Connective Tissue Nevus

Connective Tissue Nevus

“…Their case also had an atrophic clinical appearance and an increase of elastic tissue histologically. However, the lesion was small, asymmetric and not on the face [8]. An increased number of hair follicles has not been previously reported and the number of follicles was noted to be sparse in at least one report [10].…”

“…Histologically the increase in elastic tissue and lack of elastorrhexis would rule out the primary and secondary macular atrophies. There was no evidence of systemic abnormality, other significant skin lesions or familial incidence and the absences of these make it unlikely that this condition would be associated with tuberous sclerosis or osteopoikilosis [8,10].…”

“…A lesion characterized by nodular accumulations of fat in the dermis has also been considered a type of connective tissue nevus [7], Since L e w a n d o w sk y 's report of 3 cases in 1921 [I] several case reports and reviews of the subject have been published [4,8,[10][11][12][13][14]. Attempts to cate gorize the lesions depending on the predominant tissue type has generally not been acceptable because of the great amount of overlap and the lack of modification of the clinical significance [8,10]. Rocha and W in kelm ann [8] in delineating the connective tissue nevus as a distinct entity have emphasized the lack of association with collagen dysplasias and dermal sclerosis.…”

“…Attempts to cate gorize the lesions depending on the predominant tissue type has generally not been acceptable because of the great amount of overlap and the lack of modification of the clinical significance [8,10]. Rocha and W in kelm ann [8] in delineating the connective tissue nevus as a distinct entity have emphasized the lack of association with collagen dysplasias and dermal sclerosis. A recent general classification by R aque and W ood [6] divides the connective tissue nevus into 3 categories: without associated systemic disease; with tuberous sclerosis or adenoma sebaceum; and with osteopoikilosis.…”

Familial Incidence of Tuberous Sclerosis

“…Adenoma sebaceum (60%), shagreen patch (80%) and hypopigmented macules (60%) were the main neurocutaneous markers detected in our series. Adenoma sebaceum and hypopigmented macules are usually present from birth and shagreen patch develops after 10 years of age [2,4,5]. None of our cases had other system involvement, though lesions in eye, heart, lungs and kidney have been found frequently in other series [1].…”