[Skip to Content]
Localized Scleroderma: Morphea
Video Overview
Types of Morphea
Juvenile Scleroderma
Associated Conditions
Causes of Morphea
Complications of Morphea
Diagnosis
Photos
Research Registry
Treatments
Patient and Caregiver Stories
English
Español
Italiano

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

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...
Submit your personal story about Pansclerotic Morphea.
 
Go to CREST Scleroderma
 

SCLERO.ORG is operated by the International Scleroderma Network, which is a full-service U.S. nonprofit 501(c)(3) established in 2002. We provide stellar worldwide research, support, education and awareness for scleroderma and related illnesses, such as pulmonary hypertension. Donate or Shop Now.

Questions? Post a message in Sclero Forums or email us directly at isn@sclero.org. Or call our Scleroderma Hotline (English only), Toll Free in U.S. 1-800-564-7099 or Direct at 1-952-831-3091. Ask for our Welcoming Email.

Our headquarters postal mailing address is: International Scleroderma Network (ISN), 7455 France Ave So #266, Edina, MN 55435-4702, United States.

The most important thing in the world to know about scleroderma...is sclero.org!
Home   Medical   News   Sclero Forums   Support   Translations  Donate or Shop
Copyright 1998-2014, International Scleroderma Network. AKA Scleroderma from A to Z and SCLERO.ORG. All Rights Reserved.