Epidermal nevus syndrome associated with ocular symblepharon and gum hypertrophy – A rare variant.

Epidermal nevi are congenital hamartomas of embryonal ectodermal origin classified on the basis of their main component. The component may be sebaceous, apocrine, eccrine, follicular or keratinocytic. An estimated 1/3 of individuals with epidermal nevi have involvement of other organ systems; hence this condition is considered to be epidermal nevus syndrome. Solomon defined epidermal nevus syndrome as a sporadic neuro-cutaneous linkage of congenital ectodermal defects in the skin, brain, eyes and skeleton, now also referred to as Solomon syndrome. The syndromes are uncommon. Mortality and morbidity are associated with systemic anomalies.

A Case of Diffuse Epidermal Nevi in the Neonate

Epidermal nevi are benign congenital hamartomas derived from the embryonal ectoderm. They may also be associated with anomalies of musculoskeletal, nervous, ocular, and rarely cardiovascular and urogenital system in an epidermal nevus syndrome. To find out these abnormalities, careful history taking, thorough physical evaluation, and long-term follow-up are advisable in a patient with wide spread epidermal nevus. We report a case of diffuse epidermal nevi involving the scalp, face, trunk, and limbs in a newborn.

Systematized Epidermal Nevi Associated with Congenital Alopecia Universalis and Onychodystrophy

We report a case of a 25-year-old woman with systematized epidermal nevi associated with alopecia universalis and onychodystrophy. The histopathologic examinations by light and electron microscopies of the epidermis of the trunk and scalp lesions showed epidermal nevus with characteristic features of epidermolytic hyperkeratosis. This is a rare case of systematized epidermal nevi associated with congenital alopecia universalis and onychodystrophy. Though there is no apparent gross skin lesion on the hair bearing area, the development of epidermal nevi can affect the hair and nail units which are derived from the common epidermal germinative cells.

Epidermal nevus: a clinical and histopathological study / 대한피부과학회지

We reviewed 48 skin bicpsies obtained from 48 patients with epidermal nevus during a 10 year period from January 1981 to December 1990. The results are summarized as follows : 1) Of our 48 patients, 23 were male and 25 were female, and male to female ratio was 1 to 1.1. 2) The average of the patients was 19.8 years at the time of diagnois. 3) Lesions were present at birth in 45.7 percent of patients, and most of the remainder de veloped in childhood. The mean age of onset was 7.7 years. 4) The clinical varieties of the nevi in 48 patients were classified according to caicegories of nevus verrucosus, including systematized epidermal nevus, nevu, unius lateris, ichthyosis hystrix, epidermal nevus syndrome and inflammatory linear veirucous epidermal nevus. The most common type was nevus verrucosus(88.6%). 5) A large variety of histopathologic changes can be seen in epidermal nevi. The most common histopathologic .ype was nonspecific(31.3%), follows by common(12.5%), epidermolytic hyperkertosis(12.5%), and acanthosis nigricans(10.4%). 6) The most commonly affected area was head and neck(48.5%) followed by trunk(28.6%), upper limb(11.4%) and lower limb(2.9%).

Etretinate Therapy of Epidermal Nevi

Two cases of epidermal nevi treated with systemic etretinate are presented. Case 1, a 21-year-old female, had been suffered from brownish verrucous papules and plaques on the left inguinal area, thigh, lower leg and dorsum of the foot since her first month of life; and case 2, a 4-year-old boy, had been suffered from brownish verrucous papular plaques on the left chest, axilla and back since birth. In both cases, there was no family history of similar disease. On histopathological examination, case 1 showed the findings of epidermolytic. hyperkeratosis but case 2 did not, with the exception of clumping of keratohyaline granules. In the treatment with systemic etretinate, we observed marked clinical improvement in case 1, but not in case 2. Any particular side effects of etretinate were not observed during treatment. We consider that etretinate therapy is useful in treating epidermal nevus with epidermolytic hyperkeratosis histopathologically.