1/49. Linear melanonychia due to subungual keratosis of the nail bed: a report of two cases.Longitudinal melanonychia displaying features of keratinized acanthoma is described in two patients. In both cases, a pigmented band consisting of a subungual keratinized epithelial ridge originated in the nail bed. We have been able to show, using the clinical and histological findings, that the origin of the pigment is linked to its synthesis within the acanthoma of the nail bed. These lesions are reminiscent of pigmented seborrhoeic keratosis. The horny cysts are replaced, because of the special physiological longitudinal arrangement of the ridges in the nail bed, by a single, prominent, longitudinal, keratinized and pigmented mass.- - - - - - - - - - ranking = 1keywords = ridge (Clic here for more details about this article) |
2/49. Hypomelanosis of ito and a 'mirror image' whole chromosome duplication resulting in trisomy 14 mosaicism.We describe a female infant with multiple congenital anomalies including unusual hyperpigmentation, tetralogy of fallot, absent corpus callosum and wide prominent nasal bridge. The infant was initially seen for genetic consultation on day one after birth. Chromosome analysis from cultured lymphocytes showed a normal 46,XX karyotype. However, cultured skin fibroblasts showed mosaicism with 46,XX,add(14)(q32).ish psu dic dup(14)(q32p13)(wcp14 )/46,XX complements. A review of the published report with chromosome mosaicism and hypomelanosis of Ito (HMI) is included. We suggest that the trisomy 14 mosaicism seen in fibroblast cultures has importance in the expression of pigmentation dysplasias in this patient. Pigmentary anomaly may be due to loss or gain of specific genes that influence pigmentation located on the long arm of chromosome 14 in this patient.- - - - - - - - - - ranking = 0.5keywords = ridge (Clic here for more details about this article) |
3/49. Two cases of vulval pigmented extramammary Paget's disease: histochemical and immunohistochemical studies.We describe two Japanese female patients with pigmented extramammary Paget's disease (EMPD); one patient had a dark brown plaque and the other had a reddish patch with a pigmented area, both affecting the vulval region. Histochemical and immunohistochemical examinations confirmed EMPD with melanocyte colonization; plump tumour cells with a large nucleus and pale cytoplasm that were positive for CAM 5.2 and CEA proliferated singly or in nests in the epidermis, and scattered among the tumour cells were many dendritic cells with a large amount of melanin that were positive for HMB-45 and S-100 protein. Fontana-Masson (FM) stain showed many positive cells with well-developed dendritic processes within and around tumour nests. Histochemical and immunohistochemical studies of non-pigmented EMPD cases on the same region showed that HMB-45 positive cells were sparse or not detected at all, and that also FM staining-positive cells were decreased or not detected, and their dendritic processes were poorly formed. The present study suggests that there might be heterogeneity in EMPD in terms of relationships between Paget's cells and melanocytes.- - - - - - - - - - ranking = 0.0093580866786389keywords = process (Clic here for more details about this article) |
4/49. Dowling-Degos disease--a heat aggravated variant.A 22-year-old woman presented with a 5-year history of a micropapular eruption localized to the flexor aspect of her limbs as well as persistent reticulate pigmentation of her neck and upper chest resembling Darier's disease. The eruption was associated with pruritus that was precipitated by heat and was worse in summer. The axillae, groins and inframammary areas had multiple papules but lacked reticulate pigmentation. Multiple biopsies showed an epidermis with club- and antler-like rete ridges but no acantholysis or dyskeratosis. This distinct clinical presentation may represent an unusual heat aggravated variant of Dowling-Degos disease that clinically shares features with Darier's disease and transient acantholytic dermatosis.- - - - - - - - - - ranking = 0.5keywords = ridge (Clic here for more details about this article) |
5/49. Ring chromosome 17 in a mentally retarded boy.A six-year-old boy was found to have a ring chromosome 17. In addition to psychomotor retardation, speech delay and seizure disorders, his abnormal phenotypic features included epicanthal folds, broad depressed nasal bridge, protruding thick upper and lower lips, micrognathia, narrow high arched palate, short fifth fingers with a mild degree of clinicodactyly, multiple cafe-aui-lait spots, and abnormal dermatoglyphics.- - - - - - - - - - ranking = 0.5keywords = ridge (Clic here for more details about this article) |
6/49. Dyschromatosis universalis hereditaria.Dyschromatosis universalis hereditaria is a clinically heterogenous disorder. We report two unrelated Indian patients with dyschromatosis universalis hereditaria, who had generalized and progressive reticulate hyper- and hypo-pigmentation of the skin. The oral mucosa and tongue also showed mottled pigmentation. Intriguingly, the palms and soles were also affected with a diffuse hyper-pigmentation interspersed with spotty de-pigmented macules. Dystrophic nail changes with pterygium formation were seen in one case. Histopathology revealed a variable degree of pigmentary incontinence. Although the precise aetiology of this disorder is not yet known, the clinicopathological findings implicate an inherent abnormality of melanosomes or melanin processing.- - - - - - - - - - ranking = 0.0046790433393195keywords = process (Clic here for more details about this article) |
7/49. Bednar tumor associated with dermal melanocytosis: melanocytic colonization or neuroectodermal multidirectional differentiation?BACKGROUND: Neuroectodermal differentiation or melanocytic colonization are the opposing theories of histogenesis for the Bednar tumor or pigmented dermatofibrosarcoma protuberans (DFSP). observation: A 31-year-old African-American woman presented with a 2-cm blue-black shoulder nodule of 1-year duration. Punch biopsy revealed a CD34 , factor xiiia-DFSP, harboring numerous, pigmented spindle S100 , Mart-1 and HMB-45 cells. Subsequent wide excision demonstrated pigmented dendritic and spindled cells widely scattered throughout the dermis of the 3-cm excisional margins and punch biopsy specimens of normal skin from both shoulders. This latter process was interpreted as dermal melanocytosis (nevus of Ito). The dermal pigmented spindle cells were Mart-1 and CD34-, and were associated with non-pigmented CD34 , cytologically banal spindle cells, which were more numerous in the excisional margins than the contralateral shoulder. CONCLUSION: Reported herein is a singular case of Bednar tumor associated with dermal melanocytosis. Although the coexistence of these processes implicates colonization of the DFSP by constituent dermal melanocytes, the mixed immunophenotype (CD34 or Mart-1 cells) of dispersed dermal spindle cells hints at the possibility of a common cell of origin: the putative neuromesenchymal cell. In effect, the Bednar tumor could represent one part of a spectrum of neural crest-derived dermal tumors that includes dermal melanocytosis, cellular blue nevus and conventional DFSP.- - - - - - - - - - ranking = 0.0093580866786389keywords = process (Clic here for more details about this article) |
8/49. minocycline-induced staining of torus palatinus and alveolar bone.BACKGROUND: minocycline hydrochloride, an analog of tetracycline, is widely used in the treatment of acne. Its use has been associated with discoloration of teeth, bone, and other tissues. methods: A case is presented involving a patient with minocycline-induced staining of the torus palatinus and alveolar bone. RESULTS: No treatment was rendered since the patient was not concerned with the appearance of the discoloration. The patient's dermatologist elected to change antibiotics. CONCLUSIONS: patients on long-term minocycline therapy should be made aware of the possibility of pigmentation of bone and soft tissue that may be reversible with discontinuation of therapy; however, minocycline-induced staining of the permanent dentition may not be reversible.- - - - - - - - - - ranking = 1.0358694943841keywords = alveolar (Clic here for more details about this article) |
9/49. Leukoderma in association with giant congenital nevi: report of two cases.Two patients are presented in whom giant congenital nevi were associated with hypopigmentation. One patient has had no associated melanoma. The second patient developed hypopigmentation years before a melanoma was excised, and increased hypopigmentation was noted years later without evidence of melanoma recurrence. While the mechanism for the development of the hypopigmentation noted in these two patients in uncertain, an immunologically mediated systemic process may be responsible.- - - - - - - - - - ranking = 0.0046790433393195keywords = process (Clic here for more details about this article) |
10/49. Oral-dental findings in dyskeratosis congenita.A 13-yr-old girl with dyskeratosis congenita is presented. Besides oral leukoplakia and nail dystrophies, there was evidence of pancytopenia, growth retardation, alopecia, mental retardation and microcephaly. The oral findings included caries, gingival recession, short-blunted roots, gingival bleeding, tooth mobility and severe alveolar bone loss resembling juvenile periodontitis.- - - - - - - - - - ranking = 0.20717389887682keywords = alveolar (Clic here for more details about this article) |
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