1/19. Benign melanocytic proliferative nodule within a congenital naevus.A neonate presented with a deeply pigmented papule within a medium-sized congenital naevus. Histologically, this proved to be a benign proliferative nodule in a congenital naevus. This case is presented to highlight the occurrence of this lesion, the main differential of which is the rare entity of true congenital melanoma.- - - - - - - - - - ranking = 1keywords = naevus (Clic here for more details about this article) |
2/19. Massive abdominal and pelvic myxoma in Carney's syndrome.This report describes a massive abdominal and pelvic myxoma in a patient with Carney's syndrome. A 38 year old woman presented with abdominal distension and a palpable mass, and at operation a large pelvic and abdominal tumour was identified and resected. The surgical specimen consisted of a lobulated mass, which on cut section had a uniform gelatinous consistency. The mass surrounded both ovaries, the appendix, and the upper part of the uterus, but macroscopically did not appear to involve these organs. Histological examination showed plump stellate and spindle shaped cells set in an abundant myxoid stroma, in keeping with a myxoma. Immunohistochemical staining revealed positivity of tumour cells for vimentin, but no reactivity to desmin, alpha-smooth muscle actin, S-100 protein, CD34, or AE1/AE3. This is the first documented case of massive adominal and pelvic myxoma in a patient with Carney's syndrome. Clinicians and pathologists should be aware that myxomas in Carney's syndrome can rarely involve unusual sites other than the skin and heart.- - - - - - - - - - ranking = 0.0015266287643492keywords = spindle (Clic here for more details about this article) |
3/19. septo-optic dysplasia in combination with a pigmented skin lesion: a case report with nosological discussion.In this case report a patient with bilateral optic nerve hypoplasia, schizencephaly and a pigmented skin lesion is described. The diagnosis of de Morsier syndrome or septo-optic dysplasia is put forward on the basis of the diagnosis of optic nerve hypoplasia. The differential diagnosis with Jadassohn's naevus phakomatosis is discussed. The importance of direct ophthalmoscopy of optic nerve abnormalities is stressed, as well as of magnetic resonance imaging, which has become a guideline in the classification of this syndrome.- - - - - - - - - - ranking = 0.16666666666667keywords = naevus (Clic here for more details about this article) |
4/19. An ultrastructural study of pigmented purpuric dermatitis with special reference to fibrous long-spacing collagen.A case of pigmented purpuric dermatitis (PPD) in a Japanese man aged 59 years is reported with an interesting ultrastructural finding. Clinically, the lesions, which consisted of telangiectatic puncta and pigmentation, were irregular in shape and occurred predominantly on the lower legs without pruritus. Histologically, lymphocytic perivascular infiltrates and extravasation of red blood cells were observed in the papillary dermis. Ultrastructurally, endothelial cells with ovoid nuclei showed swelling and the lumen of the capillary became narrowed. Several banded structures, so-called fibrous long-spacing collagen (FLSC), were observed in the cytoplasm. They were spindle shaped, about 5 microm in length, and showed crossbands of 300-nm-wide intervals with fine intraperiodic bands. These structures were not observed in dermal connective tissue and fibroblasts. These results suggested that FLSC was synthesized in endothelial cells rather than being phagocytosed by endothelial cells, which might be helpful in investigating the etiology of PPD.- - - - - - - - - - ranking = 0.0015266287643492keywords = spindle (Clic here for more details about this article) |
5/19. 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 = 0.0076331438217458keywords = spindle (Clic here for more details about this article) |
6/19. Atypical mole syndrome and congenital giant naevus in a patient with celiac disease.We describe a case of a 28-year-old woman affected by celiac disease (CD) associated with rare multiple disorders of the cutaneous pigmentary system: atypical mole syndrome and congenital giant naevus. Some other rare skin lesions have been reported in association with celiac disease such as cutaneous sarcoidosic granuloma, bullous pemphigoid, ichthyosis, alopecia areata, erythema elevatum diutinum, sclero-atrophic lichen and primary cutaneous amyloidosis. This is the 1(st) report concerning celiac disease and congenital disorders of the pigmentary system.- - - - - - - - - - ranking = 0.83333333333333keywords = naevus (Clic here for more details about this article) |
7/19. Dense pigmentation of the posterior lens capsule associated with the pigment dispersion syndrome.PURPOSE: To report an unusual case of pigment dispersion syndrome associated with unilateral dense pigmentation of the posterior lens capsule. methods: Case report. RESULTS: A 59-year-old male with bilateral pigment dispersion syndrome presented with progressive decrease in visual acuity in the left eye over the past 10 to 20 years. Clinical examination revealed the typical findings of pigment dispersion syndrome including the presence of bilateral Krunkenberg spindles, iris transillumination defects, and heavy trabecular meshwork pigmentation. Of note, there was remarkably dense pigmentation of the posterior lens capsule in the eye with decreased visual acuity. CONCLUSION: Pigmentation of the posterior lens capsule may be a rare finding associated with pigment dispersion syndrome. Such a finding suggests that there may be aqueous flow into the retrolental space in some patients with this condition. The optimal treatment of this unusual condition remains undetermined.- - - - - - - - - - ranking = 0.0015266287643492keywords = spindle (Clic here for more details about this article) |
8/19. Acquired pigmentation simulating peutz-jeghers syndrome: initial manifestation of diffuse uveal melanocytic proliferation.A 74-year-old man with an occult carcinoma of the colon developed pigmentation of the mouth and penis typical of the peutz-jeghers syndrome as the first manifestation of bilateral diffuse uveal melanocytic proliferation. The simultaneous appearance of extraocular pigmented lesion and those in the uveal tract of both eyes of this patient provides further evidence that bilateral diffuse uveal melanocytic proliferation may be caused by activation of occult melanocytic naevus cells in response to either a hormone-producing carcinoma or to some other common oncogenic stimulus.- - - - - - - - - - ranking = 0.16666666666667keywords = naevus (Clic here for more details about this article) |
9/19. Familial acquired pigmented streaks in the nail.A 40-year-old man developed multiple longitudinal pigmented streaks in his nails. His sister was also found to have a single streak on a fingernail. Neither had any history of drug ingestion or recent illness and examination failed to reveal any other signs in the skin or mucosal surfaces. A biopsy of the nail matrix at the base of one streak showed no evidence of a naevus or lentigo, the principal finding being pigmentary incontinence and excess macrophages.- - - - - - - - - - ranking = 0.16666666666667keywords = naevus (Clic here for more details about this article) |
10/19. Acquired dermal melanocytosis of the face and extremities.Four cases of dermal melanocytosis with symmetrical areas of hyperpigmentation involving the face and extremities are reported. light and electron microscopic studies showed changes similar to those seen in naevus of Ota.- - - - - - - - - - ranking = 0.16666666666667keywords = naevus (Clic here for more details about this article) |
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