CALCINOZÃ SCROTALÃ IDIOPATICÃ - prezentare de caz

IDIOPATHIC SCROTAL CALCINOSIS - Case Report

VIRGIL PÃTRAªCU*, MARIA CRISTINA PREDOI*, RALUCA CIUREA**, FLORENTINA DELCEA*

65

* Departamentul Dermatologie, Universitatea de Medicinã ºi Farmacie din Craiova, România.Dermatology Department, University of Medicine and Pharmacy of Craiova, Romania.

** Departamentul de patologie, Universitatea de Medicinã ºi Farmacie din Craiova, România.Pathology Department, University of Medicine and Pharmacy of Craiova, Romania.

Rezumat

Calcinoza scrotalã idiopaticã (CSI) este o afecþiunebenignã, rarã, caracterizatã prin prezenþa de multiplinoduli calcificaþi, asimptomatici, localizaþi la nivel scrotal,în absenþa oricãror tulburãri ale metabolismului calciului.Boala debuteazã în perioada copilãriei sau la adulþii tineri.Etiologia acestei afecþiuni este incomplet elucidatã. Noiprezentãm cazul unui pacient cu calcinozã scrotalãidiopaticã cu debut în jurul vârstei de 15 ani, care fusesediagnosticat ºi cu tulburare de personalitate pe fondorganic, aproximativ la aceeaºi vârstã.

Cuvinte cheie: calcificãri, calcinozã, idiopaticã, scrot.

Summary

Idiopathic scrotal calcinosis (ISC) is a rare benigncondition consisting in the presence of multiple scrotalasymptomatic calcified nodules in the absence of anycalcium metabolism disorder. This condition occurs inchildren or in young adults. Its etiology is not yet fullyunderstood. We report the case of an ISC patient whosecondition occurred at 15 and had also been diagnosed withorganic personality disorder at the same age.

Key words: calcified, calcinosis, idiopathic, scrotum.

Intrat în redacþie: 10.01.2017Acceptat: 16.02.2017

Received: 10.01.2017Accepted: 16.02.2017

Introducere

Calcinoza idiopaticã poate fi localizatã(calcinozã vulvarã Winer; calcinozã scrotalã) saugeneralizatã (calcinosis universalis). Prezentãmun caz de calcinozã scrotalã idiopaticã.

Calcinoza cutanatã corespunde prezenþeidepozitelor dermice sau hipodermice de cristalede hidroxiapatitã de calciu sau de fosfat de calciuamorf.

Caz clinic

Pacient în vârstã de 26 ani, din mediul rural,se spitalizeazã pentru multipli noduli, dedimensiuni variabile, cuprinse între 0.5-2 cm,

Introduction

Idiopathic calcinosis can be localized(Winer’s vulvar calcinosis; scrotal calcinosis) orgeneralized (calcinosis universalis). We report acase of idiopathic scrotal calcinosis.

Calcinosis cutis consists in the presence ofdermal or hypodermal calcium hydroxyapatitecrystals or calcium phosphate amorphouscrystals.

Case report

A 26 year old male, rural environment wasadmitted for multiple firm, yellow, skin nodules,with dimensions between 0.5 and 2 cm, located

CAZURI CLINICECLINICAL CASES

66

DermatoVenerol. (Buc.), 62: 65-70

consistenþã fermã, culoare gãlbuie, nedureroºi,localizaþi la nivelul scrotului (fig. 1, 2). Pacientulafirmã debutul leziunilor în jurul vârstei de 15ani, cu creºtere progresivã în dimensiuni.

AHC: nesemnificativeAPP: Tulburare de personalitate pe fond

organic, diagnosticatã în urmã cu 10 ani, tratatãcu tiapridum, diazepam, acid valproic.

Am prelevat un nodul pentru examenhistopatologic, care a evidenþiat urmãtoareleaspecte: structurã microscopicã de þesutfibrocolagen ce delimiteazã o zonã de materialamorf cu multiple microcalcificãri, înconjurat demacrofage uni ºi multinucleate (fig 3, 4).

Investigaþiile complexe pe care le-am efectuatau exclus afecþiunile metabolice, endocrine,autoimune ºi neoplazice.

Pe baza anamnezei, examenului clinic,investigaþiilor de laborator ºi a examenuluihistopatologic am stabilit diagnosticul deCalcinozã scrotalã idiopaticã.

Tratamentul a constat în incizia chirurgicalã aleziunilor, cu eliminarea conþinutului alb, cretos,urmatã de suturã. Evoluþia la trei luni de laintervenþie a fost favorabilã, fãrã recidive.

on the scrotum (fig. 1, 2). The patient stated thatthe lesions had occurred when he was 15 andhave been growing in size ever since.

Family medical history: unremarkable.Patient’s medical history: Organic personality

disorder diagnosed 10 years ago, treated withtiapride, diazepam and valproic acid.

We excised a nodule for histologicalexamination, which revealed the followingaspects: fibrocollagenous structure delimiting anarea of amorphous material with multiplecalcifications, surrounded by uninucleated andmultinucleated macrophages (fig. 3, 4).

The complex investigations performed ruledout metabolic, endocrine, autoimmune andmalignant conditions.

Based on the clinical examination, laboratoryinvestigations and histological examination, weestablished the diagnosis of Idiopathic scrotalcalcinosis.

The patient’s treatment consisted in surgicalincision, excision of the white chalkish contentand surgical suture. Three months after surgery,the patient’s evolution was favorable, withoutrecurrence.

Fig. 1 ºi 2. Numeroºi noduli, de dimensiuni variabile, cuprinse între 0.5-2 cm, consistenþã fermã, culoare gãlbuie, nedureroºi,localizaþi la nivelul scrotului

Fig. 1 and fig. 2. Multiple firm, yellow, skin nodules, with dimensions between 0.5 and 2 cm, located on the scrotum

Fig. 1 Fig. 2

67

DermatoVenerol. (Buc.), 62: 65-70

Discuþii

Calcificãrile tisulare pot fi în raport cuurmãtoarele situaþii:- anomalie a metabolismului fosfocalcic:

• hipercalcemie, întâlnitã în intoxicaþia cuvitamina D, sindromul bãutorilor de lapte,sarcoidozã, hiperparatiroidie primarã, iatro-genã - secundarã administrãrii iv de clorurãde calciu;

• hiperfosforemie fãrã hipercalcemie întâlnitã încalcinoza tumoralã, hiperparatiroidia dinboala cronicã de rinichi;

- alterãri tisulare localizate (dupã traumatisme,dupã perfuzii cu soluþii de calciu, postradio-terapie, tumori benigne precum pilomatricomºi tricoepiteliom, tumori maligne cum ar ficarcinomul bazocelular, osteosarcomul, lipo-sarcomul)

- alterãri tisulare difuze, prezente în sclero-dermia sistemicã, dermatomiozitã, lupusuleritematos, porfiria cutanatã tardivã, pseudo-xanthomul elastic, sindromul Ehlers-Danlos.(1)

Calcinoza idiopaticã este o varietate rarã,cauza exactã a calcificãrii neputând fi explicatã.

Calcinoza scrotalã (CS) a fost descrisã pentruprima datã în 1883 de cãtre Lewinsky.

Afecteazã bãrbaþii cu vârsta cuprinsã între 20-40 ani (9-85 ani)(2). Clinic se manifestã prin

Discussion

Tissue calcification can occur in the followingsituations:- phosphocalcic metabolism disorders:

• hypercalcemia found in vitamin Dintoxication, milk drinker’s syndrome,sarcoidosis, primary hyperparathyroidismand calcium chloride administration;

• hyperphosphathemia without hypercal-cemia is found in tumoral calcinosis and inhyperparathyroidism associated withchronic kidney disease

- localized tissue disorders (posttraumatic, aftercalcium solutions iv perfusions, postradio-therapy, benign tumors such as pilomatricomaand tricoepitelioma and malign tumors such asbasal cell carcinoma, osteosarcoma andliposarcoma)

- diffuse tissue disorders found in systemicscleroderma, dermatomyositis, systemic lupuserythematosus, porphyria cutanea tarda,pseudoxanthoma elasticum and Ehlers-Danlossyndrome. (1)

Idiopathic calcinosis is a rare condition andthe exact cause of the calcifications remainsunknown.

Scrotal calcinosis (SC) was first described byLewinsky in 1883.

It affects men aged 20 – 40 years (9-85 years)(2). It consists in firm, yellowish nodules withvariable dimensions (between several mm and 7 cm). This condition is asymptomatic and it can

Fig. 3. Col HE x40. Structurã microscopicã de þesutfibrocolagen ce delimiteazã o zonã de material amorf cumultiple microcalcificãri, înconjurat de macrofage uni ºi

multinucleate

Fig. 3. Col HE x40. Fibrocollagenous structure delimitingan area of amorphous material with multiple calcifications,

surrounded by uninucleated and multinucleatedmacrophages

Fig. 4. Col HE x100. Multiple microcalcificãri

Fig. 4. Col HE x100. Multiple calcifications.

68

DermatoVenerol. (Buc.), 62: 65-70

noduli fermi la palpare, gãlbui, cu dimensiunivariabile, cuprinse între câþiva mm ºi 7 cm.Afecþiunea este asimptomaticã, rareori poatedetermina prurit, ulceraþii sau durere localã,secundarã cel mai adesea unei suprainfecþiibacteriene. Evoluþia bolii este lentã, benignã. Laexamenul histopatologic se evidenþiazãmicrocalcificãri ºi reacþie giganto-celularã.(3)

Patogeneza nu este elucidatã. Se discutã dacãaceastã CS este idiopaticã sau secundarãcalcificãrii unor chisturi epiteliale preexistente(epidermoide, sebacee, ale glandelor ecrine) orieste consecinþa calcificãrii distrofice a muºchiuluidartos. Având în vedere localizarea frecventã achisturilor epidermoide la nivel scrotal se pare cãacestea sunt cel mai frecvent implicate în apariþiacalcificãrilor de la acest nivel.(4)

Dubey ºi colab. sugereazã cã inflamaþiachistului epidermoid poate determina calcifi-carea peretelui chistic. Ulterior are loc distrugereaacestuia, microcalcificãrile predominând numaiîn leziunile vechi.(5)

Carson evidenþiazã rolul posibil al nanobac-teriilor în apariþia calcificãrilor extrascheletale.Acestea pot invada tegumentul secundarmicrotraumatismelor, fãrã a provoca manifestãriclinice de infecþie ºi se caracterizeazã prinformarea de cristale de hidroxiapatitã, înprezenþa unui nivel seric optim al Ca.(6)

King ºi colab. subliniazã posibilul rol înapariþia calcificãrilor distrofice a muºchiuluidartos. Aceºtia sugereazã faptul cã degenerareaacestui muºchi reprezintã elementul cheie înapariþia CS.(7)

Sunt propuse ºi alte teorii privind apariþia CS,reprezentate de calcificarea limfangioamelor,xantoamelor, fibroamelor, teratoamelor, gonado-blastoamelor.(4,8,9,10)

În majoritatea cazurilor publicate în literaturade specialitate nu au fost descrise asocieri alecalcinozei scrotale cu calcinozã cu altã localizare.

Cecchi ºi colab. au raportat cazul unuipacient de 54 ani care a prezentat calcinozãscrotalã asociatã cu calcinosis cutis circumscriptala nivelul faciesului. Leziunile apãruserã în jurulvârstei de 20 ani.(11) Noi nu am gãsit în literaturão asociere între CS ºi o anumitã boalã psihiatricã.Fãrã a desprinde la cazul nostru o relaþiepatogenicã directã între cele douã afecþiuni,menþionãm cã am remarcat o preocupare

rarely cause pruritus, ulcerations or local pain,usually secondary to a bacterial infection. Thecondition’s evolution is slow and benign.Histological examination reveals microcalci-fications and a giant cell reaction. (3)

SC’s pathogenesis remains unknown. It iscurrently discussed whether SC is an idiopathiccondition or whether it is secondary to thecalcification of preexisting epithelial cysts(epidermal, sebaceous or eccrine gland cysts) orto a dystrophic calcification of the dartos muscle.Taking into consideration that epidermal cystsare frequently found in the scrotum, it appearsthat these cysts are usually involved in theoccurrence of calcinosis in this location. (4)

Dubey et al. suggested that an epidermal cystinflammation can lead to the calcification of thecystic wall, followed by its destruction,microcalcifications remaining only in olderlesions. (5)

Carson outlined the possible role ofnanobacteria in the pathogenesis of extraskeletalcalcifications. These bacteria can invade the skinafter microtrauma without causing obviousinfections. In the presence of a high In thepresence of an optimal serum calciumhydroxyapatite crystals are formed. (6)

King et al. outlined the possible role of dartosmuscle dystrophic calcifications. They suggestedthe fact this is the key element in SC’spathogenesis. (7)

Other theories regarding CS’s etiology havealso been suggested, regarding calcification oflymphangiomas, xanthomas, fibromas, teratomasand gonadoblastomas. (4,8,9,10)

Most cases reported in the literature did notpresent associations between SC and calcinosiswith other locations.

Cecchi et al. reported the case of a 54 year oldpatient that presented SC associated with facialcalcinosis cutis circumscripta. The lesions hadoccurred at the age of 20. (11) We could not find anypreviously reported association between CS andpsychiatric disorders. Without a pathogenicrelationship between the two disorders, wenoticed an excessive concern and an increase of thepsychiatric suffering caused by the coexisting CS.

The differential diagnosis inveolved thefollowing conditions:

69

DermatoVenerol. (Buc.), 62: 65-70

• scrotal epidermal cysts – histopatologicalexamination establishes the diagnosis (presenceof keratin and a cyst wall);

• sebocystomatosis;• benign tumors: lipomas, fibromas, angiokera-

tomas and circumscribed lymphangiomas;• testicular tumors: teratomas, gonadoblastomas

and Leydig cell tumors. These can evolve intocalcifications.

Treatment

SC is a benign condition and treatment isrequired based on esthetic aspects. The standardtreatment consists in surgical excision, followedby histological examination. However, the risk ofrecurrence must be considered. In our case, thepatient’s evolution was favorable over a follow-up period of three months, without any localrecurrence.

Conclusion

The pathogenesis of idiopathic scrotalcalcinosis is not yet fully understood, andsurgical excision remains the standardtherapeutic management. Histological examina-tion is required to confirm de diagnosis.

excesivã ºi o accentuare a suferinþei psihiatriceprin coexistenþa CS.

Diagnosticul diferenþial l-am fãcut cu:• chisturi epidermoide scrotale - examenul

histopatologic stabileºte diagnosticul, pentruacestea pledeazã prezenþa de keratinã ºiperetele chistului;

• sebocistomatozã;• tumori benigne: lipoame, fibroame, angiokera-

toame, limfangiom circumscris;• tumori testiculare: teratoame, gonadoblas-

toame, tumori ale celulelor Leydig. Acesteapot evolua spre calcificãri.

Tratament

CS este o afecþiune benignã, iar tratamentuleste în principal dictat de aspectul inestetic.Terapia de elecþie este reprezentatã de exciziechirurgicalã urmatã de examen histopatologic,ceea ce am efectuat ºi noi. Totuºi risculrecurenþelor este de luat în consideraþie. În cazulpacientului nostru evoluþia la trei luni de laexternare a fost favorabilã, fãrã recurenþe locale.

Concluzie

Patogeneza calcinozei scrotale idiopatice nueste elucidatã, iar managementul terapeuticstandard rãmâne excizia chirurgicalã, urmatã deexamen histopatologic, necesar pentru confir-marea diagnosticului.

70

Bibliografie / Bibliography1. Galezowski A, Sin C, Rothé-Thomas F, Amy de la Bretèque M, Bilan P, Sigal M-L, Mahé E. La calcinose sous-

cutanée, une complication rare et sévère de la radiothérapie: trois observations. Annales de Dermatologie et deVénéréologie. 2015. 142: 761-766.

2. Makkawi AMM, Suliman AM, Elhassan FMA. Idiopathic Scrotal Calcinosis. Sudan Med J 2016. April 52(1): 24-27.3. Aphale V, Deepa T, Sneha JR. Idiopathic scrotal calcinosis – A case report. Indian Journal of Basic and Applied

Medical Research. 2015. 4(3): 59-62. 4. Kiremitci S, Yuksel S, Anafarta K, Tulunay O. Scrotal Calcinosis: A Case Report And Review Of Literature. Journal

Of Ankara University Faculty of Medicine 2011, 64(1):46-51.5. Dubey S, Sharma R, Maheshwari V. Scrotal calcinosis: idiopathic or dystrophic? Dermatology Online Journal.2010

16 (2): article 5. 6. Carson DA. An infectious origin of extraskeletal calcification. Proceedings of the National Academy of Sciences of

the United States of America. 1998. 95(14): 7846–7847. 7. King DT, Brosman S, Hirose FM, Gillespie LM. Idiopathic calcinosis of scrotum. Urology. 1979. 14(1):92–4.8. Lei X. Idiopathic scrotal calcinosis: report of two cases and review of literature. International Journal of

Dermatology. 2012. 61: 199-208.9. Saad AG, Zaatari GS. Scrotal calcinosis: is it idiopathic? Urology. 2001. 57(2):365.10. Shapiro L, Platt N, Torres-Rodriquez VM. Idiopathic calcinosis of the scrotum. Arch Dermatol. 1970. 102: 199-204.11. Cecchi R, Giomi A. Idiopathic calcinosis cutis of the penis. Dermatology. 1999. 198(2):174-5.

DermatoVenerol. (Buc.), 62: 63-68

Conflict de interese Conflict of interestNEDECLARATE NONE DECLARED

Adresa de corespondenþã: Prof. univ. dr. Virgil Pãtraºcu, Universitatea de Medicinã ºi Farmacie din Craiova, Str. Petru Rareº, nr. 2-4, cod 200345; tel. 0724273676; e-mail vm.patrascu@gmail.com

Correspondance address: Virgil Patrascu, MD, PhD, University of Medicine and Pharmacy from Craiova, Petru Rares Street, No 2-4, 200345, Craiova, Romania; phone: 004-0724273676; e-mail: vm.patrascu@gmail.com