1/35. Neurocutaneous melanosis with hydrocephalus, intraspinal arachnoid collections and syringomyelia: case report and literature review.Neurocutaneous melanosis (NCM) is a rare nonfamilial syndrome, characterised by large or numerous congenital pigmented nevi and excessive proliferation of melanin-containing cells in the leptomeninges. We report the MR findings in the brain and spine of a child with NCM who underwent neurosurgical treatment and was followed up for 8 years. The findings in this child (small hyperintense collections of melanocytes in both temporal lobes, mild meningeal enhancement along the spine and the development of an extensive subarachnoid CSF accumulation with cord compression and syringomyelia) are believed to be exceptionally rare.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) | ||
2/35. Diffuse melanosis arising from metastatic melanoma: pathogenetic function of elevated melanocyte peptide growth factors.The origin of diffuse melanosis resulting from metastatic melanoma is unknown. We examined the light microscopic and ultrastructural changes in the skin of an affected 35-year-old woman and determined the peripheral blood levels of melanocyte growth factors. A total of 7 biopsy specimens were examined by light and electron microscopy and immunohistology (S-100, HMB45, MART1, CD68, MAC387). serum/plasma levels of melanocyte growth factors of the patient were determined by enzyme-linked immunosorbent assays and compared with those of normal volunteers (n = 10) and amelanotic patients with metastatic melanoma (n = 10), matched to the UICC stage of the affected patient. Hyperpigmented but otherwise apparently normal skin of the patient displayed epidermal melanocyte hyperplasia, increased melanogenesis, and dermal pigment stored in histiocytes and other cells along with extracellular deposits. blood levels of alpha-melanocyte stimulating hormone, hepatocyte growth factor, and endothelin-1 were significantly elevated in the affected patient. Aberrant production of these factors may not only be responsible for activation of the pigment system in diffuse melanosis of metastatic melanoma, but also for increased proliferation, motility, and pigment incontinence of normal and malignant melanocytes.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) | ||
3/35. Neurocutaneous melanosis with epilepsy: report of one case.Neurocutaneous melanosis is a rare congenital syndrome characterized by the association of large or multiple congenital melanocytic nevi and benign or malignant melanotic tumors in the central nervous system. patients with neurocutaneous melanosis usually have neurological symptoms early in life that progress rapidly due to the development of increased intracranial pressure or malignant melanoma. We report a 2-month-old female infant with multiple congenital melanocytic nevi and frequent seizure attacks. magnetic resonance imaging of the brain demonstrated several regions compatible with melanotic deposits. During follow-up for one year, she had normal development and was seizure-free under the treatment of phenobarbital and valproic acid. We suggest that infants with large or multiple congenital melanocytic nevi should receive regular clinical check-up and brain imaging to exclude the possibility of central nervous system lesions.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) | ||
4/35. Neurocutaneous melanosis in association with encephalocraniocutaneous lipomatosis.We describe a white female infant with neurocutaneous melanosis (NCM) and encephalocraniocutaneous lipomatosis (ECCL). Multiple, giant and small congenital melanocytic nevi (CMN) were observed on the head, neck and trunk and involved 70% of body surface area. Histologic examination of several CMN revealed atypical nodular proliferations of dermal nevomelanocytes. In a small (<1 cm) truncal CMN, single and dyscohesive intraepidermal nests of atypical nevomelanocytes simulating a superficial spreading melanoma, were observed. The placenta was grossly normal and histologically demonstrated multiple banal appearing nevomelanocytes within the stroma of its villi. At the 17-month follow-up no evidence of primary or metastatic melanoma was present. This previously undescribed association of NCM, ECCL and placental nevomelanocytes provides strong support for the hypothesized causal role of anomalous neural crest morphogenesis and migration in the development of all three disorders. The genetic mechanism underlying these complex birth defects has been hypothesized to result from the action of lethal autosomal dominant genes surviving by mosaicism.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) | ||
5/35. Treatment of phacomatosis pigmentovascularis: a combined multiple laser approach.BACKGROUND: Phacomatosis pigmentovascularis (PPV) consists of a capillary malformation with a variety of melanocytic lesions, which involve various regions of the body and are difficult to treat with conventional therapeutic tools. OBJECTIVE: We described two cases with PPV (type IIa and IIb) that were successfully treated with different lasers. methods: The areas involved by both melanocytic lesions and port-wine stains were treated using the Q-switched ruby laser, the Q-switched Alexandrite laser, and the flashlamp pumped pulsed-dye laser. RESULTS: Removal of a good portion of cutaneous and vascular lesions using combined multiple laser approach was achieved after 6 sessions in the first case under general anesthesia and after 31 sessions under local anesthesia in the second case. CONCLUSION: PPV type II can be treated successfully by laser treatment. We prefer to start combined multiple laser treatment of PPV in childhood period under general anesthesia because it will reduce the number of treatment, improve the patient's quality of life, and increase the cost-effectiveness of the treatment.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) | ||
6/35. Eruptive melanotic macules and papules associated with adenocarcinoma.BACKGROUND: Malignancies may be associated with paraneoplastic cutaneous manifestations, including pigmentary disorders. methods: The clinical findings were reviewed. skin and tumor tissue samples were examined by routine histology, immunohistochemistry, and in one case also by electron microscopy. RESULTS: Two patients developed diffuse melanotic macules and papules associated with visceral adenocarcinoma. One patient was a 64-year-old man with advanced carcinoma of the distal esophagus. The other was a 62-year-old man with metastatic pulmonary adenocarcinoma. The detection of the primary tumor in both patients was preceded by the rapid onset of melanotic macules and papules in the anogenital region and in one patient also around both nipples. The pigmented lesions were histologically characterized by a lentiginous melanocytic proliferation of large and heavily pigmented melanocytes associated with hyperpigmentation of adjacent keratinocytes. Both patients had been misdiagnosed as having epidermotropic metastatic malignant melanoma. None of them had prior, concurrent, or subsequent cutaneous or extracutaneous invasive melanoma. Both patients died of metastatic adenocarcinoma. CONCLUSION: Eruptive melanotic macules and papules represent an under-recognized paraneoplastic syndrome. The cases illustrate a diagnostic pitfall for clinicians and pathologists unaware of this phenomenon.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) | ||
7/35. Neurocutaneous melanosis with transposition of the great arteries and renal agenesis.Neurocutaneous melanosis (NCM) is rare and is characterized by the proliferation of melanocytes in the central nervous system. A 6-day-old infant boy was referred to our department with giant congenital melanocytic nevi and convulsions. On physical examination the patient had a giant black-brown pigmented nevus covering his face, neck, scalp, shoulders, back, chest, and abdomen. Numerous satellite lesions were noted on the face, neck, and upper extremities. In the right bulbar conjunctiva, a brown plaque was present. magnetic resonance imaging (MRI) showed hyperintense areas in the brain on short repetition time/short echo time sequences, compatible with intraparenchymal melanin deposits. No leptomeningeal abnormality was seen. Further investigation also revealed agenesis of the right kidney and transposition of the great arteries. Transposition of the great arteries, which has never been reported in NCM, may be an incidental finding. We present a case of NCM associated with agenesis of the right kidney and transposition of the great arteries.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) | ||
8/35. Neurocutaneous melanosis.BACKGROUND: Neurocutaneous melanosis (NCM) is a rare phakomatosis characterized by a focal or diffuse proliferation of melanin-producing cells in both the skin and the leptomeninges. This syndrome is believed to result from an error in the morphogenesis of embryonal neuroectoderm. FEATURES: Two-thirds of patients with NCM have giant congenital melanocytic nevi, and the remaining third have numerous lesions but no giant lesions. Most patients present neurological manifestations early in life, which can be secondary to intracranial hemorrhages, to impairment of cerebrospinal fluid circulation, and to malignant transformation of the melanocytes. prognosis: The prognosis of patients with symptomatic neurocutaneous melanosis is extremely poor, even in the absence of malignancy. Chemotherapy has been ineffective in the few patients in whom it has been tried.- - - - - - - - - - ranking = 2keywords = life (Clic here for more details about this article) | ||
9/35. Neurocutaneous melanosis associated with malignant leptomeningeal melanoma in an adult: clinical significance of 5-S-cysteinyldopa in the cerebrospinal fluidcase report.A 35-year-old male presented with a variant of neurocutaneous melanosis with leptomeningeal malignant melanoma. He had three pigmented nevi from birth. He suffered diplopia followed by headache. T1-weighted magnetic resonance (MR) imaging revealed hydrocephalus and a small linear hyperintense lesion in the right frontal cortex. Several parts of the cortical sulci and the brain surface were slightly enhanced by gadolinium. Ventriculoperitoneal shunting was performed and extensive pigmented leptomeninges were recognized. Open biopsy established the diagnosis of leptomeningeal malignant melanoma. Combined chemoimmunotherapy was repeated every other month with monitoring of the 5-S-cysteinyldopa (5-S-CD) level in the cerebrospinal fluid (CSF). The 5-S-CD level decreased after each treatment, but the basal level steadily increased prior to the next treatment. Two years after the onset, he showed paraplegia caused by an extramedullary mass at the T-6 level. MR imaging showed that melanoma had involved the entire subarachnoid space including the whole spine. He underwent emergent removal of the spinal tumor and showed transient marked improvement. Further intensive chemotherapy was given. However, he died 31 months after the onset of massive proliferation of intracranial leptomeningeal melanoma. Measurement of CSF 5-S-CD levels is valuable for evaluating the therapeutic efficacy and for monitoring the progression of melanoma.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) 10/35. Laugier and Hunziker pigmentation: a lentiginous proliferation of melanocytes.Macular pigmentation of Laugier and Hunziker is an acquired disorder involving lips, oral mucosa, acral surfaces, nail apparatus, or a combination of these in the absence of systemic disease, and is reported to show intraepidermal melanosis without melanocytosis. We present a 69-year-old white man with typical features of this disorder, involving lips and oral mucosa, first appearing 4 years before presentation. A pigmented macule on sun-exposed lip vermilion revealed increased numbers of slightly atypical melanocytes in a lentiginous epidermal pattern. A buccal mucosa pigmented macule and adjacent nonpigmented mucosa were studied using hematoxylin and eosin staining, and S-100 and L-3,4 dihydroxyphenylalanine histochemistry. The buccal mucosa macule revealed markedly increased numbers of dendritic, L-3,4 dihydroxyphenylalanine-reactive intraepithelial melanocytes. Further studies are warranted to determine if this pigmentation disorder represents a spectrum of histopathologic change or, in fact, should be renamed mucocutaneous lentiginosis of Laugier and Hunziker.- - - - - - - - - - ranking = 4keywords = life (Clic here for more details about this article) |
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