DP12 Angioinvasive lympho matoid papulosis: a raresubtype easily mistaken for a high-gradeangioinvasive lymphoma
Published Date: 14th March 2017
Publication Authors: Walsh ML
Abstract
A 59-year-old woman presented with painful ulceration on her chin. She described that it began as a nodule that then ulcerated. On examination she had a well-defined area of ulceration measuring 2 x 2 cm with haemorrhagic crust resembling eschar. There was no lymphadenopathy. She was treated with prednisolone and the area rapidly healed with scarring. On stopping her prednisolone, two new lesions appeared on her left breast. The clinical impression was of pyoderma gangrenosum. Her full blood count, urea and electrolytes, liver function tests and autoantibodies were all negative. A computed tomography scan demonstrated two lung nodules. These appeared inflammatory in appearance, and serial scans revealed no change; she was subsequently discharged from the respiratory team. An incisional biopsy of the breast lesion revealed extensive ischaemic ulceration of the epidermis and superficial dermis. Beneath this area of ulceration, there was a dense T-cell lymphoid infiltrate, rich in eosinophils. A prominent angiocentric pattern was noted with many large atypical nuclei surrounding and infiltrating the blood vessel wall. These cells showed strong CD30 positivity. There was
a mixture of CD8 and CD4 cells, with the former dominating. Combining the histology of CD30-rich angiocentric T-cell infiltrate and clinical presentation of waxing and waning ulcerated lesions, a diagnosis of lymphomatoid papulosis (LyP) type E was made. She was subsequently put back on prednisolone, and as her dose was reduced methotrexate was added. She remains clear on methotrexate. LyP is a rare skin disorder belonging to the spectrum of primary cutaneous CD30+ lymphoproliferative disorders. In 2013, Kempf et al. described a clinically and morphologically distinct variant called type E (or angioinvasive) LyP (Kempf W, Kazakov DV, Scharer L et al. Angioinvasive lymphomatoid papulosis: a new variant simulating aggressive lymphomas. Am J Surg Pathol 2013; 37: 1-13). Clinically, it presents as waxing-waning papulonodular lesions, which rapidly turn into haemorrhagic necrotic ulcers. As the clinical presentation is rather different from the other forms of LyP, the clinical differential often includes pyoderma gangrenosum, ulcerative herpes or orf. Histologically LyP type E shows features simulating a highgrade angiocentric and angiodestructive T-cell lymphoma. Accurate categorization depends on the typical histology combined with a history of spontaneous resolution. Most lesions show spontaneous resolution. More resistant cases show a positive response to methotrexate or psoralen-ultraviolet A. This case highlights a newly described disease entity. Histologically it can simulate a high-grade lymphoma, and therefore it is vital that dermatologists and pathologists are aware of this new histological subtype of LyP.
Trehan, P; Bakshi, A; Walsh, M; Azurdia, R. (2016). DP12 Angioinvasive lympho matoid papulosis: a raresubtype easily mistaken for a high-gradeangioinvasive lymphoma . British Journal of Dermatology. 175 (Suppl S1), 138
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