Figure 1
AP radiograph of pelvis
AP view of the pelvis showing extensive calcifications projected over the pelvis and thighs.
Calcinosis cutis in dermatomyositis
SectionMusculoskeletal system
Case TypeClinical Cases
AuthorsLy DL, Khashoggi K, Le Corroller T, Hargunani R, Torreggiani WC, Munk PL
Connected authors
54 years, female
Clinical History
54-year-old female patient presenting with progressive proximal muscle weakness affecting both legs. There was no history of trauma.
Imaging Findings
A 54-year old female patient with a known diagnosis of dermatomyositis presented with worsening proximal muscle weakness affecting both legs. Plain radiographs of the pelvis as well as both hips were obtained.
Extensive sheet-like calcifications were projected over the pelvis as well as along the soft tissues of both thighs. Additional radiographs of both knees were subsequently obtained to assess the full extent of these calcifications, which were found to extend to the soft tissues posterior to both knee joints.
A contrast enhanced CT was obtained from the level of L5 to the mid-femur for further assessment. The sheet-like calcifications were confirmed to be within the subcutaneous fat and fascial planes around the pelvis and upper inner thighs. Marked calcifications were also noted surrounding the right hamstring compartment without intra-muscular involvement.
Blood work drawn at the time of assessment revealed normal electrolytes including calcium and phosphate, CBC, and liver function tests. Creatine kinase, ESR and rheumatoid factor were also all within normal limits.
Extensive sheet-like calcifications were projected over the pelvis as well as along the soft tissues of both thighs. Additional radiographs of both knees were subsequently obtained to assess the full extent of these calcifications, which were found to extend to the soft tissues posterior to both knee joints.
A contrast enhanced CT was obtained from the level of L5 to the mid-femur for further assessment. The sheet-like calcifications were confirmed to be within the subcutaneous fat and fascial planes around the pelvis and upper inner thighs. Marked calcifications were also noted surrounding the right hamstring compartment without intra-muscular involvement.
Blood work drawn at the time of assessment revealed normal electrolytes including calcium and phosphate, CBC, and liver function tests. Creatine kinase, ESR and rheumatoid factor were also all within normal limits.
Discussion
Dermatomyositis is a systemic connective tissue disease of unknown aetiology characterised by symmetric proximal muscle weakness accompanied by characteristic cutaneous manifestations. One of the cutaneous manifestations is calcinosis in the skin or muscles. It is found more commonly in juvenile dermatomyositis than in adults and may be present in 30-70% of cases [1].
Soft-tissue calcifications may be metastatic, dystrophic, idiopathic, or iatrogenic [1]. Calcinosis cutis is a deposition of insoluble calcium salts in the skin and is most commonly due to dystrophic causes in connective tissue diseases; hence, the calcium and phosphate metabolism is normal. The exact mechanism of calcium deposition is unclear but is presumed to be due to damaged, inflamed, or necrotic skin.
Imaging typically begins with plain radiographs but CT provides the advantage of determining the plane of calcification. The calcium deposits may be intracutaneous, subcutaneous, fascial, or intramuscular [2]. Compared to conventional radiography, CT is also more specific and sensitive in the detection of deeper soft-tissue calcifications [1]. MR images and whole-body scintigraphy with 99mTc medronate have been used to assess calcifications but it is not clear that they have improved detection over thin-slice CT.
Soft tissue calcinosis, particularly when florid, has been associated with pain, cosmetic disfigurement, and persistent ulceration with infection but most often is asymptomatic and found as an incidental finding on radiographic examination [2]. The natural history of calcinosis cutis is highly variable and unpredictable with cases ranging from spontaneous resolution to marked progression.
First-line therapy for localised disease begins with topical agents such as antipruritics and/or corticosteroids and systemic treatment courses for widespread disease [3]. For significant skin disease, antimalarials are usually used as first-line treatment, followed by other anti-inflammatory agents, systemic corticosteroids, corticosteroid-sparing immunosuppressants, biologics, and immunoglobulins.
Soft-tissue calcifications may be metastatic, dystrophic, idiopathic, or iatrogenic [1]. Calcinosis cutis is a deposition of insoluble calcium salts in the skin and is most commonly due to dystrophic causes in connective tissue diseases; hence, the calcium and phosphate metabolism is normal. The exact mechanism of calcium deposition is unclear but is presumed to be due to damaged, inflamed, or necrotic skin.
Imaging typically begins with plain radiographs but CT provides the advantage of determining the plane of calcification. The calcium deposits may be intracutaneous, subcutaneous, fascial, or intramuscular [2]. Compared to conventional radiography, CT is also more specific and sensitive in the detection of deeper soft-tissue calcifications [1]. MR images and whole-body scintigraphy with 99mTc medronate have been used to assess calcifications but it is not clear that they have improved detection over thin-slice CT.
Soft tissue calcinosis, particularly when florid, has been associated with pain, cosmetic disfigurement, and persistent ulceration with infection but most often is asymptomatic and found as an incidental finding on radiographic examination [2]. The natural history of calcinosis cutis is highly variable and unpredictable with cases ranging from spontaneous resolution to marked progression.
First-line therapy for localised disease begins with topical agents such as antipruritics and/or corticosteroids and systemic treatment courses for widespread disease [3]. For significant skin disease, antimalarials are usually used as first-line treatment, followed by other anti-inflammatory agents, systemic corticosteroids, corticosteroid-sparing immunosuppressants, biologics, and immunoglobulins.
Differential Diagnosis List
54-year-old female patient with soft tissue calcinosis related to dermatomyositis.
Final Diagnosis
54-year-old female patient with soft tissue calcinosis related to dermatomyositis.
References
[1] Agarwal V, Sachdev A, Dabra AK (2007) Case 104: Calcinosis in juvenile dermatomyositis. Radiology 242(1):307-11 (PMID: 17185675)
[2] Lobo IM, Machado S, Teixeira M, Selores M (2008) Calcinosis cutis: a rare feature of adult dermatomyositis. Dermatol Online J 14(1):10 (PMID: 18319027)
[3] Quain RD, Werth VP (2006) Management of cutaneous dermatomyositis: current therapeutic options. Am J Clin Dermatol 7(6):341-51 (PMID: 17173468)
Case information
| URL: | https://www.eurorad.org/case/8744 |
| DOI: | 10.1594/EURORAD/CASE.8744 |
| ISSN: | 1563-4086 |
Figure 2
Plain radiograph of right hip
Right hip radiograph showing subcutaneous and sheet-like calcifications at the pelvis extending along the length of the femur within the subcutaneous tissues.
Figure 3
AP view of both knees
AP view of both knees showing extensive subcutaneous and fascial sheet-like calcifications bilaterally, more significantly on the right.
Figure 4
Lateral view Right Knee
Lateral view of the right knee with subcutaneous sheet-like calcifications located posterior to the femur extending to the knee.
Figure 5
Lateral view Left Knee
Lateral view of the left knee with further calcifications located posteriorly behind the knee.
Figure 6
Axial CT Pelvis
Axial CT image at the level of the hip joints confirming sheet-like calcifications in the subcutaneous tissues and fascial planes but without intramuscular involvement.
Figure 7
Coronal CT pelvis and both thighs
Coronal CT image, demonstrating sheet-like calcifications throughout the subcutaneous layers around both hips including involvement of the medial thighs.
Figure 8
Plain radiograph of left hip
Left hip radiograph with sheet-like calcifications surrounding the head and neck of the femur and extending partially down the shaft.
AP radiograph of pelvis
AP view of the pelvis showing extensive calcifications projected over the pelvis and thighs.
Plain radiograph of right hip
Right hip radiograph showing subcutaneous and sheet-like calcifications at the pelvis extending along the length of the femur within the subcutaneous tissues.
AP view of both knees
AP view of both knees showing extensive subcutaneous and fascial sheet-like calcifications bilaterally, more significantly on the right.
Lateral view Right Knee
Lateral view of the right knee with subcutaneous sheet-like calcifications located posterior to the femur extending to the knee.
Lateral view Left Knee
Lateral view of the left knee with further calcifications located posteriorly behind the knee.
Axial CT Pelvis
Axial CT image at the level of the hip joints confirming sheet-like calcifications in the subcutaneous tissues and fascial planes but without intramuscular involvement.
Coronal CT pelvis and both thighs
Coronal CT image, demonstrating sheet-like calcifications throughout the subcutaneous layers around both hips including involvement of the medial thighs.
Plain radiograph of left hip
Left hip radiograph with sheet-like calcifications surrounding the head and neck of the femur and extending partially down the shaft.