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CORRESPONDENCE |
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| Year : 2022 | Volume
: 67
| Issue : 6 | Page : 799-802 |
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| Naevus spinofiliformis: A novel variant of organoid epidermal naevus |
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Vibhu Mendiratta, Sneha Sonker
Department of Dermatology, Lady Hardinge Medical College, New Delhi, India
| Date of Web Publication | 23-Feb-2023 |
Correspondence Address:
Sneha Sonker
Department of Dermatology, Lady Hardinge Medical College, New Delhi
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/ijd.ijd_542_22
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How to cite this article:
Mendiratta V, Sonker S. Naevus spinofiliformis: A novel variant of organoid epidermal naevus. Indian J Dermatol 2022;67:799-802 |
Sir,
Epidermal naevi (EN) are benign congenital hamartomas that result from post-zygotic mutations and comprise organoid and non-organoid (keratinocytic) types.[1] We report an unusual organoid naevus that has so far, to our knowledge, not being recognized as a distinct entity. We propose the term 'naevus spinofiliformis' for this novel presentation with multiple spinous projections and filiform hyperkeratotic lesions.
An 8-year-old boy born to a non-consanguineous marriage presented to us with multiple hyperkeratotic spiny lesions arranged in a linear pattern predominantly over the left upper part of the body. The lesions were present since the age of 1 year and were asymptomatic, but the parents had cosmetic concern. Starting at the age of 7 years, he developed nodular lesions within these hyperkeratotic lesions on the left upper back and shoulder. Developmental history elicited from the parents revealed delayed milestones in terms of walking at age of 3.5 years and delayed speech. There was no history of any systemic involvement. The family history was non-contributory.
On physical examination, there was presence of multiple tiny filiform hyperkeratotic papules and spinous projections, following the lines of Blashcko's in a linear pattern extending from face (including forehead and left lower part of the face), neck, shoulder [Figure 1] left arm and upper back [Figure 2]. There were firm cyst-like nodules of size up to 1 cm amidst the multiple filiform hyperkeratoses on the left upper back and shoulder [Figure 3]. Signs of inflammation were not discernible. Limb length measurement revealed a length discrepancy of 2 cm in both upper limb and lower limb (left > right). On detailed IQ assessment, patient had borderline intellectual and social adaptive behaviour (including delayed milestones, i.e., walking at 3.5 years, delayed speech, average in school performance, slow in writing and reading). On systemic evaluation, ophthalmological, auditory, dental aspects were found to be normal. Skeletal examination revealed non-rigid, flexible dorso-lumbar scoliosis with major curve with right dorsal convexity with compensatory lumbar curve with left convexity without any neurological deficit in lower limb [Figure 3]. | Figure 1: Multiple filiform hyperkeratotic spinous projections following the lines of Blashcko's in a linear pattern extending from face (including left side of forehead and lower part of the face) and neck
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 | Figure 2: Multiple filiform hyperkeratotic spinous projections following the lines of Blashcko's in a linear pattern extending over left shoulder, upper back and arm
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 | Figure 3: Firm cyst-like nodules amidst the multiple filiform hyperkeratosis over the left upper back and shoulder
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Biopsy specimens were obtained from the filiform hyperkeratoses and the nodular lesions on the back. Histopathology from the filiform hyperkeratotic lesions showed the presence of epidermis lined by stratified squamous epithelium and basket weave orthokeratosis, focal papillomatosis and foci of big keratin plugs. Related to the keratin plugs, there was hypergranulosis with mild acanthosis [Figure 4]. Microscopic examination from the nodule was suggestive of calcified sebaceous cyst. Radiograph of dorso-lumbar spine on AP view revealed dorso-lumbar scoliosis [Figure 5]. MRI brain was normal. | Figure 4: Histopathological examination revealed infolding of epithelium at the base of lesion along with thickened granular layer
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 | Figure 5: Radiograph of dorso-lumbar spine on antero-posterior view revealed dorso-lumbar scoliosis
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Nodular lesions (calcified sebaceous cysts) present over back were surgically excised under local anaesthesia, topical retinoids for the hyperkeratotic lesions and oral supplementation with vitamin D was advised.
Epidermal naevi are congenital hamartomas, derived from the multipotent germinative cells of the embryonic ectoderm. Their occurrence has been commonly reported at birth or early childhood, and in 13% of the cases, the lesion is widespread. Happle proposed the classification of epidermal naevi into two major types: nonorganoid (common EN, epidermolytic EN, CHILD naevus) and organoid (naevus sebaceous, naevus comedonicus, porokeratotic eccrine ostial duct naevus).[2]
The origin of these naevi is exactly not known. It is likely that this naevus originated from a somatic mutation occurring at an early stage of embryogenesis. The presence of hyperkeratosis, papillomatosis and acanthosis with lengthening of the ridges is the commonest histological pattern for epidermal naevi.[3]
Variable extracutaneous abnormalities involving most commonly central nervous system, skeletal and ocular system have been reported with organoid naevi such as naevus sebaceous, naevus comedonicus, etc.
The case being reported here had an onset at 1 year of age with spatial congruence of spinous filiform projections over left side of face and trunk, few sebaceous cysts over the upper back along with extracutaneous findings such as dorso-lumbar scoliosis, borderline intellectual impairment and limb length discrepancy.
Literature review of epidermal naevus did not show any case exhibiting similar cutaneous morphological presentation only as multiple aggregates of spinous filiform projections with intervening skip areas over the sites described. This was the predominant, uniform characteristic presentation without any verrucous papules or plaques suggestive of classical morphological pattern of epidermal naevus. However, spinous filiform projections in epidermal naevus have been described in earlier case reports as shown in [Table 1].[1],[3],[4],[5],[6] | Table 1: Reported cases with filiform hyperkeratosis as a clinical finding similar to our case[1],[2],[4],[5],[6]
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Our case describes a unique morphological variant of epidermal naevus which we term as 'naevus spinofiliformis' to denote the characteristic hallmark appearance without any semblance to the known variants of epidermal naevus described earlier. Future experience with similar case reports along with genetic studies (chromosomal analysis for mutation) shall be able to validate the status of this rare entity.
In conclusion, however, few clinical and histopathological features were shared by other organoid naevi, but the spatial congruence of filiform hyperkeratoses, spinous projections with sebaceous cyst is unique for naevus spinofiliformis. Hence, more cases would be required to validate this entity.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
 References | |  |
| 1. | Singh S, Taneja N, Arava S, Bhari N. Nevus trichilemmocysticus. Int J Dermatol 2017;56:1483-6.  |
| 2. | Ferreira PS, Valente NY, Nico MM. Multiple filiform keratoses and nodules in a 10-year-old girl. Pediatr Dermatol 2013;30:261-2. |
| 3. | Zamora MI, Vazquez-Doval FJ. Epidermal naevi associated with trichilemmal cysts and chromosomal mosaicism. Br J Dermatol 1997;137:821-4. |
| 4. | Tantcheva-Poor I, Reinhold K, Krieg T, Happle R. Trichilemmal cyst nevus: a new complex organoid epidermal nevus. J Am Acad Dermatol 2007;57:S72-7. |
| 5. | Lang SC, Bauer B, Bröcker EB, Hamm H. Naevus trichilemmocysticus–the first paediatric case of a newly delineated organoid naevus. J Eur Acad Dermatol Venereol 2010;24:1485-7. |
| 6. | Larralde M, Boggio P, Abad ME, Corbella C, Happle R. Nevus trichilemmocysticus: Report of a new case of a recently recognized entity. Pediatr Dermatol 2011;28:286-9. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]
[Table 1] |
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