İdiyopatik skrotal kalsinozis

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Introduction
Idiopathic scrotal calcinosis (ISC) is a rare benign
condition which presents with multiple, asymptomatic
and painless nodules on the scrotal skin wall. Various
theories regarding the etiology and pathogenesis of this
condition have been proposed although none has been
widely accepted. Although calcinosis of the scrotum
was first described by Lewinski in 1883 (1), its cause
and nature have remained elusive. Hutchinson has
emphasized that these calcific masses appear with no
evidence of underlying cause (2), and finally the term
"idiopathic scrotal calcinosis" used by Shapiro in 1970
has most commonly been accepted (3).
Histologically, ISC is characterized by the presence
of calcium deposits that are variable in size within the
dermis, often surrounded by a foreign body-type granulomatous reaction. Some cysts reveal calcification of
their keratin contents with little evidence of active
inflammation.
Because of its rarity and controversial pathogenesis of
the disease, we present a case of ISC with multiple nodules over the scrotal skin.
Case Report
A 21-year-old man was referred to the outpatient
clinic of urology service with one year history of painless multiple cystic bumps over both sides of the scrotum. The lesions were measured 0.5 to 2 cm in diameter and skin-colored to white-yellow and moveable
beneath the skin surface. The patient stated that he did
not experience itching, pain or discharge. The testes
were normal. Blood chemistry including serum electrolytes, urea, creatinine, calcium, phosphate, uric acid
* Department of Urology, Derince Military Hospital, Kocaeli
** Department of Urology, Special Ersoy Hospital, Ýstanbul
***Department of Pathology, Mareþal Çakmak Military Hospital,
Erzurum
Reprint request: Dr. Fahri Sümer, Derince Asker Hastanesi Üroloji
Servisi, Derince-41900, Kocaeli
E-mail: doktorfahri@yahoo.com
Date submitted: November 15, 2006
Accepted: March 06, 2007
OLGU SUNUMU/CASE REPORT Gülhane Týp Dergisi 2007; 49: 259-261
© Gülhane Askeri Týp Akademisi 2007
Summary
Idiopathic scrotal calcinosis is a rare benign disease characterized by multiple, asymptomatic and painless nodules
on the scrotum. Over 100 cases have been reported to
date. We herein report our case with this rare disease and
shortly review the literature.
Key words: Calcinosis, scrotum
Özet
Ýdiyopatik skrotal kalsinozis
Ýdiyopatik skrotal kalsinozis skrotum derisi üzerinde
görülen, birden çok sayýda asemptomatik ve aðrýsýz
nodüller ile karakterize benign bir hastalýktýr. Bugüne kadar
100’den fazla olgu bildirilmiþtir. Burada nadir görülen
hastalýða sahip olgumuz sunularak, bu konudaki literatür
bilgisi kýsaca gözden geçirilmiþtir.
Anahtar kelimeler: Kalsinozis, skrotum260 · Aralýk 2007 · Gülhane TD Sümer et al.
and alkaline phosphatase and parathyroid hormone levels were normal. The patient complained of poor
cosmesis and wanted the lesions be removed, and the
nodules were extirpated surgically under local anesthesia. The recurrence was not observed after a 6-month
follow-up period. Histologic examination of an excised
nodule stained with hematoxyline and eosin showed a
thin layer of epidermis overlying a dermal lesion made
of multiple cystic spaces of varying sizes. These spaces
were distended by large amorphous granular basophilic
material consistent with ICS (Figures 1,2).
Discussion
ICS is a rare condition characterized by multiple,
slowly growing and distinct nodular masses embedded
within the dermis of scrotal skin, and was first described
by Lewinski in 1883 (1). The lesions have been attributed as sebaceous cysts, calcified steatocystoma, fibroma, atheroma and xanthoma. Shapiro et al. reviewed
the histologic data and found no evidence of an epithelial lining, residual cysts and lipid or organisms, and
concluded that the calcification was idiopathic introducing the term "idiopathic scrotal calcinosis" (2).
ISC occurs mainly between 20 to 40 years of age, and
the youngest and the oldest patients were 9 and 85 years
old, respectively (4). The pathogenesis is unclear and
controversy exists as to whether the condition is idiopathic or the result of dystrophic calcification of preexisting epidermal cysts (5-8).
Akosa et al. (9) and recently Saad and Zaatari (4) have
reported that scrotal calcinosis might result from
inflammation of epidermal cysts followed by dystrophic calcification within the keratin of the cyst or dermis
adjacent to a ruptured cell wall. Others support the theory of epidermal conclusion cysts playing a major role
in the pathogenesis of the disease (5). In fact the idiopathic etiology is based upon the absence of the epithelial lining within the lesions (5,6,8).
Swinehart and Golitz also described this epithelial
lining surrounding the calcified material and offered a
model for ICS (10). Veress and Malik (11) and Feinstein
et al. (12) have suggested that minor trauma plays an
important role as the starting point of dystrophic calcification. And recently Pabuccuoglu et al. have reported
that dartoic muscle degeneration and necrosis are the
most important factors in the process of this pathology
(13).
Most of the cases are asymptomatic and patients usually seek medical advice for cosmetic reasons as in our
case. Surgical excision and histopathologic examination
are necessary to confirm the diagnosis. Recurrence is
unusual.
We think that the cause of the condition is most likely multifactorial. There is evidence supporting the calcification of the ruptured epithelial cysts, and the cause
of ICS may be the degeneration process of the dartoic
muscle. For the remainder of the cases, there is still difficulty in finding a preexisting lesion and the use of the
term "idiopathic" may be appropriate.