¿Cutis verticis gyrata secundaria en un paciente con esclerosis tuberosa? / Secondary cutis verticis gyrata in a patient with tuberous sclerosis?

No disponible

Porokeratotic eccrine ostial and dermal duct nevus - revisited.

We hereby report a rare case of a 14-year-old girl presenting with asymptomatic pitted papules over the flexor aspect of her right 4th and 5th digits. This was histopathologically proven to be porokeratotic eccrine ostial and dermal duct nevus (PEODDN).

Hemimegalencephalic variant of epidermal nevus syndrome: case report and literature review.

The epidermal nevus syndrome (ENS) is an uncommon neurocutaneous disorder in which epidermal nevi are found in association with congenital abnormalities of the brain, eye, and/or skeleton. The association of epidermal nevi and neurologic abnormalities was comprehensively described by Schimmelpenning in 1957. Pavone et al. (1991) identified a homogeneous variant of ENS with hemimegalencephaly, gyral malformation, mental retardation, seizures and facial hemihypertrophy. A 13-year-old boy with the neurologic variant of ENS with hemimegalencephaly, facial asymmetry, febrile seizures and mental retardation is reported. Additionally, we performed a literature review using the search terms "epidermal nevus syndrome" and "hemimegalencephaly", including secondary sources of data such as reference lists of articles reviewed. We found 57 previously reported cases with the hemimegalencephalic variant of epidermal nevus syndrome, in which the most frequent associated features are severe epilepsy, in about half of cases with neonatal onset, mental retardation/developmental delay, ocular/visual involvement, and facial abnormalities.

Porokeratotic eccrine ostial and dermal duct nevus.

Porokeratotic eccrine ostial and dermal duct nevus (PEODDN) is a rare variant of porokeratosis with characteristic histological feature of cornoid lamella involving the acrosyringium. We report a classic case of a 20-year-old male, who clinically presented to us with keratotic papules and plaque with pits, few having comedo like plugs, on right palm and sole since 1 year of age. A punch biopsy from palm was diagnostic as well as confirmatory showing cornoid lamella involving an eccrine duct which is the characteristic histopathological feature of PEODDN.

Letter: Cerebriform intradermal nevus presenting as secondary cutis verticis gyrata.

Cutis verticis gyrata is a rare skin condition characterized by ridges and furrows resembling the surface of the brain. It can be considered as a manifestation of a variety of diverse causes such as cerebriform intradermal nevus. We report a 48-year-old man with cerebriform and soft folds on the left parietal and temporal areas. Histology showed solitary or clusters of nevus cells in the dermis. The diagnosis of cerebriform intradermal nevus was confirmed.

Epidermal nevus syndrome and intraspinal hemorrhage.

The epidermal nevus syndrome (ENS) is a rare neurocutaneous disease characterized by extensive epidermal nevi and a wide variety of abnormalities involving brain, eyes, and skeleton. Neurological symptoms in ENS include seizures, paresis, and mental retardation and are usually ascribed to hemimegalencephaly and various migration disorders. It was suggested that in some patients neurological symptoms might be secondary to vascular abnormalities. We report a case of a patient with diagnosed ENS without any primary CNS lesions, who developed paraplegia resulting from spinal cord hemorrhage. The patient presented many vascular and skeletal anomalies.

Linear congenital Becker nevus.

Becker nevus is an acquired disorder that usually manifests in late childhood or adolescence as a hyperpigmented hypertrichotic patch usually located on the upper trunk or proximal upper extremities. Only a few cases of congenital and familial Becker nevus have been described. Although the lesions may have various shapes, they consistently have a geographic or blocklike configuration in an irregular fashion; a linear pattern has rarely been reported. We describe a case of linear congenial Becker nevus following Blaschko line that appeared at birth on the right shoulder, with hypertrichosis developing 4 years later.

Elevated serum antimelanocyte antibody level in cerebriform intradermal nevus with vitiligo.

We describe a rare, but typical case of cerebriform intradermal nevus associated with vitiligo. A 45-year-old man had a patch of alopecia over his vertex scalp for 15 years. The microscopic findings of the biopsy revealed a typical deep-seated intradermal nevus and neuroid differentiation with a few pigments. Three hypopigmented patches developed on the forehead, cheek and index finger five years after the scalp lesion, with loss of both melanocytes and melanins. In addition, no dopa reactions were present. Compared to normal controls, the serum anti-melanocyte antibody level in the patient was elevated as determined by cellular enzyme-linked immunosorbent assay (cellular ELISA). This is the first reported case with elevation of serum antimelanocyte antibody level of cerebriform intradermal nevus with vitiligo. This antibody's presence may be related to the occurrence of the vitiligo in patient with cerebriform intradermal nevus.

Cutis verticis gyrata secondary to a cerebriform intradermal nevus.

We report the case of a 30-year-old black man with a large mass consisting of longitudinal parallel ridges and furrows on the left parietal region. A small, single, hyperpigmented macule was present at birth and gradually grew and extended over the years. Cutis verticis gyrata was suspected, and an investigation was performed to discharge the possibility of pachydermoperiostosis. However, results of biopsies obtained from 3 different lesional areas showed the same histopathologic features--deep-seated hair follicles and clusters of nevus cells concentrated in the dermis. Cerebriform intradermal nevus is a rare cause of cutis verticis gyrata. Early diagnosis is extremely important to prevent the development of malignant melanoma.

Seborrheic keratosis with compound nevus, junctional nevus and basal cell carcinoma in the same lesion.

Seborrheic keratosis can be associated with different neoplasms such as basal cell carcinomas, squamous cell carcinomas and melanomas. We describe an unusual case of a man who presented with a brown plaque on his back. The clinical diagnosis was melanoma. Histopathologic examination of the lesion revealed four neoplasms: a compound nevus, a junctional nevus, a superficial basal cell carcinoma and a seborrheic keratosis. Although this association most likely represents a chance phenomenon, we discuss the possibility that the seborrheic keratosis developed from the nevus, and that subsequently the junctional nevus and the basal cell carcinoma developed from the seborrheic keratosis.