Calcinosis cutis universalis

We report the case of a 49-year-old female who complained of hardening of the skin, with onset about 1.5 years before presentation. The laboratory data showed normal biochemistry profile. Routine haematochemical examinations showed slight anaemia, an increased erythrocyte sedimentation rate and negative rheumatological markers. Calcium excretion in a 24-h urine sample was normal, but the phosphate excretion was slightly low. The clinical diagnosis was verified by soft tissue ultrasound examination showing subcutaneous calcifications. X-ray examination of bones evidenced no abnormal calcification. Mammography revealed deep seated bilateral reticular calcifications, even in the axillary region. Histological examination showed calcinosis cutis. On these grounds, the diagnosis of idiopathic universal calcinosis cutis was made. The authors describe the clinical and histological picture and discuss the laboratory findings.     

Disseminated linear calcinosis cutis associated with the Koebner phenomenon in an infant with congenital acute monocytic leukaemia

We present a unique case of an infant with acute monocytic leukaemia who presented at birth with multiple rubbery, erythematous to violaceous subcutaneous nodules secondary to leukaemia cutis. As these infiltrates regressed with chemotherapy, numerous white to yellow linear confluent papules appeared in a scratch-like pattern. These lesions were widely disseminated but were concentrated across her face, trunk and extremities with relative sparing of the napkin area and back. We propose that these lesions represent a form of dystrophic calcinosis cutis that occurred secondary to koebnerization in an infant with congenital leukaemia cutis.     

Calcinosis cutis following contact with calcium chloride solution

Calcinosis cutis is the deposition of insoluble calcium in the cutaneous tissue. Calcinosis cutis can be classified as metastatic, dystrophic, idiopathic or exogenous. We report a 48‐year‐old white man who was dismantling a portable ice skating rink when calcium chloride solution from the pipes spilt onto his clothing. Several days later, he started to develop mildly pruritic erythematous papules, some studded with white deposits and some with umbilication over the exposed areas corresponding to the spillage of the calcium chloride solution. Histological features revealed interstitial fibrohistiocytic reaction with calcium‐encrusted degenerated collagen bundles in the dermis which was further confirmed by von Kossa stain. He was commenced on topical corticosteroid cream twice daily and the lesions cleared completely between 6 to 10 weeks.     

Calcinosis cutis dermatologic toxicity associated with fibroblast growth factor receptor inhibitor for the treatment of Wilms tumor

Small‐molecule inhibitors (nibs) have revolutionized cancer therapy with the emergence of clinically efficacious treatment for advanced‐stage malignancies. Fibroblast growth factor receptor (FGFR) inhibitors have shown therapeutic efficacy in malignancies with molecular‐genetic alterations in the FGFR/fibroblast growth factor pathway. In a phase 1 clinical trial, erdafitinib, a pan FGFR inhibitor, was well tolerated with a manageable toxicity profile. Hyperphosphatemia was a frequent adverse event in patients treated with erdafitinib; however, no serious complications were observed with this therapy. Here, we report the development of calcinosis cutis dermatologic toxicity in a patient with hyperphosphatemia while treated with a novel selective FGFR inhibitor, INCB 54828‐101. Awareness of this form of dermatologic toxicity from an FGFR inhibitor will be important for close monitoring of serum levels of phosphate, FGF23, vitamin D, and calcitriol, the management of adverse serum chemistry with chelators, and treatment decisions to either reduce dose or withhold FGFR inhibitor.     

Calcinosis cutis in systemic lupus erythematosus: a case report and review of the published work

Calcinosis cutis is a common clinical feature of dermatomyositis and scleroderma but rarely reported in association with systemic lupus erythematosus (SLE). Calcinosis cutis in SLE occurs without calcium and phosphorus metabolic abnormalities and may be localized or generalized. The pathophysiology remains unclear and no effective therapy is currently available. We report a 30-year-old woman with a 13-year history of SLE who developed multiple calcinosis cutis around both knees and we review the relevant published work.     

Solitary Milialike Idiopathic Calcinosis Cutis: A Case Unassociated with Down Syndrome

We report a unique case of solitary milialike idiopathic calcinosis cutis (MICC) in a healthy Korean woman, which is not associated with Down syndrome. This case of MICC would be a form of idiopathic calcinosis cutis, which can be solitary or multiple, sporadic or associated with Down syndrome.     

Raynaud's phenomenon with gangrene of the digits as the revealing sign of an antiphospholipid syndrome

We report the case of a 37-year-old woman who suffered for years of Raynaud's phenomenon associated with gangrene of digits. The patient displayed high levels of circulating antiphospholipid (aPL) antibodies, which on treatment with aspirin decreased significantly along with resolution of both Raynaud's phenomena and distal ischemic necrosis. To our knowledge this is the first report of an aPL syndrome featuring severe Raynaud's phenomenon as the only clinical sign. Possible pathogenetic correlations and the favourable therapeutic effect of aspirin are discussed.     

Diagnosis of Raynaud's phenomenon by thermography

Background/aims: The aim was to clarify whether cold fingers before a moderate cold stress test can predict a prolonged delay (more than 20 min) in rewarming, as diagnostic for Raynaud's phenomenon.
Methods: A retrospective study was conducted on 71 patients suspected of suffering from Raynaud's phenomenon. The thermal gradient from the metacarpophalangeal joints to the finger tips was calculated for each finger and cold fingers were defined by a temperature difference of more than −0.5°2C.
Results: Combining the frequencies of cold fingers with a diagnosis Raynaud's phenomenon resulted in a sensitivity of 78.4%, a specificity of 72.4% and diagnostic accuracy of 74.0%.
Conclusion: Based on a positive predictive value of 58.5%, it was concluded, that a prolonged delay of rewarming after a cold stress test cannot be predicted sufficiently by the presence of cold fingers alone, and that a cold stress test is necessary to confirm the diagnosis objectively.     

Two cases of dystrophic calcinosis cutis in burn scars

Dystrophic calcinosis cutis is defined as the abnormal deposition of insoluble calcium salts in dead or degenerated cutaneous tissues in the absence of abnormal serum calcium or phosphate concentrations. Although dystrophic calcification can occur in various diseases, its occurrence on a burn scar has rarely been reported in the dermatologic literature. Herein we describe two patients who presented with a solitary non-healing ulcer in a postburn scar, with histopathologic evidence of calcium deposition in the dermis.     

Cold-induced Raynaud's phenomenon ameliorated by intravenous administration of ketanserin: a double-blind cross-over study

Summary Fifteen patients suffering from Raynaud's phenomenon (RP) were exmined. They were placed in a climate, chamber (a small room in which the temperature and humidity could be varied). The temperature was gradually decreased, while the humidity was kept constant. After 60–90 min, distinct RP was induced and intravenous medication was administered. The whole trial was performed in a double-blind, crossover fashion. Every patient participated in two experiments performed 2 days apart. Either ketanserin or placebo was given during the first experiment. In the second experiment, the medication (or placebo) which had not been given was administered. The effects of ketanserin were highly significant: the bluish pallor changed into bright erythema and the skin temperature rose significantly. This was interpreted as a spasmolytic effect. Intravenously administered ketanserin has a place in the clinical treatment of acutely deteriorating RP of diverse etiologies.     

雷诺现象的转归研究

将可供长期随访的雷诺现象患者８０例分为两组：原发性雷诺现象组及疑继发性雷诺现象组。经平均６．０３年随访后，前组５２例中仅２例发展为结缔组织病，后组２８例中有１３例发展为结缔组织病，两组差别显著（Ｘ２＝１１．３７，Ｐ＜０．００５）。分析结果示，雷诺现象伴甲皱循环异常，抗核抗体阳性及γ－球蛋白升高与发展为结缔组织病有密切关系。     

A case of hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber's disease) with dystrophic calcinosis cutis and retinal lesions

A 65-year-old Japanese female developed a nodule on the distal interphalangealjoint of her right thumb. She also had multiple telangiectasias on her face, oral mucosa, tongue, nasal mucosa and upper extremities. A lip biopsy showed irregularly dilated capillaries and venules lined by flat endothelial cells. The nodule in the right thumb was resected. Histopathology revealed calcium deposits in the dermis. This case was diagnosed as dystrophic calcinosis cutis within hereditary hemorrhagic telangiectasia. Ophthalmologic examination showed bilateral retinal vascular lesions with dilated and tortuous retinal venules.     

Study of a myo-inositol hexaphosphate-based cream to prevent dystrophic calcinosis cutis

Background Calcinosis cutis is a disorder caused by abnormal deposits of calcium phosphate in the skin and is observed in diverse disorders. Myo‐inositol hexaphosphate (InsP₆) is a diet‐dependent molecule found in all mammalian fluids and tissues, which exhibits an extraordinary capacity as a crystallization inhibitor of calcium salts. Objectives To establish the effects of topically administered InsP₆ cream on artificially provoked dystrophic calcifications in soft tissues. Methods Fourteen male Wistar rats were randomly assigned into two groups: control and treated groups. Rats were fed with an InsP₆‐free or phytate diet. Plaque formation was induced by subcutaneous injection of 0·1% KMnO₄ solution. From 4 days before plaque induction to the end of the experiment, control rats were treated topically with a standard cream, whereas treated rats were treated with the same cream with 2% InsP₆ or phytate (as sodium salt). Calcification of plaques was allowed to proceed for 10 days. InsP₆ in urine was determined. The plaques were excised and weighed. Results It was found that when InsP₆ was administered topically through a moisturizing cream (2% InsP₆‐rich), the plaque size and weight were notably and significantly reduced compared with the control group (1·6 ± 1·1 mg InsP₆‐treated, 26·7 ± 3·0 mg control). The InsP₆ urinary levels for animals treated with the InsP₆‐enriched cream were considerably and significantly higher than those found in animals treated topically with the cream without InsP₆ (16·96 ± 4·32 mg L^?1 InsP₆‐treated, 0·06 ± 0·03 mg L^?1 control). Conclusions This demonstrates the important capacity of InsP₆ as a crystallization inhibitor and also demonstrates that it is possible to propose topical use as a new InsP₆ administration route.     

Hypereosinophilic syndrome presenting as cutaneous necrotizing eosinophilic vasculitis and Raynaud's phenomenon complicated by digital gangrene

Cutaneous necrotizing eosinophilic vasculitis is a recently identified type of vasculitis that is characterized by an eosinophil-predominant necrotizing vasculitis affecting small dermal vessels. Clinically, it presents with pruritic erythematous and purpuric papules and plaques, peripheral eosinophilia and a good response to systemic steroid therapy. This vasculitis can be idiopathic or associated with connective tissue diseases. Although the pathogenic roles of eosinophil-derived granule proteins and interleukins have been documented in diseases associated with eosinophilia, a role of CD40 (a glycoprotein of the tumour necrosis factor receptor superfamily) has rarely been described. We describe two patients with idiopathic hypereosinophilic syndrome (HES) presenting with multiple erythematous patches and plaques on the lower extremities and Raynaud's phenomenon. They satisfied the criteria for the diagnosis of HES by clinical and laboratory investigations. Histopathology of the cutaneous lesions revealed prominent eosinophilic infiltration with local fibrinoid change in vessel walls in the dermis and subcutis. Immunohistochemical detection of CD3, CD4, CD8 and CD40 was performed. Infiltrating eosinophils were strongly stained by anti-CD40 monoclonal antibody. One patient improved with prednisolone, pentoxifylline and nifedipine, without recurrence. The other patient initially improved with steroids, but after self-withdrawal of steroid developed digital ischaemia that evolved to severe necrosis and required amputation. Cutaneous necrotizing eosinophilic vasculitis, Raynaud's phenomenon and digital gangrene may develop as cutaneous manifestations of HES. CD40 may play a part in the pathogenesis of eosinophilic vasculitis in HES.     

Case of dystrophic calcinosis cutis in epidermal cyst arising from verrucous epidermal nevus

Dystrophic calcinosis cutis is diagnosed when calcium is deposited into previously damaged tissue by connective tissue disease, panniculitis, pseudoxanthoma elasticum or trauma. We report a case of dystrophic calcinosis cutis arising from the lesion of an epidermal cyst on the verrucous epidermal nevus. A 20‐year‐old woman presented with a polypoid pinkish tumor on a brownish, verrucous plaque. Histopathological findings of the pinkish tumor showed calcium deposits as amorphous, basophilic material lining the true epidermis in the upper dermis, which were compatible with dystrophic calcinosis cutis and the plaque was diagnosed as a verrucous epidermal nevus.     

Unilateral Beau's lines associatd with a fingertip crushing injury

Beau's lines are transverse depressions or ridgings of the nail surface that are the result of the temporary arrest of nail matrix formation. They are caused by systemic diseases, local trauma, drugs, and infection. Generally, Beau's lines after a fingertip injury occur on only the affected finger, not on the unilateral extremity. In this report, we present the case of a 36-year-old man who developed transverse ridgings in the nail plates of all five fingers of the affected extremity after a fingertip (thumb) crushing injury. During the follow up, the growth rate of affected fingernails was normal. The transverse ridgings advanced with the linear growth of the nails and eventually disappeared. To the best of our knowledge, no association between unilateral Beau's lines and fingertip injury has been reported in the English literature.     

Dystrophic Calcinosis of the Penis

A 22-year-old man had had a dome-shaped tumor on his penis for one year. Neither his particular past history nor family history was available. The excised specimen contained numerous von Kossa-positive deposits. Four types of histologic pattern were identified: 1) a cystic structure filled with amorphous material lined by a few layers of epithelial cells, 2) a cyst containing calcified deposits in the keratinous material, 3) a large calcified nodule lined by attenuated epithelial cyst walls, 4) numerous calcium collections without an epithelial wall. The cystic structure showed the histologic features of syringoma or sweat duct milia. The luminal cells of the cyst showed positive immunoreactivity for both keratin and carcinoembryonic antigen. These findings suggested that the keratinous contents of syringoma had gradually calcified, the cyst wall had been attenuated, and, finally, numerous calcium collections without an epithelial wall were formed. Our case further supported the hypothesis that penile calcinosis as well as scrotal calcinosis might derive from syringoma.