calcinosis universalis
Calcinosis universalis is a condition characterized by long bands or sheets of symmetrical calcifications in skin, subcutaneous tissues, muscles and tendons . It is considered a diffuse form of calcinosis cutis.
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 along deep fascial planes. Tendon and ligamental 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 organized bone formation and the lack of previous trauma or injury.
Treatment and prognosis
No established therapy is recognized, 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
Consider:
- fibrodysplasia ossificans progressiva
- myositis ossificans
- calcinosis circumscripta
- localized rather than bilateral and symmetrical
See also
Siehe auch:
Assoziationen und Differentialdiagnosen zu Calcinosis cutis universalis:
