1/18. Nevoid basal cell carcinoma syndrome.A case report of a young girl with nevoid basal cell carcinoma syndrome is presented. The patient showed cutaneous and skeletal findings characteristic of the syndrome. Multiple basal cell carcinomas, rib abnormalities, along with clinical evidence of frontal bossing and ocular hypertelorism were the primary features of the syndrome in this patient. It is suggested that other characteristics of the syndrome, such as jaw cysts, palmar and plantar pitting and calcification of the falx cerebri will develop as the patient grows older. Careful observation, particularly for medulloblastoma and malignant degeneration and invasiveness of basal cell carcinomas, will be an integral part of this young patient's care.- - - - - - - - - - ranking = 1keywords = palm (Clic here for more details about this article) |
2/18. 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 = 1keywords = palm (Clic here for more details about this article) |
3/18. An unusual presentation of a linear epidermal nevus.We report an unusual presentation of a linear epidermal nevus in an adolescent male. Epidermal nevi most commonly appear in infancy and early childhood. They often are found in association with other organ system anomalies. We describe a palmar linear epidermal nevus that caused impairment of the patient's use of his hand. Therapeutic management involved surgical excision of the nevus and reconstruction of the area with a full-thickness skin graft.- - - - - - - - - - ranking = 1keywords = palm (Clic here for more details about this article) |
4/18. An acantholytic dyskeratotic epidermal naevus with other features of Darier's disease on the same side of the body.Many epidermal naevi with the histology of Darier's disease have been reported. In the absence of associated features of Darier's disease, they cannot be assumed to have a common pathogenesis with it, and it has been suggested that they are better classified as acantholytic dyskeratotic epidermal naevi rather than naevoid Darier's disease. We describe a patient with such a naevus who had typical nail and palmar changes of Darier's disease on the same side of the body. We suggest that in at least some cases the naevus has the same genetic defect(s) as generalized Darier's disease, and discuss the possibility that a patient with such a naevus could occasionally transmit Darier's disease to an offspring.- - - - - - - - - - ranking = 1keywords = palm (Clic here for more details about this article) |
5/18. A case of porokeratotic eccrine ostial and dermal duct naevus of late onset.We describe the case of a 21-year-old man with an 8-year history of porokeratotic eccrine ostial and dermal duct naevus (PEODDN) of late onset. The patient had pruritic, keratotic papules on the dorsal surface of his left hand, bilaterally on his palms and multiple yellowish lesions on the plantar surface of his feet. On histopathological examination cornoid lamella-like parakeratotic columns above eccrine sweat ducts were observed. The acrosyringium was also dilated. The lesions were refractory to treatment with salicylic acid in petrolatum and acitretin for 2 months. This entity should be taken into consideration in the differential diagnosis of linear keratotic cutaneous eruptions.- - - - - - - - - - ranking = 1keywords = palm (Clic here for more details about this article) |
6/18. Eruptive nevi of the palms and soles.The sudden eruption of atypical and benign melanocytic nevi has been associated with a number of disease states and primary skin conditions. Most case reports and series of patients have linked eruptive nevi with blistering skin disease or immunosuppression. Subsets of patients in the immunosuppressed category have remarkably increased numbers of nevi on the palms and soles. We describe a case of multiple eruptive nevi of the palms and soles in association with immunosuppression, and the potential underlying mechanisms promoting such nevogenesis are explored. Although both the absolute number of nevi and presence of dysplastic nevi have been correlated with an increased relative risk of melanoma, actual risk of melanoma in patients with eruptive nevi is unknown.- - - - - - - - - - ranking = 6keywords = palm (Clic here for more details about this article) |
7/18. proteus syndrome. Ultrastructural study of linear verrucous and depigmented nevi.proteus syndrome is a rare hamartomatous disorder characterized by multifocal overgrowths that can involve any structure of the body. Clinical manifestations include macrodactyly, hemihypertrophy, subcutaneous masses, exostosis, cerebroid thickening of palms and soles, and linear skin lesions. About 50 cases have been described, but the ultrastructural features of the linear skin lesions have not been characterized. We describe the clinical, histologic, and ultrastructural findings for a 30-year-old patient who had a mild form of proteus syndrome with linear lesions characterized by a mixed pattern of hyperkeratosis and depigmentation. light microscopy of the linear nevus showed acanthosis and hyperorthokeratosis. Electron microscopy revealed extensive vacuolation at the interface between melanocytes and keratinocytes, with large aggregations of densely packed granules in the intercellular space. melanocytes showed only slight degenerative changes. An immunohistochemical study of the expression of epidermal growth factor receptors revealed no significant abnormalities.- - - - - - - - - - ranking = 1keywords = palm (Clic here for more details about this article) |
8/18. Porokeratotic eccrine ostial and dermal duct nevus.A case of porokeratotic eccrine ostial and dermal duct nevus (PEODDN) affecting all 4 extremities is described. Clinically, palmo-plantar lesions resembled those of nevus comedonicus and the remaining lesions those of linear verrucous epidermal nevus. Histologically, all lesions were identical with cornoid lamellae in the eccrine ostia and epidermal ducts.- - - - - - - - - - ranking = 1keywords = palm (Clic here for more details about this article) |
9/18. Porokeratotic eccrine ostial and dermal duct nevus.We report 2 cases of congenital porokeratotic eccrine nevus in a 5-year-old girl and a 7-year-old boy. The clinical manifestations were those of nevus comedonicus palmaris. The histologic picture consisted of comedo-like dilatations and cornoid lamellae involving the eccrine ostia and ducts. One of the cases showed anomalies in the dermal eccrine ducts. We believe that these lesions are eccrine hamartomas unrelated to porokeratosis. We review similar cases from the literature and discuss the differential diagnosis.- - - - - - - - - - ranking = 1keywords = palm (Clic here for more details about this article) |
10/18. Porokeratotic eccrine ostial and dermal duct nevus.Comedo-like lesions of the palm, present since birth, are studied electronmicroscopically and their eccrine nature demonstrated. We suggest their inclusion under the name porokeratotic eccrine-ostial and dermal duct nevus previously proposed by Abell and Read.- - - - - - - - - - ranking = 1keywords = palm (Clic here for more details about this article) |
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