Abstract
CD30+ primary cutaneous T-cell lymphoproliferative disorders have been classified on the basis of their clinical behavior into lymphomatoid papulosis (LyP), primary cutaneous anaplastic large cell lymphoma (pcALCL), and borderline cases. LyP typically has a recurrent, self-healing course, with an excellent prognosis whereas pcALCL has the potential to spread to regional lymph nodes and must be distinguished from systemic disease. Intralymphatic cutaneous ALCL/LyP is a rare and newly described entity, with only one case series in the literature, which highlights the surprisingly indolent nature of this entity. Herein, we report a 79-year-old male with a history of Kaposis sarcoma, multiple non-melanoma skin cancers, and recurrent multifocal CD30+ lymphoproliferative lesions previously treated with radiotherapy from 2015-2019. He presented with several new self-resolving nodules on the scalp and upper trunk ranging from 0.5 to 1.5 cm, which were biopsied demonstrating dense CD30+ mononuclear cells resembling pcALCL but behaving clinically like LyP. The patient then underwent radical scalp excision which demonstrated multiple loci of a dense nodular proliferation of atypical CD30+/ALK- mononuclear cells with extensive invasion of the superficial and deep lymphatic vasculature, as highlighted by CD31 and D2-40 immunostains. A diagnosis of intralymphatic ALCL/LyP was made. In contrast to other intravascular lymphomas which are usually aggressive clinically, his case like those reported has followed a much more indolent course. Therefore, our case seeks to bring further awareness to this newly described entity and highlight a lymphotropic behavior recently observed among cutaneous CD30+ lymphoproliferative disorders, which is not necessarily associated with a bad prognosis.
Financial Disclosure:
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