Disabling pansclerotic Morphea L94.1

Rare, aggressive, profound, linear ligamentous circumscritic scleroderma prone to extensive sclerosis of skin, subcutis, fascia, muscle, and bone. This "disabling" type of scleroderma, usually leading to significant contractures and, less commonly, mutilating atrophy, is very rare. Only a few isolated cases have been described.

Especially children before adolescence. More rarely the first manifestation is in early adulthood; m:w=1:2/3;

General susceptibility to infections which can lead to bronchopneumonia and sepsis. Development of contractures and immobility. There is also the risk of chronic ulcer formation on the tight sclerosis plaques. In persistent ulcers, there is an above average risk of carcinoma formation even in early adulthood.

The following drugs with varying degrees of success are listed as system therapies (only individual cases)

• Methotrexate
• Mycophenolate mofetil
• Sildenafil
• Bosentan

1. Iqbal MPet al (2007) Disabling pansclerotic and plaque morphoea. J Coll Physicians Surg Pak 17:495-496.
2. Jamalpur I et al (2018) Disabling pansclerotic morphoea of childhood. BMJ Case Rep:bcr2017222132.
3. Kura MM et al.(2013) Disabling pansclerotic morphea of childhood with extracutaneous manifestations. Indian J Dermatol 58:159
4. Maragh SH et al (2005) Disabling pansclerotic morphea: clinical presentation in two adults. J Am Acad Dermatol 53 (2 Suppl 1): 115-119.
5. Odhav A et al (2014) Pansclerotic morphea with features of eosinophilic fasciitis: distinct entities or part of a continuum? Pediatr Dermatol 31:e 42-47
6. Singh A et al (2014) Adult-onset unilateral disabling pansclerotic morphea. Indian J Dermatol 59:316
7. Tekin NS et al (2010) Disabling pansclerotic morphoea: a case report. Int J Clin Pract 64: 99-101
8. Wollina U et al (2007) Disabling pansclerotic morphea of childhood poses a high risk of chronic ulceration of the skin and squamous cell carcinoma. Int J Low Extrem Wounds 6: 291-298.