1/378. melanosis in association with metastatic malignant melanoma: report of a case and a unifying concept of pathogenesis. An unusual case of melanosis associated with metastatic malignant melanoma is reported. This was characterized by progressive blue/gray discoloration of the skin of the chest and abdomen in an elderly patient, 1 year after removal of a polypoid malignant melanoma from the right arm. A biopsy of involved skin revealed perivascular aggregates of melanin-laden histiocytes throughout the dermis, the histopathologic hallmark of melanosis. An unusual aspect of the case was the coincidental finding of a tumor embolus within a small dermal vessel, probably a lymphatic. To date, neoplastic melanocytes have been detected in only a small minority of skin biopsies with features of melanosis. This case and a distillation of related information in the literature lead to the conclusion that the essence of melanosis, and the feature that distinguishes this from conventional metastatic melanoma, is the persistent and cumulative dissemination of melanin, via the bloodstream, throughout the body. This in turn leads to progressive pigmentation of all internal organs and the skin. Only continuous access to the circulation by neoplastic melanocytes could explain such a phenomenon. Potential mechanisms by which this could arise are discussed in the context of existing knowledge. ( info) |
2/378. Erythrosis pigmentosa mediofacialis (Brocq) and erythromelanosis follicularis faciei et colli in the same patient. Erythrosis pigmentosa peribuccalis (Brocq) (or erythrosis pigmentosa mediofacialis) and erythromelanosis follicularis faciei et colli, have been regarded as different disorders, mainly because the first occurs on the mediofacial area and is common in women and the second mostly occurs pre-auricularly in men. Both conditions show histological signs of abnormal follicular keratinization with teleangiectasia and round cell infiltrate. An increase in the level of melanin has been seen in some patients. We describe here a woman in whom lesions started in the middle of the face and later became evident in the pre-auricular area. This suggests that the two conditions are in fact the same disease. As a neutral term for this not uncommon disorder we propose erythrosis pigmentosa faciei et colli. ( info) |
3/378. The uptake of gallium 67 in colonic macrophages. A case is presented in which a patient with a well-differentiated adenocarcinoma showed high gallium concentration in the segment with melanosis coli proximal to the obstruction. Although in this case the gallium was associated with an increased number of faecal pigment containing macrophages it is unlikely that macrophages are the main factor in tumour uptake of gallium compounds. ( info) |
4/378. oral manifestations of idiopathic lenticular mucocutaneous pigmentation (Laugier-Hunziker syndrome): a clinical, histopathological and ultrastructural review of 12 cases. OBJECTIVE: To present a clinical, histopathological and ultrastructural study on a group of patients affected by idiopathic mucocutaneous pigmentation (Laugier-Hunziker syndrome: LHS). MATERIALS AND methods: Twelve patients were investigated: clinical examination, laboratory tests, and X-ray studies together with light microscopy and electron microscopy were performed in order to diagnose LHS. RESULTS: All cases showed acquired, benign, macular hyperpigmentation of buccal mucosa lips and nails. Histologically, pigmentations are due to an accumulation of melanin in the basal layer keratinocytes and an increase in the number of melanophages in the submucosa and/or papillary dermis. Ultrastructurally there were increased numbers of normal-appearing melanosomes in keratinocytes of the lower epithelium. No evidence of malignant changes were detected. CONCLUSIONS: The importance of this condition relates to it being included in the differential diagnoses of pigmentary disorders of the oral mucosa with associated nail involvement. It is important to recognize this acquired benign disorder to avoid unnecessary investigations and treatments. ( info) |
5/378. Neurocutaneous melanosis presenting with intracranial amelanotic melanoma. We describe imaging findings in a 2-year-old girl with neurocutaneous melanosis and malignant cerebral melanoma. Because the cerebral melanoma in this child was of the amelanotic type, high-signal intensity on unenhanced T1-weighted images was not present. The cutaneous lesions played a crucial role in establishing a correct (presumed) histopathologic diagnosis on the basis of the imaging findings. To our knowledge this is the first report describing an intracranial amelanotic malignant melanoma in association with neurocutaneous melanosis. ( info) |
| Two cases of hyperthyroidism with hyperpigmentation are presented. In both cases, hyperpigmentation was seen on the lower extremities, most strikingly on the shins, backs of the feet and the nail bed. histology of the pigmented skin showed basal melanosis and heavy deposition of haemosiderin around dermal capillaries and sweat glands. Treatment with mercazol in both cases resulted in no significant waning of pigmentation. Distribution of hyperpigmentation, haemosiderin deposition and poor response to the treatment may be characteristic features of the pigmentation caused by hyperthyroidism, and may represent differences from the pigmentation seen in Addison's disease. ( info) |
| The most characteristic lesions of neurofibromatosis are the extremely large plexiform neurofibromas involving an entire extremity, which give rise to the condition known as elephantiasis neuromatosa. In this article, we present a patient who was diagnosed as elephantiasis neuromatosa with Becker's melanosis clinically and review the literature briefly. ( info) |
8/378. A Laugier-Hunziker syndrome associated with esophageal melanocytosis. A 62-year-old Japanese woman came to our clinic because of melanotic macules on the lip, palatoglossal arch, lingual margin and palm. Endoscopic examination revealed a melanotic macule on the midesophageal mucosa but no polyposis in the gastrointestinal tract. Histologically, the specimens taken from the labial, esophageal and palmar lesions showed an acanthosis and basal hyperpigmentation in the epithelium. The patient had not taken any medication which could lead to pigmentation. As far as we know, this is the first case report of an esophageal melanocytic macule which occurred in a patient with Laugier-Hunziker syndrome. When confronted with an isolated pigment spot, we emphasize the necessity of systematic examinations for others. Because the pathologic relationship between Laugier-Hunziker syndrome and the esophageal melanocytic lesion is not proven, further studies should clarify this issue. ( info) |
9/378. Longitudinal melanonychia associated with hydroxyurea therapy in a patient with essential thrombocytosis. Occurrence of skin lesions during long-term therapy with hydroxyurea are well described, but there have been only a few case reports of longitudinal melanonychia associated with hydroxyurea treatment. We report herein longitudinal melanonychia associated with hydroxyurea therapy in a subject with essential thrombocytosis. The prolonged time lapse (1 year) between initiation of hydroxyurea therapy and the onset of the longitudinal melanonychia should be noted. ( info) |
10/378. Primary invasive signet-ring cell melanoma. The histopathological variants of malignant melanoma include the common type (lentigo maligna, superficial spreading melanoma, nodular melanoma, acrolentiginous melanoma), spindle cell, desmoplastic, balloon cell, pleomorphic (fibrohistiocytic), myxoid, small cell melanoma and malignant blue nevus. Recently, signet-ring cell melanoma was introduced as an additional cytologic variant. We describe a 72-year-old patient with a primary signet-ring cell melanoma of the skin located on the upper arm. Histopathologic examination disclosed a melanocytic tumor extending from the epidermis to the deep reticular dermis. Numerous pleomorphic tumor cells showed large, intracellular vacuoles and oval to spindle-shaped nuclei at their periphery. Mitotic figures and multinucleated melanocytes were also observed. Some of the signet-ring cells exhibited cytoplasmatic periodic acid-Schiff (PAS)-positivity. immunohistochemistry showed positive reaction of the tumor cells for S-100, HMB-45 protein and vimentin, confirming their melanocytic differentiation. Tumor cells were negative for cytokeratins, epithelial membrane antigen (EMA), and carcinoembryonic antigen (CEA). The signet-ring cell melanoma disclosed an invasion to Clark Level IV and tumor thickness of 2.2 mm. Signet-ring cell melanoma is a rare morphologic variant of melanoma. Its recognition is important for differentiation from other tumors featuring signet ring cells. ( info) |