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CASE REPORT
Year : 1991  |  Volume : 57  |  Issue : 1  |  Page : 41-42

Facial hemiatrophy of Romberg and Parry



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  Abstract 

Two cases of facial hemiatrophy of Romberg and Parry are reported. Both presented with atrophy of subcutaneous tissue, muscle and bones of left of the face. Case 1 with onset at 17 years in a female is a classical presentation, while case 2 with onset at 5 years in a boy is an uncommon presentation.


Keywords: Facial hemiatrophy, Romberg′s disease, Facial hemiatrophy of Romberg′s and Parry


How to cite this article:
. Facial hemiatrophy of Romberg and Parry. Indian J Dermatol Venereol Leprol 1991;57:41-2

How to cite this URL:
. Facial hemiatrophy of Romberg and Parry. Indian J Dermatol Venereol Leprol [serial online] 1991 [cited 2020 May 27];57:41-2. Available from: http://www.ijdvl.com/text.asp?1991/57/1/41/3619


Facial hemiatrophy is a disorder of subcu­taneous tissue, muscles and bone first de­scribed by Parry in 1825 and Romberg in 1847 and named after them by Eulemberg in 1871. [1] Romberg and Parry's disease has to be differ­entiated from facial hemiatrophy due to scleroderma, coup de sabre type. In contrast to scleroderma, in Romberg's disease the change is much more diffuse, the skin remains soft and nonadherent and there is no loss of hair. There is atrophy of the subcutaneous tissue, muscle and bones on one side of the face leading to asymmetry and distortion. Inspite of the muscle atrophy, the muscle power is very good. Aetiology of the disease remains unknown. It commonly affects adolescent girls with no apparent hereditary pattern .[1]


  Case Reports Top


Case 1

A 19-year-old female presented with asymmetry and distortion of left side of the face since last two years. There was no history of trauma, local infection, epilepsy or neuralgic pains and no family history of similar illness. Clinical examination revealed asymmetry and depression on the left side of the face. Skin was thin, soft and nonadherent, with loss of subcutaneous tissue giving ghost like appear­ance to the left side of the face. There was no hypo- or hyperpigmentation and hair were nor­mal. Left masseter and temporal muscles were atrophied with decreased muscle bulk but were not soft or flabby and contracted normally. There was no weakness of facial muscles or muscles of mastication and there was no sensory deficit, peripheral nerve thickness or pupillary abnor­mality.

Skiagram of the skull showed rarefaction of left half of the mandible, maxilla, zygomatic process of temporal bone and zygomatic bone.

Case 2

A 10-year-old boy presented with asym­metry and depression of left side of the face since last five years. He also had no history of injury, infection, epilepsy or neuralgia and had no family history of similar illness. Clinical ex­amination showed findings exactly similar to the case 1 [Figure - 1]. Skiagram of face showed rar­efaction of bones on the left side of the face but less marked than in the above case.


  Comments Top


Facial hemiatrophy of Romberg and Parry being more common is adolescent girls, case 1 is a classical presentation, while onset at 5 years in a male child is an uncommon presen­tation as is seen in case 2. [1]

Though local trauma and infection have been suggested as precipitating factors and lesions of ipsilateral trigeminal nerve or cervi­cal sympathetic as causative factors, the exact cause of the disease is not known. [2] Some cases had associated syringomyelia or cerebral atherosclerosis suggesting an underlying cen­tral nervous system defect, but in 90% of cases there was no such association. [3] Recently Asher and Berge [4] investigated three cases by com­puted tomography. In one case it suggested vascular malformation in the ipsilateral hemi­sphere, in the second an ill-defined abnormality in the opposite hemisphere and in the third case there was no abnormality. In both our patients there was no inheritance pattern, no precipitat­ing factor and no neurological association.

 
  References Top

1.Fleischmajor R and Matus NR : Diseases of the corium and subcutaneous tissue, in : Dermatology Second edition, editors, Meschella SL and Hurley HJ : WB Saunders Company, Philadelphia, 1985 ; P 1136-1181. ,  Back to cited text no. 1    
2.Walton J : Brain's Diseases of the Nervous System, 9th edition, Oxford University Press Publications, Oxford, 1985; P 387-388.  Back to cited text no. 2    
3.Cunliffe WJ : Disorders of connective tissue, in Textbook of Dermatology, 3rd edition, Editors, Rook A and Wilkinson DS : Backwell Scientific Publica­tions, Oxford, 1979; P 1611- 1653.  Back to cited text no. 3    
4.Asher SW and Berg Bo : Progressive hemifacial atrophy. Report of three cases including one ob­served over 43 yrs and computed tomography findings, Arch Neurol, 1982; 39 : 44-46.  Back to cited text no. 4    


    Figures

[Figure - 1]

This article has been cited by
1 Unilateral linear pansclerotic morphea affecting face and limbs
Padmavathy, L., Rao, L.L.
Indian Journal of Dermatology, Venereology and Leprology. 2005; 71(3): 192-194
[Pubmed]



 

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