Idiopathic calcinosis of the scrotum: report of five cases and review of the literature

Although calcinosis of the scrotum was first described in 1883 by Lewinski [1] its nature and cause have remained elusive. Hutchinson in 1888 [2], emphasized that these calcific masses appear with no evidence of underlying cause. Various theories regarding the etiology and pathogenesis of the condition have been proposed, non has been widely accepted. “Idiopathic scrotal calcinosis” (ICS), a term applied by Shapiro in 1970 [3], is most commonly used. About 118 cases have been reported. A series of five cases is reported in which scrotal calcinosis developed. A review of the literature and a discussion on the etiology in this disease is also included. Received: 28 July 1997 / Accepted: 16 December 1997     

Unusual cause of calcinosis cutis

An unusual cause of calcinosis cutis is reported in a 73-year-old woman following subcutaneous fluid replacement, 36 years before consultation. Late secondary infection of the calcium deposits, 35 years after the causative event, was followed by local and systemic symptomatology. Healing was only achieved after wide surgical excision of the involved tissue and skin grafting. Correspondence to: Y Ullmann     

Tumoral calcinosis: a case report

Tumoral Calcinosis is a distinct clinical and histological entity that is characterized by large periarticular deposits of calcium resembling a neoplasm and is found foremost in the region of hip, shoulder and elbow. We report a case of Tumoral Calcinosis in a 25-year-old male who presented to us with gradually increasing swelling of right axilla, and both hips of nearly two years duration. It was diagnostic enigma for the treating surgeons but with the help of an astute pathologist we diagnosed this rare condition and successfully treated it surgically.     

Electrochemotherapy for digital chondrosarcoma

Electrochemotherapy (ECT) delivers nonpermeable anticancer drugs to cell interiors by temporally increasing the permeability of the cytoplasmic membrane under locally applied pulsating electrical stimuli. This treatment results in consistent and enhanced pharmacological effects of drugs on the targeted tissue. ECT has been used for surface skin cancer but never for musculoskeletal tumors. This report describes a clinical trial of ECT for digital chondrosarcoma. A 74-year-old woman with a digital chondrosarcoma was administered electric stimulation with two surface electrodes 10 min after intratumoral multiple injection of bleomycin sulfate and 15 s after intraarterial perfusion of bleomycin sulfate. Biopsy performed after ECT showed 90% tumor necrosis. Marginal resection of the tumor was followed by autologous bone grafting to fill the bone defect. Although the follow-up period was short (3 years), the patient remained disease-free after ECT and was satisfied that amputation of the affected finger could be avoided. This preliminary result suggests that ECT is a viable modality for limb-preserving treatment of patients with sarcoma of the extremities. Received: August 16, 2002 / Accepted: November 29, 2002 Offprint requests to: T. Nikaido     

Scrotal calcinosis: A case report

Scrotal calcinosis (SC) was a rare and benign condition characterized by multiple calcific substances deposits occurring in scrotum and formed nodules and lumps within scrotal skin. A case of a 49-year-old male patient with a 7-year history of scrotal calcinosis was reported. Histopathological findings had not showed evidences of epithelial structures. In our case, no evidence of cystic structure was found around calcified materials. It was indicated that SC might be idiopathic.     

Popliteal pterygium syndrome associated with sacrococcygeal agenesis

A case of popliteal pterygium syndrome (PPS) associated with total sacrococcygeal and partial lumbar vertebrae agenesis is described. This is the first report of this combination. The case was sporadic; both legs were involved and were treated surgically. The possible etiological factors, various clinical presentations and available methods of treatment are discussed. Received: 25 August 1999 / Accepted: 17 January 2000     

Focal mineralization defect during disodium etidronate treatment of calcinosis

Summary The use of disodium etidronate (EHDP) for the treatment of calcinosis is complicated by the threat of drug-induced inhibition of skeletal mineralization. Adults with Paget's disease of bone treated for 6 months with 10–20 mg/kg/day of EHDP have been reported to show both a marked delay in mineralization and a diffuse excess of unmineralized bone matrix. Drug-induced bone disease is, however, a function of growth as well as of the dose and duration of therapy. Therefore, children treated with EHDP may respond differently to the drug-induced mineralization defect. A 10-year-old girl with dermatomyositis developed incapacitating ectopic calcification. After 9 months of therapy with 12 mg/kg/Day of EHDP, a small decrease in the calcinosis was accompanied by a dramatic increase in joint mobility. Bone mineral content of the radial diaphysis showed a failure to gain mineral density as expected with prepubertal growth (8 cm/year). Bone biopsy revealed a patchy excess of osteoid. Although the percentage of osteoid surface labeled by tetracycline was reduced, normal mineralization was evident in the double-labeled areas. In children, the mineralization defect occurring with EHDP treatment may be focal.     

Biochemical analysis of collagen in adhesive tissues formed after digital flexor tendon injuries

 Restrictive adhesion is a major problem following flexor tendon surgery. We investigated crosslinks and types of collagen in two adhesive tissue models in chickens, characterized by loose adhesion and dense adhesion. The flexor digitorum profundus tendon was cut and sutured in two ways: (1) the severed tendon was sutured outside the tendon sheath (loose adhesion model), and (2) the tendon sheath was excised and the severed tendon was sutured on the damaged bony floor (dense adhesion model). Biochemical analysis of collagen was done for each group after tenorrhaphy. In the outside-sheath group, the ratio of the collagen crosslinks, dihydroxylysinonorleucine/hydroxylysinonorleucine, was lower than that in the sheath-excised group at 12 weeks, while the collagen crosslinks ratio remained considerably high in the sheath-excised group. The ratio of type III collagen to type I collagen in the outside-sheath group was higher than that in the sheath-excised group at 12 weeks and approached an almost normal value at 24 weeks. The collagen type III/I ratio remained low in the sheath-excised group. The current study shows that biochemical properties are different between loose and dense adhesions. In dense adhesive scars, pathological collagen turnover occurs even at 24 weeks after tenorrhaphy. Received: November 2, 2001 / Accepted: June 3, 2002 Offprint requests to: K. Masuda, Asahikawa Kosei Hospital, 24-111, 1-jo-dori, Asahikawa, Hokkaido 078-8211, Japan     

Poland syndrome associated with renal agenesis

Poland syndrome is characterized by unilateral aplasia or hypoplasia of the sternocostal portion of the pectoralis major muscle and ipsilateral syndactyly. In some cases other associated anomalies, including renal malformations, dextrocardia, and vertebral abnormalities, have been reported. We report a 7-month-old girl with Poland syndrome who also presented with ipsilateral renal agenesis. This report suggests that renal structural anomaly may be an integral part of this syndrome. We recommend renal imaging studies be performed on all children with Poland syndrome. Received: 23 July 2001 / Revised: 23 October 2001 / Accepted: 23 October 2001     

Apolipoprotein E polymorphism in childhood nephrotic syndrome

Recent clinical reports have demonstrated that the progression and prognosis of renal diseases are possibly influenced by apolipoprotein E (apoE) genotypes and alleles. In this study we investigated whether apoE genotypes and alleles can be a prognostic criterion for the steroid responsiveness in childhood nephrotic syndrome. One hundred and seven pediatric patients with primary idiopathic nephrotic syndrome and 83 healthy volunteers were enrolled in the study. Eighty-seven of the patients had steroid-sensitive nephrotic syndrome (SSNS) and 20 had steroid-resistant nephrotic syndrome (SRNS). The ɛ2 allele frequency and ɛ2/3 genotype frequency of the SNRS group were statistically higher when compared with SSNS and control groups (P<0.05). The higher frequency of the ɛ2 allele in steroid resistant nephrotic patients suggests that the ɛ2 allele gives a possible genetic predisposition to steroid resistance in our population, but further studies are needed to clarify this subject. Received: 15 November 2000 / Revised: 15 November 2001 / Accepted: 18 November 2001     

Disseminated autoimmune disease during levamisole treatment of nephrotic syndrome

Side effects such as cutaneous vasculitis, which occur during prolonged levamisole treatment, may discourage the utilization of the drug in relapsing nephrotic syndrome. We describe a child who developed disseminated vasculitis during prolonged treatment with levamisole. The acute phase was characterized by hepatosplenomegaly, hemolytic anemia, IgM anticardiolipin and p-antineutrophil cytoplasmic antibodies. One month after withdrawal of therapy all symptoms had disappeared and tests normalized. This case report, together with other reports on cutaneous vasculitis, suggest caution and close monitoring during prolonged levamisole therapy. Received: 24 June 1998 / Revised: 17 November 1998 / Accepted: 20 November 1998     

Murray-Puretic syndrome: a report of two cases

The Murray-Puretic Syndrome is also known as juvenile hyaline fibromatosis. It is a congenital disorder characterized by skin lesions, joint contractures, gingival hypertrophy and bone lesions. Two siblings with this syndrome are presented. Received: 28 June 1996 / Accepted: 23 March 1998     

A pharmacokinetic study of Neoral in childhood steroid-dependent nephrotic syndrome

Seven children with steroid-dependent nephrotic syndrome who were on stable remission under Sandimmun therapy were switched to Neoral at the same dosage. During the 4-month follow-up period, two patients relapsed, due to poor compliance in one of them. Serum creatinine remained stable in all patients. Pharmacokinetic profiles were performed at day 0 while on Sandimmun and 4 weeks after conversion to Neoral. Following conversion to Neoral, the peak concentration occurred earlier (2±1.4 h vs 3.9±2.4 h), and the maximum concentration (677±386 ng/ml vs 488±265 ng/ml) and the area under the curve (3082±1536 ng/ml/h vs 2201± 889 ng/ml/h) were higher. We conclude that Neoral results in an increased bioavailability of cyclosporine (CsA) as compared to Sandimmun in patients with steroid-dependent nephrotic syndrome in remission. Received: 28 April 2000 / Revised: 5 October 2000 / Accepted: 5 October 2000     

Hypercalciuria and urolithiasis in a case of Costello syndrome

 Costello syndrome is characterized by postnatal growth deficiency, mental retardation, curly hair, coarse characteristic face, and loose skin of hands and feet. Patients with this syndrome have a high incidence of cardiac involvement, including arrhythmia, atrial septal defect, and hypertrophic cardiomyopathy. We report a 16-year-old adolescent female with Costello syndrome who presents with hypercalciuria and urolithiasis. Received: 15 October 1997 / Revised: 31 March 1998 / Accepted: 31 March 1998     

Inclusional body myositis localized to wrist and digital flexors of the left upper limb

A clinical case of inclusion body myositis, an infrequent type of inflammatory myositis, is reported. In addition to the rarity of the disease, this case had a unique clinical presentation. A full account of this condition is presented, including its epidemiological aspects, clinical presentation, etiopathogenesis, laboratory and histopathological findings, differential diagnosis and treatment. Received: 4 December 1997 / Accepted: 2 February 1998     

The proximal dorsal digital flap

In 9 patients, 10 flaps based on the dorsal branches of the digital artery from the dorsum of the proximal phalanx were used to cover tissue defects on the volar aspect of the fingers. Full-thickness skin grafts were applied to the donor defects. All flaps survived completely with no donor site morbidity. This flap appears to be an easy and reliable way of covering the flexor tendon, the digital nerve and the digital artery after contracture release or for skin defects on the volar surface of the proximal phalanx. Received: 15 December 1997 / Accepted: 31 March 1998     

Relationship between iron, calcium and phosphate during experimental cutaneous calcinosis

Calcification of the skin (calciphylaxis) induced by the subcutaneous administration of iron chloride to dihydrotachysterol (DHT)-sensitized rats is preceded by the selective deposition of iron (visible with electron microscopic techniques) on the surface of collagen fibrils. Electron probe X-ray microanalysis indicates that the injectionof iron alone suffices to increase local concentrations of calcium and phosphorus; however, apatite formation occurs only in animals pretreated with DHT. The amount and degree of crystallinity of the inorganic material is very similar on the sixth and thirtieth day after the beginning of the experiment.

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Zusammenfassung Durch elektronenmikroskopische Untersuchungen wurde festgestellt, daß die durch subcutane Verabreichung von Eisenchlorid bei Dihydrotachysterin-sensibilisierten Ratten herbeigeführte Hautverkalkung durch eine selektive Eisenablagerung auf der Oberfläche der Kollagenfibrillen eingeleitet wird. Die Röntgenmikroanalyse zeigt an, daß die lokale Calciumund Phosphorkonzentration bereits nach Eiseninjektion allein ansteigt, während Apatitbildung nur in den mit DHT vorbehandelten Tieren erfolgt. Ausmaß und Menge der Kristallisation sind am 6. und 30. Tage nach Versuchsbeginn fast gleich.

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Résumé La calcification de la peau (calciphylaxie) provoquée par l'administration sous-cutanée de chlorure de fer chez des rats sensibilisés par le dihydrotachystérol (DHT) est précédée par une déposition sélective de fer (décelable à la microscopie électronique) à la surface des fibres collagènes.Les analyses à la microsonde prouvent que les taux de calcium et de phosphore sont augmentés à l'endroit de l'injection du chlorure de fer même sans l'administration préalable de DHT; cependant, l'apatite ne se forme que chez les animaux prétraités par le DHT.La quantité et le degré de cristallinité de la matière inorganique sont presque identiques au sixième et au trentième jour de l'expérience.

     

Abortive cryptophthalmos and cup ear deformity (Fraser syndrome): case report and literature review

Cryptophthalmos is a rare eyelid anomaly that is part of Fraser syndrome (cryptopthalmos-syndactyly syndrome). The two major features of this syndrome, cryptophthalmos and syndactyly, may be absent in some cases. In this paper, a case of Fraser syndrome without syndactyly is presented. The importance of ear and nose anomalies in this syndrome is emphasized. Received: 20 July 1998 / Accepted: 22 September 1998     

Steroids in the hemolytic uremic syndrome

To determine whether steroids could be of clinical benefit in the treatment of the hemolytic uremic syndrome (HUS), we conducted a randomized, double-blinded, placebo-controlled trial of methylprednisone (5 mg/kg per day in four oral doses over 7 days), in children with HUS during the acute phase. Ninety-two patients with typical HUS (47 receiving placebo and 45 receiving steroids) were investigated for neurological, hematological, and nephrological variables. There were no differences between groups in the number of convulsive episodes or transfusion requirements during the hospital stay. Serum creatinine levels were slightly increased on day 10 in the placebo group compared with the steroid group (P = 0.06) and declined significantly between days 1 and 10 only in the steroid group (P = 0.001). In the 51 patients with anuria (24 placebo, 27 steroids), median serum creatinine levels were reduced in the steroid group compared with the placebo group on the 10th day (P = 0.01). Differences in median days of oliguria [11.5 versus 8 (P = 0.28)], anuria [5 versus 7 (P = 0.20)], and dialysis [12 versus 10 (P = 0.26)] for the placebo and the steroid group respectively were not significant. Our data suggest that oral steroids are not able to modify hematological, neurological, or nephrological clinical parameters during the acute phase of childhood HUS, even though they do seem to be associated with a more rapid decline in serum creatinine levels. Received April 9, 1997; received in revised form September 8, 1997, accepted September 10, 1997