Rhinoscleroma: A Growing Concern in the United States?Mayo Clinic Experience

RAFAEL ANDRACA, M.D.,* RANDALL S. EDSON, M.D., AND EUGENE B. KERN, M.D.

Rhinoscleroma is a chronic, progressive, granulomatous infection of the upper airways caused by thebacterium Klebsiella rhinoscleromatis. Although most cases occur in developing countries, recentimmigration patterns have led to an increasing number of patients with rhinoscleroma in the UnitedStates. Rhinoscleroma may mimic various inflammatory and neoplastic processes, including leprosy,paracoccidioidomycosis, sarcoidosis, basal cell carcinoma, and Wegener's granulomatosis. Currenttherapy consists of a combination of surgical debridement and prolonged antimicrobial therapy.Rhinoscleroma should be added to the list of opportunistic infections that can occur in patients withhuman immunodeficiency virus.

In this retrospective study, we reviewed the medical litera­ture on rhinoscleroma (RS) from 1965 to the present and themedical records of patients who were diagnosed with ortreated for RS at the Mayo Clinic from January 1900 toNovember 1991. Only two such patients were born in theUnited States (cases 5 and 6); the others were from aroundthe world.

Of the six Mayo Clinic patients who were identified ashaving RS, three had a definite diagnosis (cases 1,2, and 3),two had a probable diagnosis (cases 4 and 5; the diagnosiswas never fully established, but they responded to treat­ment), and one (case 6) had a possible diagnosis. For theMayo Clinic cases, RS was diagnosed on the basis of posi­tive results of culture, report of pathologic abnormalities, orpositive results of an immunoperoxidase test.

The following case reports illustrate the initial clinicalmanifestations, course, and treatment of RS (Tables 1through 5).

From the Department of Otorhinolaryngology (RA., E.B.K.) and DivisionofInfectious Diseases and Internal Medicine (RS.E.), Mayo Clinic Roches­ter, Rochester, Minnesota.

*Current address: Hospital Central Sur de Alta Especialidad, Mexico City,Mexico.

Address reprint requests to Dr. R S. Edson, Division ofInfectious Diseases,Mayo Clinic Rochester, 200 First Street SW, Rochester, MN 55905.

REPORT OF ILLUSTRATIVE CASESCase 1.-A 33-year-old woman was admitted to the MayoClinic in July 1946. Seven years earlier, RS had beendiagnosed while she was living in Shanghai. Treatment withKlebsiella rhinoscleromatis vaccine was unsuccessful. Shehad a history of a spontaneous pneumothorax in 1938 and oftuberculosis in 1941 (managed with thoracotomy andpneumonectomy). In addition, she had a 15-year history ofnasal crusting, stuffiness, purulent nasal discharge, tinnitus,and a 15.9-kg weight loss in 10 years. Three months beforeadmission, she began to have increasing dysphagia andhoarseness.

On physical examination, the patient had nasal obstruc­tion with bloody secretions, cicatricial tissue, and atrophy ofthe nasal mucosa. Moreover, scarring of the pharynx andgranulomatous tissue in the epiglottis were noted. The diag­nosis of rhinoscleroma was confirmed by culture and biopsy,both of which had positive results. Treatment with strepto­mycin (1 g/day for 65 days) yielded a good response and anuneventful recovery. After almost 1 year of follow-up, noevidence of reactivation of the disease was noted.

Case 3.-A 39-year-old man from the Soviet Union cameto the Mayo Clinic in December 1944. Eight years previ­ously, he had undergone surgical correction for a 10-yearhistory of chronic sinusitis and a nasal septal deviation. He

Mayo Clin Proc 1993; 68:1151-1157 1151 © 1993 Mayo Foundation for Medical Education and Research

1152 RHINOSCLEROMA Mayo CIiDProc,December 1993, Vol 68

Table I.-Demographic Data for Six Mayo PatientsWith Rhinoscleroma, Stratified by Type of Diagnosis

Table 2.-Medical Histories of Six Mayo PatientsWith Rhinoscleroma

had a 3-year history of nasal crusting associated with foultaste and smell, a 6.8-kg weight loss during a 2-year period,and progressive hoarseness, dyspnea, and cough.

Findings on physical examination were laryngeal andsubglottic obstruction in conjunction with scarring, cicatri­cial tissue, and 'atrophy of the larynx. Results of culture werepositive, but the biopsy results were doubtful because of thelarge amount of scarring and cicatricial tissue ..The patientwas treated with irradiation (224 rad) for one session, fol­lowed by two sessions of electrocoagulation and topicalapplication of acriflavine for 3 months. During a 4-yearfollow-up, he was without evidence of recurrence. He diedin 1951 of unrelated causes.

REVIEW OF LITERATURE t

RS is a chronic, slowly progressive, granulomatous diseasethat affects the upper and lower airways and has a preferencefor the nose. It has a limited capacity to spread throughoutthe body and is extremely difficult to cure.

History.-RS (or scleroma) has affected humanity formore than a thousand years. Early evidence of this diseasewas found on a small terra-cotta head from the Mayan cul­ture (Guatemala), dating from AD 300 to 600,! with thecharacteristic Hebra nose. In more recent times, moulagesmade in Krakow, Poland, by Bierkowski in 1840 alsoshowed features suggestive of RS. Ferdinand von Hebra ofVienna provided the first complete description of the dis­ease. Although von Hebra and Kaposi considered the dis­ease of neoplastic origin, Geber and Mikulicz thought that itwas of inflammatory origin.' In 1882, von Frisch describedthe causative organism now known as K. rhinoscleromatis.'In 1932, at the Second International Congress of Oto­rhinolaryngology held in Madrid, Spain, a change in thename ofthe disease from "rhinoscleroma" to "scleroma" wassuggested, to emphasize that the disease can involve most ofthe respiratory tract.' Alternatively, however, retention of

33 F Siberia Shanghai 7/8/4633M Guadalajara, Mexico l/23/5939M SovietUnion ? 12/5/44

23M Arequipa, Peru 4/2l/8236 F Texas,USA 9/28/75

32M Oklahoma, USA 1120/40

Geographic data

the prefix "rhino-" has been considered useful for avoidingconfusion with scleroderma or multiple sclerosis."

Etiology.-Since von Frisch's description of the bacillusfound in the lesions of RS, extensive data have been pre­sented about the role of this bacterium in the pathogenesis ofRS. Although earlier studies suggested two types of caus­ative organisms," not until 1961 (when all Koch's postulateswere fulfilled") was a better understanding of K.rhinoscleromatis possible. K. rhinoscleromatis is a gram­negative, encapsulated, nonmotile, glucose-fermenting dip­lobacillus. It is also a facultative anaerobe that can growintracellularly or extracellularly'' and whose only host ishumans. Its antigenic formula is 02K3' in which °is thesmooth somatic antigen and K is the capsular antigen." Thislatter antigen forms the basis of the immunoperoxidase test.It can persist in the human host for months, years, and evendecades if untreated.

Epidemiology.-RS, a global disease, has been reported.in more than 68 countries (primarily in the tropics) as bothsporadic and endemic cases." More than 80% of the reportedcases have occurred in five endemic foci;'? eastern andcentral Europe (Poland, Hungary, Russia, and Romania);Mexico, Central America, and South America (Brazil, Co­lombia, Cuba, El Salvador, Guatemala, Nicaragua, and Ven­ezuela); Africa-where 5% of the world's cases have beenfound!' (East Africa, Egypt, and the region of LakeVictoria); India; and Indonesia.P''? The disease does nothave a uniform distribution, affecting mainly rural areas.

*See text for detailedcase report.

1* Pneumothorax in 1938Tuberculosis diagnosedin 1941 (treatedwith

thoracotomy and pneumonectomy)

2 Medical student

3* Eight years beforeadmission: surgicalcorrectionfor a10-yearhistoryof chronicsinusitisand septal defect

4 Allergicto penicillinPapillomain the soft palate in 1978(treatedwith

cryosurgery)Schizophrenia-paranoia diagnosedin 1982

5 Tonsillectomy in 1928Diabetessince 1954Hysterectomy and radiotherapy for cervicalcancer in

1963Pemphigus since 1964Tracheostomies in 1954,1958,and 1968becauseof

tracheal stenosis

6 Malaria in 1930(treatedwith chloroquine)Chronicprostatitisin 1938

Case HistoryDate of~ssion

Residence (mo/day/yr)Birth­place

Age (yr)and sex

Definite123

Probable45

Possible6

Case

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Table 3.-Initial Symptoms and Signs in Six Mayo PatientsWith Rhinoscleroma

Case* No. ofSymptom or sign 2 3t 4 5 6 patients

Crusts + + + + + + 6Stuffiness + + + + + 5Foul taste and smell + + + + + 5Hyposmia + + + + 4

.Hoarseness + + + + 4,-----Purulent discharge 4+ + + +Bloody discharge + + + 3Epistaxis + + 2Cough + + 2Dysphagia + + 2Dyspnea + + 2Tinnitus + IAdenopathy + 1Lacrimal involvement + 1Weight loss + + + 3

(kg) (15.9):j: (6.8)§ (9)f Mean loss,23.3

Duration (yr) 15 2 3 7 2mo 21 Mean, 8

*Plus sign indicates presence of sign or symptom.t'This patient had primary laryngeal scleroma at time of initial assessment.:j:In 10 years.§In 2 years.§In 12 years.

Most of the sporadic cases are due to migration from en­demic regions. l3 The frequency in an endemic zone ofPoland was approximately 0.5%.10

Until recently, RS was rarely reported in the UnitedStates. Before 1968, RS had been diagnosed in approxi­mately 4,000 patients worldwide, only 171 of whom re­ceived the diagnosis in the United States (47 of these patientswere born in the United States). From 1966 to 1980, 24

cases were diagnosed in the United States, and only 6 of thepatients were born in the United States." Because of thescarcity of RS, the Centers for Disease Control and Preven­tion in Atlanta, Georgia, has not maintained statistics on it.This situation will likely change in the near future becausethe United States currently has many immigrants from en­demic nations and also because of the increased US militarypresence in Central America and the Middle East.5•14 The

", Table 4.-Findings on Physical Examination in Six Mayo PatientsWith Rhinoscleroma

Case

Finding 2 3 4 5 6

Obstruction Nose Nose Larynx, Nose Nose Nosesubglottis

Cicatricial Nose Larynx Nose,tissue larynx

Scarring Pharynx Interarytenoid Septum Larynx,area trachea

Granulomatous Epiglottis Nose Larynx, Middle Nosetissue subglottis turbinate

Adenopathy CervicalCrusting Nose Nose Nose NoseAtrophy Nasal Laryngeal Nasal

mucosa mucosa mucosaBloody

secretions Yes Yes Yes

1154 RHINOSCLEROMA Mayo Clin Proc, December1993,Vol 68

Table 5.-Method of Diagnosis, Treatment, and Follow-Up Data for Six Mayo Patients With Rhinoscleroma

Case

2

3

4

5

6

Diagnosis

Shanghai, 1939: biopsy + cultureMayo, 9/13/46: biopsy + culture

Mexico, 1110/59: biopsy + culture

Mayo, 12/5/44: biopsy doubtful,culture positive

Mayo, 4/21182: biopsy, chronicinflammation; culture positivefor Staphylococcus aureus andCorynebacterium spp

Mayo,9/29/75: biopsy, chronicinflammation; culture positivefor Klebsiella spp

Mayo, 1123/40: biopsy, chronicinflammation; no culture resultreported

Treatment

Vaccine, 1939Streptomycin, 1 g/day

intramuscularly, 9/22/46-11125/46(65 days)

Radiotherapy, 1959 (1,000 rad twice)+ neomycin (dose?), 3 mo beforecoming to Mayo

Radiotherapy, 12/10/44 (224 rad)Electrocoagulation, 12/18/44 and

Feb 1945Topically applied acriflavine, Feb 1945

Only reassurance

Tracheal stoma, 6/4/75Nasal valve operation, 10/26/81Tracheal end-to-end anastomosis, 3/10/86Segmental tracheal resection + primary

closure, 7/28/86

Radiotherapy (300 rad), three occasions;1/24/40, 1/27/40, and 1130/40

Potassium iodide, 25 drops three timesa day

Follow-up

11/25/46,3/18/47,7/25/47: biopsy andculture negative

None

Feb 1945, Dec 1945, Feb 1947, Jan1949: all cultures and biopsiesnegative

Died 1951

None

Died, 8/13/86

None

number of cases has already increased in the southern borderstates; for example, the Los Angeles County-University ofSouthern California Medical Center now examines a meanof six patients with RS per year.'

The person-to-person transmission of this disease mostlikely occurs through airborne secretions and requires pro­longed intimate contact, similar to Hansen's disease (lep­rosy). The requirements for transmission include activedisease," subjects living in close quarters," and presence ofpredisposing factors. IS The most common predisposing fac­tors include low socioeconomic conditions, poor hygiene,poor nutrition, and young age (typically, persons in the sec­ond or third decade of life). RS also tends to affect womenslightly more often than men (female:male ratio, 1.3:1),12 Inaddition, iron deficiency" and immunologic factors maypredispose to the acquisition of RS.

Pathophysiology.-Although the pathogenesis of RS isnot clearly established, the disease process probably beginsat the junction between two epithelia-the stratified squa­mous epithelium of the vestibule and the respiratory epithe­lium of the nose. Pharyngoscleroma begins at the junctionbetween the respiratory and squamous epithelia of the phar­ynx, whereas laryngoscleroma arises below the vocal cordswhere the stratified squamous epithelium adjoins the respira­tory epithelium in the subglottic area.'? In otoscleroma, the

disease begins between the respiratory epithelium and theflattened nonciliated epithelium ofthe middle ear."

Iron deficiency alters epithelial regeneration and thuscauses squamous metaplasia (a common finding in RS).Because both menstruating and pregnant women with RSseem to have a more severe course than do other patients,iron deficiency can be considered a predisposing factor inRS by altering the normal integrity of the epithelium."

Many theories have been proposed to explain thepathophysiologic aspects of this disease, but most investiga­tors agree that the mucopolysaccharide in the capsule of thebacterium may be responsible for most of the damage. Thecapsule protects the bacteria by inhibiting phagocytosisthrough a complex series of events. 19,20

The humoral immunologic response in patients with RS isnormal;" however, cellular immunity seems impaired inthese patients. The granulomas in RS are ineffective histio­cytic granulomas-that is, the macrophages are inactivatedand cannot transform into epidermoid cells that kill the bac­teria." A person with RS exposed to antigens from variousstrains of K. rhinoscleromatis responds with an increase inserum antibodies but does not form antibodies to the infect­ing strain of Klebsiella." seemingly demonstrating a patternof "tolerance" to the disease. The CD4:CD8 ratio is alteredin RS: the CD4 lymphocytes are decreased and the CD8

Mayo Clin Proc, December 1993, Vol 68

lymphocytes are increased. In addition, CD4 lymphocytesshow a minimal response to a stimulus with interleukin 2.This lack of response could explain why histiocytic granulo­mas are formed instead of epidermoid cell granulomas.Thus, the disease causes some type of acquired cellularirnmunodeficiency.t':"

RS is believed to be spread by contiguity, the lymphaticsystem, or direct contact. The squamous metaplasia noted inRS may protect from an intense contiguous disseminationand thus may explain the long incubation period of thedisease.v" Lymphatic spread can be limited by the fibroustissue surrounding the granuloma; this feature also explainswhy cervical lymphadenopathy is an uncommon clinicalfinding.P-"

Pathology.-The histologic characteristics of the lesionsof RS depend on the clinical stage of the disease. Clinicallyand pathologically, RS has been classified into three or fourstages: catarrhal, atrophic (this stage is sometimes consid­ered part of the catarrhal stage), granulomatous, and scle-

, rotic. In the catarrhal-atrophic stage, the typical finding isepithelial squamous metaplasia in conjunction with asubepithelial infiltrate of polymorphonuclear cells and somegranulation tissue." During the granulomatous stage (themost characteristic stage), pseudoepitheliomatoushyperplasia (hypertrophy of the epithelium by downwardgrowth) and a chronic inflammatory reaction with manymonocytes, lymphocytes, and histiocytes are evident. Occa­sionally, the infiltrate tends to be angiocentric and to cause avasculitis-like picture with hyalinization of the vessels."Some studies have also reported the existence of amyloid­like deposits, which are thought to be caused by the chronicinfection." Characteristically, Russell bodies are found dur­ing the chronic, granulomatous stage.

Mikulicz cells can be found beneath the basal lamina;they are large histiocytes that have a single nucleus withdiffuse chromatin. The nucleus is displaced to the peripherybecause of the numerous vacuoles in the cytoplasm. Viablebacilli may be present in some of these vacuoles. WithinMikulicz cells, two types of granules-"A" and "B"-havebeen found. Type A granules are mainly in vacuoles thatcontain bacilli and are believed to be a large quantity ofantibodies against them, whereas type B granules arethought to be a cluster of antigens with a large amount ofmucopolysaccharide inside them."

Alternatively, the Russell bodies are structures in thecytoplasm of the plasma cells. These bodies are 20 to 40 urn,homogeneous, and extremely eosinophilic.

The sclerotic stage manifests with large amounts of fi­brous and cicatricial tissue, residual stenosis, and few or noplasma cells, Mikulicz cells, or Russell bodies.

Clinical Manifestations.-RS is a slowly progressive,granulomatous, infectious disease. Although it can be dis-

RHINOSCLEROMA 1155

figuring, it is rarely lethal, unless it obstructs either thelarynx or the trachea. It affects primarily young persons inthe second or third decade of life. Because the diseaseprogresses slowly, the initial manifestations can be detectedin adolescence. As already described, the disease can beclassified into three or four clinical stages.

The Catarrhal-Atrophic Stage.-The catarrhal-atro­phic stage manifests as purulent, fetid rhinorrhea of longduration (weeks or months), unilateral or bilateral nasal ob­struction, and crusting (although not as intense as withWegener's granulomatosis). ' Physical examination maydemonstrate atrophy and crusting of the nasal mucosaor interarytenoid hyperemia and exudates if the larynx isinvolved.

The Granulomatous Stage.-The granulomatous stageis characterized by epistaxis, nasal deformity (depending onthe duration), labial edema, involvement of the paranasalsinuses, hoarseness (if the larynx is affected), anosmia, anes­thesia of the soft palate, and epiphora (affecting the lacrimalapparatus). On physical examination, the initial finding willbe a bluish red and rubbery granulomatous lesion, whichsubsequently evolves into a pale and indurated gran­ulomatous mass. Bony destruction and, in some cases, cervi­cal node involvement can occur."

The Sclerotic Stage.-The sclerotic stage is similar tothe granulomatous stage but with increased deformity andstenosis. Physical examination may demonstrate gran­ulomatous lesions surrounded by dense fibrotic tissue.

Pattern of Involvement and Related Prognosis.-RScan begin anywhere in the respiratory tract. The nose isaffected in 95 to 100% of cases, the pharynx in 18 to 43%,the trachea in 12%, and the bronchi in 2 to 7%.17,20,30.33 Theparanasal sinuses can also be affected (26.4% of cases)y,34Rare sites of involvement include the eustachian tube," themiddle ear (manifesting as otorrhea and anakusis"), and oneor both eyes (blindness, lacrimal dysfunction.v-r'-" and eventhe orbital apex syndrome"). Skin involvement occurs pri­marily on the upper lip, the nasal dorsum, and, rarely, theback.F-" Cerebral dissemination has also been described."

The earlier the diagnosis of RS, the better the prognosis.Unfortunately, patients tend to consult a physician when thelesions arein an advanced stage. In one study," the signsand symptoms when patients were initially examined bytheir physicians included nasal obstruction in 94%, nasaldeformity in 32%, hoarseness in 12%, epistaxis in 11%,swelling ofthe lip in 10%, sore throat in 6%, and epiphora in4%.

Complications.-Malignant degeneration has been de­scribed" but poorly documented in RS. The most commoncomplication is relapse," which necessitates prolongedtherapy. Stenosis is another common complication; it occursbecause of cicatricial tissue and can be fatal if the upper

1156 RHINOSCLEROMA

airways become obstructed. This complication necessitatestracheostomy and surgical debridement."

Differential Diagnosis.-Various granulomatous pro­cesses of the head and neck can mimic RS. Infectious,neoplastic, and autoimmune diseases are included in thedifferential diagnosis. Bacterial diseases such as tuberculo­sis, actinomycosis, syphilis, and leprosy can all producegranulomas in the upper airways. Fungal infections such ashistoplasmosis, blastomycosis, paracoccidioidomycosis, andsporotrichosis can also produce similar lesions, but the use ofappropriate cultures or silver stains can help establish anaccurate diagnosis. Mucocutaneous leishmaniasis can alsoproduce granulomatous lesions that mimic RS.5,1I

Malignant lesions such as the usual lymphomas of thenose, unusual lymphomas such as T-cell lymphoma (for­merly called polymorphic reticulosis), and sinushistiocytosis in conjunction with cervical adenopathy cansimulate RS. 41 Sarcoidosis and vasculitis, especiallyWegener's granulomatosis, should be considered in the as­sessment of a patient with a possible diagnosis of RS.

Diagnosis.s-:To establish an accurate diagnosis, the clini­cian must obtain a complete history from the patient, includ­ing birthplace and residence, family history, hygienic andnutritional habits, and duration of the disease .. A thoroughphysical examination is also helpful, especially when thelesions are in the catarrhal-atrophic or granulomatous stage.

Biopsy and bacterial culture remain the most helpful toolsfor diagnosis. Routine cultures on blood or MacConkey agarwill be positive for K. rhinoscleromatis in 50 to 60% ofpatients."

Imaging techniques, especially computed tomography,can help establish the extent of the disease.'? Likewise,endoscopy can provide valuable information about the ex­tent of RS and can also be useful in its treatment. 33

Biopsy results, although not pathognomonic for RS, arehelpful and are characterized by Mikulicz cells, Russell bod­ies, pseudoepitheliomatous hyperplasia, and squamousmetaplasia; when these are considered in combination withthe results of immunoperoxidase staining, specificity ap­proaches 100%.13,39 Other useful stains include periodicacid-Schiff," Giemsa, and Warthin-Starry. Serologic stud­ies such as the complement-fixation test have cross-reac­tions, but they can be of help in assessing the response totreatment.42

Treatment.-RS is difficult to treat, and the relapse rate ishigh. Numerous treatment options have been described.Initial therapy included mercurials, caustics (such as zincchloride, silver nitrate, and salicylic acid), cautery, arseni­cals, and methylene blue. Radium and radiotherapy werelater used. Recently, antibiotics, ranging from systemicallyadministered streptomycin to locally administered rifampinor acriflavine, have been used. Surgical debridement has

Mayo Clin Proc, December 1993, Vol 68

always played a part in treatment, and laser therapy is cur­rently one of the best surgical methods." Even a vaccine hasbeen proposed as a potential therapeutic modality." Despitethe many treatment options, relapse is common, and oftenthe symptoms can only be mitigated and the evolution of thedisease restricted."

Radiotherapy for RS was used during the first half of thiscentury, primarily before the advent of antimicrobial agents.This type of therapy provided symptomatic relief for longperiods (months to years), but most patients eventually had arelapse.

Currently, treatment typically is a combination of pro­longed antimicrobial therapy and surgical debridement. An­timicrobial therapy must be given for prolonged periods­from months to years in some cases." In the absence ofcontrolled clinical trials, treatment is guided by antimicro­bial susceptibility data." The systemically administered anti­biotics that have been used are streptomycin, tetracycline,rifampin, second- and third-generation cephalosporins, andagents such as sulfonamides and clofazimine." Antimicro­bial agents have been used alone or in combination.

Streptomycin has been used as a single agent to treat RSwithout involvement of any site other than the nose, but thehigh doses needed ultimately produced ototoxicity andnephrotoxicity. When used in combination with tetracy­clines or other antibiotics, the dose of streptomycin can bedecreased; thus, toxicity is reduced." Tetracyclines are areasonable choice because they act intracellularly and areinexpensive; however, their use is contraindicated in chil­dren, infants, or pregnant women.P-" Agents such asclofazimine were recently reported to yield promising thera­peutic results, mainly if used in the early (catarrhal-atrophicand granulomatous) stages of the disease." Rifampin hasproduced microbiologic resolution of the disease, but it mustbe used for prolonged periods." Second- and third-genera­tion cephalosporins are effective against K. rhinoscleromatisbut necessitate parenteral therapy; thus, long-term outpatientuse is limited.' Despite the absence of published clinicaltrials, the new orally administered fluoroquinolone antimi­crobial agents have excellent activity against gram-negativebacteria, low toxicity, and limitations similar to those of thetetracyclines. Topical therapy with acriflavine (an acridinederivative used for treating giardiasis) or rifampin has shownpromising results; response rates have exceeded the usualresponse rate of 60 to 70%.20,25,43-45

In patients with RS who have cosmetic deformity orfunctional obstruction of the airway, surgical therapy is indi­cated. Operation should be delayed either until the diagnosisis definite (through biopsy or culture) or until no residualdisease activity is evident in the tissue to be debrided. Ifdisease activity exists, medical treatment should be institutedand completed before operation because the risk of recur-

Mayo Clio Proc, December 1993, Vol 68

renee or dissemination is substantial. 12•26.45.46 Laser therapymay be used when debridement is necessary; it will decreasethe amount of cicatricial tissue and thus minimize dissemina­tion of the disease and postoperative edema.8.9.47

ADDENDUMAfter our manuscript was submitted for publication, Pauland associates" described two patients with human immuno­deficiency virus (HIV) infection and RS. This report pro­vides further evidence of the association between RS andaltered T-cell immunity. RS should be added to the list ofopportunistic infections that can potentially occur in HIV­positive patients who either visit or reside in endemic areas.

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