Calcinosis universalis
Updates to Article Attributes
Calcinosis universalis is a condition characterised by long bands or sheets of symmetrical subcutaneous calcification.
Clinical presentation
It usually presents <20 years of age, and is more common in women.
- palpable calcific plaques in subcutaneous or deeper tissue
- fatigue, muscle pain, and stiffness
- possible high levels of γ-carboxyglutamic acid in tissues and urine
~1/3 of cases are associated with scleroderma, dermatomyositis, polymyositis, and systemic lupus erythematosus.
Radiographic features
Calcinosis universalis manifests as long bands of symmetrical subcutaneous calcification, seen extending into deeper connective tissuesalong deep fascial planes. TendonsTendon and ligaments may become involvedligamental involvement has been described.
Unlike tumoral calcinosis, it is plaque or sheet like rather than mass like. The calcifications tend to be coarser and larger than those seen in hyperparathyroidism or chronic renal disease. It can be distinguished from heterotopic ossification by the absence of organised bone formation and the lack of previous trauma or injury.
Treatment and prognosis
No established therapy is recognised, but calcium chelates (EDTA) bisphosphonates, and steroids have been used with little consistent benefit. Local surgical excision of symptomatic calcinosis has been shown to relieve symptoms in cases.
Differential diagnosis
- fibrodysplasia ossificans progressiva
- myositis ossificans
-
calcinosis circumscripta
- localised rather than bilateral and symmetrical
-</ul><p>~1/3 of cases are associated with <a href="/articles/scleroderma">scleroderma</a>, <a href="/articles/dermatomyositis">dermatomyositis</a>, <a href="/articles/polymyositis">polymyositis</a>, and <a href="/articles/systemic-lupus-erythematosus">systemic lupus erythematosus</a>.</p><h4>Radiographic features</h4><p>Calcinosis universalis manifests as long bands of symmetrical subcutaneous calcification, extending into deeper connective tissues. Tendons and ligaments may become involved.</p><h4>Treatment and prognosis</h4><p>No established therapy is recognised, but calcium chelates (EDTA) bisphosphonates, and steroids have been used.</p><h4>Differential diagnosis</h4><ul>- +</ul><p>~1/3 of cases are associated with <a href="/articles/scleroderma">scleroderma</a>, <a href="/articles/dermatomyositis">dermatomyositis</a>, <a href="/articles/polymyositis">polymyositis</a>, and <a href="/articles/systemic-lupus-erythematosus">systemic lupus erythematosus</a>.</p><h4>Radiographic features</h4><p>Calcinosis universalis manifests as long bands of symmetrical subcutaneous calcification seen extending along deep fascial planes. Tendon and ligamental involvement has been described.</p><p>Unlike <a href="/articles/tumoral-calcinosis">tumoral calcinosis</a>, it is plaque or sheet like rather than mass like. The calcifications tend to be coarser and larger than those seen in hyperparathyroidism or chronic renal disease. It can be distinguished from heterotopic ossification by the absence of organised bone formation and the lack of previous trauma or injury.</p><h4>Treatment and prognosis</h4><p>No established therapy is recognised, but calcium chelates (EDTA) bisphosphonates, and steroids have been used with little consistent benefit. Local surgical excision of symptomatic calcinosis has been shown to relieve symptoms in cases.</p><h4>Differential diagnosis</h4><ul>
-<li><a title="dermatopolymyositis" href="/articles/dermatopolymyositis">dermatopolymyositis</a></li>- +<li>
- +<a href="/articles/calcinosis-circumscripta">calcinosis circumscripta</a><ul><li>localised rather than bilateral and symmetrical</li></ul>
- +</li>
References changed:
- 4. Valenzuela A, Chung L. Calcinosis: pathophysiology and management. Current opinion in rheumatology. 27 (6): 542-8. <a href="https://doi.org/10.1097/BOR.0000000000000220">doi:10.1097/BOR.0000000000000220</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/26352733">Pubmed</a> <span class="ref_v4"></span>
- 3. Stewart VL, Herling P, Dalinka MK. Calcification in soft tissues. JAMA. 250 (1): 78-81. <a href="https://www.ncbi.nlm.nih.gov/pubmed/6854888">Pubmed</a> <span class="ref_v4"></span>
Image 1 X-ray (Frontal) (create)
