Connective tissue naevus
M Ramesh Bhat
Department of Skin, STD and Leprosy, Father Muller's Medical College, Kankanady, Mangalore - 575 002
|How to cite this article:
Fernandes CZ, Martis J, Bhat M R, Shetty J N. Connective tissue naevus. Indian J Dermatol Venereol Leprol 2002;68:102
AbstractA case of non hereditary connective tissue naevus without any association with systemic complaints, in a 20 - year old male is reported.
Connective tissue naevus represents a hamartoma in which the amount of collagen is increased but the amount of elastic tissue may be increased, normal or decreased.
A 20 -year-old man presented with asymptomatic, raised, black-coloured, skin lesions on the back of 3 years duration. The lesions were gradually progressive. There was no family history of similar lesions. [Figure - 1]
Clinical examination revealed multiple hyperpigmented papules and nodules of varying sizes on the back No systemic abnormalities were detected. Histopathology of the skin lesion showed thickened and homogenized collagen bundles. Special staining with Vangieson Verhoeff′s stain showed an increase in collagen and elastic fibres in the dermis. Radiological examination of pelvis, hands and feet revealed no bony abnormalities. The lesions were excised.
Connective tissue naevi are circumscribed hamartomatous malformations of the dermal extracellular matrix, that is of collagen, elastic fibres or glycosaminoglycans. Present at birth, appearing within the first few years of life, they appear as elevated, soft to firm tumours of varying size. They may be grouped, linear or irregularly distributed over the body suface. The colour may be flesh, brown hypopigmented or yellow. Aconnective tissue naevus can occur a) without alterations in other organs and without being genetically determined b) With alterations in other organs, being inherited as an autosomal dominanttrait. c) with osteopoikilosis being inherited in an autosomal dominant pattern.
The autosomal dominantly inherited disorder of familial cutaneous collagenoma is characterised bya symmetric "eruption" of collagen type nodules and plaques occurring predominantly over the upper back in adolescence.
Connective tissue naevi associated with osteopokilosis is referred to as Buschke Ollendroff syndrome. The skin lesions in this syndrome consist of multiple firm pale papules and plaques in an asymmetric distribution.
Histologicallya hamartomatous collection of various amounts of collagen, elastic and possibly adipose tissue′s present in the dermis. There is no increase in fibroblasts.
Connective tissue naevi are divided into two types, namely the adventitial and reticular. Naevi of adventitial connective tissue are usually small and situated on the extremities. Naevi of reticular connective tissue are usually situated on the trunk.
In this case, patient had any connective tissue naevus, without any association with systemic complaints. It may be considered as non - hereditary, because there was no family history of connective tissue naevus.
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