Background: Despite common endothelial origins, angiosarcoma and Kaposi’ s sarcoma are clinically and histologically distinct vascular proliferations. The development of angiosarcoma in a chronically edematous abdomi...Background: Despite common endothelial origins, angiosarcoma and Kaposi’ s sarcoma are clinically and histologically distinct vascular proliferations. The development of angiosarcoma in a chronically edematous abdominal pannus is extremely uncommon. Similarly, tumors with the combined histologic features of angiosarcoma and Kaposi’ s sarcoma have rarely been described. Methods: We reviewed the literature on angiosarcoma arising in a lymphedematous abdominal pannus and evaluated an 81-year-old morbidly obese woman who had profound, long-standing edema of the lower abdominal wall in which an aggressive vascular tumor developed. Results: Three clinically similar cases were identified in the literature. All patients were women who generally experienced rapid disease progression. In addition, in our patient, sequential cutaneous sampling from different lesional sites demonstrated disparate histologic changes, ranging from those of classic Kaposi’ s sarcoma to high-grade angiosarcoma, to areas with combined features of the two tumors. A polymerase chain reaction performed on lesional tissue was negative for human herpesvirus-8 DNA. Conclusion: It is important to note that angiosarcoma may develop in the abdomen in association with chronic lymphedema, as demonstrated by the cases noted in this report. In addition, our case highlights the difficulty in differentiating histologically angiosarcoma from Kaposi’ s sarcoma in some situations, and demonstrates the value of close clinicopathologic correlation and sequential tissue sampling in evaluating problematic cases.展开更多
We describe a 53-year-old white female with distinctive clinical and histologic changes of involutional lipoatrophy on the right elbow that developed 6 months following a steroid injection to the site. Morphologically...We describe a 53-year-old white female with distinctive clinical and histologic changes of involutional lipoatrophy on the right elbow that developed 6 months following a steroid injection to the site. Morphologically, immunohistochemically and ultrastructurally macrophages were seen in close proximity to the altered adipocytes. The macrophages displayed an activated phenotype andwere observed engulfing segments of altered adipose and stromal tissue. Other inflammatory cells were lacking and up-regulation of the intra-epidermal Langerhan cells and dermal dendrocytic populations were not observed suggesting non-immune causal mechanisms in the disease process. We believe that the progression model of involutional lipoatrophy is similar to that of glomerulonephritis in which macrophage accumulation can result from non-immune mechanisms such as trauma and lead to the production of several cytokines that induce tissue injury and subsequent fibrosis and sclerosis.展开更多
A 4- year- old Caucasian girl with pili trianguli et canaliculi had distinctive findings. These included unique ultrastructural alterations consisting of tonofilament- desmosomal detachment and tonofilament clumping w...A 4- year- old Caucasian girl with pili trianguli et canaliculi had distinctive findings. These included unique ultrastructural alterations consisting of tonofilament- desmosomal detachment and tonofilament clumping within inner root sheath cells. We believe that the hair anomaly in this condition may be due to a compromised cytoskeleton with subsequent configurational changes of the inner root sheath. Despite these configurational changes keratinization of the inner root sheath occurs. As inner root sheath keratinization typically precedes keratinization of the hair shaft, the abnormally configured inner root sheath determines the surface characteristics of the hair shaft in pili trianguli et canaliculi.展开更多
Background: Diffuse dermal angiomatosis is rare and usually considered a variant of reactive angioendotheliomatosis. It generally involves the extremities of patients with severe vascular disease and other comorbiditi...Background: Diffuse dermal angiomatosis is rare and usually considered a variant of reactive angioendotheliomatosis. It generally involves the extremities of patients with severe vascular disease and other comorbidities. Two patients with breast involvement have been described; however, neither had a relevant medical history or a vaso-occlusive disorder, but both had large pendulous breasts, and 1 was positive for IgM anticardiolipin and antinuclear antibodies. Observations: A 53-year-old woman had a reticulated, erythematous plaque with superficial ulceration and underlying tender nodules on her left breast. She had a history of cardiovascular disease and was a heavy smoker. Biopsy of the lesion showed diffuse proliferation of additional endothelial cells and small bland vessels within the papillary and upper reticular dermis. Angiography showed almost complete occlusion of the subclavian artery proximally. Diffuse dermal angiomatosis was diagnosed. With isotretinoin therapy, the lesions improved.One month later, after percutaneous subclavian arterial revascularization, the lesion resolved completely. A literature review suggested that a history of heavy smoking, in addition to a history of vascular disease, may be important in the pathogenesis of diffuse dermal angiomatosis. Conclusions: Clinical acumen is crucial to diagnose diffuse angiomatosis of the breast. Appropriate treatment to alleviate hypoxiamay improve the patient’ s condition.展开更多
Background: Skin is involved in 25% of cases of sarcoidosis. The lesions are specific and nonspecific depending on the presence or absence of granulomas, respectively. Specific lesions are not thought to have prognost...Background: Skin is involved in 25% of cases of sarcoidosis. The lesions are specific and nonspecific depending on the presence or absence of granulomas, respectively. Specific lesions are not thought to have prognostic significance and are not associated with systemic disease. Objective: We sought to evaluate for the presence or absence of systemic disease in patients with subcutaneous sarcoidosis. Methods: With diagnostic criteria of subcutaneous sarcoidosis, 33 cases were identified in the literature and 21 cases in our institutional database. A retrospective clinical and pathologic review of these cases was conducted. Results: Subcutaneous sarcoidosis is characterized by a peak incidence during the fourth decade; female predisposition; asymptomatic to slightly tender lesions typically involving the upper extremities; cutaneous lesional clustering and multiplicity; autoimmune disease associations at time of diagnosis in a subset of cases; systemic disease associations at diagnosis in most patients, typically consisting of bilateral hilar adenopathy; and a favorable response to oral corticosteroid therapy. Limitations: Retrospective analysis with inadequate documentation of therapeutic regimens and their responses in some cases is a limitation of this study. Conclusions: The confirmatory diagnosis of subcutaneous sarcoidosis depends on identifying pannicular noninfectious sarcoidal or epithelioid granulomas with minimal lymphocytic inflammation. Subcutaneous sarcoidosis is the only specific subset of cutaneous sarcoidosis frequently associated with systemic disease.展开更多
Mammary-like glands are a poorly recognized structure of normal vulvar skin. Adenocarcinomas arising from these glands are rare and represent a distinct clinicopathologic entity that must be distinguished from metasta...Mammary-like glands are a poorly recognized structure of normal vulvar skin. Adenocarcinomas arising from these glands are rare and represent a distinct clinicopathologic entity that must be distinguished from metastatic tumors. We reviewed 19 cases reported in the literature and describe a 51-year-old patient with a long-standing nodule on her right interlabial sulcus. Excisional biopsy and Mohs micrographic surgery demonstrated an infiltrating adenocarcinoma of the mammary-like glands involving the dermis and subcutis. On review of all 20 cases, the mean age at diagnosis was 59.6 years, the labia majora were involved in 13 cases (65%), and the mean lesional size was 2.5 cm. The tumor’s histologic patterns varied greatly, although the histomorphologic similarity among this neoplasm, sweat gland carcinomas, and invasive extramammary Paget disease suggested a morphologic spectrum among these tumors. Criteria for establishing the diagnosis of adenocarcinoma of mammarylike glands include identifying transition zones between normal mammary-like glands and adenocarcinomatous areas. This tumor is best categorized as a locally aggressive neoplasm with a low risk for widespread metastasis. Use of aggressive surgical therapeutic regimens, particularly in the case of tumors localized to the skin, must be reassessed, especially given the likelihood of long-term morbidity with such regimens. In our patient, successful Mohs micrographic surgery suggests that alternate management may be adopted for tumors localized to the skin, especially in elderly patients.展开更多
文摘Background: Despite common endothelial origins, angiosarcoma and Kaposi’ s sarcoma are clinically and histologically distinct vascular proliferations. The development of angiosarcoma in a chronically edematous abdominal pannus is extremely uncommon. Similarly, tumors with the combined histologic features of angiosarcoma and Kaposi’ s sarcoma have rarely been described. Methods: We reviewed the literature on angiosarcoma arising in a lymphedematous abdominal pannus and evaluated an 81-year-old morbidly obese woman who had profound, long-standing edema of the lower abdominal wall in which an aggressive vascular tumor developed. Results: Three clinically similar cases were identified in the literature. All patients were women who generally experienced rapid disease progression. In addition, in our patient, sequential cutaneous sampling from different lesional sites demonstrated disparate histologic changes, ranging from those of classic Kaposi’ s sarcoma to high-grade angiosarcoma, to areas with combined features of the two tumors. A polymerase chain reaction performed on lesional tissue was negative for human herpesvirus-8 DNA. Conclusion: It is important to note that angiosarcoma may develop in the abdomen in association with chronic lymphedema, as demonstrated by the cases noted in this report. In addition, our case highlights the difficulty in differentiating histologically angiosarcoma from Kaposi’ s sarcoma in some situations, and demonstrates the value of close clinicopathologic correlation and sequential tissue sampling in evaluating problematic cases.
文摘We describe a 53-year-old white female with distinctive clinical and histologic changes of involutional lipoatrophy on the right elbow that developed 6 months following a steroid injection to the site. Morphologically, immunohistochemically and ultrastructurally macrophages were seen in close proximity to the altered adipocytes. The macrophages displayed an activated phenotype andwere observed engulfing segments of altered adipose and stromal tissue. Other inflammatory cells were lacking and up-regulation of the intra-epidermal Langerhan cells and dermal dendrocytic populations were not observed suggesting non-immune causal mechanisms in the disease process. We believe that the progression model of involutional lipoatrophy is similar to that of glomerulonephritis in which macrophage accumulation can result from non-immune mechanisms such as trauma and lead to the production of several cytokines that induce tissue injury and subsequent fibrosis and sclerosis.
文摘A 4- year- old Caucasian girl with pili trianguli et canaliculi had distinctive findings. These included unique ultrastructural alterations consisting of tonofilament- desmosomal detachment and tonofilament clumping within inner root sheath cells. We believe that the hair anomaly in this condition may be due to a compromised cytoskeleton with subsequent configurational changes of the inner root sheath. Despite these configurational changes keratinization of the inner root sheath occurs. As inner root sheath keratinization typically precedes keratinization of the hair shaft, the abnormally configured inner root sheath determines the surface characteristics of the hair shaft in pili trianguli et canaliculi.
文摘Background: Diffuse dermal angiomatosis is rare and usually considered a variant of reactive angioendotheliomatosis. It generally involves the extremities of patients with severe vascular disease and other comorbidities. Two patients with breast involvement have been described; however, neither had a relevant medical history or a vaso-occlusive disorder, but both had large pendulous breasts, and 1 was positive for IgM anticardiolipin and antinuclear antibodies. Observations: A 53-year-old woman had a reticulated, erythematous plaque with superficial ulceration and underlying tender nodules on her left breast. She had a history of cardiovascular disease and was a heavy smoker. Biopsy of the lesion showed diffuse proliferation of additional endothelial cells and small bland vessels within the papillary and upper reticular dermis. Angiography showed almost complete occlusion of the subclavian artery proximally. Diffuse dermal angiomatosis was diagnosed. With isotretinoin therapy, the lesions improved.One month later, after percutaneous subclavian arterial revascularization, the lesion resolved completely. A literature review suggested that a history of heavy smoking, in addition to a history of vascular disease, may be important in the pathogenesis of diffuse dermal angiomatosis. Conclusions: Clinical acumen is crucial to diagnose diffuse angiomatosis of the breast. Appropriate treatment to alleviate hypoxiamay improve the patient’ s condition.
文摘Background: Skin is involved in 25% of cases of sarcoidosis. The lesions are specific and nonspecific depending on the presence or absence of granulomas, respectively. Specific lesions are not thought to have prognostic significance and are not associated with systemic disease. Objective: We sought to evaluate for the presence or absence of systemic disease in patients with subcutaneous sarcoidosis. Methods: With diagnostic criteria of subcutaneous sarcoidosis, 33 cases were identified in the literature and 21 cases in our institutional database. A retrospective clinical and pathologic review of these cases was conducted. Results: Subcutaneous sarcoidosis is characterized by a peak incidence during the fourth decade; female predisposition; asymptomatic to slightly tender lesions typically involving the upper extremities; cutaneous lesional clustering and multiplicity; autoimmune disease associations at time of diagnosis in a subset of cases; systemic disease associations at diagnosis in most patients, typically consisting of bilateral hilar adenopathy; and a favorable response to oral corticosteroid therapy. Limitations: Retrospective analysis with inadequate documentation of therapeutic regimens and their responses in some cases is a limitation of this study. Conclusions: The confirmatory diagnosis of subcutaneous sarcoidosis depends on identifying pannicular noninfectious sarcoidal or epithelioid granulomas with minimal lymphocytic inflammation. Subcutaneous sarcoidosis is the only specific subset of cutaneous sarcoidosis frequently associated with systemic disease.
文摘Mammary-like glands are a poorly recognized structure of normal vulvar skin. Adenocarcinomas arising from these glands are rare and represent a distinct clinicopathologic entity that must be distinguished from metastatic tumors. We reviewed 19 cases reported in the literature and describe a 51-year-old patient with a long-standing nodule on her right interlabial sulcus. Excisional biopsy and Mohs micrographic surgery demonstrated an infiltrating adenocarcinoma of the mammary-like glands involving the dermis and subcutis. On review of all 20 cases, the mean age at diagnosis was 59.6 years, the labia majora were involved in 13 cases (65%), and the mean lesional size was 2.5 cm. The tumor’s histologic patterns varied greatly, although the histomorphologic similarity among this neoplasm, sweat gland carcinomas, and invasive extramammary Paget disease suggested a morphologic spectrum among these tumors. Criteria for establishing the diagnosis of adenocarcinoma of mammarylike glands include identifying transition zones between normal mammary-like glands and adenocarcinomatous areas. This tumor is best categorized as a locally aggressive neoplasm with a low risk for widespread metastasis. Use of aggressive surgical therapeutic regimens, particularly in the case of tumors localized to the skin, must be reassessed, especially given the likelihood of long-term morbidity with such regimens. In our patient, successful Mohs micrographic surgery suggests that alternate management may be adopted for tumors localized to the skin, especially in elderly patients.
