Malacoplakia of the Retroperitoneum in a Girl with Systemic Lupus Erythematosus

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ventilation of infants of less than 1501 gm birth weight:

Health, growth and neurologic sequelae. J Pediatr 88:531,

1976

9. Krishnamoorthy KS, Fernandez RA, Momos KJ, et al:

Eval-uation of neonatal intracranial hemorrhage by computerized

tomography. Pediatrics 59:165, 1977

10. Papile LA, Munsick G, Weaver N, et al: Cerebral

intraven-tricular hemorrhage (CVH) in infants <1500 grams:

Devel-opmental follow-up at one year. Pediatr Res 4:528, 1979 1 1. Dykes FD, Lazzara A, Ahmann P, et al: Intraventricular

hemorrhage: A prospective evaluation of etiopathogenesis.

Pediatrics 66:42, 1980

12. Ballard JL, Kazmaier K, Driver M: A simplified assessment

of gestational age. Pediatr Res 1 1:374, 1977

13. Lubchenco LO, Hansman C, Boyd E: Intrauterine growth:

Weight, length and head circumference as estimated from

live births at gestational ages from 26 to 42 weeks. Pediatrics

37:403, 1966

14. Amiel-Tison C: Neurologic evaluation of the maturity of

newborn infants. Arch Dis Child 43:89, 1968

15. Saint-Anne Dargassies S: Neurodevelopmental symptoms

during the first year of life. I. Essential landmarks for each

key-age. II. Practical examination and the application of this

assessment method to the abnormal infant. Dec Med Child

Neurol 14:235, 1972

16. Bayley N: Bayley Scales oflnfant Development. New York,

The Psychological Corp., 1969

17. Kitchen WH, Ryan MM, Richards A, et al: A longitudinal

study of very low-birthweight infants. Intraventricular hem-orrhage: An overview of performance at eight years of age.

Dev Med Child Neurol 22:172, 1980

18. Davies PA, Stewart AL: Low-birth-weight infants:

Neuro-logic sequelae and later intelligence. Br Med Bull 31:85, 1976

19. Drillien CM, Thomson AJM, Burgoyne K: Low-birthweight

children at early school-age: A longitudinal study. Dev Med

Child Neurol 22:26, 1980

20. Fitzhardinge P, Ramsey M: The improving outlook for the

small prematurely born infant. Dev Med Child Neurol

15:447, 1973

21. Drillien CM: Abnormal neurologic signs in the fu-st year of life in low-birthweight infants: Possible prognostic

signifi-cance. Dev Med Child Neurol 14:575, 1972

22. Krishnamoorthy KS, Todres ID, Kuehnles KJ, et al:

Neu-rologic sequelae in the survivors of neonatal intraventricular

hemorrhage, in Perinatal Intracranial Hemorrhage

Con-ference Syllabus. Columbus, OH, Ross Laboratories Publi-cations, 1980, pp 697-710

23. Schechner 5, Ross G, Auld P: Developmental follow-up at

one year post-term of infants with intracranial hemorrhage, in Perinatal Intracranial Hemorrhage Conference

Sylla-bus. Columbus, OH, Ross Laboratories Publications, 1980,

pp 724-735

Malacoplakia

of the

Retroperitoneum

in a Girl

with

Systemic

Lupus

Erythematosus

Malacoplakia is a rare chronic inflammatory

dis-ease, usually involving the bladder but occasionally

affecting other organs. However, only two cases of

malacoplakia of the retroperitoneum and ureter

have been reported in the English language

litera-ture.”2 Herein, we describe the third case of

mala-coplakia of the retroperitoneum and ureter in a

patient with systemic lupus erythematosus who had

been receiving prednisone therapy for two years.

Modification by the steroid of phagocytosis and of

the normal inflammatory response to bacterial

in-fection, and the systemic lupus erythematosus, may

have been of major importance in the genesis of

malacoplakia in this patient.

CASE REPORT

A 14-year-old girl was hospitalized in 1978 at the age of

12 years because of a two-month history of joint pains

and fever. At that time she had a typical butterfly rash,

which involved the malar areas and extended over the

Reprint requests to (M.S.A.) Pediatric Services Division,

Dhah-ran Health Center, Box 76, Dhahran, Saudi Arabia.

American Academy of Pediatrics.

bridge of the nose. Measurements of hemoglobin,

urinal-ysis, serum creatinine, and creatinine clearance all yielded

normal values. The erythrocyte sedimentation rate

(ESR) was 63 mm/hr and the level of $c globulin was

markedly decreased. An LE cell preparation and

anti-DNA test were positive. Renal biopsy revealed immune

complex-mediated glomerulonephritis with abundant

mesangial and numerous subepithelial deposits typical of

lupus erythematosus. The patient was treated with

pred-nisone, 60 mg/day for four weeks, followed by the same

dose given every other day. She showed partial response

and an eight-week course of cyclophosphamide was given

to keep her symptom-free with normal kidney function.

Two years after diagnosis, while the patient was still

receiving prednisone, 60 mg every other day, she was seen with a one-week history of lower abdominal pain

associ-ated with urinary urgency. Temperature was 39 C and

she appeared ill. There was tenderness on deep palpation

ofthe right lower quadrant of the abdomen. The following

laboratory values were obtained: hemoglobin, 12.5 gm/

100 ml; ESR, 49 mm/br; leukocyte count, 10,200/cu mm

with 77% polymorphonuclear cells, 4% band forms, 19%

lymphocytes; BUN, 6 mg/100 ml; serum creatinine, 1 mg/

100 ml; creatinine clearance, 75 ml/min/1.73 sq m; routine

urinalysis, normal; the $,c globulin was normal and

anti-bodies to DNA were absent. Tests to demonstrate

im-munodeficiency or reduced phagocytic function were not

performed. Cultures of the blood, urine, and stool grew

no organisms. An excretory urogram revealed delayed excretion and persistent nephrogram with faint and

di-lated pyelogram on the right side. On cystoscopic

exami-nation, the bladder mucosa was found to be unremarkable

and retrograde catheterization and pyelogram of the right

ureter revealed extramural compression at the level of

the pelvic brim and the absence of intraureteral

(2)

Fig 1. Right retrograde pyelogram demonstrates extramural compression at level of pelvic brim and absence of

intraureteral obstruction.

EXPERIENCE AND REASON 297

During exploratory surgery, the distal portion of the

right ureter was found to be surrounded and invaded by

a tumor mass extending from the retroperitoneum. A

frozen section was reported as dense connective tissue

only. Both the distal section of the right ureter and the

tumor mass were excised with reimplantation of the

re-maining portion of the ureter into the bladder. The

mi-croscopic findings were extensive infiltrates of plasma

cells and histiocytes in both the mass and in the wall of

the ureter. There was no involvement of the mucosa of

the ureter. The histiocytic cells contained abundant

gran-ular eosinophiic cytoplasm which, on special stain,

re-vealed calcium-containing concentrically laminated

structures typical of Michaelis-Gutmann bodies,

charac-teristic of malacoplakia (Fig 2).

Convalescence was uneventful and the patient was

discharged from the hospital 12 days after surgery on a

regimen of trimethoprim-sulfamethoxazole for two

months. The excretory u.rogram ten weeks after surgery

showed marked improvement of hythoureteronephrosis

on the right side. The patient remains well six months

later.

DISCUSSION

Malacoplakia is an infrequently reported

granu-lomatous disease that occurs most commonly in the

urinary bladder. Involvement of the kidney, ureter,

urethra, prostate, testes, retroperitoneum, stomach,

colon, adrenal glands, lungs, or bone is rare. The

lesion occurs four times as often in women as in

men. It may occur at any age and has occurred in

children as young as 6 weeks of age.9 However, it

appears most often in the fifth decade. The lesion

derives its name from the Greek words malakos

(soft) and p/ax (plaque), which describes its

mac-roscopic appearance. Grossly, the lesions are

yel-lowish-brown plaques that range in size from 0.1

mm to 3.0 cm, although they may occasionally

reach the proportions of large masses requiring

surgical resection, as in our case. Microscopically,

the lesions of malacoplakia possess certain

distin-guishing characteristics. They contain an

aggrega-tion of large histiocytic cells with an eosinophiic

granular cytoplasm. In addition, these histiocytic

cells contain intracytoplasmic laminated basophiic

calcospheru.les, the pathognomonic

Michaelis-Gut-mann bodies. Electron microscopy of the large

his-tiocytes reveals two types of cytoplasmic inclusions.

The first are phagolysosomes that have been shown

to contain phagocytosed bacteria in varying degrees

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Fig 2. Microscopic appearance of retroperitoneal mass lesion demonstrating Michaelis-Gutmann bodies (arrow) in

large macrophages (periodic acid-Schiff, x500).

of degradation. The second type of inclusion, the

laminated Michaelis-Gutmann body, is thought by

some to represent crystalline deposits of calcified,

incompletely digested, bacterial

Although the pathogenesis of malacoplakia is not

completely clear, the present evidence that

mala-coplakia is an infectious process is supported by the

fact that most of the reported cases involving the

genitourinary system were associated with

Gram-negative urinary tract infections and by the

dem-onstratiom of bacteria within the phagocytic

vacu-oles of histiocytes. This is also supported by the

report of usefulness of

trimethoprim-sulfamethox-azole in the treatment of ‘ The

asso-ciation of some cases of malacoplakia with

sarcoid-osis, diabetes mellitus, chronic liver disease, cancer,

and, as in our case, lupus erythematosus may

sug-gest an underlying immunologic defect as a

prereq-uisite for the development of malacoplakia.

Re-cently, monocytes from one patient with

malaco-plakia were shown to have low levels of cyclic

guanosine monophosphate.’2 This deficiency results

in the impaired ability of the macrophages to

release lysosomal enzymes necessary for the

diges-tion of phagocytosed bacteria. In addition, this

de-fective monocyte function appears to be reversible

by treatment with cholinergic agonists such as

beth-anechol chloride. This finding, however, remains to

be confirmed. Our patient has systemic lupus

ery-thematosus and has been receiving prednisone for

the last two years in a dose sufficient to keep her

symptom-free. Patients with systemic lupus

ery-thematosus are vulnerable to infection, and steroids

are known to interfere with phagocytosis by

mac-rophages.” Such modification of phagocytosis and

the normal inflammatory response to bacterial

in-fection may have had an important role in the

genesis of retroperitoneal malacoplakia in this case.

ACKNOWLEDGMENT

We thank Lynne Gould for her help in the preparation

and typing of this manuscript.

JAHED A. HAMDAN, MD

MOHAMMED S. AHMAD, MD

ABDEL RAHMAN SA’ADI, MD

Departments of Pediatrics and Pathology

Dhahran Health Center

Dhahram, Saudi Arabia

REFERENCES

1. Reiner HA, Conway GF, Goodman PA: Retroperitoneal ma-lacoplakia. Urology 10:276, 1977

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EXPERIENCE AND REASON 299 malacoplakia with trimethoprim-sulfamethoxazole. Urology

13:70, 1979

3. Cadnapaphornchai P, Rosenburg BF, Taher S, et al: Renal

parenchymal malacoplakia. N Engl J Med 299:1 1 10, 1978

4. McClure J: A case of urethral malacoplakia associated with

vesical disease. J Urol 122:705, 1979

5. Joyeuse R, Lott JV, Michaelis M, et al: Malacoplakia of the

colon and rectum: Report of a case and review of the litera-ture. Surgery 81:189, 1977

6. Kawamura N, Murakami Y, Okada K: Three cases of mala-coplakia of the prostate. Urology 15:77, 1980

7. Nieh PT, Althausen AT: Malacoplakia of the ureter. J Urol

122:701, 1979

8. Feldman SF, Levy LB, Prinz LM: Malacoplakia of the

blad-der causing bilateral ureteral obstruction. J Urol 123:588,

1980

9. Sinclair-Smith C, Kahn LB, Cywes 5: Malacoplakia in

child-hood: Case report with ultrastructural observations and

re-view of the literature. Arch Pathol 99:198, 1975

10. Lou TY, Teplitz C: Malacoplakia: Pathogenesis and

ultra-structural morphogenesis: A problem of altered macrophage

(phagolysosomal) response. Hum Pathol 5:191, 1974

11. Lewin KJ, Harell GS, Lee AS, et al: An electron-microscopic

study: Demonstration of bacilliform organisms in malaco-plakia macrophages. Gastroenterology 66:28, 1974 12. Abdou NI, Napomberja C, Sagawa A, et a!: Malacoplakia:

Evidence for monocyte lysosomal abnormality correctable

by cholinergic agonist in vitro and in vivo. N Engl J Med

297:1413, 1977

13. Zurier RB, Weissman G: Anti-immunologic and

anti-infiam-matory effects of steroid therapy. Med Clin North Am

57:1295, 1973

Dysuria

in Adolescent

Girls:

Urinary

Tract

Infection

or

Vaginitis?

Dysuria is a common presenting complaint of

adolescent girls. Because physicians often assume

a bacterial urinary tract infection (UTI) is present,

the patient may be treated with antibiotics without

thorough evaluation. In previous studies only half

of adult women complaining of dysuria had

bacte-riuria with greater than iO organisms per

miii-liter.’2 Vaginitis,3’4 vulvitis,3 Neisseria

gonor-rhoeae,5 Chiamydia trachomatis,9 and bacteriuria

with less than iO organisms per milliiter”2’9 are

responsible for the symptoms in many of the

re-maining subjects. A recent study in adult women

found that a history of external dysuria (pain felt as

the urine passes over the inflamed vaginal labia)

suggested vaginal infection, whereas a history of

internal dysuria (pain felt inside the body)

sug-gested bacterial UTI.4

No study has been undertaken to identify the

causes of dysuna in adolescent girls or to ascertain

whether the history cam be similarly helpful to the

pediatrician caring for this group of patients.

METHODS

Fifty-three females 12 to 21 years of age with a

chief complaint of dysuria who were seen at the

adolescent outpatient clinics of The Children’s

Hos-pital Medical Center from January to December

1980 were evaluated. Informed consent was

ob-tamed. The history included inquiries regarding the

Reprint requests to (S.J.E.) Adolescent/Young Adult Medicine

Unit, The Children’s Hospital Medical Center, 300 Longwood

Aye, Boston, MA 02115.

American Academy of Pediatrics.

presence and duration of internal vs external

dy-suria, urgency, frequency, suprapubic and flank

pam, fever, vaginal discharge, odor and irritation,

vulvar lesions, recent antibiotic use, sexual

experi-ence, contraceptive use, and previous UTI or other

renal disease. Method of payment (Medicaid vs

non-Medicaid) as an approximation of

socioeco-nomic status was recorded. Mean age of the study

population was 17.5 years; 42/53 (79%) were

sex-ually experienced; and 33/53 (62%) had Medicaid

insurance.

A complete physical examination was performed,

including inspection of the genitalia. A pelvic

ex-amination was performed in all sexually

experi-enced patients and in seven of 11 virginal patients.

In four adolescents whose introitus was too small

to admit a small Huffman speculum without

dis-comfort, samples of vaginal secretions were

ob-tamed with a saline-moistened cotton-tipped

appli-cator gently inserted into the vagina.

Laboratory evaluation included urinalysis, two

clean voided midstream urine specimens cultured

quantitatively on blood and eosin-methylene blue

agar or a Uricult dipslide (Orion Diagnostica,

Hel-sinki, Finland), am endocervical culture for N

gon-orrhoeae streaked directly onto a modified

Thayer-Martin Jembec plate, a vaginal culture on

Nicker-son’s medium (Ortho or Biggy agar [Scott] can now

be used) for Candida, and microscopic examination

of 10% KOH and saline preparations of vaginal

secretions.

Six subjects whose initial evaluation did not

re-veal a cause for the dysuria and who continued to

have symptoms were recalled within 72 hours;

swabs from the urethra and cervix were cultured

for

C

trachomatis. The urethral swab was also

cultured for N gonorrhoeae. Serum samples were

obtained at this time and again after three to four

weeks of convalescence for determination of

mi-croimmunofluorescent antibody titers to

C

tra-chomatis.

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1982;70;296

Pediatrics

Jahed A. Hamdan, Mohammed S. Ahmad and Abdel Rahman Sa'adi