Papulolinear collagenoma

Abstract

Connective tissue nevi are circumscribed hamartomas of the dermal extracellular matrix. Documented connective tissue nevi of the collagen type include familial cutaneous collagenomas, shagreen patches, eruptive collagenomas, and isolated collagenomas.1Uitto J Santa Cruz D.J Eisen A.Z Connective tissue nevi of the skin.J Am Acad Dermatol. 1980; 3: 441-461Abstract Full Text PDF PubMed Scopus (208) Google Scholar During the last 10 years, we have followed a patient with a unique variant of connective tissue nevi, characterized by both papular and linear skin-colored lesions affecting the dorsal aspects of the hands and fingers. Histologic examination revealed an increase of coarse collagen fibers in the dermis and diminished elastic fibers. A 45-year-old white Brazilian woman had multiple papules and linear skin-colored plaques that involved the dorsum of the hands and fingers. The lesions were asymptomatic. No history of trauma or inflammation preceding the appearance of the lesions could be elicited. The patient's relatives were not available for evaluation. Clinical examination revealed multiple linear skin-colored lesions, ranging in size from 0.4 to 6 cm, associated with tiny papules affecting the hands and fingers of the patient (Fig 1). Histologically, both papules and linear lesions were characterized by accumulation of dense, coarse collagen fibers in the dermis. The epidermis and adnexal structures appeared normal and there were no signs of inflammation (Fig 2). Van Gieson stain demonstrated enlarged but essentially normal collagen tissue. Elastic-tissue stains revealed a paucity of elastic fibrils.Fig 2Biopsy specimen of a linear lesion on the hand. Hematoxylin-eosin stain shows normal epidermis and reticular dermis. Papillary dermis exhibits both compact and coarse collagen fibers, running parallel to the dermoepidermal junction. Preserved eccrine sudoriparous ducts can be seen surrounded by dense collagen fibers. (Original magnification ×100.)View Large Image Figure ViewerDownload (PPT) Connective tissue nevi are hamartomas consisting of the various components of the dermal connective tissue. On the basis of clinical, histologic, and genetic consideration, the connective tissue nevi can be classified into defined categories. Association with extracutaneous features or a characteristic topography allows further delineation of these disease entities and aids in establishing an accurate diagnosis.2Beylot C, Bioulot-Sage P, Doutre MS, Vendeaud-Busquet M. Les dysplasies héréditaires du tissu conjonctif. Encycl Méd Chir (Paris-France). Dermatologie 1988:12660 A10, 1-21Google Scholar, 3Uitto J Santa Cruz D.J Eisen A.Z Familial cutaneous collagenoma genetic studies on a family.Br J Dermatol. 1979; 101: 185-195Crossref PubMed Scopus (66) Google Scholar, 4Smith L.R Bernstein B.D Eruptive collagenoma.Arch Dermatol. 1978; 114: 1710-1711Crossref PubMed Scopus (26) Google Scholar We report a unique variant of connective tissue nevi of the collagen type. Morphologically, slightly elevated skin-colored papules vary in size from 0.1 to 0.5 cm in diameter and elevated, well-demarcated, asymptomatic linear lesions extend up to 6 cm. The lesions predominated on the dorsum of the hands and fingers. Associated abnormalities were absent. Clinically, the lesions closely resembled scars. The patient emphatically denied any history of trauma or inflammation preceding the onset of the disorder. Histologic examination revealed a normal epidermis associated with an accumulation of dense, coarse collagen fibers in the dermis and diminished elastic fibers, characterizing an hamartomatous proliferation of the collagen.5Nogues I Le Gall F Chevrant-Breton J A hardened patch on the thigh. Conjuntival nevus of the collagen type which is also called conjunctival nevus of the fibromatous type, collagenoma, collagen hamartoma or fibromatous hamartoma.Ann Pathol. 1995; 15: 69-70PubMed Google Scholar, 6Rocha G Winkelman R.K Connective tissue nevus.Arch Dermatol. 1962; 85: 727-729Google Scholar In scars, the normal collagen bundles are replaced by fibrillary collagen. The epidermis is usually thin with a diminished pattern of epidermal rete ridges. In scars, a decreased number of adnexal epithelial structures can usually be observed. In summary, we believe our case represents a variant of connective tissue nevi of the collagen type and further suggest the designation papulolinear collagenoma. Additional cases will certainly help to classify best this unusual disorder of the collagen tissue. We thank Henry W. Randle, MD, PhD, Mayo Clinic, Jacksonville, for his assistance.