Angioinvasive Lymphomatoid Papulosis

Abstract

Lymphomatoid papulosis (LyP) belongs to the spectrum of primary cutaneous CD30-positive lymphoproliferative disorders. Clinically, LyP is characterized by a variable number of self-healing papulo-nodular lesions, with the typical waxing and waning course. Histologically, 4 types (A, B, C, and D) have been delineated. Angioinvasive growth and large ulcers are rare findings in LyP and simulate aggressive lymphoma. We retrospectively analyzed the clinicopathologic and molecular features of angioinvasive LyP in a series of 16 patients. This new form of LyP is characterized by oligolesional papules that rapidly ulcerate and evolve into large necrotic eschar-like lesions with a diameter of 1 to 4 cm and an angiocentric and angiodestructive infiltrate of small-sized to medium-sized atypical lymphocytes expressing CD30 and frequently CD8. As in other forms of LyP, the lesions underwent spontaneous regression after a few weeks. Recurrences were common, but the prognosis was excellent with no extracutaneous spread or disease-related deaths. Complete remission occurred in 9 of 16 patients (56%). This LyP variant should be distinguished from aggressive forms of angiocentric and angiodestructive and cytotoxic T-cell lymphomas. We propose the term LyP type E for this clinically and histologically unusual variant.

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

“…Up to date 18 cases were reported in the literature [3]. All of them had severe clinical features, with a typical progressive course.…”

“…All of them had severe clinical features, with a typical progressive course. During follow-up, they all experienced several relapses like current patient [3]. …”

“…Histopathologically, there are well-known 4 LyP types (type A-wedge-shaped infiltrate containing eosinophils and histiocytes, type B-epidermotropism, resembling mycosis fungoides, type C-cohesive sheets of CD30 (+) cells, resembling anaplastic large cell lymphoma, and type D-CD8 (+), resembling primary cutaneous aggressive epidermotropic CD8 (+) cytotoxic T-cell lymphoma) [2]. A new variant of LyP which was termed “type E” by Kempf et al was recently described with similar clinical and histological features to angiocentric and angiodestructive T-cell lymphoma [3]. …”

Significance of CD30 Expression by Epidermotropic T Cells in Lymphomatoid Papulosis and Lymphomatoid Pityriasis Lichenoides

“…The immunophenotype of these atypical cells was CD8+CD4-CD30+ indicating the possibility of aggressive primary cutaneous epidermotropic CD8+ cytotoxic T-cell lymphoma, also known as Berti's lymphoma. [10,11] The infiltrate was not angiocentric nor was vasculitis present, features characteristic of LyP-E. [12] Epidermotropic T cells were CD8+ and approximately one-half expressed of CD30. Although the patient's disease responded well to low doses of methotrexate, her disease would abruptly recur when methotrexate doses were reduced.…”

Acitretin combined with NB‐UVB in the treatment of cutaneous CD30‐positive anaplastic large cell lymphoma

“…There was no occurrence of erythemas in the follow up 20 months later. (Kempf, 2017;Kempf, Kazakov, Scharer, et al, 2013).…”