| Localized Scleroderma: Morphea |
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Pansclerotic Morphea |
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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 |
| Localized Scleroderma or Morphea? (Article no longer available.) The most severe form of localized morphea (scleroderma) is disabling pansclerotic morphea of childhood. Pansclerotic morphea usually occurs in children before 14 years of age. In this disease, rapid progression of cutaneous fibrosis expands over large body surface areas. The prognosis in terms of quality of life is poor, with the disease at times even taking a fatal course. Complications of disabling pansclerotic morphea include cutaneous ulcerations, joint contractures, and mutilating deformities and loss of the extremities (Stucker et al., 1999). Ref: Stucker, M., Schreiber, D., Gruss, C. Freitag, M., von Kobyletzki, G., Kerscher, M., & Altmeyer, P. (1999). Severe course of a mutilating pansclerotic circumscribed scleroderma in childhood. Clinical aspects and therapy. Hautarzt, 50(2), 131-135. Medicaltalk.org. |
| The "tank top sign": a unique pattern of skin fibrosis seen in pansclerotic morphea. Between 2000 and 2008, three men with pansclerotic morphea presented to the Johns Hopkins Scleroderma Center with striking similarity in the distribution of fibrotic plaques on the upper chest that spared the areolae and lateral pectoral region and involved the whole back. Ann Rheum Dis 2009;68:1511-1512 doi:10.1136/ard.2008. |
| Disabling pansclerotic morphea: clinical presentation in two adults. Disabling pansclerotic morphea involves all layers of the skin, extending through the dermis and subcutaneous tissues to involve muscle, tendon, and bone. It is distinguished from generalized scleroderma by its lack of systemic involvement. PubMed. J Am Acad Dermatol. 2005 Aug;53(2 Suppl 1):S115-9. |
| Disabling pansclerotic morphea. A 12 year old boy presented with pain and stiffness in large joints with tautness of skin, dysphagia and respiratory distress of 1 year duration. There was osteopenia on X-ray of limbs. Histopathology revealed features of morphea. Indian J Dermatol 2005;50:164-5. |
| Pansclerotic Morphea. The experience with children is limited. There are only two reported cases in the literature, an 8-year-old girl and a 16-year-old boy with pansclerotic morphea. This is a variant of localized scleroderma characterized by a rapid progression of cutaneous fibrosis with extension to joints and fascia. Severe joint contractures and cutaneous ulcers are common. 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. Expired article. Pedrheumonlinejournal.org. Division of Rheumatology,Universidade de Santo Amaro (UNISA). |
Treatments for Pansclerotic Morphea |
| Resolution of pansclerotic morphea after treatment with antithymocyte globulin. His fibrotic skin and hematologic conditions dramatically responded to antithymocyte globulin 40 mg/kg daily for 4 days, plus 10 mg/kg ciclosporin and methylprednisolone 1 mg/kg per day. Nat Rev Rheumatol. 2009 Sep;5(9):513-6. |
| Efficacy of Bosentan in Treatment of Unresponsive Cutaneous Ulceration in Disabling Pansclerotic Morphea in Children. In the context of other data in scleroderma, bosentan may be a promising option in the treatment of PM. J Rheumatol 2006 December;33:2538-40. Case Report. |
| PUVA therapy in disabling pansclerotic morphea of children. Mihailov I., Trashlieva M., Gospodinov D., Valkova S., Hajdoudova Chr., Konova E., Eur. J. Pediat. Dermatol. Vol. 9, n. 3, 1999. |
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 |
| 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, vol. 19, Issue 2, pg. 151, March/April 2002. |
Causes of Pansclerotic Morphea |
| Pleomorphic, variably acid-fast bacteria in an adult patient with disabling pansclerotic morphea. Sclerodermatous skin specimens from a 24-year-old woman with DPM were studied for the presence of acid-fast bacteria in bacteriologic culture and in microscopic sections. On three of four occasions, a highly pleomorphic organism was cultured from the skin lesions. The morphologic forms observed in vivo were similar in appearance to some of the growth forms of the microbe observed in vitro, suggesting that such an organism might be implicated to the pathogenesis of DPM. (Expired article.) A. R. Cantwell Jr, J. E. Jones and D. W. Kelso, Archives of Dermatology, Vol. 120 No. 5, May 1984. |
Case Reports of Pansclerotic Morphea |
| Pansclerotic Morphea in Childhood. The present case report concerns a boy who died at 16 years of age. In the 5th year of life, he experienced weight loss and developed multiple, firm, partially atrophic plaques in the skin of the extremities. These plaques gradually became confluent and extended over the whole torso and head. Plaque ulceration resulted in massive mutilations to the body. Later the patient's cachexia worsened and he developed keratose, moderately differentiated squamous cell carcinoma of the right leg. The prognosis of pansclerotic morphea for children is worse than for adults. No successful therapy is known. Pediatric Surgery International, vol.19, no. 5, July 2003. |
Personal Stories about Pansclerotic Morphea |
| Ariel D: Pansclerotic Morphea My first treatment was chemotherapy, then steroids, then light treatment... |
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