RESUMEN
Angiokeratomas are vascular malformations that usually appear as multiple or solitary cutaneous plaques. Several clinical variants have been described, with the same underlying histopathological lesion. Mucosal involvement, including the oral cavity, is occasionally found either as a component of the systemic variety, called angiokeratoma corporis diffusum, or associated with cutaneous lesions in more locations. Isolated oral involvement seems to be rather infrequent and only five cases have been described in adults in the world literature. We herein report another case of this rare entity affecting a 62-year-old woman in the dorsum at the tip of the tongue. This is the first report including an immunohistochemical study to discard a lymphatic origin of the tumor.
Asunto(s)
Angioqueratoma/patología , Neoplasias de la Lengua/patología , Adulto , Angioqueratoma/inmunología , Angioqueratoma/metabolismo , Antígenos CD34/metabolismo , Diagnóstico Diferencial , Femenino , Humanos , Inmunohistoquímica , Linfangioma/diagnóstico , Persona de Mediana Edad , Molécula-1 de Adhesión Celular Endotelial de Plaqueta/metabolismo , Neoplasias de la Lengua/inmunología , Neoplasias de la Lengua/metabolismo , Proteínas de Transporte Vesicular/metabolismoRESUMEN
BACKGROUND: Pseudolymphomatous angiokeratoma (PA), originally termed 'acral pseudolymphomatous angiokeratoma of children', is a disorder characterized clinically by development of red nodules on the extremities and histologically by a subepidermal dense lymphocyte infiltrate. METHODS: We report three cases of PA, with characteristically dense, nodular infiltrate composed predominantly of small lymphocytes, and thick-walled vessels. RESULTS: Immunohistochemical investigation revealed a dense accumulation of CD20+ cells with CD3+ cells in one case. Infiltrate in the other two cases was mainly composed of CD3+ cells and a mixture of CD4+ and CD8+ cells, with a few cells expressing CD20. CONCLUSION: Our immunohistological results reveal a wide spectrum of cellular infiltrate compositions ranging from T-cell to B-cell predominance.
Asunto(s)
Angioqueratoma/patología , Linfocitos B/patología , Neoplasias Cutáneas/patología , Linfocitos T/patología , Adulto , Angioqueratoma/inmunología , Linfocitos B/inmunología , Niño , Femenino , Humanos , Inmunidad Celular/inmunología , Inmunohistoquímica , Neoplasias Cutáneas/inmunología , Linfocitos T/inmunología , Adulto JovenRESUMEN
Immunohistochemical staining for factor VIII-related antigen (FVIII-RAG) with the peroxidase-antiperoxidase technic was used as a marker for endothelial cells in a variety of nevoid, reactive, and malignant vascular cutaneous proliferations. The endothelial cells of small normal cutaneous vessels gave the strongest reaction. The cells of hemangioma and angiokeratoma generally were stained, but with less consistency. In lymphangioma, pyogenic granuloma, and pigmented purpuric dermatosis, positivity was either patchy or of lesser intensity. Numerous strongly positive endothelial cells lining well-formed blood vessels were present in lesions of Kaposi's sarcoma, but the proliferating spindle cells forming "vascular slits" were uniformly negative. The cells of angiosarcoma were essentially negative, except for isolated elements in the better-differentiated areas. Plasma was also strongly positive; this may aid in distinguishing vascular from lymphatic channels. Anti-FVIII-RAG immunoperoxidase staining is a helpful aid in evaluating cutaneous vascular proliferations.
Asunto(s)
Antígenos/inmunología , Endotelio/inmunología , Factor VIII/inmunología , Piel/irrigación sanguínea , Enfermedades Vasculares/inmunología , Angioqueratoma/inmunología , Vasos Sanguíneos/inmunología , Endotelio/patología , Granuloma/inmunología , Hemangioma/inmunología , Humanos , Técnicas para Inmunoenzimas , Linfangioma/inmunología , Linfangiosarcoma/inmunología , Púrpura/inmunología , Sarcoma de Kaposi/inmunología , Enfermedades de la Piel/inmunología , Enfermedades de la Piel/patología , Neoplasias Cutáneas/inmunología , Neoplasias Cutáneas/patología , Enfermedades Vasculares/patología , Factor de von WillebrandRESUMEN
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.
Asunto(s)
Angioqueratoma/patología , Seudolinfoma/patología , Enfermedades de la Piel/patología , Angioqueratoma/inmunología , Niño , Femenino , Humanos , Seudolinfoma/inmunología , Enfermedades de la Piel/inmunologíaRESUMEN
Acral pseudolymphomatous angiokeratoma of children (APACHE) is a new clinical entity that is characterized by angiomatous papules on the extremities. We report a case APACHE in a 14-year-old Japanese girl with asymptomatic red and violaceous papules and nodules on the ring finger of the left hand. Histological and immunohistochemical studies of our patient indicated that APACHE is a pseudolymphoma rather than an angiokeratoma.
Asunto(s)
Angioqueratoma/patología , Neoplasias Cutáneas/patología , Adolescente , Angioqueratoma/inmunología , Antígenos de Diferenciación de Linfocitos T/análisis , Femenino , Dedos/patología , Humanos , Liquen Plano/patología , Linfoma/inmunología , Linfoma/patología , Piel/patología , Neoplasias Cutáneas/inmunologíaRESUMEN
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.