| 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, 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. |
| 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. |
| 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 |
| 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. |
| Pansclerotic
Morphea Treated with UVA: A Case Report. An 8-year-old
girl with pansclerotic morphea of childhood, a rare, severe
variant of localized scleroderma. We used UVA therapy in
the treatment of this case. PubMed. J Dermatol. 2003 Aug;30(8):625-7. |
| Severe
Ankle Deformity Secondary to Pansclerotic Morphea in a 9-Year-Old
Girl: Correction Involving Arthrodesis and Free Flap Coverage. (Abstract
for purchase.) Pediatric Dermatology, November 2002, vol.
19, no. 6, pp. 560-563(4). |
| 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. 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... |
| Submit
your personal story about Pansclerotic Morphea. |
