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Pansclerotic Morphea

Overview of Pansclerotic Morphea
Symptoms of Pansclerotic Morphea
Treatments for Pansclerotic Morphea
Squamous Cell Carcinoma

Overview of Pansclerotic Morphea

Pansclerotic morphea is an exceedingly rare and severe form of morphea, which can be very disabling and sometimes even fatal. There are only a few reported cases of pansclerotic morphea in medical literature.

Although it most often occurs in young children, it can also occur in adults. In isolated cases, it has been associated with hypogammaglobulinemia, thrombocytopenia (low blood platelets), squamous cell carcinoma, and a highly unusual pleomorphic acid-fast bacteria.

Symptoms of Pansclerotic Morphea

Disabling pansclerotic Morphea of childhood with extracutaneous manifestations. Disabling pansclerotic morphea (DPM) of childhood is a rare generalized type of localized scleroderma (LS) that is known to follow an aggressive course with pansclerotic lesions leading to severe joint contractures and consequent immobility. Indian Journal of Dermatology 03/05/2013.

Treatments for Pansclerotic Morphea

For UVA1 treatment, first contact your scleroderma expert to see if it they will recommend it for you. If they recommend and prescribe it for you, try to find UVA1 facilities near you. Some dermatology offices (usually large offices or with major health centers) have UVA1 equipment (not UVB). It may also be possible to buy UVA1 phototherapy equipment for home use. It is manufactured by Daavlin and distributed worldwide. Home users must be monitored by a physician, who will prescribe the necessary dosage and monitor for possible side-effects, such as skin cancer.

Successful Treatment of Pansclerotic Morphea with Imatinib Mesylate in a Pediatric Patient. To our knowledge, this is the first successful use of imatinib mesylate in a pediatric patient as a component of combination therapy to treat pansclerotic morphea. Ann Paediatr Rheum, 2013; 2(1): 43-49.

Disabling pansclerotic morphea of childhood with extracutaneous manifestations. In spite of treatment with methotrexate, corticosteroids, and PUVA therapy, she ultimately succumbed to her illness due to sepsis. Indian J Dermatol 2013;58:159.

(Expired Article) UVA1 Phototherapy for Pansclerotic Morphea. There are only two reported cases in the literature, an 8-year-old girl and a 16-year-old boy with pansclerotic morphea. The children received UVA1 irradiation for 6 months and 2 months, respectively. At the end of treatment, the patients showed healing of ulcers, improvement of joint mobility and important reduction of cutaneous sclerosis. Pedrheumonlinejournal.org. Division of Rheumatology,Universidade de Santo Amaro (UNISA).

Hypogammaglobulinemia and Pansclerotic Morphea

Disabling pansclerotic morphea of childhood and hypogammaglobulinemia: a curious association. A 1.5-year-old girl who presented with multiple episodes of infection since early infancy and stiffening of limbs for 2 months. Biopsy of the involved areas showed features consistent with pansclerotic morphea. Immunoglobulin estimation revealed hypogammaglobulinemia. This unique combination of pansclerotic morphea with hypogammaglobulinemia has not been reported before. Expired article. Rheumatology International, vol. 21, no. 4, Jan. 2002.

Squamous Cell Carcinoma and Pansclerotic Morphea

Case Report: Squamous Cell Carcinoma in Pansclerotic Morphea of Childhood. Squamous cell carcinoma (SCC) has only occasionally been reported in patients with systemic sclerosis. It is very rare in morphea. Herein we describe SCC presenting as malignant ulcers in pansclerotic morphea of childhood in a 16-year-old boy. Pediatric Dermatology.

Personal Stories about Pansclerotic Morphea

Ariel D: Pansclerotic Morphea My first treatment was chemotherapy, then steroids, then light treatment…

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