|Year : 2017 | Volume
| Issue : 3 | Page : 237-240
A unique porokeratotic variant of inflammatory linear verrucous epidermal nevus
Anup Kumar Tiwary, Dharmendra Kumar Mishra
Department of Dermatology, Venereology and Leprosy, Rajendra Institute of Medical Sciences, Ranchi, Jharkhand, India
|Date of Web Publication||7-Jun-2017|
Anup Kumar Tiwary
Flat No. 103, Maa Enclave Block B, Cheshire Home Road, Bariatu, Ranchi - 834 009, Jharkhand
Source of Support: None, Conflict of Interest: None
Inflammatory linear verrucous epidermal nevus (ILVEN) is a rare variant of nevoid linear inflammatory dermatoses which appear most often in the first 6 months of life and persists for many years or even whole life. Clinically, it often shares many features with linear psoriasis, linear porokeratosis, and other linear dermatoses which can usually be differentiated by an established set of clinical and histopathological criteria. Herein, we are reporting an interesting and rare case fulfilling all the clinical and histopathological criteria of ILVEN in a 13-year-old boy which uniquely revealed cornoid lamella on histopathology so we termed it as “porokeratotic variant of inflammatory linear verrucous epidermal nevus.” To the best of our knowledge, only two such cases were reported previously in the literature and none from our country.
Keywords: Inflammatory linear verrucous epidermal nevus, linear dermatoses, nevoid, porokeratotic variant
|How to cite this article:|
Tiwary AK, Mishra DK. A unique porokeratotic variant of inflammatory linear verrucous epidermal nevus. Indian J Paediatr Dermatol 2017;18:237-40
|How to cite this URL:|
Tiwary AK, Mishra DK. A unique porokeratotic variant of inflammatory linear verrucous epidermal nevus. Indian J Paediatr Dermatol [serial online] 2017 [cited 2018 Jun 23];18:237-40. Available from: http://www.ijpd.in/text.asp?2017/18/3/237/206088
| Introduction|| |
Inflammatory linear verrucous epidermal nevus (ILVEN) is clinically characterized by linear, erythematous and intensely pruritic scaly psoriasiform papules coalescing to form plaques following the Blaschko's lines usually involving the lower limb unilaterally. ILVEN usually has early life onset, and characteristically, the lesions are persistent and resistant to treatment. Here, we are reporting a unique case of ILVEN as a “porokeratotic variant of ILVEN” in a 13-year-old boy. To the best of our knowledge, only two such cases were reported in the previous literature and none from our country.
| Case Report|| |
A 13-year-old boy came at the outpatient department presenting with multiple, extremely itchy papules, and plaques in linear fashion on the left lower limb since his infancy. During the infancy, lesions started to develop first on the dorsum of the left foot which subsequently extended to involve leg and posterior aspect of the thigh in linear pattern over the next 3 years. No other body sites were affected with such lesions, and there was no such family history.
On local cutaneous examination, multiple, intensely pruritic, psoriasiform and hyperkeratotic (at some places) coalescing papules and plaques on the lateral side of the left leg and posterior aspect of the left thigh were noted on the hypopigmented and erythematous background which were arranged in linear pattern along the Blaschko's lines. On the dorsum of the left feet, verrucous nodules were the predominant lesions [Figure 1]a and [Figure 1]b. The size of the lesions, erythema, and scaling was variable. Examination of hair, nails, teeth, mucosa, palm, and soles was normal. The child was normal on systemic examination, and all laboratory parameters were within normal limits.
|Figure 1: (a) Verrucous nodules on dorsum of the left foot, (b) Hyperkeratotic coalescing papules and plaques on the lateral side of the left leg along the Blaschko's lines|
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Clinically, ILVEN, linear psoriasis, and linear porokeratosis were kept as differential diagnoses and to reach a final diagnosis, punch biopsy sample was taken and sent for the histopathological examination (HPE).
HPE revealed spongiosis, acanthosis, elongation of rete ridges, and alternating areas of ortho-hyperkeratosis and hypergranulosis with parakeratosis and hypogranulosis. In two small foci, the epidermis showed an invagination having a column of poorly staining parakeratotic corneocytes (cornoid lamella). The granular layer was absent at the floor and wall had hypergranulosis. A moderately dense superficial perivascular patchy lichenoid lymphocytic infiltrate was seen in the upper dermis [Figure 2]a,[Figure 2]b,[Figure 2]c.
|Figure 2: (a) Spongiosis, acanthosis, and alternating areas of ortho-hyperkeratosis and hypergranulosis (bold arrow) with parakeratosis and hypogranulosis, respectively (H and E, ×20). (b) Focal interface dermatitis with patchy lichenoid lymphocytic infiltrates in the upper dermis (H and E, ×40). (c) Epidermal invagination having a column of poorly staining parakeratotic corneocytes (cornoid lamella; H and E, ×20)|
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In the past few years, the child was treated with topical emollients, potent topical corticosteroid, and topical tacalcitol, but none of them appeared to be effective.
The detailed history, clinical features, histopathological findings, and marked failure of response to antipsoriatic treatment favored the diagnosis of ILVEN to be made, and this case was suggested as porokeratotic variant of ILVEN. The patient was started on oral isotretinoin therapy and advised for follow-up.
| Discussion|| |
In 1896, the term “inflammatory linear verrucous epidermal nevus” was proposed by Unna, but this rare linear dermatosis was first described separately by “Kaidbey and Kurban,” and “Altman and Mehregan” in 1971. Usually, it presents as markedly pruritic, variably erythematous, scaly, coalescing papules, and plaques along the Blaschko's lines typically involving the single lower limb and persists for many years of life.,
The etiopathogenesis of ILVEN is still unclear but owing to its sporadic occurrence, extremely rare familial cases, and resemblance to other nevoid inflammatory linear dermatoses, it was thought to be the result of an early, potentially lethal, and unidentified postzygotic dominant mutation rescued by genetic mosaicism. Hypothetically, in 2002, Happle has suggested that ILVEN might be a reflection of the action of a “retrotransposon” that is partly expressed and partly silenced at an early developmental stage. Based on genetic and molecular markers, there are some suggested cascade of underlying pathological changes which eventually results in the clinical manifestation and they are: (1) Elevated intercellular adhesion molecule-1, extracellular adhesion molecule-1, and Human Leukocyte Antigen-DR and keratin ten expression; (2) increased interleukin-1 (IL-1), IL-6, and tumor necrosis factor-α; (3) upregulation of involucrin expression in orthokeratotic area and decreased expression in parakeratotic area; and (4) clonal dysregulation of growth of keratinocytes.
To differentiate from other pathogenetically and clinically related nevoid linear inflammatory dermatoses, following distinct diagnostic clinical criteria have been given by Altman and Mehregan in 1971 which were later modified by Morag and Metzker in 1985. Further histopathological criteria were also proposed by Dupre and Christol in 1977 to increase the diagnostic accuracy.
Altman and Mehregan's Clinical Criteria
- Early age of onset (75% of cases with onset before 5 years of age, and 50% before 6 months)
- Predominance in women in a 4:1 proportion (omitted in the modified criteria)
- Left side more commonly affected
- Psoriasis-like morphology
- Persistence and resistance to treatment.
Dupre and Christol's Histopathological Criteria
- Psoriasiform changes - parakeratosis, acanthosis, elongation of rete ridges, thinned suprapapillary plates, tortuosity of the dermal capillaries, and upper dermal lymphocytic infiltrates
- Sharply demarcated alternate areas of parakeratosis with agranulosis or hypogranulosis and orthokeratosis with hypergranulosis.
Clinically, following are some linear dermatoses which should be kept as close differential diagnoses: linear psoriasis, linear lichen planus, linear porokeratosis, lichen striatus, and ichthyosiform nevus in congenital hemidysplasia with ichthyosiform nevus, and limb defects syndrome.
All these dermatoses can be ruled out by the above-mentioned criteria. Although linear psoriasis is the closest differential but with the help of clinical criteria of ILVEN such as very early age of onset, extremely rare family history, intense pruritus, and resistance to antipsoriatic treatments (as noted in our case) it can be ruled out. Furthermore, wherein the facility is available, the genetic and quantitative immunohistochemical markers can also be used such as increased keratin ten expression and reduced Ki-67, CD4, CD8, CD450RO, CD2, CD25, CD94, and CD161 positive cells in ILVEN compared to psoriasis.
In our case, all the clinical and histopathological features were much more in favor of ILVEN than the rest. The mere presence of cornoid lamella was not enough to allow reckless ignorance of all those features which were all in favor of ILVEN. Moreover, cornoid lamellation is by no means pathognomonic of porokeratosis as it can be found in a range of inflammatory, hyperplastic, and neoplastic skin conditions as described by Wade and Ackerman in 1980. Further in 1982, Su identified the porokeratotic changes in epidermal nevus and gave the term “porokeratotic epidermal nevus.” Therefore, the presence of cornoid lamella does not necessarily mean that the condition is primarily porokeratosis.
As our case fulfilled all the required established criteria of ILVEN and also showed the presence of cornoid lamella, it was better to consider this case as porokeratotic variant of ILVEN rather than making a hasty diagnosis of linear porokeratosis.
For therapeutic point of view, ILVEN is notoriously resistant to treatment, occasionally having a fortunate course of spontaneous resolution. Potent topical corticosteroids applied under occlusion and intralesional injections of triamcinolone acetonide may provide symptomatic relief. Topical retinoids and Vitamin D analogs (calcipotriol, tacalcitol) appear to provide either no benefit or merely reduce the itching and redness.,,
Systemic treatments in the form of oral administration of acitretin and etanercept have been proved effective. Treatment with dermabrasion or cryotherapy is commonly associated with recurrence after superficial procedures and scarring following deeper procedures.
Surgical excision may be curative if done up to the appropriate depth but postoperative scarring is the obvious sequelae. Successful treatment has also been reported with the erbium-yttrium aluminum garnet ablation laser, 585-nm flash lamp-pumped pulsed tunable dye laser in some cases.
The authors sincerely thank Dr. Uday Khopkar for their contribution by giving valuable suggestions for the histopathological diagnosis.
Financial Support and Sponsorship
Conflicts of Interest
There are no conflicts of interest.
| References|| |
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[Figure 1], [Figure 2]