Retrospective evaluation of 28 cases of inflammatory linear verrucous epidermal nevus

Background and Design: Inflammatory linear verrucous epidermal nevus (ILVEN) is a rare, chronic dermatosis characterized by linear erythematous scaly plaques following Blaschko’s lines. This study aims to comprehensively evaluate ILVEN cases, focusing on demographic, clinical, and histopathological features to improve understanding of its presentation and management.
Materials and Methods: This retrospective cross-sectional study included 28 patients diagnosed at our center with clinically and histopathologically confirmed ILVEN between 2011 and 2022. Data on patient demographics, age at lesion onset, duration before diagnosis, lesion location, histopathological findings, and treatment approaches were collected from hospital records and supplemented by patient interviews.
Results: Our study had a male predominance (63.4%), with a median age of lesion onset at 7.5 years and a median age of 14 years, indicating a prolonged delay in diagnosis. The mean duration from lesion onset to diagnosis was 9.86 years. Lesions were most frequently located on the lower extremities, following a Blaschkoid distribution, with 73.3% of patients reporting pruritus. Key histopathological findings included orthokeratosis with hypergranulosis (60.7%), parakeratosis overlying hypogranulosis (46.4%), and psoriasiform epidermal hyperplasia (46.4%). Topical corticosteroids were the primary treatment modality, while spontaneous regression was observed in 14.3% of untreated cases.
Conclusion: This study highlights the clinical and histopathological features of ILVEN, emphasizing the importance of early recognition in reducing diagnostic delays. Further research is warranted to enhance understanding of ILVEN’s clinical course and to optimize diagnostic and therapeutic strategies.

Enflamatuvar lineer verrüköz epidermal nevüs: 28 olgunun retrospektif deðerlendirmesi

Amaç: Enflamatuvar lineer verrüköz epidermal nevüs (ILVEN), Blaschko çizgilerini izleyen lineer eritematöz, skuamlý plaklarla karakterize nadir ve kronik bir dermatozdur. Bu çalýþma, ILVEN’in sunumu ve yönetimi hakkýnda daha fazla bilgi saðlamak amacýyla olgularý demografik, klinik ve histopatolojik özellikler açýsýndan kapsamlý bir þekilde deðerlendirmeyi amaçlamaktadýr.
Gereç ve Yöntem: Bu retrospektif kesitsel çalýþmaya, 2011-2022 yýllarý arasýnda merkezimizde klinik ve histopatolojik olarak ILVEN tanýsý doðrulanmýþ 28 hasta dahil edilmiþtir. Hastalarýn demografik verileri, lezyon baþlangýç yaþý, tanýya kadar geçen süre, lezyon yerleþimi, histopatolojik bulgular ve tedavi yaklaþýmlarýna iliþkin veriler hastane kayýtlarýndan toplanmýþ ve hasta görüþmeleri ile desteklenmiþtir.
Bulgular: Çalýþmamýzda erkek hastalar çoðunluktaydý (%63,4) ve lezyon baþlangýç yaþý ortancasý 7,5 yýl, taný yaþý ortancasý 14 yýl olarak bulunmuþ olup, tanýda belirgin bir gecikme olduðunu göstermektedir. Lezyon baþlangýcýndan tanýya kadar geçen ortalama süre 9,86 yýl olarak saptanmýþtýr. Lezyonlar en sýk alt ekstremitelerde, Blaschkoid daðýlýmda bulunmuþ ve hastalarýn %73,3’ü kaþýntý bildirmiþtir. Ana histopatolojik bulgular hipergranülozis ile ortokeratoz (%60,7), hipogranülozis üzerinde parakeratoz (%46,4) ve psoriasiform epidermal hiperplazi (%46,4) þeklindedir. Ana tedavi yöntemi topikal kortikosteroidler olup, tedavi almayan hastalarýn %14,3'ünde spontan gerileme gözlenmiþtir.
Sonuç: Bu çalýþma, ILVEN’in klinik ve histopatolojik özelliklerini vurgulayarak erken tanýnýn taný gecikmelerini azaltmadaki önemine dikkat çekmektedir. ILVEN’in klinik seyrini daha iyi anlamak ve taný ile tedavi stratejilerini optimize etmek için daha fazla araþtýrmaya ihtiyaç vardýr.