ABSTRACT
Inflammatory linear verrucous epidermal nevus (ILVEN) is an uncommon variant of verrucous epidermal nevus, clinically characterized by erythematous and hyperkeratotic verrucous papules with a typical linear arrangement. Most reported cases present as solitary lesions, but systematized ILVEN is rare. Epidermal nevus syndrome is a congenital disorder in which epidermal nevi are associated with abnormalities in other organ systems. However, ILVEN has been reported rarely in association with such abnormalities, including those of the skeleton. Here we report a rare case of systematized ILVEN presenting with concomitant mandibular hypoplasia.
Subject(s)
Congenital, Hereditary, and Neonatal Diseases and Abnormalities , Nevus , Nevus, Sebaceous of Jadassohn , SkeletonABSTRACT
Inflammatory linear verrucous epidermal nevi (ILVEN) were first described in the literature, in 1971, by Altman and Mehregan. Most cases were reported as solitary lesions. In contrast, systematized ILVEN, involving wide areas of the integument, has only rarely been reported. A variety of treatment modalities has been reported, ranging from topical medications, such as potent corticosteroids or tretinoin 0.1% to variable procedures, including CO2 and pulsed dye laser, cryotherapy, and surgical excision. However, multifocal skin lesion is more difficult to treat. An 8-year old boy was presented with an extensive thick scaly plaques and patches, affecting the trunk and the four extremities sparing face. It developed when he was 3 years old, and he complained pruritus. On physical examination, linear, or whirl-like scaly plaques were seen, along with Blaschko lines. Pathologic finding was in accordance to ILVEN. We treated him with topical calcipotriol, tacrolimus and systemic acitretin. After 8 months, the lesion and symptoms improved.
Subject(s)
Acitretin , Adrenal Cortex Hormones , Calcitriol , Cryotherapy , Extremities , Lasers, Dye , Nevus , Nevus, Sebaceous of Jadassohn , Physical Examination , Pruritus , Skin , Tacrolimus , TretinoinABSTRACT
El NEVIL es la variedad inflamatoria del nevo epidérmico; es un tumor benigno hamartomatoso, que se distribuye siguiendo las líneas de Blaschko. Estas líneas representarían mosaicismos cutáneos del desarrollo embriológico. El NEVIL suele aparecer en la infancia. Son generalmente unilaterales y en ocasiones muy pruriginosos. El diagnóstico diferencial es frecuentemente dificultoso. Presentamos una paciente en la cual la manifestación de esta patología se produce en forma tardía, siendo esto poco frecuente. El tratamiento es un desafío, se han probado múltiples modalidades terapéuticas, con resultados en ocasiones desalentadores. La cirugía, puede utilizarse en lesiones de pequeño tamaño. Los tratamientos con luz láser podrían ser de utilidad.
The ILVEN is the inflammatory variety of epidermal nevi, benign hamartomatous tumor that distributed following Blaschko lines. These lines represent cutaneous mosaicism in embryologic development. The ILVEN usually appear in the infancy. Generally unilateral and occasionally is very pruriginous. The differential diagnosis is often difficult. We present a patient in which the manifestation of her disease is produced in late onset, being this infrequent. Management is a challenge, multiple therapeutic modalities have been tested, with discouraging results. Surgery can be utilized in small size lesions. Laser therapy could be of utility.
Subject(s)
Humans , Female , Adult , Nevus/diagnosis , Nevus/pathology , Diagnosis, Differential , Hamartoma/pathology , Nevus/therapyABSTRACT
Inflammatory linear verrucous epidermal nevus(ILVEN) is a rare variant of epidermal nevus characterized by an early age of onset, an unilateral pruritic inflammatory psoriasiform lesion, and relative refractoriness to treatment. It has been reported that ILVEN mainly occur in the lower extremities but develop in the upper extremities, neck and trunk. Herein, we present a case of ILVEN occurring in the left temporal scalp of a 32-year-old woman which, in our review of literature, has not been reported as a development site.
Subject(s)
Adult , Female , Humans , Age of Onset , Lower Extremity , Neck , Nevus , Nevus, Sebaceous of Jadassohn , Scalp , Upper ExtremityABSTRACT
Inflammatory linear verrucous epidermal nevus(ILVEN) is a relatively rare skin disorder, characterized by chronic inflammatory psoriasiform pattern. This chronic skin disorder is generally believed to be resistant to various treatments, although some authors have reported success with 13-cis-retinoic acid and 585 nm pulsed laser therapy. We experienced a 15-month aged female ILVEN patient who has been suffered from linear erythematous verrucous pathes mainly located on left inguinal area, hand, and foot with persistent itching sense. This skin lesion have been developed at birth, and more spread out toward right extremity after 1 year old age . We initially treated this skin lesion with topical corticosteroids and oral anti-histamines, but this treatment was not successful. So we retreated this patient with etretinate(oral retinoid) and topical 13-cis-retinoic acids, we found that therapeutic responses to these applications were more remarkable. Now, we closely observe this patient's clinical course further more.
Subject(s)
Female , Humans , Adrenal Cortex Hormones , Etretinate , Extremities , Foot , Hand , Isotretinoin , Laser Therapy , Nevus, Sebaceous of Jadassohn , Parturition , Pruritus , SkinABSTRACT
A 5- year old female patient visited our clinic due to linear erythernatous and verrucous patches on the left extremities of 3 years duration, She was born with a small erythematous macule on the lateral side of left little finger. This macule, over 2 years, developed into pruritic, linear, and verrucous patches stretching from left little finger to lefr, axillai y area, and also erythematous, linear patches on the left thigh. On follow-up visit on October 1986, she has generalized psoriatic eruption with aggravation of originnl skin lesions. Digriosis was based on the clinical features and histopathnlogic findings of the skin lesions.