Lymphomatoid papulosis is a chronic, clinically benign skin disorder that, when examined histologically, is seen to include numerous large, atypical lymphoid cells that display antigenic markers of T lymphocytes. To investigate the disparity between the clinical behavior of this disease and its malignant histologic appearance, we analyzed the DNA from skin lesions of six patients for rearrangements of beta and gamma T-cell receptor genes. Lesions from five of these patients showed between one and three clonal rearrangements for at least one T-cell receptor gene. Three separate biopsy specimens from a single patient showed different patterns of rearrangements for the beta gene in each specimen. Our results indicate that lymphomatoid papulosis is a clonal T-cell lymphoproliferative process that may possibly be multiclonal in origin. We conclude that this disease has both biologic and histologic features consistent with a malignant T-cell neoplasm despite its indolent course.

Clonal T-cell populations in lymphomatoid papulosis. Evidence of a lymphoproliferative origin for a clinically benign disease.

SUMMARY

A review of sixty-four skin 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 finding 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 nucleoti 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.

The clinical and histological spectrum of lymphomatoid papulosis.

Lymphomatoid papulosis (LyP) is a disorder which generally runs a benign course, but can sometimes be associated with a malignant lymphoma. Information about the prognosis of these LyP-associated lymphomas is, however, fragmentary. In this study, the clinical data of 50 LyP-associated malignant lymphomas, including 11 patients of our own group and 39 reported in the literature, are evaluated. Three main groups of LyP-associated malignant lymphomas could be distinguished: cases associated with mycosis fungoides (19/50 cases). Hodgkin's disease (12/50 cases) and (CD30+) large-cell lymphomas (16/50). The results of this study demonstrate that patients with mycosis fungoides. Hodgkin's disease, and (CD30+) large-cell lymphomas limited to the skin have a favourable prognosis. However, the prognosis of patients developing a systemic (CD30+) large-cell lymphoma proved generally poor. The results of this study also indicate that the risk of an individual LyP patient developing systemic lymphoma is less than 5%.

The prognosis of patients with lymphomatoid papulosis associated with malignant lymphomas.

Herein we review the Mayo Clinic experience with thirty-one cases of lymphomatoid papulosis seen since 1965 All patients had chronic, recurrent, and self-healing erythematous papulonodular lesions, which often became pustular, ulcerated, and resolved with scarring The clinical features often corresponded to those seen in Mucha-Habermann disease; however, the predominant histopathologic feature was an infiltrate composed primarily of atypical lymphoid cells suggestive of malignant lymphoma In six patients, a lymphoproliferative disorder was eventually diagnosed There were two cases of mycosis fungoides (stage I), one case of nodular sclerosing Hodgkin's disease, and three cases of malignant lymphoma-one diffuse mixed large and small cell type with features of T-immunoblastic type, one diffuse large cell type, and one follicular small cleaved cell type The clinical course of the lymphomatoid papulosis was unaffected by chemotherapy for the lymphoproliferative disorder Our data indicate that, with sufficient duration of follow-up, malignant lymphoma may develop in some patients with lymphomatoid papulosis

The clinicopathologic spectrum of lymphomatoid papulosis: Study of 31 cases

Parapsoriasis is a group of uncommon but not rare disorders that was created in 1902 as part of a now long forgotten scheme to classify all inflammatory dermatoses. This artificial grouping has led to an enormously confused nosology of these disorders, that are, for the most part, otherwise unrelated. The use of a number of different terms at different institutions and by different physicians to denote the same diseases, together with the use of particular, single terms to denote different diseases, has caused much unnecessary confusion, In this review these terms are examined and an attempt is made to propose a new, unambiguous classification. Using this system, physicians with different views regarding which of the parapsoriases constitute distinct entities should have no difficulty communicating, and should have a clearer frame of reference within which to work. An attempt is made to evaluate critically which of the parapsoriases are distinct entities and whether one or more of them should be considered an early form of cutaneous lymphoma in light of currently available data.

Rudner Ej

Papers

Lymphomatoid papulosis eventuating in mycosis fungoides.