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Indian Journal of Dermatology
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CASE REPORTS
Year : 2005  |  Volume : 50  |  Issue : 3  |  Page : 164-165
Disabling pansclerotic morphea


Department of Dermatology, NRS Medical College, Kolkata, India

Correspondence Address:
Mousumi Nandy
AD- 193, Sector- I, Salt Lake City, Kolkata - 700064, WB
India
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Source of Support: None, Conflict of Interest: None


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   Abstract 

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. There was no history of Raynaud's phenomenon.


Keywords: Pansclerotic morphea, Osteopenia


How to cite this article:
Nandy M, Samanta A, Ghosh A, Mandal K, Barbhuiya JN. Disabling pansclerotic morphea. Indian J Dermatol 2005;50:164-5

How to cite this URL:
Nandy M, Samanta A, Ghosh A, Mandal K, Barbhuiya JN. Disabling pansclerotic morphea. Indian J Dermatol [serial online] 2005 [cited 2020 Sep 23];50:164-5. Available from: http://www.e-ijd.org/text.asp?2005/50/3/164/18934



   Introduction Top


Disabling pansclerotic morphea which is the most severe form of morphea may develop from its linear variant. The condition is progressive and may even be fatal.[1] Here we present such a case due to rarity of the condition.


   Case report Top


A 12 year old boy presented with tautness of skin over face and extremities with difficulty in opening the mouth. At the onset, 1 year back there was pain and stiffness in the knee joints followed by similar involvement of the elbow joints and spine. The child thus attained a stooping posture [Figure - 1]. The patient had no history of fever or Raynaud's phenomenon. On examination, the skin over face and extremities was bound down. The ­limbs were thinned. The child had dysphagia, respiratory distress, glossitis and angular cheilitis. Investigations revealed a very low Hb (6 gm%) count and a raised ESR (80 mm/hr). Liver function test, blood urea, creatinine, serum electrolytes were within normal limits as were urine and stool for routine and microscopical examination. RA factor and ANF were negative. X-ray of upper and lower limbs showed osteopenia. Barium swallow showed aperistaltic dilatation of the lower third of esophagus. Histopathological examination of skin biopsy specimen showed features of scleroderma. The child improved considerably with d-penicillamine therapy and physiotherapy.


   Discussion Top


Disabling pansclerotic morphea is a mutilating form of morphea that involves skin, subcutaneous tissue, muscles and even bones. It usually starts before the age of 14 years.[2] Superficial and deep cutaneous sclerosis occur involving trunk, extremities, face, scalp and gives rise to various deformities with limitation of joint movement.[3] Systemic manifestations like respiratory distress and dysphagia are often seen. Cyclosporine, d-­penicillamine, and diphenylhydantoin are somewhat effective as are PUVA and low dose UVA.[4] The rapid clinical progression within 1 year in a child less than 14 years of age in the absence of Raynaud's phenomenon pointed to the clinical diagnosis of pansclerotic morphea. The osteopenic changes seen in the X-ray of long bones were further diagnostic.



 
   References Top

1.Chakraborty J, Coondoo A, Banerjee R. Disabling pansclerotic morphoea of children. Indian J Dermatol 2001;46: 234-5.  Back to cited text no. 1    
2.Rowell NR, Goodfield MJD. The connective tissue disease. In: Champion RH, Burton JL, Burns DA, et al. eds. Textbook of Dermatology. Vol.3, 6th ed. Oxford: Blackwell scientific publication, 1998;2506-7.  Back to cited text no. 2    
3.Stucker M, Schreiber D, Grass C. Severe course of a mutilating pansclerotic circumscribed scleroderma in childhood. Clinical aspect and therapy. Hautarzt 1999; 50: 131-5.   Back to cited text no. 3    
4. Grass C, Struker M, von Kobyletsky G, et al . Low dose, UVA1 phototherapy in disabling pansclerotic morphea of childhood. Br J Dermatol 1997;136:293-4.  Back to cited text no. 4    


Figures

[Figure - 1], [Figure - 2]



 

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    Abstract
    Introduction
    Case report
    Discussion
    References
    Article Figures

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