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    Abstract
    Introduction
    Case Report
    Discussion
    References

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CASE REPORT
Year : 2002  |  Volume : 68  |  Issue : 2  |  Page : 97-98

Multiple lentigenes syndrome


Department of Dermato-Venereology, L.L.R.M. Medical College, Meerut (UP), India

Correspondence Address:
L-S, Shastri Nagar, Meerut - 250 004, India

   Abstract 

A 23-year-old male with multiple lentigenes, pulmonary stenosis, hyperelasticity of skin, hypermobile metacarpophalangeal joints is reported as a forme fruste form of leopard syndrome.

How to cite this article:
Sharma R P, Singh S P. Multiple lentigenes syndrome. Indian J Dermatol Venereol Leprol 2002;68:97-8


How to cite this URL:
Sharma R P, Singh S P. Multiple lentigenes syndrome. Indian J Dermatol Venereol Leprol [serial online] 2002 [cited 2020 Jun 1];68:97-8. Available from: http://www.ijdvl.com/text.asp?2002/68/2/97/12609



   Introduction Top

Multiple lentigenes are associated with wide range of developmental defects. It js determined by autosornal dominant gene with variable expressivity.[1] Gorlin et al[2] gave the mnemonic term  LEOPARD syndrome More Details from the principal components of this syndrome, letter denotes. L-lentigenosis, E-electrocardiographic defects, O-Ocular hypertelorism, P-pulmonary stenosis, A-abnormalities of genitalia, Rretardation of growth, D-deafness. Of these cardiac and cutaneous involvement are more frequent.[3] Lentigenes usually present at birth or start developing at early life and increase in number up to the puberty. They mainly involve the neck and upper trunk but may extend to other parts including scalp, genitalia, palms and soles. The mucous membranes are usually spared.[3] Cardiac abnormalities include conduction defects, pulmonary stenosis, subaortic stenosis, and obstructive cardjomyopathy.[3]

   Case Report Top

A 23-year-old male presented with multiple light brown to dark brown flat lesions of variable sizes ranging from 2-5mm. These lesions were present over body sparing mucous membranes. At birth lesions were present over left side of the neck and medial side of the neck and medial side of left thigh. The lesions progressively increased in numbers and invoived all body parts including plams and soles. [Figure - 1]
Examination revealed: height 160 cm, weight 38 kg., normal intelligence, multiple lentigenes present all over body except mucous membranes, hyperelastic skin, hypermobile metacarpophalangeal joints, no abnormalities of the eye, ear and genitalia. Histopath- ological examination of hyperpigmented macular lesion revealed features of lentigenosis. Clinical, electrocardiographic and echocardio- graphic findings were suggestive of pulnomary stenosis. All routine investigations viz haematologic, liver function tests, urinalysis were within normal limits.

   Discussion Top

Although multiple lentigenes syndrome has been named as  LEOPARD syndrome More Details this is very variable syndrome, having a wide range of manifestations. Voron et al[4] tried to enlist all possible abnormalities: cutaneous, cardiac, neurologic, ocular, cephalofacial dysmorphism, stature, and skeletal. Of these, cutaneous and cardiac abnormalities are more frequent.[3] That is why, this syndrome is also known as cardio-cutaneous syndrome.[5] Guha et al[5] also reported a case of incomplete  LEOPARD syndrome More Details with lentigenosis, vitiliginous patch over left half of face, hypertrophic cardiomyopathy, short stature, spina bifida oculta, hypertelorism, epistaxis. Several patients with this syndrome have died at an early age from obstructive cardiomy- opathy.[1] Hence it is suggested that in case of multiple lentigenes investigations to rule out cardiac abnormalities must be carried out. 

   References Top

1.Bleehen SS. Disorders of skin colour. In: Champion RH, Burton JL, Burn DA, Breathnach SM (eds), Textbook of Dermatology, Blackwell Scientific Publication, London 1998;2:1768-1769.  Back to cited text no. 1    
2.Gorlin RJ, Anderson RD, Blow M. Multiple lentigene syndrome. Am J Dis Child 1969.  Back to cited text no. 2    
3.Dutta AK, Datt PK. Pigmentary disorders. In: IADVL Textbook and Atlas of Dermatology. Bhalani Publication, Bombay 1994;1:541.  Back to cited text no. 3    
4.Voron DA, Hatfiled HH, Kalkhoff RK. Multiple lentigenes syndrome. Am J Med 1976;60:447-456.  Back to cited text no. 4    
5.Guha PK, Barbhuiya JN, Maity SG. Progressive cardiomyopathic lentigenosis. Indian J Dermatol Venereal Leprol 1991;57:144-145.  Back to cited text no. 5    

 

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