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inflammation, neoangiogenesis, and fibroblasticproliferation.4

The absence of any serious side effects in ourstudy could be explained by the fact that pulsesteroid therapy has been found to have a highersafety profile than daily steroids.2 Furthermore, bothsirolimus (2 mg/day)5 and octreotide4 are regardedas drugs with reasonable safety margins and minimaldrug interactions.

Accordingly, this study offers a possible effectiveand safe therapeutic strategy for this recalcitrant andpotentially fatal disorder. Still larger scale studies withlonger follow-up are needed to confirm the efficacyand safety profiles of this triple therapy in cases of SM.

Mohammad Ali El-Darouti, MD, Rehab Aly Hegazy,MD, Marwa Mohamed Fawzy, MD, Sara BahaaMahmoud, MD, and Dina Ahmed Dorgham, MSc

From the Dermatology Department, Faculty ofMedicine, Cairo University, Egypt

Funding sources: None.

Conflicts of interest: None declared.

Correspondence to: Sara Bahaa Mahmoud, MD, 16El Shaheed Abd El Moneim Hafez-Almaza-Heliopolis-Cairo, Egypt

E-mail: bahaa.sara@yahoo.com

REFERENCES

1. Heymann WR. Scleromyxedema. J Am Acad Dermatol

2007;57:890-1.

2. EL-Darouti MA,Mashaly HM, EL-Nabarawy E, EL-Tawdy AM, Fawzy

MM, Salem DS, et al. Comparative study of the effect of a daily

steroid regimen versus a weekly oral pulse steroid regimen on

morphological changes, blood sugar, bone mineral density and

suprarenal gland activity. J Dermatolog Treat 2012;23:4-10.

3. Swaminathan S, Arbiser JL, Hiatt KM, High W, Abul-Ezz S, Horn

TD, et al. Rapid improvement of nephrogenic systemic

fibrosis with rapamycin therapy: possible role of phospho-70-

ribosomal-S6 kinase. J Am Acad Dermatol 2010;62:343-5.

4. Cozzi R, Attanasio R. Octreotide long-acting repeatable for

acromegaly. Expert Rev Clin Pharmacol 2012;5:125-43.

5. Su TI, Khanna D, Furst DE, Danovitch G, Burger C, Maranian P,

et al. Rapamycin versus methotrexate in early diffuse systemic

sclerosis: results from a randomized, single-blind pilot study.

Arthritis Rheum 2009;60:3821-30. http://dx.doi.org/10.1016/j.

jaad.2013.07.043.

http://dx.doi.org/10.1016/j.jaad.2013.07.043

Emperipolesis is not pathognomonic forRosai-Dorfman disease: Rhinoscleromamimicking Rosai-Dorfman disease, a clinicalseries

To the Editor: Rhinoscleroma is a chronic granulom-atous infection of the upper respiratory tract, partic-ularly affecting the nose, caused by the bacterium

Klebsiella pneumoniae subspecies rhinoscleromatis.In contrast, Rosai-Dorfman disease (RDD) is anuncommon histiocytic proliferative disorder withcharacteristic emperipolesis but unknown origin,although immune dysregulation might be involved.Several reports described patients who were previ-ously diagnosed with rhinoscleroma and developedRDD de novo or nodal RDD several years later.1-3 Wepresent 3 cases of rhinoscleromawith emperipolesis,a characteristic feature of RDD.

Case 1, a 57-year-oldman, presentedwith 1 tendernodule on right perioral skin for 2 weeks. Neithercervical lymphadenopathy nor fever was observed.Clinical impression was an inflamed epidermal cyst.An incision revealed only minimal serous discharge.Further skin biopsy specimen showed mixed inflam-matory cells, some of which were phagocytosed byfoamy histiocytes (emperipolesis) (Fig 1,A). Becausecommon clinical features of fever and cervical lym-phadenopathy were absent, the diagnosis of RDDwas questioned. Further examination showed thesefoamy histiocytes negative for S-100 and CD1a, butpositive for CD68, contradicting the diagnosis ofRDD. Giemsa stain revealed aggregated short rodbacilli within the histiocytes (Fig 1, B). The tissuecultures grew K pneumoniae. The diagnosis ofrhinoscleroma was rendered based on the demon-stration of bacilli, CD681 histiocytes, and growthfrom the tissue culture.

Case 2, a 57-year-old man, presented with anodule on left perioral skin. Pathological examina-tion showed abscess formation with prominent em-peripolesis. S-100 and CD1a stains were negative,although Giemsa stain revealed many bacilli withinhistiocytes.

The nodules of cases 1 and 2 shrank after treatmentwith oral trimethoprim/sulfamethoxazole, whichwasselected according to antimicrobial susceptibility test-ing. No sign of recurrence was observed after 1 year.

Case 3, a 32-year-old man, had a recurrent nasalmass for 8 years. Pathologic examination revealedgranulomatous inflammation with foamy histiocytesand emperipolesis. Giemsa staining revealed diplo-cocci within histiocytes. They were positive for CD68but negative for S-100 and CD1a. Bacterial culturesyielded K rhinoscleromatis.

Rhinoscleroma and RDD actually share similari-ties, including a predilection for the respiratory tractand cervical lymph nodes, protracted courses inyounger patients, biclonal plasma cell infiltrate, andspecial histiocytes.2 A high antibody titer againstKlebsiella species was reported in a typical case ofRDD.4 Recently, coexistence of rhinoscleroma andRDD was reported at the same site from a patient.1

Emperipolesis has been reported in diseases

Fig 1. A, Emperipolesis. Numerous neutrophils, plasma cells, and erythrocytes, many of whichare phagocytosed by aggregates of S-100� histiocytes in the dermis and upper subcutis.(Hematoxylin-eosin stain; original magnification:3400.) B, Giemsa stain revealed short rods ofbacilli aggregated within the foamy cytoplasm of histiocytes, consistent with Mikulicz cells.Bacterial cultures yielded Klebsiella pneumoniae. (Giemsa stain, oil immersion; originalmagnification: 31000.)

Table I. Criteria to be scheduled into the urgentcare clinic

Immunocompromised patientAcute skin problem (\1 mo)Eruption of vesicles or blistersSuspected severe drug reactionNew or changing mole strongly suspicious for melanoma

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other than RDD, including recently described Hsyndrome, cancers, and several brain pathologies.However, RDD is the only condition to demonstrateemperipolesis and a distinctive S-100/CD681 andCD1a� immunohistochemical profile.5

Distinguishing rhinoscleroma from RDD by ap-plying S-100, CD1a, Giemsa stain, and microbiologiccultures in patient care is crucial. The treatments forRDD include steroids or simply waiting for self-regression, whereas treatments for rhinoscleromausually involve antibiotics and surgical treatments orotherwise facing possible destructive consequences.RDD should be a diagnosis of exclusion, even in thepresence of characteristic emperipolesis. Clinicalawareness, Giemsa staining, and tissue culturesshould be considered in future cases of RDD.

Tsai-Ching Chou, MD,a Kun-Bow Tsai, MD,b andChih-Hung Lee, MD, PhDc

Departments of Dermatologya and Pathology,b

Kaohsiung Municipal Hsiao-Kang Hospital andKaohsiung Medical University Hospital; Depart-ment of Dermatology, Kaohsiung Chang GungMemorial Hospital and Chang Gung UniversityCollege of Medicine, Kaohsiung,c Taiwan,Republic of China

Funding sources: None.

Conflicts of interest: None declared.

Correspondence to: Chih-Hung Lee, MD, PhD, De-partment of Dermatology, Kaohsiung ChangGungMemorial Hospital and Chang Gung UniversityCollege of Medicine, 123 Dapi Rd, Niaosng Dist,Kaohsiung City 83301, Taiwan, Republic of China

E-mail: dermlee@gmail.com

REFERENCES

1. Kumari JO. Coexistence of rhinoscleroma with Rosai-Dorfman

disease: is rhinoscleroma a cause of this disease? J Laryngol

Otol 2012;126:630-2.

2. Kasper HU, Hegenbarth V, Buhtz P. Rhinoscleroma associated

with Rosai-Dorfman reaction of regional lymph nodes. Pathol

Int 2004;54:101-4.

3. Chen QR, Yang F, Wang MW. Rosai-Dorfman disease misdiag-

nosed as rhinoscleroma: a case report [in Chinese]. Zhonghua

Er Bi Yan Hou Tou Jing Wai Ke Za Zhi 2010;45:338-9.

4. Lampert F, Lennert K. Sinus histiocytosis with massive lym-

phadenopathy: fifteen new cases. Cancer 1976;37:783-9.

5. Nishie M, Mori F, Houzen H, Yamaguchi J, Jensen PH,

Wakabayashi K. Oligodendrocytes within astrocytes (‘‘emperi-

polesis’’) in the cerebral white matter in hepatic and hypogly-

cemic encephalopathy. Neuropathology 2006;26:62-5.

http://dx.doi.org/10.1016/j.jaad.2013.08.036

Dermatology urgent care clinic: A survey ofreferring physician satisfaction

To the Editor: Access to and satisfaction with dermato-logic care are critical concerns givenprovider shortagesand patient dissatisfaction with current lengthy waittimes. Estimates of average wait times for new ap-pointments range from 33 to 38 days1-3; 25% reportedthe average wait to be more than 60 days, and mayexceed 120 days in certain states.2 More alarming,patient acuity appears not to impact wait time.1,3