|LETTERS TO EDITOR
|Year : 2016 | Volume
| Issue : 2 | Page : 159-160
Diagnostic dilemma between linear psoriasis and inflammatory linear verrucous epidermal nevus
Nibir Biswas1, Dipayan Sengupta1, Ishad Agarwal2, Prerna Pathak1
1 Department of Dermatology, Venereology and Leprosy, School of Tropical Medicine, Kolkata, West Bengal, India
2 Department of Dermatology, Institute of Post Graduate Medical Education and Research, Kolkata, West Bengal, India
|Date of Web Publication||30-Mar-2016|
Male Resident Hostel, School of Tropical Medicine, 108, C.R. Avenue, Kolkata - 700 073, West Bengal
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Biswas N, Sengupta D, Agarwal I, Pathak P. Diagnostic dilemma between linear psoriasis and inflammatory linear verrucous epidermal nevus. Indian J Paediatr Dermatol 2016;17:159-60
|How to cite this URL:|
Biswas N, Sengupta D, Agarwal I, Pathak P. Diagnostic dilemma between linear psoriasis and inflammatory linear verrucous epidermal nevus. Indian J Paediatr Dermatol [serial online] 2016 [cited 2020 Aug 8];17:159-60. Available from: http://www.ijpd.in/text.asp?2016/17/2/159/172476
Inflammatory linear verrucous epidermal nevus (ILVEN) is a rare congenital disease characterized by a pruritic linear lichenoid lesion that appears shortly after birth. However, it is difficult clinically to distinguish from linear nevoid psoriasis. In a rare occasion, they can even present together. Several attempts have been made in the past to differentiate between these two conditions on the basis of clinical or histological features, and even by molecular markers. Here, we describe a 2-year-old girl presented with linear lichenoid lesion which clinically and histologically resembles ILVEN, but responded to a topical corticosteroid, a feature, which is usually seen in linear psoriasis (LP).
A 2-year-old girl child was presented at the outpatient department with the complaint of multiple confluent erythematous papules involving anterior and posterior trunk and all the four extremities but sparing scalp, face, neck, palm, and soles. The lesions started to develop 2 months back from the dorsolateral aspect of her hand in a linear fashion.
On examination, multiple well-defined areas of grouped papules on a hypopigmented background arranging in geographic shape (some linear, especially those were on the distal limbs) were observed. They were of various sizes and often accompanied by a moderate degree of scaling and erythema [Figure 1].
|Figure 1: Clinical photograph showing linear lesion at the back of leg (a), improvement after treatment with topical corticosteroids (b)|
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Hair, nail, and mucosa examination were normal. No family history of the similar lesion was present. Birth and developmental history was normal. However, she gave a history of having cough and cold easily.
A histopathological examination from the lesion shows acanthosis and an alternating band of hyperkeratosis and parakeratosis. Hypergranulosis was seen beneath hyperkeratotic column and hypogranulosis beneath the parakeratotic column. Rete-ridges showed psoriasiform hyperplasia. Perivascular mono-nuclear infiltrate was seen in the upper dermis [Figure 2]. HLA-Cw*0602 was tested which came back as negative.
|Figure 2: Photomicrograph showing histopathological feature of alternating hyperkeratosis and parakeratosis associated with hypergranulosis and hypogranulosis, respectively, (a) Scanner view (b) ×10 view (c) ×40 view|
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The patient was treated with topical emollients, oral antihistaminics for pruritus along with mid-potent topical corticosteroid, and topical tacalcitol alternately (changed every 2 weeks). Marked improvement of the most of the lesions was seen at the 16th week of treatment.
ILVEN is a rare congenital disease characterized by lichenified linear plaque that can morphologically resemble psoriasis., Clinical criteria for ILVEN was proposed by Altman and Mehregan (Early age of onset, 4:1 female preponderance, frequent involvement of left lower extremity, pruritus, distinct, and inflammatory linear appearance, following the lines of Blaschko and persistent lesions showing marked refractoriness to treatment). Histological criteria are later established. Unfortunately, the most cases do not show this classic criteria making the diagnosis difficult. However, attempts have been made to differentiate it from LP clinically (clinically ILVEN is characterized by early age of onset: During the 1st month of life, slow progression, presence of pruritus, and unresponsiveness to treatment. However, LP tends to develop later in life, progresses rapidly, only occasionally pruritic, and responds well to anti-psoriatic treatment. Furthermore, scalp and nail involvement favors the possibility of LP), and histologically. Also, some authors postulated the use of genetic and molecular markers (keratin, Ki-67, CD markers, and involucrin ,,) to differentiate between these two conditions. However, clearly an application of such markers in clinical setting is not always possible.
The most interesting hypothesis was put forward by Hoferwho have argued that that such as psoriasis, ILVEN may also represent the segmental Types 1 and 2 mutations that phenotypically represent four distinct types of clinical variants: (1) ILVEN with or without concomitant psoriasis, only in part reacting to antipsoriatic treatment, (2) ILVEN without concomitant psoriasis, (3) LP with concomitant psoriasis vulgaris, with both Groups 2 and 3 reacting successfully to antipsoriatic treatment, and (4) LP without concomitant psoriasis vulgaris and with no family history of psoriasis (very rarely reported).
Our case has mostly shown clinical characteristics of ILVEN and has shown classical features of ILVEN histologically (hypergranulosis and parakeratosis alternating with hypogranulosis and orthokeratosis). However, contrary to the usual notion, it responded well to topical anti-psoriatic medication. We classify this case as Hoffer Type 1 ILVEN phenotype and report due to the paucity of a similar report in the literature.
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[Figure 1], [Figure 2]