The clinical and histological spectrum of lymphomatoid papulosis

Abstract

A review of sixty-four biopsies from sixteen patients with lymphomatoid papulosis revealed two characteristic histological types (type A and type B), which also had a different clinical behaviour. In lymphomatoid papulosis type A lesions, four histological patterns corresponding with the age of the lesion could be distinguished. Such a relationship was not found in type B lesions. The findings of transitional forms in some biopsy specimens, showing histological features of both type A and type B, and the presence of both types in different but concurrent lesions, suggests that these two types are not different entities but rather represent the ends of a spectrum. At least two different populations of atypical cells can be distinguished in lymphomatoid papulosis. Apart from the atypical cerebriform mononuclear cells, which are T-lymphocytic in origin and predominant in type B lesions, large atypical cells with vesicular nuclei, prominent nucleoli and abundant cytoplasm are found, particularly in type A lesions. Preliminary immunohistochemical and cytochemical investigations suggest that these cells are not lymphoid in origin, but are related to the Langerhans cell series.

Single-cell analysis of CD30+ cells in lymphomatoid papulosis demonstrates a common clonal T-cell origin

“…Furthermore, patients with associated lymphoma were not representative of the vast majority of patients with LyP, because transformation into malignant lymphoma only rarely occurs. 4 A total of 387 single CD30 ϩ cells isolated from 14 biopsies gave rise to 123 specific PCR products. Nucleotide sequence analyses of all PCR products showed identical TCR-␥ gene rearrangements within each individual case.…”

“…From 10% to 20% of LyP cases are associated with malignant lymphoma, especially mycosis fungoides, CD30 ϩ cutaneous ALCL, or Hodgkin disease, which can precede, coexist with, or follow LyP and can also appear in the lymph nodes. [4][5][6][7][8] In many of these cases the same clonal T-cell receptor (TCR) rearrangements have been found in the LyP as well as in the associated lymphoma, identifying LyP as a precursor lesion. 6,[9][10][11][12] Most LyP cases are, however, not associated with T-cell lymphomas of various types.…”

“…6,[9][10][11][12] Most LyP cases are, however, not associated with T-cell lymphomas of various types. 4,7 In these cases, clonal T-cell populations were detectable only in a proportion of analyzed lesions, [13][14][15][16] which might, in addition, be different when taken at various times. 13 Therefore, it was concluded that LyP represents in most instances a reactive skin disease that can, under certain circumstances, present as a localized clonal lymphoid disorder.…”

Prognosis of Lymphomatoid Papulosis

“…In comparison, absolute frequencies between 5% and 24% have been reported in the literature [3][4][5][6][7][8][9][10] (Table 2). However, rather than calculating the absolute frequency in a given LyP patient cohort, an analysis of the course of the disease indicates that there is a considerably increased risk for progression when LyP is followed up for extended time periods (Table 3).…”

The History of Lymphoma Classifications with Special Consideration of Cutaneous Lymphomas

“…In 1982, histologic subtyping in A, B or C was performed in a doctoral thesis by Willemze [104]. These subtypes are included in the WHO-EORTC classification, although they exhibit-without prognostic significance -different monoclonality of the T-cell receptor depending on the type and are often represented simultaneously within one lesion [1].…”