1/64. 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. ( info) |
2/64. Angiokeratomas in juvenile dermatomyositis. Juvenile dermatomyositis (JDM) is an uncommon disease that features muscle weakness, a characteristic rash, and vascular changes in skin, muscle, and other organs. We report a patient with JDM who developed multiple angiokeratomas, one of which was calcified. Electron microscopy of an angiokeratoma revealed altered connective tissue consistent with abnormal collagen. To our knowledge, angiokeratomas occurring in association with JDM have not been previously reported. Abnormal collagen distinguishes the angiokeratomas in our patient from those seen in other conditions. We propose that the angiokeratomas resulted from a progressive compensatory response to the obliterative angiopathy of JDM. ( info) |
3/64. 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. ( info) |
4/64. 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. ( info) |
5/64. Renal metastases simulating Kaposi sarcoma. Radionuclide scanning, an aid in diagnosis and treatment planning. A case of renal cutaneous metastases simulated Kaposi sarcoma and the condition was misdiagnosed as Kaposi sarcoma. A new technique was employed using the isotope technetium 99m as the compound sodium pertechnetate tc 99m for the diagnosis and evaluation of the extent of Kaposi sarcoma. The failure to demonstrate a positive isotope scan in our case was a clue to the incorrect diagnosis of Kaposi sarcoma. ( info) |
6/64. Severe predominantly acral variant of angiokeratoma of Mibelli: response to long-pulse Nd:YAG (1064 nm) laser treatment. The classification of vascular abnormalities is difficult. We describe a child with a disorder that is best described as angiokeratoma of Mibelli. This unusual, severe, predominantly acral case has been improved by long-wave Nd:YAG laser treatment, but treatment is still ongoing. ( info) |
| In 1988, Ramsay et al proposed an entity of acral pseudolymphomatous angiokeratoma of children (with an abbreviation of apache) for the unilateral multiple angiomatous papules affecting the acral region of the extremities of children. We report here similar lesions that developed in the acral portions of 1 female adolescent and 2 women. Histopathologically, they showed pseudolymphomatous features rather than those of angiokeratoma. Thus, the term should be acral angiokeratoma-like pseudolymphoma would be more appropriate than apache originally proposed. ( info) |
| Two men presented with asymptomatic diffuse redness of the scrotum. Examination showed angiokeratoma of Fordyce (AF), an associated finding. In our practice, AF and diffuse scrotal redness co-occur in 50% of patients. Providing reassurance to the patient is appropriate if this clinical link is detected. ( info) |
9/64. 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. ( info) |
10/64. 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. ( info) |