CORRESPONDENCE COLUMN |
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Year : 2007 | Volume
: 52
| Issue : 4 | Page : 210-212 |
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Extensive nevus comedonicus |
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Maria Kuruvila, M Kumaresan
Department of Skin and STD, Kasturba Medical College, Mangalore, Karnataka, India
Correspondence Address: M Kumaresan Department of Skin and STD, Kasturba Medical College, Mangalore, Karnataka India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0019-5154.37732
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How to cite this article: Kuruvila M, Kumaresan M. Extensive nevus comedonicus. Indian J Dermatol 2007;52:210-2 |
A 20-year-old female presented with extensive comedones of four years duration. The lesions started on the back and then progressed to involve the face, chest, arms. There was no history of similar complaints in the family. On examination there were multiple dilated follicular openings filled with keratin plugs diffusely involving the face, chest, back, arms [Figure - 1],[Figure - 2],[Figure - 3],[Figure - 4]. Palms and soles were spared. No skeletal ocular or central nervous system abnormalities were found. A clinical diagnosis of nevus comedonicus was made. Biopsy from the lesions showed multiple invaginations of epidermis, filled with concentric lamella of keratin and few rudimentary sebaceous glands opening into lower portion of the invaginations [Figure - 5], confirming the diagnosis of nevus comedonicus. Topical retinoic acid (0.025%) was given as treatment.
Discussion | |  |
Nevus comedonicus (NC) was first described by Kofmann in 1895. [1] Many consider it as a type of adnexal hamartoma, with abnormal differentiation of the epithelial portion. The follicular structure that results is unable to form terminal hair or sebaceous glands and is capable of producing only soft keratin. [2] Others consider this lesion to be an epidermal nevus involving the hair follicle or an appendageal nevus of sweat ducts. Its name may be a misnomer since, according to some, true comedones are not present. [3]
Nevus comedonicus comprises of groups of pits filled with black kerationous plugs resembling black heads. The intervening epidermis may appear normal, hyperkeratotic, hypo or hyperpigmented. [2],[4] There may be one or several lesions in a linear, unilateral [2],[5],[6] or rarely bilateral distribution. [7] Lesions may be very extensive. [2],[6] The commonest site is face followed by the neck, trunk and upper arm. Palms, soles and glans penis may be involved. The lesion usually manifests at birth or by the age of 15 years or rarely as late as middle age with no particular sex predilection. [2]
Beck et al. [2] had reported a case of extensive NC involving left arm, trunk and legs. Extensive NC covering up to half the body surface had been reported by Anderson NP. [5] Our case had extensive bilateral NC involving face, chest and arms.
Epidermal nevus syndrome is a kind of neurocutaneous syndrome that is associated with epidermal nevus and a variety of congenital CNS disorders. Clinical presentations include seizures, paresis, mental retardation and developmental delay. [8] NC usually occurs by itself but may be linked with a variety of systemic findings such as skeletal or ocular anomalies. Nevus comedonicus syndrome (NCS) is a well-defined disorder within the large group of epidermal nevus syndromes. [9] In patients suffering from NCS, the nevus is associated with noncutaneous developmental abnormalities, including ipsilateral cataract, skeletal malformation, CNS abnormalities and trichelemmal cysts. [2],[10],[11] Our patient did not have any systemic findings.
The characteristic histological feature is deep, wide invagination of acanthotic epidermis, filled with concentric lamella of keratin. These probably represent dilated hair follicles, as hair shafts are occasionally seen in the lower portion of the invagination and rudimentary sebaceous glands may open into them. [4] Inter-follicular epidermis may be normal or may show epidermolytic hyperkeratosis. [12] In our case, interfollicular epidermis was normal.
The various topical preparations used for the treatment are retinoic acid, 12% ammonium lactate, tazarotene and calcipotriene. [2],[13],[14] Surgical excision is more effective in the long term than superficial shaving or dermabrasion. [2],[15] Our patient did not report for follow-up.
Our patient is a case of extensive bilateral nevus comedonicus and we report this case for its rarity.
References | |  |
1. | Kofmann S, Ein Fall Von, Seltener. Localization and verbretung of comedones. Arch Dermatol Syphilis 1895;32:177-8. |
2. | Beck MH, Dave VK. Extensive nevus comedonicus. Arch Dermatol 1980;116:1048-50. [PUBMED] |
3. | Lefkowitz A, Schwartz RA, Lambert WC. Nevus comedonicus. Dermatology 1999;199:204-7. [PUBMED] [FULLTEXT] |
4. | Nabai H, Mehregan AH. Nevus comedonicus: A review of the literature and report of twelve cases. Acta Derma Venereol 1973;53:71-4. |
5. | Anderson NP. Comedonicus nevus of extensive distribution. Arch Dermatol Syphilol 1946;53:433-4. |
6. | Rodriguez JM. Nevus comedonicus. Arch Dermatol 1975;111:1363-4. [PUBMED] |
7. | Fritisch P, Wittels W. A case of bilateral naevus comedonicus. A contribution on histogenesis. Hautarzt 1971;22:409-12. |
8. | Zhang W, Simos PG, Ishibashi H, Wheless JW, Castillo EM, Breier JI, et al . Neuroimaging features of epidermal nevus syndrome. AJNR Am J Neuroradiol 2003;24:1468-70. [PUBMED] [FULLTEXT] |
9. | Patrizi A, Neri I, Fiorentini C, Marzaduri S. Nevus comedonicus syndrome: A new pediatric case. Pediatr Dermatol 1998;15:304-6. [PUBMED] [FULLTEXT] |
10. | Whyte HJ. Unilateral comedo nevus and cataract. Arch Dermatol 1968;97:533-5. [PUBMED] |
11. | Engber PB. The Nevus comedonicus syndrome: Case report with emphasis on associated internal manifestations. Int J Dermatol 1978;17:745-9. [PUBMED] |
12. | Barsky S, Doyle JA, Winkelmann RK. Nevus comedonicus with epidermolytic hyperkeratosis. Arch Dermatol 1981;117:86-8. [PUBMED] |
13. | Milton GP, DiGiovanna JJ, Peck GL. Treatment of Nevus comedonicus with ammonium lactate lotion. J Am Acad Dermatol 1989;20:324-8. [PUBMED] |
14. | Deliduka SB, Kwong PC. Treatment of nevus comedonicus with topical tazarotene and calcipotriene. J Drugs Dermatol 2004;3:674-6. [PUBMED] |
15. | Marcus J, Estrkly NB, Bauer BS. Tissue expansion in a patient with extensive Nevus comedonicus. Ann Plast Surg 1992;29:362-6. |
[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5] |
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