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CASE REPORT
Year : 1992  |  Volume : 58  |  Issue : 3  |  Page : 208-209

Lipodystrophia centrifugalis abdominalis infantalis




Correspondence Address:
C Balachandran


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  Abstract 

An eight year old girl presented with an atrophic lesion in the left inguinal region from the age of 3 years which was gradually spreading centrifugally. Biopsy showed absence of subcutaneous fat with normal appendages and dermal collagen.


Keywords: Lipodystrophia Centrifugalis Abdominalis Infantalis


How to cite this article:
Balachandran C, Srinivas C R, Shenoi S D, Sandhya, C. Lipodystrophia centrifugalis abdominalis infantalis. Indian J Dermatol Venereol Leprol 1992;58:208-9

How to cite this URL:
Balachandran C, Srinivas C R, Shenoi S D, Sandhya, C. Lipodystrophia centrifugalis abdominalis infantalis. Indian J Dermatol Venereol Leprol [serial online] 1992 [cited 2020 May 28];58:208-9. Available from: http://www.ijdvl.com/text.asp?1992/58/3/208/3797



  Introduction Top


Lipodystrophia centrifugal is abdominalis infantalis (LCAI) is a peculiar atrophic skin disorder described mainly in Japanese children. [1] The characteristic features of this disease are a depression of the skin resulting from the loss of subcutaneous fat which involves the greater part of the abdomen and the neighbouring region, centrifugal enlargement of the depressed area and slightly erythematous and scaly changes in the surrounding area. The onset of this disorder usually occurs before the age of 3 and no other significant abnormalities occur in any other skin surface or organ. [1],[2],[3]


  Case Report Top


The parents of an 8 year old girl noticed an asymptomatic depressed patch in left inguinal region at the age of 3 years, which was gradually spreading centrifugally. She was delivered as a full term normal baby. The parents were non-consanguinous and no other family member was affected. There was no history of trauma. The child was active and her. milestones were normal. Examination revealed a depressed atrophic area in the left inguinal region extending to labia majora and thigh [Figure - 1]. Skin was slightly hyperpigmented over the lesion, but not indurated.

Prominent subcutaneous blood vessels were seen over the area. Systemic examination revealed no abnormality. Routine blood and urine investigations were within normal limits. Biopsy of the lesion showed thinning of the epidermis and absence of subcutaneous fat beneath the dermis. Dermal collagen and appendages were normal. There was minimal perivascular- infiltrate of lymphocytes and histiocytes in upper dermis.


  Comments Top


The term LCAI for this peculiar skin condition was proposed by Immamura et al in 1971 in 5 Japanese children. [1] Only a few cases have been reported outside Japan. [4],[5] The etiology and pathogenesis of this disease are unknown. The probable cause could be mechanical irritation. Oral or topical corticosteroids though sometimes effective against the inflammatory changes in the surrounding area, were not very effective in stopping the enlargement of the depressed lesion. Spontaneous cessation of the lesion occurs in most of the cases by the age of 13 after which the depressed area tends to recover. 9 The characteristic clinical presentation and histopathology distinguishes this condition from macular atrophy, atrophoderma idiopathica progressiva (Pasini-Pierini), panatrophy of gowers and adiponecrosis subcutanea neonatorum. [1] Our case had the same clinical and histopathological features as described by Immamura et al except the erythematous and scaly changes in the surrounding area.

 
  References Top

1.Immamura S, Yamada M, Ikeda T. Lipodystrophia centrifugalis abdominalis infantalis. Arch Dermatol 1971;104:291-8.  Back to cited text no. 1    
2.Immamura S, Yamada M, Yamamoto K. Lipodystrophia centrifugal is abdominalis infantalis. J Amer Acad Dermatol 1984;11:203-9.  Back to cited text no. 2    
3.Makino K, Inoue T, Shimao S, et al:. LipodystrQphia centrifugalis abdominalis infantalis. Arch Dermatol 1972;106:899-900.  Back to cited text no. 3    
4.Giam YC, Rajan VS, Hock OB. Lipodystrophia centrifugalis abdominalis infantalis. Br J Dermatol 1982;106:461-4.  Back to cited text no. 4  [PUBMED]  
5.Zachary CB, Wells RS. Centrifugal Lipodystrophy. Br J Dermatol 1984;110:107-10.  Back to cited text no. 5  [PUBMED]  


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