Clinical Analysis Of Connective Tissue Nevi

Connective tissue nevi are uncommon hamartomas that may be acquired or may manifest an autosomal dominant pattern of inheritance. These lesions characteristically occur on the trunk, most often in the lumbosacral area. They may be solitary, but are often multiple. The lesions are papules, nodules, or plaques, varying in color from yellowish to skin-colored. The hereditary types of connective tissue nevi include primarily shagreen patch seen in tuberous sclerosis ( TS) , Buschke-Ollendorff syndrome and familial cutaneous collagenoma.In the article, we reported a family with connective tissue nevi. The clinical , histological and genetic features of a family and 9 patients reported in Chinese literature since 1980 were summarized and analyzed. We discussed the differential diagnosis of this disease with tuberous sclerosis, Buschke-Ollendorff syndrome, familial cutaneous collagenoma, pseudoxanthoma elasticum, xanthomatosis and dermatofibroma. At last, we generalized the lesions features of 27 patients with Buschke-Ollendorff syndrome searched incompletely from Pubmed.Our patients presented multiple yellowish, pignut-size, firm papules and nodules which partly coalesced in plaques on the trunk and the thigh. Histopathologic examination of the lesions demonstrated increased amount of collagen and elastin. The family consists of 13 family members, in which 3 individuals are affected. Female and male can be affected in three continuous generations of the family. The results from clinical analysis of the 9 patients are as following: i ) The incidence is equal for males and females; the average age of the onset was 6.5 years, ii ) The lesions are mostly asymmetric plaques in various diameter...