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Year : 2013  |  Volume : 58  |  Issue : 5  |  Page : 411
Isolated pedunculated collagenoma (collagen nevi) of the scalp


Department of Dermatology, Topiwala National Medical College and B.Y.L. Nair Hospital, Mumbai, India

Date of Web Publication30-Aug-2013

Correspondence Address:
Chitra Nayak
Department of Dermatology, Topiwala National Medical College and B.Y.L. Nair Hospital, Mumbai
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.117367

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How to cite this article:
Madke B, Doshi B, Nayak C, Prasannan R. Isolated pedunculated collagenoma (collagen nevi) of the scalp. Indian J Dermatol 2013;58:411

How to cite this URL:
Madke B, Doshi B, Nayak C, Prasannan R. Isolated pedunculated collagenoma (collagen nevi) of the scalp. Indian J Dermatol [serial online] 2013 [cited 2020 Jul 13];58:411. Available from: http://www.e-ijd.org/text.asp?2013/58/5/411/117367


Sir,

Connective tissue nevi of the skin are hamartomatous proliferative lesions consisting chiefly of one of the components of the extracellular matrix, namely, collagen, elastin, or glycosaminoglycans. [1] Connective tissue nevi with predominant collagen and elastic component are referred to as Lipschutz type and Lewandowsky type respectively. [2] Multiple collagenomas are a feature of several distinct diseases and syndromes, including tuberous sclerosis, Buschke-Ollendorf syndrome (dermatofibrosis lenticularis disseminata), eruptive collagenomas, and familial cutaneous collagenoma. [3] Isolated cerebriformcollagenoma of the palm and sole is a well known entity and has been reported in association with Proteus syndrome. [4],[5] We hereby report a case of isolated pedunculatedcollagenoma of the scalp for its unusual location and absence of other syndromic features.

A 20-year-old unmarried male presented to our out-patient department with an asymptomatic skin colored mass on the scalp region of three years duration which was gradually increasing in size. The patient did not report any previous history of trauma at the involved area. There was no family history of any cutaneous disorder. Dermatological examination showed a soft skin to pink colored mass on vertex of the scalp and measured about 5 centimeter in diameter. [Figure 1] We considered a differential diagnosis of sebaceous nevus, nevus lipomatosis and appeandegeal tumor. There was no evidence of hypopigmented macule on wood's lamp examination or skin lesions suggestive of tuberous sclerosis. There was no significant past medical or surgical history. Baseline laboratory investigations including urinalysis were within normal limits. A skin biopsy obtained from the lesion showed focal acanthotic epidermis and significant increased density of collagen bundles in the deep reticular dermis. [Figure 2] Masson's trichrome stain confirmed the presence of dense collagen bundles with decreased elastic fibers. [Figure 3] Abdominal ultrasound did not reveal any renal masses. Chest roentgenogram did not show any abnormality. Radiography of the pelvis and long bones were normal with no evidence of circumscribed areas of increased bone density suggestive of osteopoikilosis. Thus, a diagnosis of sporadic type of isolated collagenoma of the scalp was made.
Figure 1: A single pedunculated tumor like mass on vertex of scalp

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Figure 2: H and E stained section from pedunculated mass showing acanthotic epidermis and dense collagen bundles in the deeper dermis arranged in a haphazard manner. (×40)

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Figure 3: Masson's trichrome stained section showing green colored staining collagen bundles suggesting excessive collagen. (x40)

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Collagenoma (collagen nevus) has been classified into four distinct groups: (i) familial cutaneous collagenoma (FCC); (ii) the Shagreen patch of tuberous sclerosis; (iii) eruptive collagenoma; and (iv) isolated collagenoma. [6] Depending upon the classification of the genetic inheritance pattern, collagenomas are classified as either inherited or sporadic. [7],[8] Inherited group includes dermatofibrosis lenticularis disseminata in the Buschke-Ollendorf syndrome, familial cutaneous collagenoma (FCC), and shagreen patches seen in tuberous sclerosis-all are autosomal dominant in inheritance. The acquired group includes eruptive collagenomas and isolated collagenomas. Eruptive collagenomas is characterized by multiple asymptomatic skin colored papules and nodules occurring symmetrically on trunk and upper limbs. FCC also in combination with multiple collagenomas has in addition autosomal inheritance pattern and cardiac disorders like cardiomyopathy and conduction abnormalities. [9],[10] Shagreen patches (plaques of collagenoma) are present in tuberous sclerosis with the classic skin findings of facial angiofibromas, periungual fibromas (koenen's tumor), gingival fibromas, fibrous plaque of forehead and ash-leaf macules.

Isolated collagenomas are sporadic and are localized to a single body region as in our case. Cerebriform plantar nevi are considered to be a pathognomic cutaneous marker of Proteus syndrome (a type of epidermal nevus syndrome). [11] However, many authors have reported the occurrence of plantar collagenoma without any co-existent features of Proteus syndrome. A collagenoma on the scalp can give the clinical morphology of cutis verticisgyrata and deserves special mention. [8] Collagenomas have also been reported in association with pseudohypoparathyroidism, [12] Down syndrome [13] and hypogonadism. [14] The pathogenesis of collagenomas is not clear. In a proven case of collagenoma or connective tissue nevi, a further work-up is necessary to rule out any underlying systemic disorder. [Table 1] gives the diagnostic evaluation to be done in cases suspected of collagenoma.
Table 1: Diagnostic work up to be done in case of collagenoma

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Histopathology of collagenoma shows increased amount of collagen fibers, with either normal or decreased elastic tissue. Management of extensive collagenoma especially over the plantar surface is difficult and is primarily a surgeon's domain. In our case we could excise the entire mass due to small size of the lesion.

 
   References Top

1.Ju Q, Song N, Sun J. Eruptive cutaneous collagenoma in a Chinese patient. J Dermatol 2011;38:399-401.  Back to cited text no. 1
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2.Mukhi SV, Kumar P, Yuvarajkumar D, Raghuveer CV. Eruptive collagenoma. Indian J Dermatol Venereol Leprol 2002;68:98-9.  Back to cited text no. 2
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3.Dawn ME, Deng AC, Petrali J, Wessely C, Jaffe D, Gaspari AA. Familial cutaneous collagenoma. Skinmed 2008;7:43-5.  Back to cited text no. 3
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4.Nico MM, Valente NY, Machado KA. Isolated plantar collagenoma. Acta Derm Venereol 2003;83:144.  Back to cited text no. 4
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5.Beachkofsky TM, Sapp JC, Biesecker LG, Darling TN. Progressive overgrowth of the cerebriform connective tissue nevus in patients with Proteus syndrome. J Am Acad Dermatol 2010;63:799-804.  Back to cited text no. 5
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6.Uitto J, Santa Cruz DJ, Eisen AZ. Connective tissue nevi of the skin. Clinical, genetic, and histopathologic classification of hamartomas of the collagen, elastin, and proteoglycan type. J Am Acad Dermatol 1980;3:441-61.  Back to cited text no. 6
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7.Ishii N, Maeyama Y, Nakama T, Hashimoto T. A case of solitary collagenoma localized on the upper lip mimicking mucocele. Clin Exp Dermatol 2009;34:243-4.  Back to cited text no. 7
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8.Laxmisha C, Thappa DM, Jayanthi S. Isolated scalp collagenoma mimicking cutis verticisgyrata. Indian J Dermatol Venereol Leprol 2006;72:309-11.  Back to cited text no. 8
[PUBMED]  Medknow Journal  
9.Amato L, Mei S, Gallerani I, Moretti S, Cipollini EM, Palleschi GM, et al. Familial cutaneous collagenoma: Report of an affected family. Int J Dermatol 2005;44:315-7.  Back to cited text no. 9
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10.BoenteMdel C, Primc NB, Asial RA, Winik BC. Familial cutaneous collagenoma: Aclinicopathologic study of two new cases. Pediatr Dermatol 2004;21:33-8.  Back to cited text no. 10
    
11.Viljoen DL, Saxe N, Temple-Camp C. Cutaneous manifestations of the Proteus syndrome. Pediatr Dermatol 1988;5:14-21.  Back to cited text no. 11
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12.Kakinuma Y, Endo H, Tsukahara T, Futoeda T, Saito Y, Shinkai H. Collagenoma with pseudohypoparathyroidism. Br J Dermatol 2000;143:1122-4.  Back to cited text no. 12
    
13.Togawa Y, Nohira G, Shinkai H, Utani A. Collagenoma in Down syndrome. Br J Dermatol 2003;148:596-7.  Back to cited text no. 13
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14.Sacks HN, Crawley IS, Ward JA, Fine RM. Familial cardiomyopathy, hypogonadism, and collagenoma. Ann Intern Med 1980;93:813-7.  Back to cited text no. 14
[PUBMED]    


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