A 74-year-old female presents with palpable lump over the right axillary region.
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Diagnosis: Systemic Sclerosis
• Imaging with bilateral MLOs demonstrate arterial calcifications.
• Lobulated ovoid soft tissue abnormality with heavy calcifications and intense posterior acoustic enhancement. Similar sonographic findings at the lower posterior back at the site of patient’s complaint of a large palpable mass.
• PA and lateral images of the chest demonstrate coarse soft tissue calcifications projecting over the left and right shoulder, right axilla and soft tissues of the lower back.
• AP mages of the hands demonstrate varying degree of osseous erosion along the tufts of the digits. There are multiple groupings of smooth, rounded soft tissue calcifications. On the right, these project along thumb and distal third digit. On the left, these project along the distal thumb, distal second digit, distal fifth digit, as well as along the radial and dorsal aspects of the carpus.
Systemic sclerosis (SSc), also known as Scleroderma, is a multisystem disease characterized by widespread vascular dysfunction and progressive fibrosis of the skin and internal organs. Diagnosis is dependent on clinical presentation and findings, and confirmed by specific serologic abnormalities. SSc can be further subcategorized into diffuse cutaneous and limited cutaneous, based on the extent of skin involvement and distribution of internal organ involvement. The incidence for SSc is 0.6 to 122 per million persons per year.
The pathogenesis of SSc is poorly understood and is thought to be related to immune activation, vascular damage and excessive synthesis of extracellular matrix, with an increase in abnormal deposition of structurally normal collagen.
Skin involvement is a universal feature of SSc. The fingers, hands and face are normally the first areas of the body that become involved. Other common manifestations of the disease are puffy or swollen fingers, hand stiffness, and painful distal digital ulcers under or close to the nail bed. Extracutaneous organ involvement is common in SSc and includes organ systems such as: Musculoskeletal, respiratory, renal, gastrointestinal, and cardiac.
Diffuse cutaneous SSc is characterized by sclerotic skin located on the chest, abdomen, or upper arms and shoulders. Patients with diffuse SSc are more likely to develop internal organ damage due to ischemic injury or fibrosis than those with limited cutaneous SSc. Patients that demonstrate limited cutaneous SSc, demonstrate skin sclerosis limited to the hands, distal forearm, and to a lesser extent, the face and neck. It is commonly associated with CREST syndrome (Calcinosis Cutis, Raynaud Phenomenon, Esophageal dysmotility, Sclerodactyly and Telangectasia).
The following serologic tests are most often performed: Antinuclear antibody, Antitopoisomerase I (formerly known as anti-scl-70), Anticentromere antibody and Anti-RNA polymerase III antibody. If these are positive, it can support the diagnosis of SSc.