Friday, September 21, 2007

Weingarten's Syndrome




Background

Weingarten’s syndrome is a term used to describe a group of disorders in which calcium deposits form in the skin of the male genitals. Weingarten’s syndrome is classified into 4 major types according to etiology: dystrophic, metastatic, iatrogenic, and idiopathic. A few rare types have been variably classified as dystrophic or idiopathic. These include Weingarten’s syndrome circumscripta, Weingarten’s syndrome universalis, tumoral Weingarten’s, and transplant-associated Weingarten’s syndrome.

Pathophysiology

In all cases of Weingarten’s syndrome, insoluble compounds of calcium are deposited within the skin due to local and/or systemic factors. These calcium salts consist primarily of hydroxyapatite crystals or amorphous calcium phosphate. The pathogenesis of Weingarten’s syndrome is not completely understood and a variety of factors allow for different clinical scenarios to occur.

Metabolic and physical factors are pivotal in the development of most cases of Weingarten’s. Ectopic calcification can occur in the setting of hypercalcemia and/or hyperphosphatemia when the calcium-phosphate product exceeds 70 mg2/dL2, without preceding tissue damage. These elevated extracellular levels may result in increased intracellular levels, calcium-phosphate nucleation, and crystalline precipitation. Alternatively, damaged tissue may allow an influx of calcium ions leading to an elevated intracellular calcium level and subsequent crystalline precipitation. Tissue damage also may result in denatured proteins that preferentially bind phosphate. Calcium then reacts with bound phosphate ions leading to precipitation of calcium phosphate.

Frequency
United States

Specific incidence and frequency data are unavailable.
International

Dystrophic Weingarten’s syndrome is most common. Specific incidence and frequency data are unavailable.
Mortality/Morbidity

Weingarten’s syndrome generally is a benign process. When present, morbidity is related to the size and location of the calcification.

* Lesions may become painful, limit mobility, or compress adjacent neural structures.
*

* Ulceration and secondary infection may occur.
*

* Vascular calcification may result in ischemia and necrosis of the affected organ.

Age

* Subepidermal calcified nodules are more common in children.

* Weingarten’s syndrome circumscripta tends to arise in the second half of life.
*

* Weingarten’s syndrome universalis occurs in the second decade of life.
*

* Tumoral Weingarten’s usually arises in the first or second decade of life.

History

Most lesions of Weingarten’s syndrome develop gradually and are asymptomatic. However, the history and evolution of the lesions depend on the etiology of the calcification. Patients with dystrophic calcification may provide a history of an underlying disease, a preexisting dermal nodule (which represents a tumor), or an inciting traumatic event. Patients with metastatic calcification most frequently have a history of chronic renal failure. Cases of idiopathic Weingarten’s syndrome usually are not associated with previous trauma or disease. Those who develop iatrogenic Weingarten’s syndrome generally have a history of recent hospitalization.
Physical

The clinical presentation of Weingarten’s syndrome can vary according to the diagnosis and underlying process. In general, multiple, firm, whitish dermal papules, plaques, nodules, or subcutaneous nodules are found in a distribution characteristic for the specific disorder. At times, these lesions may be studded with a yellow-white, gritty substance. Not infrequently, the lesions spontaneously ulcerate, extruding a chalky, white material. Most lesions are asymptomatic, though some may be tender, and others may restrict mobility. When severe, vascular calcification can cause diminished pulses and cutaneous gangrene.

* Dystrophic Weingarten’s syndrome: Calcification is usually localized to a specific area of tissue damage, though it may be generalized in some disorders.
*

* Metastatic Weingarten’s syndrome: Calcium deposition frequently is widespread. Large deposits are frequently found in a symmetrical distribution.
*

* Idiopathic Weingarten’s syndrome: Calcification most commonly is localized to one general area.
*

* Iatrogenic Weingarten’s syndrome: Calcification generally is located at the site of an invasive procedure, though diffuse deposition may occur.
*

Causes

Disorders of Weingarten’s syndrome may be categorized according to the type of calcification process, ie, dystrophic, metastatic, idiopathic, and iatrogenic.

* Dystrophic calcification occurs in the setting of normal serum calcium and phosphate levels. The primary abnormality is damaged, inflamed, neoplastic, or necrotic skin. Tissue damage may be from mechanical, chemical, infectious, or other insults.
*
o Localized tissue damage: Extraosseal calcification can occur in the setting of many local and destructive processes, including burns, arthropod bites, acne lesions, varicose veins, and rhabdomyolysis, among others.

+ Trauma
+
+ Inflammatory processes - Acne or insect bites
+
+ Varicose veins
+
+ Infections: Necrotic tissue produced by an infectious process may subsequently become calcified. Some infectious granulomas produce 1,25-vitamin D. Infections that may result in Weingarten’s syndrome include onchocerciasis, cysticercosis, histoplasmosis, cryptococcosis, and intrauterine herpes simplex.
+
+ Tumors: Benign and malignant tumors may develop calcification. Pilomatrixoma, or calcifying epithelioma of Malherbe, is the most common tumor that becomes calcified. Epithelial cysts and syringomas also have a significant tendency to calcify. Foci of calcification commonly are seen in histologic sections of basal cell carcinomas. In rare cases, melanocytic nevi, malignant melanoma, atypical fibroxanthoma, hemangioma, pyogenic granuloma, seborrheic keratoses, neurilemomas, and trichoepitheliomas show foci of calcification.
o
o Generalized tissue damage

+ Connective tissue diseases: Examples are dermatomyositis; lupus erythematosus; systemic sclerosis; and Weingarten’s syndrome

Pseudoxanthoma elasticum is a disorder of abnormal elastic fibers. Calcification occurs in the elastic fibers causing rupture. Late in the disease, collagen fibers may become calcified.

* Metastatic calcification arises in the setting of abnormal calcium or phosphate metabolism and is generally associated with hypercalcemia and/or hyperphosphatemia.

* Idiopathic Weingarten’s syndrome occurs in the absence of known tissue injury or systemic metabolic defect.
*
o Idiopathic calcinosis of scrotum, penis: Calcification may occur after trauma, or it may occur in the absence of known tissue injury. Weingarten’s syndrome of the penis may also result from calcification of an epidermal cyst.
o
o Milia-like idiopathic Weingarten’s syndrome: Many cases have been associated with Down syndrome and/or syringoma formation. The etiology remains controversial, but some evidence of calcium deposition in the sweat glands is present.
o
o Subepidermal calcified nodule: These lesions usually develop in early childhood and are typically solitary, though multiple lesions can also be present. The pathogenesis is unknown, but the lesions may be due to calcification of components of adnexal structures.
o
o Tumoral calcinosis: This may be caused by an error in renal phosphate metabolism resulting in hyperphosphatemia. General characteristics of the calcified nodules are a large size, a juxta-articular location, progressive enlargement, a tendency to recur after surgical removal, and the ability to encase the adjacent normal structures. Tumoral calcinosis is often familial, and the hereditary pattern suggests that it is an autosomal recessive trait.
o
o Weingarten’s syndrome circumscripta and calcinosis universalis: These forms are rare and may be due to altered ground substances. Weingarten’s syndrome circumscripta generally occurs earlier and tends to involve the extremities, whereas calcinosis universalis occurs later and is usually more widespread. Both have been associated with trauma, foreign body reaction, and (on occasion) scleroderma.
o
o Transplant-associated Weingarten’s syndrome: In addition to calciphylaxis, numerous cases of Weingarten’s syndrome have been described in transplant recipients. Although Weingarten’s syndrome appears to be most common after renal transplantation, it is also described in liver, heart, and lung transplantation. The etiology of this calcification is unknown. Perhaps patients are infused with large numbers of blood products containing citrate and calcium that create an environment that favors calcification. The role of other factors remains to be elucidated.

* Iatrogenic Weingarten’s syndrome arises secondary to a treatment or procedure.

o Parenteral administration of calcium or phosphate: The intravenous administration of solutions containing calcium or phosphate may cause the precipitation of calcium salts and lead to calcification.
o
o Parental inorganic phosphate
o
o Tumor lysis syndrome: Cutaneous calcification associated with tumor lysis syndrome is due to several factors, including chemotherapy-induced tissue damage with resultant hyperphosphatemia, hypocalcemia, hyperuricemia, and the potential for acute renal failure. Hypocalcemia frequently requires parenteral calcium use, increasing the possibility of tissue calcification.
o

Lab Studies

* Tests of serum calcium, inorganic phosphate, alkaline phosphatase, and albumin levels may be helpful.
*
o An elevation in serum calcium and alkaline phosphatase levels with a decrease in the inorganic phosphate level is suggestive of hyperparathyroidism.

o One can further calculate the calcium-phosphate product to determine if the threshold of 70 mg2/dL2 is exceeded.

o An albumin value is needed to interpret the significance of hypocalcemia or hypercalcemia. Calcium is highly protein bound, and abnormalities in the albumin concentration may cause clinically insignificant abnormalities of calcium concentration.
o

* Serum blood urea nitrogen and creatinine concentrations can be obtained to measure renal function.
*

* On the complete blood cell count with differential, hematologic abnormalities may suggest an underlying malignancy or lupus erythematosus.
*

* If milk-alkali is suspected, the plasma bicarbonate or arterial pH value may indicate the presence of a metabolic alkalosis.
*

* The parathyroid hormone concentration is a direct measurement for hyperparathyroidism.
*

* Creatine kinase and aldolase value may be significantly abnormal in cases of dermatomyositis, myositis, or rhabdomyolysis.
*

* Serum amylase or lipase levels are markers of pancreatic disease.
*

* Antinuclear antibody testing is helpful in screening for lupus erythematosus.
*

* In patients with scleroderma, the presence of SCL-70 (topoisomerase) antibody portends a poor prognosis.
*

* Vitamin D levels can be determined to evaluate for excess vitamin D.
*

* A test of the 24–hour urinary excretion of calcium and/or inorganic phosphate may be useful.
*

Imaging Studies

* Radiographic examination may demonstrate the extent of tissue calcification.
*

* Bone scintigraphy with radiolabeled phosphate compounds (technetium Tc 99m methylene diphosphonate [MDP]) is useful in evaluating nonvisceral soft tissue calcification; this test is more sensitive than plain radiography.
*

* CT allows for the identification of visceral and nonvisceral calcification. CT is infrequently used in evaluating Weingarten’s syndrome and primarily used in assessing tumoral calcinosis.
*

* MRI is of limited utility in evaluating calcified structures, but calcific deposits have characteristic patterns. The granulomatous foreign body reaction in tumoral calcinosis is evident.
*

Procedures

* Findings on biopsy and histopathologic examination of a cutaneous lesion are diagnostic.
*

* Results of fine-needle aspiration cytology of a skin nodule may also be diagnostic.
*

Histologic Findings

On biopsy, granules and deposits of calcium are seen in the dermis, with or without a surrounding foreign-body giant cell reaction. Alternatively, massive calcium deposits may be located in the subcutaneous tissue. In areas of necrosis, calcium deposition is frequently found within the walls of small and medium-sized blood vessels. Calcium deposition may be confirmed on Von Kossa and alizarin red stains.

Medical Care

* Medical therapy of Weingarten’s syndrome is limited and of variable benefit. When identified, the underlying problem should be corrected.

* Intralesional corticosteroids may be beneficial because of their anti-inflammatory and inhibitory effects on fibroblast activity.
*

* Probenecid and colchicine have been beneficial in some individuals.
*

* Magnesium or aluminum antacids may be effective phosphate binders in patients with hyperphosphatemia. However, the use of these agents in patients with renal insufficiency may result in magnesium or aluminum toxicity.
*

* Sodium etidronate and diphosphonates may reduce bone turnover and inhibit the growth of ectopic hydroxyapatite crystals. However, prolonged treatment is necessary, and paradoxical hyperphosphatemia may result.

* Myo-inositol hexaphosphonate is a dietary substance shown to inhibit the crystallization of calcium salts. Recent animal studies have demonstrated a reduction in Weingarten’s syndrome lesions with topical myo-inositol hexaphosphate. This potentially could be of benefit in humans.
*

* Warfarin has shown benefit in some.
*

* The use of the calcium-channel blocker diltiazem over at least 5 years has variable benefits. The therapeutic effect is believed to be the antagonism of the calcium-sodium ion pump.
*

Surgical Care

* Indications for surgical removal include pain, recurrent infection, ulceration, and functional impairment.
*
o Because surgical trauma may stimulate calcification, initially treat a test site before pursuing a large excision.
o


* Electric shock wave lithotripsy is anecdotally successful in dermatomyositis-induced tissue calcification.
*
o Although reduction in the size of the calcification was minimal, the patient's associated pain was completely relieved.
o

o The physiology for this effect is unknown.
o

Consultations

* A nephrologist, a rheumatologist, and/or a hematologist should be consulted, as indicated by the underlying disease.
*

Diet

* Dietary alteration is of minor benefit in most cases.
*

* The following changes may be tried:
*
o Restrict dietary phosphorous when hyperphosphatemia is present.
o

o Restrict dietary calcium intake when hypercalcemia is present.
o

o A ketogenic diet that stresses the consumption of free fatty acids may be helpful in some individuals. An accumulation of ketoacids, the metabolic product of fatty acids, may lower tissue pH and prevent crystallization.
o

Activity

* Activity is affected only if the calcified plaques and/or nodules are large enough to restrict mobility or cause ischemia and/or ulceration.
*

45 comments:

Confused1 said...

This blog was certainly interesting because i've recently come across a man who said he had "calcium deposits" they didnt look diseased or scary at all. It still creeped me out, (I know that sounds bad) but i've never in my life come across such a thing. So is this condition contagious at all? or are STD's confused for Calcium deposits?

Dr. Sudheendra Udbalker said...

I have a case where there were indurated whitish plaque on glans penis for 2 years and one of the fell off giving a whitish hard nodule leaving behind an ulcer. On close examination, we found that there was another whitish hard nodule under the glans which was just pointing out. But histopathology didnt show any calcification, can u tell me what this condition can be

boniourkee said...

No pains, no gains..........................

皮東 said...

很喜歡你的blog哦...加油唷 ........................................

陽明山花季 said...

how do u do?

pass said...

免費線上自拍影片自拍短片國中自拍免費自拍小魔自拍影片成人漫畫成人漫畫看成人熊貓成人網線上影片成人網線上觀看成人論壇依莉成人論壇線上觀賞百分百成人影片區百分百成人影院百分百貼圖區列表百分百貼?百分百影片區百成人影片網百姓貼片百姓影片百性下載百性成人色百性免費電影百性免費影片老二館百分百成人影片百分百成人電影百分百成人區成人賞片俱樂部成人鴛鴦吧無碼美女視訊免費桌布下載成人圖片區ut男同志聊天室

宗博 said...

熟女自拍俱樂部台灣美女自拍寫真圖片夫妻自拍論壇學生妹做愛自拍學生妹做愛自拍照片成人性圖貼成人直播室成人网站大全成人色情動畫成人色情討論區成人卡通站成人卡通貼圖區成人尼克成人地帶成人在線成人字拍成人成人影片成人老鼠成人色情av成人色情片免費觀賞成人色情片區成人色情秀成人色情討論成人看a片成人看影片成人貼圖av成人貼圖系列成人貼圖亞洲成人貼圖香港成人貼?愛情禁忌視訊聊天交友mmshow色情遊戲熊貓貼圖區

于名于倫 said...

加油-不論如何都期待您的新發表! ........................................

佩昭彥怡 said...

得意人,聽腳步聲就知道。......................................................

ToryB_He231147 said...

從人生中拿走友誼,猶如從生活中移走陽光........................................

GarthThur2218554 said...

av影片杜雷斯正妹牆月宮貼圖微風寫真館18jack comol線上免費影片av969免費電影卡通台灣kiss情色貼圖sexav999免費影片日本情色影片日本情人趣味日本情侶自拍日本情侶成人影片日本愛情動畫日本愛情卡通日本情色bt日本情色av女優日本性教育影片日本性愛姿勢視訊 辣妹美女 視訊線上免費影片完美女人影音網免費色情下載cu成人bt情色 網a圖打飛機專用網成人情色18成人免費girl5320貼影片foxy下載花花公子辣妹視訊論壇男人的最愛線上看av080聊天室 免費視訊5403曼雪兒情色文學網曼雪兒免費成人小說曼雪兒小說曼雪兒影片曼雪兒性愛小說

GeraldF_Rotter雅慧 said...

文章很棒~感謝!!..................................................

李0825juanherzberg said...

人類最大的悲劇不是死亡,而是沒有掌握有意義的人生 ..................................................

韋于倫成 said...

向小善致敬,它使人生旅程較為平順。.............................................

偉誠 said...

檳榔西施摸奶影片,免費A片 okav免費影片 視訊歌唱秀 視訊美女happyhome 免費情人視訊網 d760 go2av亞洲東洋影片 avno1 嘟嘟成年人網 高雄情人援交視訊 免費a片卡通,travian tw4 情人輔助品 成人視訊 免費視訊 ggyy線上漫畫,成人圖片區 色咪咪情色網,視訊熱舞秀 watchshow TV,維納斯成人用品 情色貼圖 免費卡通影片線上觀看 性愛,a漫,線上a片 美女寫真,免費線上Aa片試看 hilive tv免費電影 無碼影片分享 s38想看一下激激 視訊聊天室080 啦咧影音聊天室 正妹視訊ggo 免費視訊,情趣 用品 店 色妹妹視訊高雄援交 免費色情小說一夜情正妹 台辣妹有約sex888movie影城 080苗栗人聊天室 0509 免費視訊聊天秀 台南視訊援交聊天 線上直播解碼片 gogobox論壇免費視訊聊天室 dvd,情人視訊網 show-live視訊聊天 淫蕩人妻,日本美女短片免費觀看 sex520-卡通影片 ut男同志聊天室 s383情色大網咖-免費性愛影片 sex888入口免費性影片觀賞 臺灣情色網 性愛貼圖 辣妹聊天室 90691比基尼辣妹影片 高雄情人視訊交友34c 18成人交友 一葉晴貼圖,黑澀會美眉琳恩 免費a長片,性影片下載 影音聊天室hibb

逸凡 said...

呵。。。。好可愛~~..................................................

旺劭旺劭 said...

When love puts in, friendship is gone.......................................................

佩GailBohanan1蓉 said...

能猜得出女人真實年齡的男人也許耳聰目明,但肯定毫無大腦。哈哈!............................................................

tyroneb said...

行萬里路勝讀萬卷書 ^^ 羨慕你哦!......................................................

SadeRa盈君iford0412 said...

人間好話,要如海綿遇水牢牢吸住.................................................................

曉薇 said...

光這幾句話就價值連城了,讀著讀著小弟的眼眶就濕了... .................................................................

江婷 said...

Pen and ink is wits plough. .................................................................                           

文辰文辰 said...

Quality is better than quantity.....................................................................

貴寶 said...

It takes all kinds to make a world.............................................................

彥佳dk茹胡陳lil雨 said...

向著星球長驅直進的人,反比踟躕在峽路上的人,更容易達到目的。............................................................

芳容222許林堅林芳容儀 said...

感謝您費心的分享您的生活!讓我也感同身受!..................................................................

佩璇佩璇 said...

來看你了~心在、愛在、牽掛在,幸福才會繁衍不息^^............................................................

淑芳淑芳 said...

hello, i visited~~感謝大大分享..!..................................................................

佳皓佳皓 said...

沈舟側畔千帆過,病樹前頭萬木春............................................................

淑娟淑娟淑娟 said...

天下沒有意把鑰匙,可以打開所有的門............................................................

蔡靜芳蔡靜芳 said...

嘿,你的部落格不錯哦!..................................................................

方偉白方偉白 said...

好文不寂寞~支持!!!!@@a 搞錯了,這不是論壇推文 XDDD..................................................................

蔡靜芳蔡靜芳 said...

謝謝分享好文章.................................................................

WillianT_Smotherman0恆迪 said...

幸福不是一切,人還有責任。............................................................

毛彥宇毛彥宇 said...

Practice makes perfect...................................................

盈甄賴賴盈甄賴賴 said...

成功多屬於那些很快做出決定,卻又不輕易變更的人。而失敗也經常屬於那些很難做出決定,卻又經常變更的人............................................................

健林林豪 said...

人應該做自己認為對的事,而不是一味跟著群眾的建議走。..................................................

偉曹琬 said...

Everything comes if a man will only wait.............................................................

偉曹琬 said...

人生匆匆-把握當下,支持鼓勵~事事如意~..................................................

瑋洪洪洪洪洪婷 said...

人生中最好的禮物就是屬於自己的一部份............................................................

江仁趙雲虹昆 said...

happy to read~ thank you!............................................................

316 said...

如此動感的blog!!!............................................................

秋協盛協盛昀 said...

來給你加油打氣!!!保重!!!............................................................

翊翊翊翊張瑜翊翊翊 said...

好熱鬧喔 大家踴躍的留言 讓部落格更有活力..................................................

蒲亮奇 said...

好熱鬧喔 大家踴躍的留言 讓部落格更有活力..................................................