Abstract
Disabling pansclerotic morphea of childhood is a rare variant of deep morphea that shows a different course from all other types of localized scleroderma. The majority of patients follow an aggressive course with deep sclerotic lesions leading to joint contractures and immobility. Scarring alopecia, secondary anonychia and ectropion of the eyelids can be seen. Later complications are from chronic nonhealing ulcers. Internal organ manifestation has been reported in several patients including pulmonary and esophageal involvement and cardiomyopathy. The disease does not present with nephritis, Raynaud’s phenomenon or arthritis – symptoms that are seen in systemic sclerosis. The disease shows a rapid progression that may lead to severe handicaps within a period of months to a few years. Another complication is the development of squamous cell carcinoma. Mortality is due to complications of the disease such as bronchopneumonia, sepsis or gangrene. There is no specific laboratory finding. Treatment has not been standardized. There is some evidence for the efficacy of UVA or psoralen plus UVA therapy in the earlier stages, probably in combination with topical calcipotriol. The combination of pulsed methylprednisolone and low-dose methotrexate therapy is promising in other types of childhood morphea. Phosphodiesterase-5 inhibitors and endothelin-receptor antagonists might be of some benefit in chronic ulcerations and in the improvement of sclerotic skin.
Financial & competing interests disclosure
The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.
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