Zusammenfassung
Prim?r kutane B-Zell-Lymphome (primary cutaneous B-cell lymphomas: PCBCL) stellen extranodale Non-Hodgkin-Lymphome reifer B-Lymphozyten dar, die die Haut als Zielorgan haben und dort proliferieren. Sie zeigen eine gro?e Bandbreite klinischer und histologischer Erscheinungsformen. Ca. 22?% der kutanen Lymphome (cutaneous lymphomas: CL) entfallen auf kutane B-Zell-Lymphome (cutaneous B-cell lymphomas: CBCL), 73?% auf kutane T-Zell-Lymphome (cutaneous T-cell lymphomas: CTCL) und 10?% werden seltenen Formen von CL zugeordnet. Die Inzidenz der CL ist weiterhin zunehmend und wird basierend auf den Daten aus den USA auf eine Neuerkrankung pro Jahr und 100?000 Einwohner gesch?tzt.
Die Diagnose eines PCBCL erfolgt in der Regel anhand klinischer und histologischer bzw. immunhistologischer Untersuchungen. Nicht immer jedoch erlauben diese Untersuchungen eine zweifelsfreie Diagnose. Vor allem fr?he Stadien eines PCBCL ?hneln sowohl klinisch als auch histologisch oft benignen entz?ndlichen Hauterkrankungen. Aufgrund der Heterogenit?t der PCBCL mit unterschiedlicher biologischer Potenz, die von einem indolenten Verlauf bis hin zu einem fatalen Verlauf reicht, ist eine eindeutige Diagnosestellung unabdingbar. Hier spielt insbesondere die Dermatohistologie, in Kombination mit der Immunhistologie und Molekularbiologie, eine entscheidende Rolle.
We describe a case of neutrophil rich variant of primary cutaneous anaplastic large cell lymphoma (PCALCL) and the clinicopathological features of cases reported in the literature. In all cases the male to female ratio is 1.4?:?1. The age at presentation ranges between 12 and 82 years with a mean age of 50.73 years. Various clinical lesions including nodules (47.8?%), tumormasses (34.8?%), plaques (21.7?%) and unusual variants (13.4?%) are noted. 47.8?% of lesions are associated with ulceration. Multifocal lesions are seen in about 18.4?% of cases. Lesions are located in extremities (44.7?%), trunk (36.8?%), head and neck (34.2?%). Around 10.5?% of patients are immunocompromised. About 5.3?% of cases show peripheral neutrophilia. Histopathological examinations show diffuse dermal infiltrate of large anaplastic (53.5?%), pleomorphic (27.9?%) or small to medium sized atypical lymphocytes (18.6?%) in inflammatory background. Subcutaneous tissues are involved in about 43.5?% of biopsies. Some biopsies show epidermal changes as epidermotropism (32.6?%), hyperplasia (20.9?%) or ulceration (7?%). 18.6?% of specimens show necrosis and angiodestruction. We try to increase awareness of this rare variant.
A 78-year-old patient presented with multiple indolent scrotal vesicles that had fi rst appeared more than a year earlier.There was no evidence of allergic contact dermatitis. The patient denied taking any new drugs or having experienced B symptoms. He had a history of a left-sided testicular tumor (1968) for which he had undergone orchiectomy, radical lymphadenectomy, and cobalt radiation. Said treatment had led to complete remission.
Clinical findingsClinical examination revealed multiple clear scrotal vesicles, measuring 2-3 mm in diameter. Some of the vesicles had a yellowish hue. One day after a diagnostic punch biopsy (4 mm), the previously clear, bright lesions had turned into multiple, black vesicles on an erythematous-violaceous base (Figure 1 ).
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