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Christopher D M Fletcher - One of the best experts on this subject based on the ideXlab platform.
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fibroblastic connective tissue nevus a rare Cutaneous Lesion analyzed in a series of 25 cases
2012Co-Authors: Sebastien De Feraudy, Christopher D M FletcherAbstract:Fibroblastic connective tissue nevus (FCTN) represents a rare and distinct benign Cutaneous mesenchymal Lesion of fibroblastic/myofibroblastic lineage, which broadens the spectrum of Lesions presently recognized as connective tissue nevus. A series of 25 cases of FCTN has been analyzed to further ch
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Fibroblastic connective tissue nevus: a rare Cutaneous Lesion analyzed in a series of 25 cases
2012Co-Authors: Sebastien De Feraudy, Christopher D M FletcherAbstract:Fibroblastic connective tissue nevus (FCTN) represents a rare and distinct benign Cutaneous mesenchymal Lesion of fibroblastic/myofibroblastic lineage, which broadens the spectrum of Lesions presently recognized as connective tissue nevus. A series of 25 cases of FCTN has been analyzed to further characterize the clinicopathologic spectrum and immunohistochemical features of this entity. Sixteen patients were female (64%) and 9 were male (36%), with age at presentation ranging from 1.5 months to 58 years (median, 10 y). Most patients presented with a solitary, slowly growing, painless plaque-like or nodular skin Lesion. Eleven cases (44%) arose on the trunk, 9 (36%) on the head and neck, and 5 (20%) on the limbs. The Lesion was present for a median duration of 11.5 months (mean, 13.2 mo). Grossly, the Lesions were tan-brown to tan-white, smooth, and firm. Their size ranged from 0.3 to 2.0 cm in greatest dimension (mean size, 0.67 cm; median, 0.6 cm). All tumors showed poor circumscription and were situated primarily in the reticular deep dermis, extending into the superficial subcutis in 13 cases (52%). The Lesion was associated with papillomatous epidermis in 17 cases (70%) and the presence of adipose tissue in the reticular dermis in 14 cases (60.9%). All tumors were composed of a proliferation of bland intradermal fibroblastic/myofibroblastic cells with indistinct palely eosinophilic cytoplasm and tapering nuclei, with no significant cytologic atypia or pleomorphism, arranged in short-intersecting fascicles and entrapping appendages. No mitoses were identified. Immunostains showed positivity for CD34 in 20 of 23 cases (87%) and weak focal positivity for smooth muscle actin in 9 of 19 cases (47%). No case stained positively for desmin or S100 protein. Clinical follow-up was obtained for 14 patients (median duration, 4 y). No tumor recurred locally, even when surgical excision was incomplete. No Lesion metastasized. FCTN occurs most commonly as a plaque on the trunk and head/neck of children, involves deep dermis and superficial subcutis, and stains mainly for CD34. FCTN most likely represents a localized developmental dermal anomaly; it is entirely benign and should not be confused with dermatofibrosarcoma protuberans or other neoplasms such as dermatomyofibroma.
Sebastien De Feraudy - One of the best experts on this subject based on the ideXlab platform.
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fibroblastic connective tissue nevus a rare Cutaneous Lesion analyzed in a series of 25 cases
2012Co-Authors: Sebastien De Feraudy, Christopher D M FletcherAbstract:Fibroblastic connective tissue nevus (FCTN) represents a rare and distinct benign Cutaneous mesenchymal Lesion of fibroblastic/myofibroblastic lineage, which broadens the spectrum of Lesions presently recognized as connective tissue nevus. A series of 25 cases of FCTN has been analyzed to further ch
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Fibroblastic connective tissue nevus: a rare Cutaneous Lesion analyzed in a series of 25 cases
2012Co-Authors: Sebastien De Feraudy, Christopher D M FletcherAbstract:Fibroblastic connective tissue nevus (FCTN) represents a rare and distinct benign Cutaneous mesenchymal Lesion of fibroblastic/myofibroblastic lineage, which broadens the spectrum of Lesions presently recognized as connective tissue nevus. A series of 25 cases of FCTN has been analyzed to further characterize the clinicopathologic spectrum and immunohistochemical features of this entity. Sixteen patients were female (64%) and 9 were male (36%), with age at presentation ranging from 1.5 months to 58 years (median, 10 y). Most patients presented with a solitary, slowly growing, painless plaque-like or nodular skin Lesion. Eleven cases (44%) arose on the trunk, 9 (36%) on the head and neck, and 5 (20%) on the limbs. The Lesion was present for a median duration of 11.5 months (mean, 13.2 mo). Grossly, the Lesions were tan-brown to tan-white, smooth, and firm. Their size ranged from 0.3 to 2.0 cm in greatest dimension (mean size, 0.67 cm; median, 0.6 cm). All tumors showed poor circumscription and were situated primarily in the reticular deep dermis, extending into the superficial subcutis in 13 cases (52%). The Lesion was associated with papillomatous epidermis in 17 cases (70%) and the presence of adipose tissue in the reticular dermis in 14 cases (60.9%). All tumors were composed of a proliferation of bland intradermal fibroblastic/myofibroblastic cells with indistinct palely eosinophilic cytoplasm and tapering nuclei, with no significant cytologic atypia or pleomorphism, arranged in short-intersecting fascicles and entrapping appendages. No mitoses were identified. Immunostains showed positivity for CD34 in 20 of 23 cases (87%) and weak focal positivity for smooth muscle actin in 9 of 19 cases (47%). No case stained positively for desmin or S100 protein. Clinical follow-up was obtained for 14 patients (median duration, 4 y). No tumor recurred locally, even when surgical excision was incomplete. No Lesion metastasized. FCTN occurs most commonly as a plaque on the trunk and head/neck of children, involves deep dermis and superficial subcutis, and stains mainly for CD34. FCTN most likely represents a localized developmental dermal anomaly; it is entirely benign and should not be confused with dermatofibrosarcoma protuberans or other neoplasms such as dermatomyofibroma.
Philip W Allen - One of the best experts on this subject based on the ideXlab platform.
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Kaposiform Hemangioendothelioma: Five Patients with Cutaneous Lesion and Long Follow-Up
2001Co-Authors: Fernand Mac-moune Lai, Paul C L Choi, Ping Chung Leung, Shekhar M Kumta, Patrick P M Yuen, Wing Yin Lam, Annie N Y Cheung, Philip W AllenAbstract:Kaposiform hemangioendothelioma is a rare locally aggressive vascular tumor of the skin, deep soft tissue, and bone in children, characterized by infiltrating nodules and sheets of spindle cells, and unmistakable resemblance to Kaposi’s sarcoma. More than 60 patients with such tumor have been reported so far, and while many have died as a result of extensive disease and severe coagulopathy, the long-term biologic behavior of this tumor remains undetermined. We describe five patients with kaposiform hemangioendothelioma and a mean follow-up of 19 years, ranging from 8 to 35 years. This report emphasizes on the importance of Cutaneous Lesions being the most commonly affected site, but also for its clinical diversity. Early diagnosis is possible even for a small skin Lesion, which may be critical for the treatment of a potentially fatal deep-seated extensive tumor. All five patients are well, and three of them with persistent vascular tumor, which has carried two patients from childhood to adult. Although the behavior of this tumor might have been modified by radiation or interferon in three patients, this series indicates that kaposiform hemangioendothelioma is incapable of metastasis, despite a protracted course of many decades with no tendency for spontaneous regression.
Peter Kind - One of the best experts on this subject based on the ideXlab platform.
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radiation lentigo a distinct Cutaneous Lesion after accidental radiation exposure
1997Co-Authors: Ralf U Peter, Petra Gottlober, Natalia Nadeshina, Gertraud Krahn, Gerd Plewig, Peter KindAbstract:Background: Accidental exposure of skin to ionizing radiation leads to long-term alterations such as fibrosis, keratosis, and teleangiectasias. Also, noncharacteristic hyperpigmentation and hypopigmentation may be noted. Observations: A distinct Lesion is described on the calves of a white male survivor of the 1986 nuclear accident at Chernobyl, Ukraine. Several years after the accident at Chernobyl, characteristic pigmented macules developed in the areas of skin that had previously been exposed to ionizing radiation: there was a marked, sharply demarcated lentiginous hyperpigmentation of epidermal and basal keratinocytes and melanocytes, as well as an increase in the number of melanocytes. No cellular atypia was noted. Conclusions: This case demonstrates the potential of high single doses of ionizing radiation to induce pigmented Lesions with similar clinical and histological features as they have been described after exposure to natural UV radiation or radiation from a tanning bed or sunlamp or after therapy with oral psoralen with long-wave UV-A radiation (PUVA), described as solar, tanning bed, and PUVA lentigines. The absence of cellular atypia may account for a favorable prognosis and enables clear distinction from more serious diagnoses such as lentigo maligna melanoma. Arch Dermatol. 1997;133:209-211
Fernand Mac-moune Lai - One of the best experts on this subject based on the ideXlab platform.
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Kaposiform Hemangioendothelioma: Five Patients with Cutaneous Lesion and Long Follow-Up
2001Co-Authors: Fernand Mac-moune Lai, Paul C L Choi, Ping Chung Leung, Shekhar M Kumta, Patrick P M Yuen, Wing Yin Lam, Annie N Y Cheung, Philip W AllenAbstract:Kaposiform hemangioendothelioma is a rare locally aggressive vascular tumor of the skin, deep soft tissue, and bone in children, characterized by infiltrating nodules and sheets of spindle cells, and unmistakable resemblance to Kaposi’s sarcoma. More than 60 patients with such tumor have been reported so far, and while many have died as a result of extensive disease and severe coagulopathy, the long-term biologic behavior of this tumor remains undetermined. We describe five patients with kaposiform hemangioendothelioma and a mean follow-up of 19 years, ranging from 8 to 35 years. This report emphasizes on the importance of Cutaneous Lesions being the most commonly affected site, but also for its clinical diversity. Early diagnosis is possible even for a small skin Lesion, which may be critical for the treatment of a potentially fatal deep-seated extensive tumor. All five patients are well, and three of them with persistent vascular tumor, which has carried two patients from childhood to adult. Although the behavior of this tumor might have been modified by radiation or interferon in three patients, this series indicates that kaposiform hemangioendothelioma is incapable of metastasis, despite a protracted course of many decades with no tendency for spontaneous regression.
