Dermatology Online Journal
- Author(s): Batra, Priya
- Loyd, Aaron
- Patel, Rishi
- Walters, Ruth
- Stein, Jennifer A
- et al.
Eruptive collagenomasDepartment of Dermatology, New York University, New York, New York
Priya Batra MD, Aaron Loyd MD, Rishi Patel MD, Ruth Walters MD, Jennifer A Stein MD PhD
Dermatology Online Journal 16 (11): 3
A 24-year-old woman presented with hypopigmented papules of the abdomen that had been present for four years without a family history of similar cutaneous findings or associated medical problems. Histopathologic features confirmed the diagnosis of a connective-tissue nevus that was composed of collagen. Eruptive collagenomas are a rare form of acquired collagenomas, which are characterized by the sudden appearance of asymptomatic papules and nodules on the lower trunk and extremities; the lesions are composed of haphazardly arranged collagen fibers. The pathogenesis is unknown, lesions are persistent, and therapeutic options have not been reported.
|Figure 1||Figure 2|
A 34-year-old woman presented to the Charles C. Harris Skin and Cancer Pavilion in April, 2009, with asymptomatic, hypopigmented papules on the abdomen that had been present for four years. They appeared suddenly during a stressful period in her life. She had consulted multiple dermatologists in the past but had not been given a diagnosis. Similar findings are not present in any members of her family. She has not developed any new lesions since the first ones appeared. Medical history includes depression for which she takes sertraline.
Hypopigmented, well-demarcated, papules and thin plaques without scale were present on the abdomen.
Within the reticular dermis there are areas with thick and haphazardly-oriented collagen bundles. There is a sparse, perivascular, lymphocytic infiltrate. A Verhoeff-van Gieson stain shows preservation of elastic fibers.
Connective-tissue nevi are hamartomas in the dermis that consist of one of the components of the extracellular matrix: elastin, collagen, or proteoglycans . Collagenoma is a form of connective-tissue nevus that is composed primarily of collagen. These may be further categorized as localized or generalized, acquired or inherited, isolated or associated with internal malformations. These hamartomas may be autosomally dominantly inherited as in familial cutaneous collagenomas (FCC) and the shagreen patch of tuberous sclerosis or acquired as in multiple eruptive collagenomas or isolated solitary collagenoma [1, 2, 3, 4].
Only a few cases of eruptive collagenoma have been reported, with the first case reported in 1966 . The disease is characterized by the sudden appearance of raised, cutaneous, firm, white-to-skin-colored papules or nodules that vary in size but that are usually less than 1 cm [4, 6]. The age of onset is commonly within the first decade of life, but lesions also have been reported to appear in early adulthood and beyond. Male and female individuals are equally affected. No successful therapeutic options have been reported and lesions have persisted in all patients . The incidence and pathogenesis of this skin finding is unknown.
Histopathologic examination of eruptive collagenomas shows randomly-arranged, thick collagen bundles, with the loss of the normal empty spaces between bundles and with either diminished, altered, or absent elastic tissue . The diminished elastin may represent a dilution phenomenon caused by the increased amount of collagen . Because of the presence of decreased elastic tissue, it has been suggested that eruptive collagenomas, nevus anelasticus, and papular elastorrhexis may be the same disease entity or different points in the same disease spectrum . In addition to similar histopathologic findings, these three entities present with similar clinical manifestations.
The lesions of FCC differ from those in the eruptive variant. FCC is characterized by the development of asymptomatic nodules that are symmetrically distributed on the upper trunk in the second and third decades of life and that may be associated with possible cardiac findings, such as progressive cardiomyopathy or conduction defects [2, 8]. Eruptive collagenomas are asymptomatic papules and nodules that are usually less than 1 cm and that are distributed on the lower trunk and extremities without any associated systemic findings. Lesions may be numerous in FCC and reach over a hundred lesions , but they are much less numerous in the eruptive variety. In both FCC and eruptive collagenomas, the lesions are symmetrical whereas isolated collagenoma presents in one area of the body, such as the palm or sole [9, 10], without any associated systemic abnormalities. The shagreen patch of tuberous sclerosis is a well-characterized manifestation of multi-organ disease  and lesions are located asymmetrically mainly in the lumbosacral area.
Our patient’s collagenomas were classified as eruptive collagenomas because of the lack of collagenomas in her family, the presence of multiple lesions, and the absence of systemic findings. With the paucity of reported cases and need for additional research into the pathogenesis of connective-tissue nevi, there is a possibility that this case cannot be differentiated from papular elastorrhexis based on histopathologic and clinical findings.
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