A Severe Case of Lymphomatoid Papulosis Type E Successfully Treated with Interferon-Alfa 2a

Abstract

Lymphomatoid papulosis (LyP) is a benign papulonodular skin eruption with histologic features of malignant lymphoma. A new variant of LyP which was termed “type E” was recently described with similar clinical and histological features to angiocentric and angiodestructive T-cell lymphoma. LyP type E is characterized with recurrent papulonodular lesions which rapidly turn into hemorrhagic necrotic ulcers and spontaneous regression by leaving a scar. None of the available treatment modalities affects the natural course of LyP. For therapy various modalities have been used such as topical and systemic steroids, PUVA, methotrexate, bexarotene, and IFN alfa-2b. Here we present a severe and devastating case with a very rare variant of LyP type E, which is, to our knowledge, the first case successfully treated with IFN alfa-2a. Now disease has been maintaining its remission status for six months.

Angiodestructive lymphomatoid papulosis lasting more than 45 years

“…To our knowledge, our case shows the longest course of LyP type E reported to date 2, 6. The disease course is benign and indolent despite its aggressive, malignant appearance on histology, which highlights the importance of clinicopathologic correlation for diagnosis of angiodestructive LyP.…”

“…Bexarotene and topical mechlorethamine, both of which are approved by the US Food and Drug Administration for mycosis fungoides/Sézary syndrome, can also be used in cases of extensive LyP 10 . Case studies have also shown successful management of LyP using interferon alfa-2a 2 . For cases refractory to methotrexate, national guidelines suggest low-dose brentuximab vedotin 10…”

“…LyP belongs to a spectrum of CD30 + lymphoproliferative disorders, which also includes primary cutaneous anaplastic large cell lymphoma. Although it is associated with mycosis fungoides, primary cutaneous anaplastic large cell lymphoma, systemic anaplastic large cell lymphoma, Hodgkin disease, 2 chronic lymphocytic leukemia, 3 multiple myeloma, and myelodysplastic syndrome, 4 the disease itself is considered benign.…”

Primary cutaneous peripheral T‐cell lymphoma, not otherwise specified, associated with lymphomatoid papulosis after a 9‐year follow up: A case report

“…However, its histology and immunophenotypes were consistent with LyP, and were clearly different from the later lymphoma. A severe clinical course, in terms of a large tumor size and an increased number of ulcerative lesions sized more than 2 cm, has been reported in LyP type E 10,11 . Although angiodestruction was not apparent in the present case, other types of LyP may also present with severe clinical characteristics; this aspect is important to consider to prevent overdiagnosis.…”

Lymphomatoid papulosis types D and E: a multicentre series of the French Cutaneous Lymphomas Study Group

“…14 LyP E is an even rarer subtype, with 22 cases having been described since its first description in 2013. 7,[20][21][22][23][24] The reported cases show a male predominance, with an age range of 8-77 years. 7,[20][21][22][23][24] In our series, we confirm the previously described clinical aspects of the lesions, i.e.…”