1/77. Malignant melanoma of the choroid in neurofibromatosis.A 60-year-old white woman with generalized neurofibromatosis and multiple melanocytic hamartomas of the iris developed an unusual choroidal mass, with secondary sensory retinal separation in the left eye. Ophthalmoscopically the tumor had a peculiar donut configuration that was caused by a large focus of central necrosis within a spindle B melanoma.- - - - - - - - - - ranking = 1keywords = melanocytic (Clic here for more details about this article) |
2/77. Linear congenital smooth muscle hamartoma with follicular spotted appearance.Congenital smooth muscle hamartoma (CSMH) with follicular spotted appearance is a rare clinical variant of CMSH in which patients have marked perifollicular papules in the patches. A linear distribution of CSMH is also extremely rare. We report a 16-year-old Korean girl with this uncommon form of CSMH who had linearly arranged, hyperpigmented lesions with follicular papules extending from the right flank to the right lower leg from birth. Pathological findings, including immunohistochemical stains, were consistent with smooth muscle hamartoma. To date, there are only four reports on this rare, follicular form and one report on the linear form of CSMH in the literature. This paper describes the first combined occurrence of follicular spotted lesions and linear arrangement in CSMH.- - - - - - - - - - ranking = 0.1064386562452keywords = pigmented (Clic here for more details about this article) |
3/77. Combined cutaneous hamartoma encompassing benign melanocytic naevus, vellus hair cyst and epidermoid cyst.We describe a combined cutaneous hamartoma in a 57-year-old man, which comprised a vellus hair cyst, an epidermoid cyst and an intradermal melanocytic naevus in a single facial tumour. The vellus hair cyst was filled with keratinous material with unusual slit-like lacunae. While numerous cases of epidermoid cyst have been reported in association with an intradermal melanocytic naevus, our case is a rare example of a lesion combining several hamartomatous elements.- - - - - - - - - - ranking = 93.646819793592keywords = naevus, melanocytic (Clic here for more details about this article) |
4/77. Acquired vulvar smooth muscle hamartoma: a case report and review of the literature.A 36-year-old Korean woman had had a flesh-colored, indurated plaque with pruritus on the labium majora for five years. The lesion was not found in association with hyperpigmented or hypertrichotic patches. Results of biopsy specimens showed an excess of haphazardly oriented smooth-muscle bundles in the mid to lower dermis with an unremarkable overlying epidermis. Our diagnosis was an acquired smooth-muscle hamartoma in the vulva. Although there have been previously reported cases of acquired smooth-muscle hamartoma, this was the first reported case in the vulva. We also describe the characteristics differing between our case and the six previously reported ones.- - - - - - - - - - ranking = 0.1064386562452keywords = pigmented (Clic here for more details about this article) |
5/77. Autosomal dominantly inherited generalized basaloid follicular hamartoma syndrome: report of a new disease in a north carolina family.BACKGROUND: An 8-year-old girl presented with hundreds of milia, measuring 1 to 2 mm; comedone-like lesions; skin-colored and hyperpigmented papules on the face, scalp, ears, neck, upper trunk, and lower arms along with diffuse scalp hypotrichosis; and pinpoint palm/sole pits. Onset was in early childhood and the disease was historically present in 6 generations. OBJECTIVE: Our objectives were to delineate the clinical and histopathologic features and mode of inheritance as a base for gene studies. methods: Eighteen family subjects were studied. Twenty-six skin biopsy specimens were examined. A detailed pedigree was constructed. A complete literature search was done concerning diseases with generalized basaloid follicular hamartomas. RESULTS: The lesions were basaloid follicular hamartomas and other folliculocentric abnormalities. Inheritance was autosomal dominant. Extensive literature search confirmed the finding of a unique genodermatosis. CONCLUSION: A new genodermatosis termed dominantly inherited generalized basaloid follicular hamartoma syndrome was defined by delineating its clinical and histopathologic features and mode of inheritance and by extensive literature review.- - - - - - - - - - ranking = 0.1064386562452keywords = pigmented (Clic here for more details about this article) |
6/77. Perioperative management of infants with the linear naevus sebaceous syndrome of Jadassohn: a report of two cases.Neurofibromatosis and tuberous sclerosis are the most well-recognized of the congential phakomatoses, a group of six hereditary neuro-oculo-cutaneous disorders. Although easily diagnosed at birth by a parasagittal line of facial sebaceous naevi, the linear naevus sebaceous syndrome (LNSS) of Jadassohn is the rarest phakomatosis, one often characterized by airway and anaesthetic considerations that do not apply to the other phakomatoses. In addition to its obvious cutaneous manifestations, LNSS is characterized by hemifacial asymmetry, an anatomic predictor of difficult trachael intubation, and intractable seizure activity, a condition that limits selection of anaesthetics. The perioperative management challenges of LNSS are depicted in the presentation of two cases of LNSS with different outcomes and contrasted with the major anaesthetic considerations in the perioperative management of other, more common phakomatoses.- - - - - - - - - - ranking = 73.03901649466keywords = naevus (Clic here for more details about this article) |
7/77. 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 = 58.431213195728keywords = naevus (Clic here for more details about this article) |
8/77. Sebaceous carcinoma, trichoblastoma, and sebaceoma with features of trichoblastoma in nevus sebaceus.A 73-year-old woman had a linear yellowish plaque on the upper part of her right ear since birth. She presented because of the sudden growth of a nodule within the plaque. The plaque was waxy and yellowish, arching around the upper part of the ear. A reddish to yellowish large nodule was seen within the central part of the arc-shaped plaque; in addition, a small pigmented nodule, a small skin-colored nodule, and a few pigmented papules were observed in the anterior half of the arched plaque. Histopathologic examination revealed the large nodule to be sebaceous carcinoma, the small pigmented nodule to be trichoblastoma, the small skin-colored nodule to be sebaceoma with the features of trichoblastoma, a few pigmented papules to be superficial trichoblastomas due to primitive follicular induction, and the linear yellowish plaque to be nevus sebaceus. Although our literature search revealed scanty reports of definite cases of sebaceous carcinoma in nevus sebaceus, the presented case demonstrated the occurrence of sebaceous carcinoma in nevus sebaceus. Malignant neoplasms occurring in nevus sebaceous seem to be extremely rare, but care should be taken when a large nodule suddenly grows in a lesion of nevus sebaceus, especially in older adults. The presented case also suggested a close relation between trichoblastoma and sebaceoma. The cytokeratin staining pattern could not distinguish between sebaceous and follicular neoplasms in our case.- - - - - - - - - - ranking = 0.4257546249808keywords = pigmented (Clic here for more details about this article) |
9/77. Melanotrichoblastoma: immunohistochemical study of a variant of pigmented trichoblastoma.Trichoblastomas (TBs) are benign skin tumors recapitulating the differentiation of hair follicles. Several pathologic variants have been described, including the rare pigmented TB. We report a deeply pigmented nodular tumor excised on the scalp of a 32-year-old African woman, which was clinically suggestive of blue nevus or melanoma. Histologically, the tumor presented features of TB, remarkable by virtue of heavy melanin deposits found within and around tumor nests. By immunohistochemistry, abundant dendritic melanocytes with features of hair follicle melanocytes (expression of S100 protein, tyrosinase, and, most importantly, gp100/HMB-45) were found within the tumor masses. By analogy with melanoacanthoma, a tumor consisting of a combined proliferation of epidermal keratinocytes and melanocytes, we called this tumor "melanotrichoblastoma" and view it as a peculiar variant of (pigmented) TB.- - - - - - - - - - ranking = 0.74507059371639keywords = pigmented (Clic here for more details about this article) |
10/77. Congenital simple hamartoma of the retinal pigment epithelium: a study of five cases.PURPOSE: To report 5 patients with presumed congenital, simple hamartoma of the retinal pigment epithelium (RPE). DESIGN: Retrospective, observational small case series. PARTICIPANTS: Five patients with a black macular tumor of the RPE. MAIN OUTCOME MEASURES: Initial tumor features, initial associated fundus features, tumor course on follow-up, and visual acuity outcome. RESULTS: In all 5 cases, the tumor involved the macula and the mean distance to the foveola was 0.4 mm (median, 0.2 mm; range, 0.2-1.2 mm). In all cases, the tumor was darkly pigmented, with full-thickness retinal and RPE involvement and minimal protrusion into the vitreous cavity. The mean ultrasonographic tumor thickness was 1.6 mm (median, 1.4 mm; range, 1.1-2.5 mm). Associated features included minimally dilated retinal feeding artery and draining vein (100%), surrounding mild retinal traction (80%), retinal exudation (20%), and vitreous pigmented cells (20%). None of the lesions had associated subretinal fluid, macular edema, or macular hole. visual acuity was 20/20 in 3 cases and slightly decreased in the other 2 cases because of foveal traction. The findings remained stable in 3 patients who had adequate follow-up. We chose to classify this lesion as congenital simple hamartoma of the RPE to differentiate it from combined hamartoma of the retina and RPE, congenital hypertrophy of the RPE, adenoma or adenocarcinoma of the RPE, and acquired hyperplasia of the RPE, which are different clinical entities. CONCLUSIONS: Congenital hamartoma of the RPE is typically a black, full-thickness retinal mass, often adjacent to the foveola. Despite such location, the visual acuity was affected minimally.- - - - - - - - - - ranking = 0.2128773124904keywords = pigmented (Clic here for more details about this article) |
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