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Year : 1993  |  Volume : 59  |  Issue : 4  |  Page : 213-215

Proteus syndrome

Correspondence Address:
Renu George

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A case of proteus syndrome in a 20 year old male is repoted. Hemihypertrophy, asymmetric megalodactyly, linear epidermal naevus, naevus flammeus, angiokeratoma, lymphangioma circumscriptum, thickening of the palms and soles, scoliosis and varicose veins were present. There are only few reports of these cases in adults. The syndrome has not been reported from India.

Keywords: Hemihypertorphy, Proteus syndrome

How to cite this article:
George R, Jacob M. Proteus syndrome. Indian J Dermatol Venereol Leprol 1993;59:213-5

How to cite this URL:
George R, Jacob M. Proteus syndrome. Indian J Dermatol Venereol Leprol [serial online] 1993 [cited 2020 Jun 1];59:213-5. Available from: http://www.ijdvl.com/text.asp?1993/59/4/213/3933

  Introduction Top

Proteus syndrome is a hamartomatous disorder described by Wiedemann et al 1983. [1] Since then about 50 cases have been reported in World literature, only 4 being in adults. [2] The typical clinical features include progressive and asymmetric megalodactyly, hemihypertrophy, subcutaneous masses, localised cerebroid thickening of the palms and soles and linear skin lesions. The occurrence is sporadic [2] In this report we describe a case of proteus syndrome in a 20 year adult male from Bihar. There are no reports to the best of our knowledge of a similar case from India.

  Case Report Top

A 20-year-old male, born full term following a normal delivery to nonconsanguineous parents was noticed to have asymmetric enlargement of the fingers and toes at birth and large red patches on the trunk, upper limbs and the left lower limb. There was progressive asymmetrical enlargement of the left half of the trunk, left lower limb and the fingers and toes as he grew up. During early childhood he developed raised linear lesions on the upper and lower limbs on the hypertrophied side. His developmental milestones were normal and scholastic performance good. He had no associated systemic symptoms except for bleeding per rectum. Mother and father had no congenital anomalies. He had no siblings.

Patient was thin built, height 160cm, arm span 162cm. There was partial hemihypertrophy of the left half of the trunk, left upper and lower limbs [Figure - 1].

The difference in girth was l cm in the arms, 2 cms in the thighs and 5 cms in the legs. There was asymmetric macrodactyly affecting the hands and feet. The palms and soles were grossly thickened with mild cerebroid hyperplasia.

The following lesions were also present:

(1) There was a large naevus flammeus involving the whole of the upper trunk and left upper and lower limb [Figure - 2] and proximal half of right upper limb. He also had a naevus flammeus over the rights soft palate. '

(2) Linear epidermal naevi on the left upper and lower limb.

(3) Angiokeratoma were present on the left posterior axillary fold, penile and scrotal skin and left popliteal fossae.

(4) Lymphangioma circumscriptum in the right cubital fossa.

There were varicose veins in the hypertrophied left lower limb. He had scoliosis. There were no bony exostoses. There were no obvious defects in the eye. Per rectal examination showed grade I haemorrhoids.

Biopsies taken from linear epidermal naevus and lymphangioma circumscriptum were confirmatory. X-ray spine showed scoliosis. Chest X-ray and ultrasound abdomen and pelvis were normal.

  Comments Top

Patient presented with several of the classical features of proteus syndrome. [2],[3] The linear epidermal naevus, varicose veins were present only on the hypertrophied side. Although the naevus flammeus was present on both sides of the trunk, the lesion was more prominent on the hypertrophied side. Lymphangioma circumscriptum was noted on the opposite upper limb. The palms and soles were grossly thickened but cerebroid appearance was not striking. Palmar or plantar masses have been noted in 64.7% (22/34) of patients reviewed in one series and have been described as fairly typical of this syndrome. [3] Subcutaneous masses, a fairly common occurrence in patients with proteus syndrome, were not seen in our patient. [3] Ultrasound of the abdomen and pelvis also did not reveal any masses. Pelvic lipomas has been reported in patients with proteus syndrome. [4] There were no clinically evident exostoses in our patient. The common sites of exostoses are on the skull, fingers and toes. Among the unusual features noted were penile hypertrophy reported only in 1 earlier case. [3]

As there are only few reports of proteus syndrome in adults the long term prognosis is not known. Serious complications evolve slowly depending on the location of tissue over growth. Testicular tumours have been reported in 2 cases, having developed at age 5 and 14 years. [5] Our patient had no evidence of malignancy at the time of examination. A close follow up of these patients is necessary because of the reported occurrence of non-epithelial neoplasms and also mechanical problems arising from tissue overgrowth. [5]

As proteus syndrome has only been recently recongnised it is possible that the apparent rarity of the syndrome from India is probably because it has been reported under different names or grouped with other conditions.

  References Top

1.Wiedemann HR, Burg GR, Aldenhoff P. The Proteus syndrome. Eur J Pediatr 1983; 140: 5-12. As quoted in Nazzaro V, Cambiaghi S, Montagnani A, et al. Proteus syndrome. J Am Acad Dermatol 1991; 25: 377-82.  Back to cited text no. 1    
2.Nazzaro V, Cambiaghi S, Montagnani A, et al. Proteus syndnrome. J Am Acad Dermatol 1991; 25: 377-82  Back to cited text no. 2  [PUBMED]  
3.Samlaska CP, Levi SW, James DW, et al. Proteus syndrome. Arch Dermatol 1989; 125: 1109-14.  Back to cited text no. 3    
4.Costa T, Fitch N, Azous EM. Proteus syndrome. A report of 2 cases with pelvic lipomatosis. Pediatrics 1985; 76: 984-9.  Back to cited text no. 4    
5.Hornstein L, Bove KE, Towbin BR. Linear nevi, hemihypertrophy, connective tissue hamartomas and unusual neoplasm in children. J Paed 1987; 110: 404-9.  Back to cited text no. 5    


[Figure - 1], [Figure - 2]

This article has been cited by
1 Reassessment of the Proteus syndrome literature: Application of diagnostic criteria to published cases
Turner, J.T., Cohen Jr., M.M., Biesecker, L.G.
American Journal of Medical Genetics. 2004; 130 A(2): 111-122
2 Unilateral generalized hypertrichosis in proteus syndrome
Asokan, N., Abdul Latheef, K.
Indian Journal of Dermatology, Venereology and Leprology. 2002; 68(5): 308-309
3 Principles for the surgical management of patients with proteus syndrome and patients with overgrowth not meeting proteus criteria
Lublin, M., Schwartzentruber, D.J., Lukish, J., Chester, C., Biesecker, L.G., Newman, K.D.
Journal of Pediatric Surgery. 2002; 37(7): 1013-1020


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