Cases reported "Angiokeratoma"

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1/11. Targetoid hemosiderotic hemangioma- a dynamic vascular tumor: report of 3 cases with episodic and cyclic changes and comparison with solitary angiokeratomas.

    BACKGROUND: Both targetoid hemosiderotic hemangiomas (THH) and solitary angiokeratomas (SAK) are acquired vascular malformations formed by superficial vascular ectasias possibly caused by trauma. OBJECTIVE: We compare the clinicopathologic findings of THHs with those of SAKs and report the clinicopathologic findings of 3 singular cases of THH affected by cyclic or episodic morphologic changes. methods: We performed a clinicopathologic study on 33 cases of THH and compared this group with 20 cases of SAK. On selected cases, histochemical and immunohistochemical analyses were evaluated. RESULTS: Overlap of all the clinical and pathologic features studied were identified for THH and SAK. Clinically, they both commonly exhibited a brown or black papule located over the lower extremities that mimicked a melanocytic lesion. Histologically, they both had ectatic papillary dermal vessels with overlying epidermal hyperplasia, and adjacent hemosiderin deposits, extravasated red blood cells, lymphocytic infiltrate, and lymphangiectases. Compared with SAKs, THHs were significantly larger (5.3 vs 3.2 mm), more often excised (elliptical excision) than shave or punch biopsied, and had deeper dermal vessel alterations, more frequent dissecting vascular spaces, and more extensive hemosiderin deposits (all P < .01). THHs presenting with episodic changes were significantly larger than those without (11 vs 4.4 mm, P =.001). CONCLUSION: THHs and SAKs differ in degree, not in type, of clinicopathologic characteristics. This finding suggests that THHs are larger variants of SAKs whose size is the cause of more extensive, prolonged, or recurrent vessel damage. The histologic findings of extravasated red blood cells, hemosiderin, telangiectases, lymphangiectases, and fibrosis implicate trauma in the cause of these acquired vascular malformations.
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2/11. Plasma cell granuloma of the oral mucosa with angiokeratomatous features: a possible analogue of cutaneous angioplasmocellular hyperplasia.

    We report a plasma cell granuloma arising in the movable mucosa of the oral cavity of a 50-year-old man. Histologically, the lesion was characterized by a dense nodular infiltrate of mature plasma cells. Immunostaining for kappa and lambda light chains confirmed a polyclonal plasma cell population. Elongated rete ridges of the overlying epithelium formed collarettes around dilated blood and lymph vessels in focal areas. Based on the overall histologic architecture, we hypothesize that these peculiar changes are secondary to local blood flow alteration with congestive vasodilation caused by a dense plasma cell infiltrate. We believe that the plasma cell population may represent the oral counterpart of the cutaneous angioplasmocellular hyperplasia.
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3/11. Cutaneous angiolymphoid hyperplasia with high endothelial venules is characterized by endothelial expression of cutaneous lymphocyte antigen.

    Two cases in which the presence of cutaneous lesions with a characteristic admixture of lymphoid hyperplasia and vascular proliferation lead to the diagnosis of so-called acral pseudolymphomatous angiokeratoma (APA) of children are reported. Owing to the prominence of its blood vessels, so striking as to be reminiscent of high endothelial venules (HEVs), APA was initially interpreted as a vascular lesion rather than a pseudolymphoma. This resemblance is further compounded by our finding that cutaneous lymphocyte antigen (CLA), an HEV marker, is also expressed in APA epithelioid blood vessels. Consequently, we believe that "cutaneous angiolymphoid hyperplasia with high endothelial venules" (ALH-HEV), which alludes to the dual nature of proliferating elements and the HEV-like phenotype of the vascular component, would be a better denomination for this entity. Additionally, we speculate that ALH-HEV lymphoid hyperplasia self-perpetuates through the transformation of dermal capillaries into HEVs, which would bind non-skin homing lymphocytes expressing l-selectin and promote their local recruitment and recirculation.
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ranking = 15.16794344657
keywords = blood vessel, vessel
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4/11. Acral pseudolymphomatous angiokeratoma of children: immunohistochemical and clonal analyses of the infiltrating cells.

    BACKGROUND: Acral pseudolymphomatous angiokeratoma of children (apache) is a disorder characterized clinically by red nodules and histopathologically by a massive subepidermal lymphohistiocytic infiltrate. Although it was initially thought to be a vascular nevus, it has never been regarded as a pseudolymphoma. Case report: We report a 7-year-old-girl with small red nodules on the dorsum of the right foot and a 73-year-old man with asymptomatic brown-red nodules on the lower extremities. RESULTS: Histopathologic examination revealed a massive lymphohistiocytic infiltrate with plasma cells, some eosinophils, or a multinucleated giant cell immediately beneath the epidermis. Thick-walled vessels were observed in the infiltrate. These characteristics are identical to those of acral pseudolymphomatous angiokeratoma of children. The infiltrate was composed mainly of equal numbers of CD4 or CD8 T cells and equal numbers of B cells stained for kappa or lambda light chains. PCR amplification of rearranged immunoglobulin heavy chain genes or T-cell receptor gamma genes showed no evidence of clonality, suggesting that these infiltrates were polyclonal both for B and T cells. CONCLUSIONS: Our data support the idea that this disorder represents a reactive process. The modified term 'papular angiolymphoid hyperplasia' would define this disorder more appropriately.
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5/11. Linear acral pseudolymphomatous angiokeratoma of children (apache): further evidence that apache is a cutaneous pseudolymphoma.

    Acral pseudolymphomatous angiokeratoma of children (apache) is a recently recognized, rare clinical entity. We report a case of apache in a 9-year-old Japanese girl with a dark red, linear plaque on the posterior area of the right lower leg. Histopathologic findings revealed further evidence that apache was a cutaneous pseudolymphoma rather than a vascular neoplasm because of the presence of distinct primary and secondary lymphoid follicles, and thick-walled, long blood vessels lined with prominent plump endothelial cells similar to high endothelial venules in the paracortical area of the lymph node. In addition to previous descriptions, the patient presented with a distinct linear lesion. Vacuolar alteration of the basal cell layer and scattered eosinophilic bodies in the epidermis were observed, which suggested a lichenoid tissue reaction. We believe that apache might show a distinct linear pattern accompanied by a lichenoid tissue reaction.
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ranking = 7.583971723285
keywords = blood vessel, vessel
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6/11. A case of galactosialidosis.

    Galactosialidosis is a lysosomal storage disease associated with a combined deficiency of beta-galactosidase and neuraminidase, caused by a defect of another lysosomal protein, the protective protein. Three subtypes are recognized: the early infantile form, the late infantile form and the juvenile/adult form. We saw a patient with galactosialidosis of the juvenile/adult form, a 51-year-old Japanese man with angiokeratomas on both elbows and knees, myoclonus, ataxia, mental retardation and macular cherry-red spots. An electron-microscopic study of a skin biopsy showed membrane-limited vacuoles in the cytoplasm of the endothelial cells, pericytes and fibroblasts. Assays of enzymatic activity in cultured fibroblasts showed a marked decrease in both beta-galactosidase and neuraminidase (sialidase). The substance contained in the cytoplasmic vacuoles appears to be glycoproteins with sialic acid, which is a terminal glycosyl residue, because the cytoplasm of the endothelial cells of the vessels and pericytes are stained by the Limax flavus agglutinin, a lectin that binds specifically with sialic acid. This technology may be useful for easy investigation of the distribution of the accumulation of such substances in the central nervous system.
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7/11. fabry disease: an atypical presentation.

    fabry disease is a rare X-linked recessive lysosomal storage disease. patients typically have angiokeratomas distributed between the umbilicus and knees, painful crises of the hands and feet, and renal, ophthalmologic, and cardiac abnormalities. An 11-year-old boy presented with a 6-year history of widespread petechial-like lesions and painful crises of the hands and feet. On physical examination, he had numerous erythematous, nonblanching pinpoint macules and rare papules with an overlying crust. These lesions were widely distributed on his trunk, palms, and soles, while sparing the area between the umbilicus and knees. Histologic evaluation of one of these lesions found several dilated, blood-filled vessels in the upper dermis beneath a thinned epidermis. The patient also had markedly decreased alpha galactosidase A levels. Although the distribution of the angiokeratomas was atypical, the clinical and histologic findings were consistent with a diagnosis of fabry disease.
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keywords = vessel
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8/11. angiokeratoma of tongue: a series of 14 cases.

    Angiokeratomas (AC) are vascular lesions which are defined histologically as one or more dilated blood vessels lying directly subepidermal and showing an epidermal proliferative reaction with ectatic capillaries in the papillary dermis. Only three other cases of isolated mucosal angiokeratoma have been reported in the indexed literature. We reviewed all cases of angiokeratoma located on the tongue, diagnosed in our department during a study period of 10 years (1995-2005). Histologically all 14 cases showed dilated and congested blood vessels in the upper papillary dermis. They lack deep dermal involvement. Hyperkeratosis and acanthosis were also seen in most of the cases. No clinical data was available to assess systemic disease. A higher incidence of of AC in tongue is seen in our study.
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ranking = 15.16794344657
keywords = blood vessel, vessel
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9/11. Rare case of recurrent angiokeratoma of Fordyce on penile shaft.

    Angiokeratomas are benign cutaneous vascular lesions characterized by dilated thin-walled blood vessels lying in the upper part of the dermis, mostly associated with an epidermal reaction such as acanthosis and/or hyperkeratosis. Angiokeratomas of Fordyce are predominantly located on the scrotum and are only rarely found on the penis and then usually on the glans penis. We report a rare case of angiokeratoma of Fordyce located on the shaft of the penis and associated with two recurrences after appropriate surgical excision.
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ranking = 7.583971723285
keywords = blood vessel, vessel
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10/11. Transepidermal elimination of thrombi in three cases of thrombotic angiokeratoma: an incidental histopathologic finding of angiokeratoma.

    Transepidermal elimination (TEE) is a well known phenomenon by which foreign or altered constituents are removed from the dermis. A thrombosis in the dilated dermal blood vessels of angiokeratoma is not unusual, hence the term thrombotic angiokeratoma. We recently reviewed the histopathologic findings of previously diagnosed cases of angiokeratoma and found that multiple histologic sections of surgical biopsy materials disclosed changes indicating TEE of thrombi in three cases. Special stains searching for an altered substance in those thrombi failed. In this report of three cases, we suggest that this phenomenon could be an incidental and long missed histopathologic finding of angiokeratoma which might explain the fate of these thrombi.
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ranking = 7.583971723285
keywords = blood vessel, vessel
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