|Year : 2013 | Volume
| Issue : 4 | Page : 444-446
Imaging features in calcinosis circumscripta, a rare type of subcutaneous calcification in localized scleroderma
Pratiksha Yadav, Sarabjit Singh Thind
Department of Radio-diagnosis, Padmashree Dr. D. Y. Patil Medical College, Hospital and Research Centre, Dr. D. Y. Patil Vidyapeeth, Pune, India
|Date of Web Publication||17-Sep-2013|
Granada A-801, Fortaleza, Kalyani Nagar, Pune, Maharashtra
Source of Support: None, Conflict of Interest: None
Calcinosis cutis circumscripta is a rare condition in which abnormal deposition of calcium seen in the dermis and subcutaneous tissue, it is associated with localized scleroderma. A 30-year-old female presented with an area of extensive calcification involving the right gluteal region, lateral aspect of right thigh and a small area on left thigh detected on radiograph with atrophy of subcutaneous tissue. Magnetic resonance imaging and computed tomography were done for further evaluation and the findings were of calcification and atrophy involving the skin and subcutaneous tissue.
Keywords: Calcinosis circumscripta, calcinosis cutis, subcutaneous calcification, morphea
|How to cite this article:|
Yadav P, Thind SS. Imaging features in calcinosis circumscripta, a rare type of subcutaneous calcification in localized scleroderma. Med J DY Patil Univ 2013;6:444-6
|How to cite this URL:|
Yadav P, Thind SS. Imaging features in calcinosis circumscripta, a rare type of subcutaneous calcification in localized scleroderma. Med J DY Patil Univ [serial online] 2013 [cited 2019 Dec 10];6:444-6. Available from: http://www.mjdrdypu.org/text.asp?2013/6/4/444/118281
| Introduction|| |
Calcinosis circumscripta is a descriptive term used for the circumscribed deposits of calcium salts in the skin and subcutaneous tissue. It was first reported in animals. In human, there are two forms of calcinosis cutis, calcinosis circumscripta, and calcinosis universalis.
| Case Report|| |
A 30-year-old female presented with complaints of blackish discoloration involving the right gluteal region and lateral aspect of right thigh and small area on the lateral aspect of left thigh since 5 months [Figure 1], which was progressively increased. It was associated with pain in the right thigh. On examination, there was blackish discoloration of the right gluteal region and right thigh, skin over the affected region was firm, adherent, and rough on touch and there was atrophy of subcutaneous tissues. No nodularity or tenderness seen on the affected region. There were no other systemic complaints present. No history of any previous illness, no evidence of any other skin lesions.
|Figure 1: Picture of the 30-year-old female showed blackish discoloration of the skin on right gluteal region since 5 months|
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Radiograph pelvis anteroposterior and lateral view revealed extensive soft-tissue calcification with atrophy of subcutaneous tissues of the right gluteal region and right thigh extended from the ilium to the mid-thigh [Figure 2]a. A small area on the lateral aspect of left mid-thigh also showed calcification with atrophy of the subcutaneous tissue [Figure 2]b. High-resolution ultrasonography was done subsequently, which revealed calcification and atrophy of the involved subcutaneous tissues; however, underlying muscles were normal in echotexture and thickness.
|Figure 2: (a and b)anteroposterior radiograph of pelvis and upper third of thighs showing extensive soft-tissue calcification involving the lateral aspect extending from mid ilium to mid-thigh. Visualized bones and joints appear normal. Small area of calcification also shows in left mid-thigh|
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Computed tomography (CT) imaging revealed areas of circumscribed calcification in the affected area. Underlying bones and both hip joints were normal [Figure 3].
|Figure 3: (a-c) Non contrast computed tomograpgy axial and coronal images showing calcification involving the subcutaneous tissue of the lateral aspect of right gluteal region and thigh, and a small area on the lateral aspect of the left thigh. Underlying bones appears normal|
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Magnetic resonance imaging (MRI) showed altered signal intensities involving the right gluteal region, lateral aspect of right thigh extended from right ilium to mid-thigh with gross atrophy of the subcutaneous tissue. A small area on the lateral aspect of left mid-thigh was also involved. These areas showed hyperintense signals with few hypointense signals on T2 weighted images and Short T1 inversion recovery (STIR) images [Figure 4]a, hypo intense signal intensity on T1weighted images [Figure 4]b that was suggestive of calcification. Hyper intense signals on STIR were suggestive of inflammation of the subcutaneous tissue [Figure 4]c. Underlying muscles and joints were normal. Histopathology revealed increased collagen bundles in supeficial dermis. Deep dermis and subcutaneous tissue showed thick collagen bundles with area of calcification suggestive of morphea (localised scleroderma) with changes of calcinosis circumscripta [Figure 5]a and b. Antinuclear antibody profile (ANA profile) was not carried out.
|Figure 4: (a-c) Magnetic resonance imaging of thigh Short T1 inversion recovery (STIR)Corona (a),T1weighted axial (b) and STIR axial images (c) showing abnormal signal intensity involving the lateral aspect of right thigh and on lateral aspect of left thigh with gross atrophy of the subcutaneous tissue however, underlying muscles and bones are normal in signal intensity|
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|Figure 5: (a-b) Histopathology showed superficial dermis with increased collagen bundles. Deep dermis and subcutaneous tissue show thick collagen bundles with area of calcification suggestive of morphea with changes of calcinosis circumscripta|
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| Discussion|| |
Calcinosis cutis is a term used to describe the abnormal calcium deposits in the skin due to local or systemic causes. There are two types of calcinosis, calcinosis circumscripta in which calcium is deposited in skin and subcutaneous tissues and calcinosis universalis in which deposition of calcium in skin, subcutaneous tissue, muscles, and tendons.  Calcium is deposited as calcium phosphate or calcium carbonate. Metabolic and physical factors are pivotal in the development of most cases of calcinosis. In cases of hypercalcemia or hyperphosphatemia elevated extracellular levels may result in increased intracellular level and subsequent crystalline precipitation.  Teissier in 1877 first described calcinosis. In 1878, Weber noted the association of calcinosis with scleroderma.  Calcinosis cutis, generally is a benign process. When present, morbidity is related to the size and location of the calcification.  Lesions may become painful, limit mobility of an adjacent joint, or compress adjacent neural structures. Ulceration and secondary infection may occur. Calcinosis circumscripta usually associated with scleroderma, Raynaud's phenomenon, and telangiectasia. It is usually seen in Middle aged patients and is more common in females (6:1). The etiology is unknown.  Localized scleroderma often termed "morphea" in dermatology represents autoimmune disorder characterized by skin thickening and increased collagen deposition with deposition of calcium, it usually affects the dermis and subcutaneous tissue, causes fibrosis and atrophic hardening of the skin; however, it can affect the muscle, fascia, tendons, joint sinovia and bones marrow. Deep involvement may result in irreversible impairment secondary to pain, loss of movement and contracture.  Morphea is classified into circumscribed, generalized, linear, and pansclerotic subtype according to clinical presentation and depth of tissue involvement. Collagen production is increased in all forms of morphea. There are no specific radiological findings of morphea, but skin, subcutaneous and muscle involvement easily recognize on imaging especially on MRI. Importance of MRI in morphea is to evaluate the extent of musculoskeletal involvement. MRI findings of morphea are thickening of subcutaneous fatty tissue septa, intramuscular septa, muscle fasciae, tenosinovitis or articular sinovitis.  Inflammatory stage of morphea reveal infiltration in the sucutaneous tissue with increase signal intensity on STIR sequences.  Depending on the pathophysiologic mechanisms, calcinosis cutis has been classified as metastatic, dystrophic, idiopathic or iatrogenic.  Calcinosis in scleroderma is more commonly of dystrophic type. Dystrophic calcinosis cutis is a deposition of calcium and phosphorus in the subcutaneous tissue and occurs in pre-existing inflammatory skin lesion. Patient with metastatic calcification most frequently have a history of chronic renal failure. Iatrogenic calcinosis cutis, generally has a history of recent hospitalization. 
Radiographs are first investigation to detect the calcification. CT scan is very important to evaluate the calcification, bones and joints. MRI has its role in the evaluation of the soft-tissue involvement and inflammatory reaction.
Findings on the biopsy and histopathology of cutaneous lesion are diagnostic. Granules and deposits of calcium are seen in dermis and subcutaneous tissues. 
Lesions of superficial circumscribed morphea usually undergo gradual spontaneous resolution in 3-5 years period. Topical steroids, photochemotherapy and phototherapy are helpful. Systemic corticosteroids needed in inflammatory lesions. Treating the inflammatory lesion in early stage is helpful in reducing the sclerosis. Physiotherapy can be used to prevent contractures.
| Acknowledgment|| |
To Department of Pathology at Dr. D. Y. Patil Medical College, Hospital and Research Centre, Pimpri, Pune, Maharashtra, India.
| References|| |
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|2.||Nunley JR, Calcinosis C, Elston DM, Calcinosis Cutis. Available from: http://www.emedicine.medscape.com/article/1103137-overview. |
|3.||Cohen SJ. Calcinosis circumscripta: Case report and review. J Foot Surg 1980;19:190-2. |
|4.||Lobo IM, Machado S, Teixeira M, Selores M. Calcinosis cutis: A rare feature of adult dermatomyositis. Dermatol Online J 2008;14:10. |
|5.||Horger M, Fierlbeck G, Kuemmerle-Deschner J, Tzaribachev N, Wehrmann M, Claussen CD, et al. MRI findings in deep and generalized morphea (localized scleroderma). AJR Am J Roentgenol 2008;190:32-9. |
|6.||Schanz S, Fierlbeck G, Ulmer A, Schmalzing M, Kümmerle-Deschner J, Claussen CD, et al. Localized scleroderma: MR findings and clinical features. Radiology 2011;260:817-24. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]