a polyclonal infiltrateand the presence of functionally activated

macrophages support a reactive process ( possiblyto infection or following an immune challenge) overa neoplastic process.1,2 Infectious agents, includinghuman herpesvirus (HHV) 6, Epstein-Barr virus(EBV), parvovirus B19, herpes simplex virus,Brucella, Klebsiella rhinoscleromatis, and Nocardiahave been implicated on the basis of temporal asso-ciation and positive serology in some affected pa-tients.2 Additionally, tissue samples from severalpatients diagnosed with RDD have demonstratedHHV6 DNA on in-situ hybridization testing, and onecase of CRD was reported to contain HHV6 DNA onpolymerase chain reaction (PCR) testing of involvedtissue3,4 Other postulated immunologic triggers in-clude autoimmune disease, lymphoma, leukemia,HIV infection, as well as scars associated with priorsurgery and herpes zoster infection.2 Similarly, stim-ulation of the immune system by pneumococcalcapsular polysaccharidesmaypresent another triggerfor the development of CRD, producing an abnormalhistiocytic infiltrate analogous to prior cases of RDDand CRD. Therefore there is not a single infectious,

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immunologic, or neoplastic process that can produceRDD/CRD. Instead, the disease likely represents animmunologic reaction pattern that can be triggeredby various agents.

In summary, our patient developed CRD afterpneumococcal vaccination. As the eruption wastemporally related and initially localized to the siteof injection, we believe that an exaggerated immu-nological response to the vaccination is a likely causefor this case of CRD. Although, to our knowledge,RDD occurring post-vaccination has not been de-scribed previously, there has been a case of exag-gerated antibody response in a child with RDDfollowing rubella vaccination.5 Our experience sug-gests that vaccine-induced immune stimulation mayprovide an alternate trigger for inducing CRD.

Anastasia V. Bassis, BS,a Janet A. Fairley, MD,b

Richard T. Ameln, MD,c and Brian L. Swick, MDb,d

University of Iowa Carver College of Medicine, IowaCitya; University of Iowa Department of Derma-tology and Iowa City VA Medical Center, IowaCityb; Dermatology Associates, PLC, Ottumwac;and the University of Iowa Department of Pa-thology, Iowa Cityd

Funding sources: None.

Conflicts of interest: None declared.

Reprint requests: Brian L. Swick, MD, University ofIowa, Department of Dermatology, 200 HawkinsDr, 40025 PFP, Iowa City, IA 52245

E-mail: brian-swick@uiowa.edu

REFERENCES

1. Paulli M, Bergamaschi G, Tonon L, Viglio A, Rosso R, Facchetti F,

et al. Evidence for a polyclonal nature of the cell infiltrate in

sinus histiocytosis with massive lymphadenopathy (Rosai-Dorf-

man disease). Br J Haematol 1995;91:415-8.

2. Lu CI, Kuo TT, Wong WR, Hong HS. Clinical and histopathologic

spectrum of cutaneous Rosai-Dorfman disease in Taiwan. J Am

Acad Dermatol 2004;51:931-9.

3. Levine PH, Jahan N, Murari P, Manak M, Jaffe ES. Detection of

Human Herpesvirus 6 in Tissues Involved by Sinus Histiocytosis

with Massive Lymphadenopathy (Rosai Dorfman Disease).

J Infect Dis 1992;166:291-5.

4. Scheel MM, Barozzi P, Garber R, Maiorana A, Bonacorsi G, Artusi

T, et al. Sinus histiocytosis with massive lymphadenopathy:

Presentation as giant granuloma annulare and detection of

human herpesvirus 6. J Am Acad Dermatol 1997;37:643-6.

5. Sumaya CV, Cherry JD, Gohd R. Exaggerated antibody response

following rubella vaccination in a child with sinus histiocytosis

with massive lymphadenopathy. J Pediatr 1976;89:81-3.

doi:10.1016/j.jaad.2011.03.019