An inflammatory verrucous epidermal nevus concomitant with psoriasis: Treatment with adalimumab
Published Web Locationhttps://doi.org/10.5070/D37ts7s0st
An inflammatory verrucous epidermal nevus concomitant with psoriasis: Treatment with adalimumabIstanbul Medipol University, School of Medicine, Department of Dermatology, Istanbul, Turkey
Mustafa Özdemir PhD, Ali Balevi PhD, Hasan Esen PhD
Dermatology Online Journal 18 (10): 11
A 42-year-old-man presented with generalized inflammatory linear verrucous epidermal nevus with concomitant psoriasis vulgaris. Because of unresponsiveness of the conventional treatment, we considered treatment with adalimumab. Adalimumab was well tolerated and no side effects were observed. Adalimumab has a limited effect in treating ILVEN lesions. However, it was effective in treating the psoriasis and pruritus related to ILVEN. For these reasons adalimumab can be used in treatment of ILVEN concomitant with psoriasis.
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A 42-year-old man presented with generalized inflammatory linear verrucous epidermal nevus with concomitant psoriasis vulgaris (Figure 1). He had pruritic, erythematous and scaly papules and plaques in a linear pattern on his left upper extremity, scapula, and chest. The lesion on the left upper extremity had been present since 7 years of age. Histological examination of the biopsy taken from the lesion of the left upper extremity showed well-demarcated zones of parakeratosis, hypergranulosis and dense inflammatory infiltrate with interface changes (Figure 2). These findings were consistent with a diagnosis of ILVEN.
Over years, the patient was prescribed many topical and systemic therapeutic agents including moisturizers, corticosteroids, calcipotriol, PUVA, acitretin, methotrexate, and cyclosporine. Although significant improvement in the psoriatic lesions was achieved with those agents, only mild to moderate improvement was seen in the ILVEN lesions. Prior to this, only mild to moderate improvement was achieved with acitretin and a potent topical corticosteroid (clobetasol propionate) However, the pruritus associated with the ILVEN did not improve with those treatment agents.
The patient’s Psoriasis Area and Severity Index (PASI) was 12. Because of unresponsiveness to the mentioned topical and systemic treatment and unwillingness to use those treatments, the patient started treatment with adalimumab. It was administered subcutaneously 40 mg every other week starting at week 1 after the 80 mg initial dose at week 0. Follow up at 24 weeks revealed that although nearly complete clearing of all psoriasis was seen after this treatment period, ILVEN lesions did not resolve completely. Mild to moderate improvement in roughness and erythema of the lesions was achieved, but the patient’s pruritus disappeared completely (Figure 3).
The pathogenesis of ILVEN is unknown. It is regarded as a genetic dyskeratotic disease reflecting genetic mosaicism. Because of its clinical and histological similarity to linear psoriasis, shared pathogenic traits such as the central involvement of T cells may be hypothesized. Some authors have suggested that similar pathways are probably mediated by interleukins 1 and 6 or tumor necrosis factor α (TNF-α) . In a recent report, a classification for the correlation of ILVEN, linear psoriasis, and epidermal nevus was suggested by Hofer et al . Although many treatment agents for ILVEN have been reported in the literature, conflicting results about efficacy have been noted. In the treatment of ILVEN, TNF-α blockers have been reported recently with varying success rates. Successful treatment with etanercept has been reported in two cases of ILVEN, one of whom had a widespread ILVEN [3, 4]. In contrast to the two reports, ILVEN lesions did not heal with etanercept in a patient with ILVEN concomitant with psoriasis . However there are no reports assessing responses of other TNF-α blockers in the disease. Although TNF-α blockers are in the same family, they have different efficacy in psoriasis and other diseases such as rheumatoid arthritis. In the present case, adalimumab was used for treatment of ILVEN concomitant with psoriasis.
Our patient exhibited localized ILVEN with concomitant psoriasis and supports the suggestion of Hofer et al noting some correlation of ILVEN and psoriasis. We achieved mild to moderate improvement with adalimumab in the present case. The complete resolution of pruritus may have allowed the decreases in roughness and erythema in the ILVEN lesions by eliminating scratching and Koebner phenomenon. Our result suggest that adalimumab has a limited effect on the ILVEN plaques, but it had an excellent effect on the psoriasis and ILVEN-related pruritus. For these reasons adalimumab can be used in treatment of ILVEN concomitant with psoriasis.
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