1/60. Epidermal naevus syndrome and hypophosphataemic rickets: description of a patient with central nervous system anomalies and review of the literature.The epidermal naevus syndrome (ENS) is a rare dermatological condition consisting of congenital epidermal nevi associated with anomalies in the central nervous system, bones, eyes, hear or genito-urinary system. We report a new case of ENS associated with hypophosphataemic rickets. The girl was born with a mixed-type epidermal naevus and skeletal anomalies. Hypophosphataemic rickets was diagnosed at the age of 2.5 years. At 14 years of age. MRI of the head demonstrated right brain hypotrophy, a left temporal arachnoid cyst and asymmetric lateral ventricles. We reviewed the literature and found 13 reported cases of ENS associated with hypophosphataemic rickets. Conclusion We report a further patient with epidermal naevus syndrome and hypophosphataemic rickets, followed from birth to the age of 15 years, who had structural central nervous system anomalies with normal intellectual functioning. A comprehensive neurological work up is recommended in patients with epidermal naevus syndrome.- - - - - - - - - - ranking = 1keywords = naevus (Clic here for more details about this article) |
2/60. Epidermal naevus syndrome.A case of the epidermal naevus syndrome is presented. Huge enlargement of both lower limbs was the presenting symptoms together with an extensive veavus unius lateris on the trunk.- - - - - - - - - - ranking = 0.625keywords = naevus (Clic here for more details about this article) |
3/60. Unusual giant comedo naevus.Comedo naevi are usually well circumscribed, and although extensive cases have been reported individual lesions crossing the midline are rare. Associated neurological, skeletal and ophthalmological abnormalities are also recognized. thus, the patient now reported is unusual in that she had an extensive systematized comedo naevus with crossing of the midline but no associated abnormalities. Topical tretinoin was helpful in improving the texture and appearance of the comedones, and various larger lesions responded to curettage.- - - - - - - - - - ranking = 0.625keywords = naevus (Clic here for more details about this article) |
4/60. Systematized porokeratotic eccrine and hair follicle naevus: report of a case and review of the literature.We report a unique case of a congenital keratinocytic naevus associated with severe alopecia, onychodysplasia and palmoplantar involvement in a 13-year-old girl. The lesions, consisting of scaly, spinous and verrucous papules and plaques, mainly followed Blaschko's lines and have remained unchanged since birth. The predominant histopathological picture was that of a column of parakeratosis overlying the eccrine ostia and hair follicles. This is the first case of a systematized keratinocytic naevus characterized by histopathology of eccrine and hair follicle porokeratosis and a widespread bilateral involvement. This may be a distinct entity to be included in the differential diagnosis of linear, hyperkeratotic dermatoses. We suggest its classification as systematized porokeratotic eccrine and hair follicle naevus.- - - - - - - - - - ranking = 0.875keywords = naevus (Clic here for more details about this article) |
5/60. A case of combined nevus: compound nevus and spindle cell Spitz nevus.Combined nevi consisting of a Spitz nevus and an acquired nevus are unusual, and, to our knowledge, the combination of a spindle cell Spitz nevus and an overlying compound nevus has not been previously reported. We report a 17-year-old girl with a nodule on the left anterior lower extremity. The nodule was asymptomatic, firm, brown, symmetrical, dome-shaped, 8 mm in diameter, and not found with ulceration. Histological findings showed proliferation of spindle-shaped cells with an overlying compound nevus. The spindle-shaped cells were large, non-pigmented, uniform in size and shape, with rare mitoses and without nuclear atypia, and arranged in a storiform pattern in thick collagen bundles. They stained positively for S-100 and negatively for HMB-45. The lesion was considered to be a new type of combined nevus consisting of a spindle cell Spitz nevus and an overlying compound nevus.- - - - - - - - - - ranking = 0.72913855624895keywords = spindle (Clic here for more details about this article) |
6/60. Epidermal naevus with Darier's disease-like changes in a patient with Gardner's syndrome.We describe the unique presentation of a linear epidermal nevus with histologic features of Darier's disease occurring in a patient with Gardner's syndrome. classification of localized forms of Darier's disease as an epidermal nevus or as a genetic mosaicism remains controversial. The association of this disorder with Gardner's syndrome has not been described in the literature before.- - - - - - - - - - ranking = 0.5keywords = naevus (Clic here for more details about this article) |
7/60. Malignant transformation in congenital sebaceous naevi in childhood.Sebaceous naevi are uncommon congenital skin lesions with a well-recognised potential for neoplastic change. They should be considered premalignant lesions as malignant degeneration, most commonly basal cell carcinoma and squamous cell carcinoma, occurs with a lifetime risk of between 5% and 22%. This incidence is equal to that of actinic keratosis and exceeds that of oral leukoplakia. Such change, however, is rare before puberty. Basal cell carcinoma may develop in children with naevoid basal cell carcinoma syndrome, xeroderma pigmentosum and rarely de novo but sebaceous naevus is the only solitary lesion in childhood associated with the development of basal cell carcinoma. We present two cases of malignant transformation in a congenital sebaceous naevus occurring in childhood and review the literature and discuss the evidence upon which to base management guidelines.- - - - - - - - - - ranking = 0.25keywords = naevus (Clic here for more details about this article) |
8/60. Naevus anaemicus with teleangiectatic vessels.We describe a 20-year-old man with naevus anaemicus on the chest where, after dermabrasion of the epidermis, enlarged teleangiectatic dark-red vessels were seen within the previously pale area. They were clearly different from those seen on dermabrasion at this site in normal skin and in patients with vitiligo where the area is lighter red with only small punctual bleedings from arterial capillaries. The naevus anaemicus and a port-wine stain (naevus flammeus) in the same location is a phenomenon of vascular twin spotting, which was revealed when the epidermis was removed. The area was transplanted with thin epidermal grafts and healed within 2 weeks. One year later the naevus looked the same as before grafting. Much thicker grafts than those used by us will be needed, but they are not cosmetically acceptable.- - - - - - - - - - ranking = 0.5keywords = naevus (Clic here for more details about this article) |
9/60. review of neurological manifestations in 196 patients with sebaceous naevi.The incidence of neurological abnormalities in patients with sebaceous naevi may not be as high as has been previously accepted. One hundred and ninety-six patients with epidermal naevi of the sebaceous type were examined for clinical neurological abnormalities. Ninety-three per cent of these patients were neurologically normal on examination. Extensive naevi were present in 21% (3/14) of patients with neurological abnormalities and in 5% (9/182) of those without. A centrofacial location of the naevus was found in 21% (3/14) of patients with abnormalities and in 2% (4/182) of neurologically normal patients. A surprising finding was the low yield of abnormalities from neuroimaging in the patients with clinical neurological abnormalities. Imaging was performed in eight of the 14 and was normal in six patients. Despite the lower than expected rate of associated abnormalities and the low yield of abnormal findings from imaging, it is strongly recommended that all patients with sebaceous naevi have a detailed neurological assessment and that imaging be performed on all those in whom clinical abnormalities are demonstrated, as well as on those patients with large naevi involving the centrofacial area.- - - - - - - - - - ranking = 0.125keywords = naevus (Clic here for more details about this article) |
10/60. Uncommon vascular naevi associated with focal acantholytic dyskeratosis.Cutis marmorata telangiectatica congenita and vascular twin naevi are rare vascular anomalies in which focal acantholytic dyskeratosis is usually not observed. We describe a 44-year-old-man who presented for evaluation of skin lesions that had been present since birth. physical examination revealed anaemic macules adjacent to a naevus telangiectaticus on the chest. Naevus anaemicus was also seen on the shoulders, arms, and left leg. There was bluish-reddish reticulate marking of the skin and cutaneous atrophy. Shortening and hypoplasia of the left leg was observed. Histologic examination of two biopsy specimens revealed focal acantholytic dyskeratosis. In vivo confocal laser scanning microscopy showed dilated capillaries and vessels of the upper dermal plexus in the telangiectatic and decreased capillary blood flow in the anaemic skin sites. The findings were consistent with a diagnosis of cutis marmorata telangiectatica congenita, vascular twin naevi, and incidental focal acantholytic dyskeratosis. The particularities of the present case are the following: firstly, the association of two rare vascular anomalies to which the genetic concept of mosaicism can be applied; secondly, the occurrence of incidental focal acantholytic dyskeratosis in sites of vascular naevi.- - - - - - - - - - ranking = 0.125keywords = naevus (Clic here for more details about this article) |
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