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Year : 2017  |  Volume : 18  |  Issue : 2  |  Page : 142-143

Multidermatomal zosteriform collagen nevus

1 Department of Dermatology, Medical College and Hospital, Kolkata, West Bengal, India
2 Department of Dermatology, Katihar Medical College, Katihar, Bihar, India

Date of Web Publication27-Mar-2017

Correspondence Address:
Anupam Das
Department of Dermatology, Medical College and Hospital, Kolkata, West Bengal
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2319-7250.193032

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How to cite this article:
Das A, Kumar P. Multidermatomal zosteriform collagen nevus. Indian J Paediatr Dermatol 2017;18:142-3

How to cite this URL:
Das A, Kumar P. Multidermatomal zosteriform collagen nevus. Indian J Paediatr Dermatol [serial online] 2017 [cited 2022 May 25];18:142-3. Available from: https://www.ijpd.in/text.asp?2017/18/2/142/193032


The term “connective tissue nevus” was brought into clinical dermatology by Lewandowsky in 1921 to designate a hamartomatous dermal proliferation of the collagen tissue. We present an interesting case of a boy with multidermatomal zosteriform connective tissue nevus.

A 4-year-old boy, otherwise healthy and well-nourished, presented with a progressively increasing number of multiple brownish asymptomatic elevated lesions on the lumbosacral region bilaterally, right shoulder, and right thigh that had been noticed by his mother since 8 months of age. The lesions appeared first on the lumbosacral area and have been increasing in number since then. Over time, new lesions developed on the right thigh and right shoulder as well. Medical, surgical, and family history were noncontributory. Cutaneous examination revealed segmental plaques with multiple confluent brownish soft to firm papules and plaques distributed in a zosteriform pattern on the lumbosacral region, right thigh, and right shoulder [Figure 1]a,[Figure 1]b,[Figure 1]c. There were no similar lesions elsewhere in the body. We did not find any cafe-au-lait macule, axillary freckling, hypopigmented macule, periungual fibroma, gingival fibroma, forehead plaque, or dental pitting. Systemic review including the central nervous system and ophthalmological examination was noncontributory. Chest X-ray, electroencephalogram, electrocardiogram, skull X-ray, radiological examination of the pelvis and long bones, and renal ultrasound did not reveal any abnormality. Sample for histopathology was collected from a representative lesion on the lumbosacral region. Histopathological examination showed thickened collagen fibers in the dermis beneath a normal epidermis [Figure 2]. There was no inflammatory infiltrate. Based on clinicopathological correlation, a diagnosis of multidermatomal zosteriform collagen nevus was made. The parents were counseled regarding the absolutely benign course of the disease, and the boy is under periodic follow-up.
Figure 1: Multiple confluent brownish soft to firm papules and plaques distributed in a zosteriform pattern over the lateral aspect of the right thigh (a), right shoulder (b), and lumbosacral region (c)

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Figure 2: Photomicrograph showing thickened collagen fibers in the dermis beneath a normal epidermis (×100, H and E)

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Connective tissue nevi (CTN) are solitary or multiple or zosteriform, sporadic or inherited, slow-growing, painless, flesh-colored, or pink nodules or plaques most commonly found in the lumbosacral area.[1]

CTN have been classified into collagen nevi, elastic nevi, proteoglycan nevi, and nevi of adventitial connective tissue. Collagen nevi may be further categorized into hereditary or acquired. The hereditary types of collagen nevi include dermatofibrosis lenticularis disseminata in the Buschke–Ollendorff syndrome, familial cutaneous collagenoma, and shagreen patch of tuberous sclerosis; all of which are inherited in an autosomal dominant pattern. Acquired type includes eruptive collagenoma and isolated collagenoma (including plantar cerebriform collagenoma alone or associated with Proteus syndrome), linear or zosteriform collagenoma, knuckle pads collagenoma, and papulolinear type of collagenoma.[2]

Eruptive collagenomas are found on the trunk and upper limbs, arranged in a symmetrical pattern. Shagreen patches are present in tuberous sclerosis with the classic skin findings of facial angiofibromas, ash-leaf macules, Koenen's tumor, gingival fibromas, dental pitting, and fibrotic forehead plaque. Isolated collagenomas are sporadic and are localized to a single body region.[2],[3] Collagenomas have also been reported in association with pseudohypoparathyroidism, Down syndrome, and hypogonadism.

Zosteriform CTN are better classified as distinctive lesions on account of a number of reasons such as atypical presentation, absence of inheritance, and absence of any systemic abnormality.[4] Till date, very few cases of zosteriform CTN have been reported. Many authors are of the opinion that linear and zosteriform CTN are actually the same entity, but another school of thought consider them different entities as linearity of distribution of lesions is not synonymous with zosteriform arrangement.[5]

The clinical differentials include nevus lipomatosus cutaneous superficialis, segmental leiomyomas, and segmental neurofibromas; all of which can be easily differentiated on histological examination.

Our case is an example of rare sporadic zosteriform connective tissue nevus affecting multiple dermatomes. Till date, only one case of zosteriform connective tissue nevus with multidermatomal involvement has been reported.[6] This makes our case the second of its kind and the first one from the Indian subcontinent, prompting us to report the case.

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Conflicts of Interest

There are no conflicts of interest.

  References Top

Xavier-Júnior JC, Batalha PK, Abbade LP, Marques ME. Linear connective tissue nevus along Blaschko's lines: Literature review and case report. Int J Dermatol 2015;54:e166-8.  Back to cited text no. 1
Kumar S, Singh SK, Bansal A, Bansal M. Isolated collagenoma on the scalp: A rare presentation. Int J Trichology 2013;5:88-90.  Back to cited text no. 2
Madke B, Doshi B, Nayak C, Prasannan R. Isolated pedunculated collagenoma (collagen nevi) of the scalp. Indian J Dermatol 2013;58:411.  Back to cited text no. 3
[PUBMED]  [Full text]  
Yeh SW, Magalhaes AM, Vasconcellos MR, Michalany NS, Tomimori Yamashita J. Zosteriform connective tissue nevus: A case report. Int J Dermatol 2003;42:720-2.  Back to cited text no. 4
Dubiel W, Kiehlmann I, Happle R. Linear collagen naevus: An unusual connective tissue disorder. Acta Derm Venereol 2014;94:361-2.  Back to cited text no. 5
Choi YJ, Lee SJ, Choi CW, Kim WS, Lee GY. Multiple unilateral zosteriform connective tissue nevi on the trunk. Ann Dermatol 2011;23 Suppl 2:S243-6.  Back to cited text no. 6


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