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Case report of subcutaneous entomophthoromycosis
with retroperitoneal invasion
Relato de caso de entomoftoromicose subcutânea
com invasão retroperitoneal
Leonora Maciel de Souza Vianna
1, 2, Marcus Vinícius Guimarães de Lacerda
1and Mário Augusto Pinto de Moraes
1ABSTRACT
Th e a u th o rs d e sc ri b e a c a se o f e n to m o p h th o ro m yc o si s i n a p re vi o u sly h e a lth y p a ti e n t, wh o p re se n te d wi th a n a b sc e ss i n th e ri gh t b u tto c k . Af te r su rgi c a l d ra i n a ge i t e vo lve d i n to a re tro p e ri to n e a l tu m o r. Th e p a ti e n t i m p ro ve d c li n i c a lly a f te r re se c ti o n o f th e m a ss a n d k e to c o n a z o le tre a tm e n t. Th e h i sto p a th o lo gi c a l a n a lysi s sh o w e d th e Sp le n d o re - Ho e p p li p h e n o m e n o n , su gge sti n g B asidio b o lus r anar um i n f e c ti o n , a zygo m yc o si s ge n e ra lly re stri c te d to th e su b c u ta n e o u s ti ssu e , wi th ra re ga stro i n te sti n a l i n vo lve m e n t.
Ke y-words: B asidio b o lus r anar um. Zygo m yc o si s. En to m o phtho ra le s. Cli n i c a l f e a tu re s. Hi sto pa tho lo gy. Sple n do re - Ho e ppli p h e n o m e n o n .
RESUMO
Os a u to re s d e sc re ve m u m c a so d e e n to m o f to ro m i c o se e m p a c i e n te p re vi a m e n te sa u d á ve l, q u e a p re se n to u a b sc e sso e m n á de ga di re i ta , e vo lu i n do , a pó s dre n a ge m c i rú rgi c a , pa ra tu m o ra ç ã o re tro pe ri to n e a l. Apó s re sse c ç ã o da m a ssa , o pa c i e n te o b te ve m e lh o ra c lí n i c a , e m u so d e c e to c o n a zo l. A a n á li se h i sto p a to ló gi c a e vi d e n c i o u f e n ô m e n o d e Sp le n d o re - Ho e p p li , su ge ri n d o i n f e c ç ã o p o r B asidio b o lus r anar um, u m a zi go m i c o se ge ra lm e n te re stri ta a o te c i d o su b c u tâ n e o , c o m ra ro c o m p ro m e ti m e n to ga stri n te sti n a l.
Pal avr as-chave s: B asidio b o lus r anar um. Zi go m i c o se . En to m o p h th o ra le s. Ca ra c te rí sti c a s c lí n i c a s. Hi sto p a to lo gi a . Fe n ô m e n o d e Ho e p p li - Sp le n d o re .
RELATO DE CASO/CASE REPORT
1 . Ce ntr o de Anato mia Pato ló gic a do Ho spital Unive r sitár io de B r asília da Unive r sidade de B r asília, B r asília, DF. 2 . Unidade de Anato mia Pato ló gic a do Ho spital Re gio nal de So b r adinho da Se c r e tar ia de Estado de Saúde do Distr ito Fe de r al, B r asília, DF.
Addr e ss to: Dr a. Le o no r a Mac ie l de So uza Vianna. Ce ntr o de Anato mia Pato ló gic a/Ho spital Unive r sitár io de B r asília. Via L2 No r te , SGAN 6 0 4 /6 0 5 , Mó dulo C, 7 0 8 4 0 - 0 5 0 B r asília, DF.
Te l. 5 5 6 1 4 4 8 -5 5 0 1 e - mail: c aphub @ unb . b r
Re c e b ido par a pub lic aç ão e m 1 0 /1 0 /2 0 0 3 Ac e ito e m 3 /5 /2 0 0 5
We refer to human zygomyc osis as polymorphic diseases c aused by fungi of the Zygo m yce te s c lass. This c lass is subdivided into two orders: Muco ra le s and Ento m o phtho ra le s1 0. Unlike the Muco ra le s order, whic h has the ability to c ause systemic disease, this last order is mainly represented by Co nidio b o lus co ro na tus, the etiologic agent of subc utaneous infec tions c onfined to the nasal muc osa and adjac ent tissues, and Ba sidio b o lus ra na rum
( also known as Ba sidio b o lus ha pto spo rus) , whic h involves the trunk and limbs, and rarely the gastrointestinal trac t4 1 5.
We r e p o r t a r a r e fo r m o f e n to m o p h th o r o m yc o s i s sugge sting Ba si d i o b o lu s ra n a ru m infe c tio n, whic h e xte nde d
to the r e tr o pe r ito ne um, afte r sub c utane o us infe c tio n in the r ight b utto c k .
CASE REPORT
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Via nna LMS e t al
systemic illness was observed. Roentgenographs of the pelvis showed a soft-tissue absc ess without bone or joint lesions. The absc ess was surgic ally drained and the lesion improved without spec ific medic ation. The c ontents of the drainage were not sent for c ulture. Histopathologic analysis of a subc utaneous tissue sample showed nonspec ific c hronic inflammation.
In February 1 9 9 0 , seven months after disc harge, the patient started to c omplain of arthralgia in the right knee, abdominal pain, malaise, unc harac teristic fever, vomiting and weight loss. He was admitte d to the same ho spital, whe r e a diagno stic proc edure was performed.
On physic al examination, the patient had a poor nutritional s ta te , we igh in g o n ly 4 5 k g, with n o r m a l vita l s ign s . No lymphadenopathywas found. The lungs and heart and neurologic examination were normal. His abdomen was tender, partic ularly in the right flank, where a palpable, nodular mass was loc ated. The liver extended 5 c m below the right c ostal border.
Hematologic laboratory data obtained on admission were as follows: hematoc rit 0 .4 1 and white-c ell c ount 1 0 .8 x 1 09/L, with
a normal differential c ount. The blood c hemistry values were normal, as well as the c oagulation profile and urinalysis. The tuberc ulin skin test was negative at 4 8 hours. Ultrasound of the abdomen c onfirmed the presenc e of a thic k mass in the right flank ( 4 0 5 c m3) .
He subsequently underwent an exploratory laparotomy, where a mass was found in the retroperitoneum, upon the right kidney. It was exc ised and sent fo r histo patho lo gic al analysis. The resec ted material c onsisted of an irregular mass, whic h was whitish, smooth, firm and measured approximately 1 0 c m in the greatest diameter. Microscopically, hematoxylin and eosin ( H&E) -stained sec tions showed a dense eosinophilic infiltrate. Many nonc aseating granulomas were present ( Figure 1 ) and multiple ar eas demo nstr ating Splendo r e-Ho eppli pheno meno n wer e o b s e r ve d, with a th ic k , e o s in o ph ilic , h ya lin ize d s h e a th surrounding the thin-walled fungal hyphae ( Figures 2 and 3 ) . No vasc ular invasion or fungal thrombosis were seen. A c ulture of the spec imen was not performed. Based on the loc alization of lesions, the a ge nt a spe c t in the tissues, and histopathologic pic ture, Ba sidio b o lus ra na rum was suggested.
The patient r ec eived keto c o nazo le ( 4 0 0 mg/d) fo r 3 5 days with a c o mple te c linic al impr o ve me nt.
DISCUSSION
Muc ormyc osis ( infec tion with fungi of the order Muco ra le s) is c harac terized by a rapidly progressive and usually fatal c ourse, primarily in immunoc ompromised patients. Common c linic al manifestations include rhinocerebral, pulmonary, gastrointestinal and c utaneous involvement as well as dissemination. Due to its vasc ulotropism, the hyphal infiltrations found near blood vessels and septic thromboembolism are c ommon6 9.
On the other hand, entomophthoromyc osis ( infec tion with fungi of the order Ento m o phtho ra le s) has a more benign c ourse and apparently no underlying disease appears to predispose to
Fi gu r e 1 - Se cti on f r om the r e se cte d r e tr ope r i ton e al tu m or, showi n g se ve r al l ar ge hyphae wi thi n m i cr oab sce sse s an d a su r r ou n di n g m on on u cl e ar i n f i l tr ate ( H&E, 4 0 x ) .
Fi gu r e 2 - Hyphal f or m of Ba sidio bo lus i s ob se r ve d i n the ce n te r of a g r a n u l o m a . Th e a m o r p h o u s m a te r i a l su r r o u n d i n g th e e m p ty-a p p e ty-a r i n g h yp h ty-a l s tr u c tu r e i s e o s i n o p h i l i c o n H&ty-amp;E s tty-a i n i n g ( Spl e n dor e -Hoe ppl i phe n om e n on ) ( 2 0 0 x ) .
Fi gu r e 3 - In thi s se cti on , the thi n wal l e d hyphae , su r r ou n de d b y Spl e n dor e -Hoe ppl i phe n om e n on , ar e m or e e vi de n t ( H&E, 2 0 0 x ) .
the infec tion. In 1 9 8 8 , of 3 3 Latin Americ an c ases reported, 8 4 % we r e de s c r ib e d in B r a zil1. Th e tis s ue r e a c tio n is a lm o s t
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The infection due to B. ra na rum is widely prevalent in tropical and subtropical regions and is often misdiagnosed or not reported. Infection usually begins as small papules following a scratch or puncture by an insect, plant, or other fungus-bearing object. The lesion commonly develops in the perineum, buttocks, trunk and thighs, probably due to the habit of using decaying plant leaves lying on the ground as toilet paper after defecating in the open7.
Sinc e the fungus c annot withstand refrigeration, biopsied material should be c ultured immediately after c ollec tion. This probably c ontributes to the absenc e of the definitive diagnosis in most of the c ases where there is no previous suspic ion of fungal infec tion7 1 0.
Visceral disease caused by B. ra na rum is rare, but cases of gastrointestinal infection have been reported worldwide3 5 8 1 3 1 5. It
is not clear how the fungus gains access to the gastrointestinal tract of patients. It is attributed to ingestion, with the portal of entry being the stomach, or to direct inoculation via minor rectal trauma.
Possibly due to protease enzymes produced by B. ra na rum
and other Ento m o phtho ra le s1 2, the fungus is able to invade soft
tissues and eventually evolve beyond a subcutaneous infection. One patient with a voluminous lesion resembling a soft tissue tumor has been reported elsewhere2. Two patients similar to ours have
been reported, one with retroperitoneal lesions and one with pe lvic c a vity le s io n s . A s ur gic a l pr o c e dur e pr io r to th e c linic al de te c tio n o f the inte r nal m asse s was o b se r ve d in b o th1 1 1 4. We c onsider that the rare forms of intra-abdominal
entomophthoromycosis without involvement of the gastrointestinal tract are probably related to invasive procedures, which possibly implant the fungus more deeply.
ACKNOWLEDGMENTS
We thank Co l. D. Sk illman, Walte r Re e d Ar my Institute o f Re se ar c h, fo r c r itic al r e vie w o f the manusc r ipt.
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