11/378. Simultaneous occurrence of multiple melanoma in situ on sun-damaged skin (lentigo maligna), solar lentigo and labial melanosis: the value of dermoscopy in diagnosis. We report on a patient developing simultaneous occurrence of lentigo maligna lesions, solar lentigines and an extensive melanosis of the oral mucosa. Diagnostically, epiluminescence microscopy had a relevant role in the preoperative assessment and selection of suspicious pigmented lesions, as the lesions histologically labelled as lentigo maligna and solar lentigo were clinically indistinguishable. We review the clinical, dermoscopic and histopathologic differential diagnosis of solar lentigo, malignant lentigo and mucosal melanosis with other melanocytic and keratinocytic lesions and discuss the possible relationship between these entities. ( info) |
12/378. Pigmented squamous cell carcinoma. Pigmented squamous cell carcinomas have been reported in the oral and ocular mucosae, but rarely in the skin. We present a case of pigmented squamous cell carcinoma of the forehead and review the current English literature. Pigmented squamous cell carcinoma can be confused with pigmented basal cell carcinomas and melanoma, especially those melanomas associated with pseudoepitheliomatous hyperplasia and should be included in the differential diagnosis of atypical pigmented lesions. ( info) |
13/378. Treatment of gingival hyperpigmentation for esthetic purposes by Nd:YAG laser: report of 4 cases. Gingival hyperpigmentation may cause esthetic problems and embarrassment, especially in patients with a gummy smile. This report presents the use of the Nd:YAG laser for gingival depigmentation. Four cases, 3 females and 1 male, ages between 24 to 28 years old, presented with the same chief complaint of unesthetic gingiva caused by melanin hyperpigmentation. The Nd:YAG laser was set at 6 watts, 60 millijoules per pulse, and 100 pulses per second. The procedures were performed with contact mode in all pigmented areas by using a handpiece with a 320 microm diameter fiber optic. Ablation of the gingival hyperpigmented areas were accomplished without any bleeding complications or significant postoperative pain. Three to 4 weeks after the procedures, the hyperpigmented gingiva appeared healthy, pink, and firm. No recurrence of hyperpigmentation had been found in 11 to 13 months of follow-up. However, in delicate areas such as the marginal gingiva, the Nd:YAG laser should be used cautiously. ( info) |
14/378. Phacomatosis pigmentovascularis type IIa successfully treated with two types of laser therapy. We describe a 28-year-old Japanese woman with phacomatosis pigmentovascularis type IIa who was treated by 27 sessions of Q-switched ruby laser irradiation to the site of dermal melanosis on her face and three sessions of dye laser irradiation to the port-wine stain on her left cheek. This is the first report of the successful treatment of a cutaneous lesion in a patient with phacomatosis pigmentovascularis. As the outcome of the treatments was excellent, we conclude that phacomatosis pigmentovascularis type IIa can be treated successfully by the combination of the two types of laser therapy. ( info) |
15/378. Epidermal nevus syndrome with hypermelanosis and chronic hyponatremia. Epidermal nevus syndrome is seldom encountered, and its association with hypermelanosis and the chronic syndrome of inappropriate antidiuretic hormone secretion (SIADH) has never been reported. A male neonate who developed intractable seizures and hyponatremia soon after birth is reported. He had alopecic patches on the scalp at birth. Large areas of skin hyperpigmentation, and epidermal nevi developed gradually. The clinical picture of hypotonic hyponatremia, high urine osmolality, elevated urine sodium, and euvolemia was compatible with SIADH. The seizures did not correlate with the hyponatremia, and no other cause for the seizures could be identified. The hyponatremia became chronic and was treated with a direct supply of sodium chloride. The development of the patient was markedly delayed at the last visit when he was 1 year of age. It is suggested that hypermelanosis and chronic SIADH may also be a variant presentation of epidermal nevus syndrome. ( info) |
16/378. An unbalanced submicroscopic translocation t(8;16)(q24.3;p13.3)pat associated with tuberous sclerosis complex, adult polycystic kidney disease, and hypomelanosis of Ito. We report on a familial submicroscopic translocation involving chromosomes 8 and 16. The proband of the family had a clinical picture suggestive of a large deletion in the chromosome 16p13.3 area, as he was affected with tuberous sclerosis complex (TSC) and had alpha thalassaemia trait, and his half brother, who also had TSC, may have suffered additionally from polycystic kidney disease (PKD). FISH studies provided evidence for a familial translocation t(8;16)(q24.3;p13.3) with an unbalanced form in the proband and a balanced form in the father and in a paternal aunt. The unbalanced translocation caused the index patient to be deleted for the chromosome 16p13.3-pter region, with the most proximal breakpoint described to date for terminal 16p deletions. In addition, FISH analysis showed a duplication for the distal 8q region. Since the index patient also had hypomelanosis of Ito (HI), either of the chromosomal areas involved in the translocation may be a candidate region for an HI determining gene. Furthermore, it is noteworthy that both carriers of the balanced translocation showed a nodular goitre, while the proband has hypothyroidism. ( info) |
17/378. Laparoscopic postmortem examination: a minimally invasive approach to the autopsy. Laparoscopic and thoracoscopic autopsies have previously only been performed on an experimental basis to determine their potential usefulness as a substitute for a conventional postmortem examination. We present the case of a patient with an unusual variant of malignant melanoma (diffuse melanosis) in whom the immediate cause of death clinically was thought to be fulminant hepatic failure, the etiology of which was unknown. The family was unwilling to consent to a conventional autopsy, but would permit a postmortem examination limited to a 2-cm abdominal incision and removal of a sample of liver. In view of the unanswered clinical questions regarding the cause of the acute hepatic failure and its possible relationship to the diagnosis of diffuse melanosis, we decided that more extensive examination of the abdominal cavity, specifically the liver, was required and that the only way that this could be accomplished was by laparoscopic techniques. Laparoscopic examination of the abdominal cavity revealed multiple melanotic nodules on the surface of the liver and studding the omentum. Multiple liver samples were easily obtained; these revealed massive diffuse necrosis of the liver parenchyma with scattered nodular deposits of dark pigment consistent with melanin. We report the first known case in which a laparoscopic autopsy was used to obtain valuable information that answered clinically relevant questions. Laparoscopic autopsy can offer the a family that is unwilling to consent to a conventional postmortem examination an alternative that can potentially provide answers to clinical questions that otherwise would have been unresolved. ( info) |
| 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. ( info) |
19/378. iris mammillations as the only sign of ocular melanocytosis in a child with choroidal melanoma. An 8-year-old girl had visual loss in her left eye over 2 months. Ocular examination showed that visual acuity was counting fingers in the left eye. The left iris was moderately pigmented and thickened with numerous confluent, dome-shaped elevations on its surface, consistent with iris mammillations arising from ocular melanocytosis. There was total retinal detachment and an inferiorly located large amelanotic choroidal mass compressing the optic nerve. A specimen from a fine-needle aspiration biopsy showed spindle and epithelioid melanoma cells. The eye was enucleated. Pathologic examination showed that the bland melanocytes comprising the anterior border layer of iris formed focal aggregates, corresponding to the iris mammillations observed clinically. The uvea was diffusely thickened. Arising from the posterior choroid and obscuring the optic nerve head was a moderately pigmented spindle and epithelioid cell choroidal melanoma with diffuse lymphocytic infiltration and high mitotic activity. This case demonstrates that iris mammillations can be the initial manifestation of ocular melanocytosis in the absence of scleral pigmentation. ( info) |
20/378. Enteric duplication cyst associated with melanosis peritonei. melanosis peritonei is usually associated with benign cystic teratomas of the ovary. We describe a one-and-a-half-year-old girl with melanosis peritonei associated with enteric duplication cyst. Melanophages were seen in aggregates in and around the serosal blood vessels, nerve bundles, and scattered within the muscular wall of the cyst. Presence of hyperplastic nerve bundles associated with melanophages suggests their origin from the neural crest. ( info) |