proteus syndrome ( ps ) is a rare and sporadic disorder that causes postnatal overgrowth of tissues in a mosaic pattern . the complications of ps include , progressive skeletal deformities , invasive lipomas , benign and malignant tumors , and deep venous thrombosis with pulmonary embolism . we report a rare case of ps that presented with hypertrophy of index and middle finger without any other abnormalities or complications . incidentally we noticed that he had enlarged index and middle fingers of both hands and thumb of right hand [ figure 1 ] . on probing patient revealed that it was present since childhood with onset around the age of 5 years and gradual progression over years to the present size . no similar tissue growth in other parts of the body and there was no one in the family with similar features . on examination hypertrophy of index and middle finger of both the hand ( a , b , c ) and thumb of the right hand ( c ) laboratory investigations revealed normal renal and liver function tests . his x - ray of hands showed hyperostosis of involved fingers [ figure 2 ] . x - ray hands showing hyperostosis of both index and middle fingure ( a , b ) and thumb of right hand ( b ) proteus who had the ability to change his shape and was proposed by wiedemann , et al . in 1983 . happle , et al . in 1987 hypothesized that the syndrome might be due to somatic alteration of a gene leading to mosaic effects that would be lethal if the mutation were carried in nonmosaic fashion . the dysregulated tissue growth in mosaic pattern results in various phenotypic presentations and hence the clinical manifestations of ps are highly variable . the tissue overgrowth is usually absent or mild at birth and progressive in nature but usually appears to plateau after adolescence . the disproportionate overgrowth of tissue is usually asymmetrical and involves the arms , legs , hands , feet , and digits . characteristic manifestations include hyperostoses , often near epiphyses with associated impaired mobility and cerebriform connective tissue nevus seen most commonly on plantar surface . other findings are lipomas , epidermal nevi and capillary vascular malformations [ table 1 ] . criteria for the diagnosis of proteus syndrome there is no specific molecular marker , or laboratory test , for the diagnosis of ps . the diagnosis is mainly based on history , clinical examination and imaging studies . because of its variable presentation , ps may be confused with other conditions . the two disorders most commonly confused with ps are klippel - trenaunay syndrome and hemihyperplasia / lipomatosis syndrome . the important points in ps that help in the differential diagnosis are : differential diagnosis of proteus syndrome 
 sporadic and progressive nature of tissue overgrowththe absent or mild tissue growth at birthabsence of bone tumor , enchondromasabsence of specific gene mutations differentiates from neurofibromatosisabsence of familial inheritance ( postzygotic somatic mutation of genes ) 
  sporadic and progressive nature of tissue overgrowth the absent or mild tissue growth at birth absence of bone tumor , enchondromas absence of specific gene mutations differentiates from neurofibromatosis absence of familial inheritance ( postzygotic somatic mutation of genes ) there are no effective treatment modalities for ps . the patients should be followed up regularly for development of complications and their management . the management is also challenging because of progressive nature of tissue growth . both benign and malignant two relatively common tumors include cystadenomas of the ovary and monomorphic adenomas of the parotid gland . presented with macrodactyly of index and middle finger of both the hands and thumb of right hand ( one criteria of category b ) and he met all the three general criteria . his skeletal survey was normal except for hyperostosis of index and middle finger and there were no associated complications except for the limitation of his affected finger movements . although the patient did not satisfy the proposed criteria [ table 1 ] , a literature search revealed that out of the 205 cases reported 90 satisfied the criteria highlighting the variability in clinical presentation in cases of ps . our patient was managed as a case of inferior wall mi and is presently on anti - ischemic medications with no new complications . in conclusion , ps is a very rare and highly variable , progressive tissue overgrowth disorder . patients should be kept under regular follow - up for the development of complications and their management .
proteus syndrome ( ps ) is a rare hamartomatous disorder characterized by various cutaneous and subcutaneous lesions , including vascular malformations , lipomas , hyperpigmentation , and several types of nevi . 
 partial gigantism with limb or digital overgrowth is pathognomonic of ps . 
 we report a rare case of ps in a 50-year - old man who presented with inferior wall myocardial infarction and was incidentally detected to have hypertrophy of index and middle fingers of both the hands .