1/130. Juvenile hyaline fibromatosis: clinical heterogeneity in three patients.BACKGROUND: Systemic hyalinoses are genetic generalized fibromatoses characterized by an accumulation of hyalin in the dermis. Two distinctive syndromes are recognized in the literature: infantile systemic hyalinosis (ISH) and juvenile hyaline fibromatosis (JHF). ISH and JHF are sometimes difficult to separate since they show significant overlap. OBSERVATIONS: We report on 3 children from two unrelated families suffering from JHF. The first child is severely handicapped by joint contracture, massive hyperplasia of the gingivae, diffuse skin papules and subcutaneous nodules occupying the scalp, face, perianal area, palms, soles and chest. At the same age, the second child only shows pearly skin papules on the face, groin and perianal area and gingival hyperplasia without joint stiffness or any other subjective complaint. The third patient, a brother of the second child, developed mild skin abnormalities by the end of the first year. The occurrence in siblings and consanguinity in the second family suggests autosomal recessive inheritance. Histological skin examination in the 3 cases showed hyaline deposition in the dermis and abnormal ultrastructure of fibroblasts. Biochemical findings showed mucopolysaccharide abnormalities in both families. CONCLUSION: Our patients do not only illustrate the different expressions of JHF but also show some overlap with ISH, suggesting a common cause for both disorders. Genetic studies will finally answer this question.- - - - - - - - - - ranking = 1keywords = palm (Clic here for more details about this article) |
2/130. 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) |
3/130. Cutaneous sclerosing perineurioma with cryptic NF2 gene deletion.Sclerosing perineurioma is a recently described variant of perineurioma that characteristically occurs in the fingers and palms of young adults. We report a cutaneous sclerosing perineurioma with preservation of the axons and schwann cells in the center of the whorls of perineurial cells, a feature that previously was reported to be typically absent in these lesions. Additionally, clonal chromosomal abnormalities of chromosome 10 and a cryptic deletion of the 5'BCR and NF2 loci on chromosome 22 were present. These findings further support the hypothesis that a gene on chromosome 22 may play a role in the pathogenesis of perineurioma. The NF2 gene is a logical candidate because of its involvement in other nerve sheath tumors.- - - - - - - - - - ranking = 1keywords = palm (Clic here for more details about this article) |
4/130. arsenic-related bowen's disease, palmar keratosis, and skin cancer.Chronic arsenical intoxication can still be found in environmental and industrial settings. Symptoms of chronic arsenic intoxication include general pigmentation or focal "raindrop" pigmentation of the skin and the appearance of hyperkeratosis of the palms of the hands and soles of the feet. In addition to arsenic-related skin diseases including keratosis, bowen's disease, basal-cell-carcinoma, and squamous-cell carcinoma, there is also an increased risk of some internal malignancies. arsenic-related diseases are common in areas of the world where the drinking water has a high arsenic content. In this paper, we describe a 35-year-old male patient who had arsenic-related keratosis, squamous-cell carcinoma in the palmar area of his left hand, and bowen's disease on his left thigh. The patient worked in a borax mine for 15 years, so he was exposed to arsenic in drinking water, airborne arsenic in his workplace, and had direct contact. The patient was treated for 11 months for arsenic-related keratosis until an axillary lymph node metastasis occurred; the lesion was excised and diagnosed to be malignant. bowen's disease was detected when the patient was being treated for cancer. No other malignancy was found. The patient is still receiving regular follow-up care.- - - - - - - - - - ranking = 6keywords = palm (Clic here for more details about this article) |
5/130. Multiple hereditary infundibulocystic basal cell carcinomas: a genodermatosis different from nevoid basal cell carcinoma syndrome.BACKGROUND: Infundibulocystic basal cell carcinoma is a recently described distinctive clinicopathologic variant of basal cell carcinoma. Histopathologic differential diagnosis among infundibulocystic basal cell carcinoma, trichoepithelioma, and basaloid follicular hamartoma has generated controversy in the literature. OBSERVATIONS: Members of 2 families with multiple infundibulocystic basal cell carcinomas are described. Each patient showed multiple papular lesions, mostly located on the face. No patient showed palmar pits or jaw cysts. Forty-two cutaneous lesions from 5 patients were studied histopathologically. Thirty-nine lesions were infundibulocystic basal cell carcinomas. This clinicopathologic variant of basal cell carcinoma consists of a relatively well-circumscribed basaloid neoplasm composed of buds and cords of neoplastic cells arranged in anastomosing fashion and with scant stroma. Some of the neoplastic cords contain tiny infundibular cysts filled by cornified cells with abundant melanin. Linkage analysis in family 2 was performed using polymorphic markers (D9S196, D9S280, D9S287, and D9S180), and the affected members shared the same haplotype. loss of heterozygosity analysis was performed in 2 affected members of this family from whom tumoral dna was available, and although these individuals were constitutively heterozygous for D9S196, they did not show loss of heterozygosity for this marker in their neoplasms. CONCLUSIONS: Multiple hereditary infundibulocystic basal cell carcinomas represent a distinctive genodermatosis different from multiple hereditary trichoepitheliomas and nevoid basal cell carcinoma syndrome. We propose clinical and histopathologic criteria to distinguish infundibulocystic basal cell carcinoma from trichoepithelioma, basaloid follicular hamartoma, and folliculocentric basaloid proliferation.- - - - - - - - - - ranking = 1keywords = palm (Clic here for more details about this article) |
6/130. Palmar basal cell carcinoma: case report and literature review.BACKGROUND: Although basal cell carcinoma (BCC) is the most common tumor of the skin, its occurrence on the palm is very rare. Only eight cases not associated with the basal cell nevus syndrome having been reported in the world literature so far. Among these eight cases, one was associated with epidermolysis bullosa dystrophica and another with previous trauma to the site of the appearance of the lesion. OBJECTIVE: The authors report a case of palmar BCC due to its rare occurrence on that site. The case is not associated with any predisposing factors such as basal cell nevus syndrome, trauma, or preexistent dermatosis. methods: An incisional biopsy was performed into the border of the palmar ulcer. The histopathologic findings of the fragment, stained by hematoxylin and eosin, were characteristic of BCC. RESULTS: Following surgical removal of the lesion, there was complete healing and the patient has presented no signs of relapse after 3 years of clinical follow-up. CONCLUSION: Palmar BCC not associated with the basal cell nevus syndrome or any other predisposing condition is very rare. Nevertheless, when facing an ulcerated lesion on the palm, BCC should be considered as a diagnostic possibility.- - - - - - - - - - ranking = 4keywords = palm (Clic here for more details about this article) |
7/130. 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) |
8/130. Spitz nevus on the palmar surface.Relatively little is known about the incidence of Spitz nevus on palmar surfaces. This report places a case study in the context of the Japanese literature regarding the occurrence of Spitz nevus on palmar surfaces. Although the proportion of palms and soles in relation to the body surface is about 5%, the incidence of the Spitz nevus was 2%. The mean age at onset was 17.8 years, and all 4 cases were women. The clinical features were a black macule or flatly elevated small modules. The size of the lesions was relatively small, extending from 3.5 mm to 8.0 mm. Although the backs of the hands and insteps have almost the same area as the palms and soles, the incidence of onset in these regions was 6.3% (13 cases). We thus concluded that Spitz nevus tends to be rare on palms and soles.- - - - - - - - - - ranking = 9keywords = palm (Clic here for more details about this article) |
9/130. Nevoid basal cell carcinoma syndrome and non-Hodgkin's lymphoma.Nevoid basal cell carcinoma syndrome (NBCCS) is a hereditary disorder with a predilection for numerous basal cell carcinomas in addition to odontogenic keratocysts, palmoplantar pitting, and skeletal malformations. NBCCS has been associated with a number of benign and malignant neoplasms. We report the first case of NBCCS in association with non-Hodgkin's lymphoma.- - - - - - - - - - ranking = 1keywords = palm (Clic here for more details about this article) |
10/130. Squamous cell carcinoma arising from lesions of porokeratosis palmaris et plantaris disseminata.We report a 63-year-old Japanese man with numerous hyperkeratotic papules of porokeratosis palmaris et plantaris disseminata (PPPD) who developed multiple squamous cell carcinomas on the lesional sites of the palms and soles. The hyperkeratotic papules, which showed tightly packed columns of parakeratotic cells in the cornified layer (cornoid lamella), lost granular layer, and dyskeratotic keratinocytes in the epidermis below the cornoid lamella histologically, had been noticed on the palms and soles from the age of 28 and 43, respectively. He has no family history of such hyperkeratotic papules. Treatment with etretinate (10-50 mg/day) was given discontinuously, and the total dose of etretinate amounted to approximately 21 g over 14 years (average: 0.07 mg/kg/day). He noticed erosions on the hyperkeratotic papules on the left sole and palm more than 9 months after cessation of treatment with etretinate. Histological findings showed numerous atypical keratinocytes in the epidermis and upper dermis with mononuclear cell infiltration seen in the upper dermis. The diagnosis of squamous cell carcinoma arising from the lesions of porokeratosis palmaris et plantaris was made. Five erosions with histologically malignant changes were removed 1 cm from the margin of the erosions. These findings suggest that etretinate may have an inhibitory action on malignant changes in PPPD.- - - - - - - - - - ranking = 9keywords = palm (Clic here for more details about this article) |
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