Inflammatory Linear Verrucous Epidermal Nevus Co-Existing with Nevoid Psoriasis Associated with Arthropathy: A Rare Association
Priya Prafulla K*
Department of Dermatology, venerology and Leprosy, Government Medical College, India
Submission: August 01, 2022;Published: August 25, 2022
*Corresponding author: Priya Prafulla K, Department of Dermatology, venerology and Leprosy, Government Medical College, India
How to cite this article: Priya Prafulla K. Inflammatory Linear Verrucous Epidermal Nevus Co-Existing with Nevoid Psoriasis Associated with Arthropathy: A Rare Association. JOJ Dermatol & Cosmet. 2022; 4(4): 555645. DOI: 10.19080/JOJDC.2022.04.555645
Abstract
Inflammatory linear verrucous epidermal nevus (ILVEN) is a keratinocytic nevus characterized by erythematous pruritic plaque along the lines of Blaschko. Epidermal nevi are associated with primarily skeletal abnormalities and less commonly central nervous system (CNS) manifestations. There have been only few case reports with the involvement of the musculoskeletal system. We present a case of ILVEN associated with a deformity of hand secondary to arthropathy.
Keywords:Nevoid psoriasis; Inflammatory verrucous epidermal nevus; Arthropathy; Deformity; Blashckoid
Introduction
Segmental mosaicism leads to variable presentation of nevoid condition leading to Blashckoid distribution of lesions [1]. ILVEN and nevoid psoriasis are two such conditions having similarities clinically and histologically which may lead to diagnostic difficulties. Arthritis has been described as a new association in the literature, which if detected early and managed promptly may prevent complications. We came across case of ILVEN associated with arthropathy which was initially thought to be nevoid psoriasis with arthropathy leading to a diagnostic dilemma.
Case Discussion
An 11-year-old female had itchy pruritic lesions predominantly involving the left side of her body since 6 years (Figure 1,2 and 4). There were episodes of exacerbation in winter. There was no involvement by the disease in any of the family members. There was a progressive loss of movement of the left little and ring finger at the distal interphalangeal joint (Figure 3) during the past 2 years. There was no preceding history of joint pain or swelling. On examination, there were multiple erythematous scaly plaques along the lines of Blaschko involving the left side of the chest, back, arm, forearm and palm intermingled with atrophic hypopigmented macules and patches. Similar erythematous scaly plaques were present on the right palm (Figure 3). A fixed flexion deformity of the left little and ring finger was observed. The scalp and nails were normal.
Clinically a diagnosis of naevoid psoriasis with arthritis was made based on the morphology and distribution of the lesions and the patient was treated with topical Flucinolone acetonide cream. On further investigation, radiological examination of the deformity revealed bony ankylosis of the left ring and little finger distal interphalangeal joint and a thinning of the middle phalanx (Figure 5). On histology, there was stratified squamous epithelium with moderate acanthosis, papillomatosis, foci of parakeratosis, elongation of rete ridges with slight spongiosis and exocytosis of lymphocytes in the epidermis and mild-to-moderate perivascular chronic inflammatory infiltrate of lymphocytes and histiocytes in the dermis (Figure 6-8). These were the features suggestive of ILVEN. It was difficult to arrive at a diagnosis in our case as there were features of both naevoid psoriasis and ILVEN. After the literature review, we considered the possibility of ILVEN and naevoid psoriasis being present concomitantly. She showed partial response to topical steroid at 6 weeks and was subsequently lost to follow-up.
Discussion
There has been a considerable debate whether ILVEN and naevoid psoriasis are distinct entities or variants of each other. Though they may have a resemblance, a few differentiating features that help in arriving at a diagnosis are given in Table 1. A diagnostic criteria for ILVEN was given by Altman and Mehregan in 1971 which had the following limbs. 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 [2]. In our case, the female predilection, early age of onset, left side of involvement and histopathology lead to a diagnosis of ILVEN. However, due to the deformity, a history of winter exacerbation, presence of skin lesions outside the lines of Blaschko (over right palm and lower back), bony ankylosis and thinning of the middle phalanx, which represents a late feature of psoriatic oligoarthritis [3], nevoid psoriasis with arthritis could not be ruled out.
Although described as separate entities, recently, it has been proposed that ILVEN and nevoid psoriasis may co-exist in the same patient [4]. Psoriasis developing on an epidermal nevus has been described, where the subjects present with typical psoriatic lesions in other parts of the body [5]. It is possible that there was an overlap of the above two conditions in this case. ILVEN has been reported to be associated with a few musculoskeletal abnormalities like supernumerary digits, congenital bony anomalies of the ipsilateral extremities, congenital dislocation of the ipsilateral hip [6], contractures, dactylitis and bony ankylosis [7].
However, our patient had an acquired deformity of the hand with concomitant features of arthropathy. The association of arthritis with ILVEN has been described recently in a few case reports [8,9]. We hypothesize that this deformity was due to associated psoriatic arthritis secondary to the co-existent naevoid psoriasis. There have been reports where arthritis associated with ILVEN has responded to methotrexate without affecting the skin lesions. It has been recommended that ILVEN-associated arthritis should be treated on the lines of psoriatic arthritis. This association has to be kept in mind while treating a case of ILVEN as early diagnosis and proper management may help in preventing deformities.
References
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