Lymphomatoid papulosis is an elusive and very rare skin disorder. It is clinically benign but histologically "atypical," and about 20% of affected patients go on to develop a lymphoreticular malignancy, usually Hodgkin's disease, or mycosis fungoides. A wide variety of treatments to prevent recurrence and improve local control have been suggested, but the results have been poor. We report on a patient with two nonhealing lymphomatoid papulosis lesions treated successfully with electron beam therapy and describe our technique. The need for close follow-up of all patients with this condition cannot be overemphasized.
[Show abstract][Hide abstract] ABSTRACT: Background. Lymphomatoid papulosis (LyP) is an uncommon disorder characterized by recurrent papulonodular cutaneous lesions that last from 4 to 5 weeks and often heal with hypopigmented or hyperpigmented scarring. Prognosis is varied, ∼O%-ZO% of patients have associated lymphomas: mycosis fungoides, T-cell immunoblastic lymphoma, or Hodgkin's disease, which can precede, occur simultaneously with, or follow the diagnosis of LyP. Anaplastic large cell lymphoma (ALCL) is histologically and phenotypically similar to LyP and also appears as part of this disease spectrum. Recent reports analyzing immunophenotype and T-cell receptor gene rearrangements in patients with both LyP and lymphoma suggest that they are derived from an identical T-cell clone, in the rare cases studied.Methods. The case histories of two patients with LyP in whom ALCL involving the skin and lymph nodes subsequently developed are presented.Results. Intensive treatment with combination chemotherapy resulted in complete remission of ALCL in both patients, followed by the recurrence of LyP. A spontaneous remission of LyP occurred in the initial patient described, whereas the second patient suffered recurrences of both LyP and ALCL despite therapy.Conclusions. The case histories presented illustrate the immunophenotypic and morphologic similarities of ALCL and LyP, and the difficulties in distinguishing between them. Both entities can occur in a single patient, as shown by this report, supporting a close relationship between these processes. However, different clinical behavior and response to therapy are apparent, which connote a fundamental difference in the biologies of these neoplastic disorders. A review of the literature concerning the association between these entities is provided.
Cancer 12/1994; 74(11):3051 - 3058. DOI:10.1002/1097-0142(19941201)74:11<3051::AID-CNCR2820741124>3.0.CO;2-P · 4.89 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: The skin is one of the organs most frequently affected by extranodal lymphomas. Cutaneous lymphomas are peculiar in many aspects. (1) In contrast to nodal lymphomas, cutaneous T-cell lymphomas are more frequent than B-cell lymphomas. (2) Cutaneous T-cell lymphomas develop in a multistep process, exhibiting distinct clinical, histologic, and molecular-biologic features. They progress very slowly over a period of years or decades. (3) The disease becomes manifest very early on. (4) The skin provides a unique structural and humoral (cytokines) microenvironment to attract T cells and B cells to home to under distinct promotional conditions. (5) Treatment strategies for cutaneous lymphomas are quite different from those for nodal lymphomas.
Current Problems in Dermatology 09/1997; 9(5-9):137-204. DOI:10.1016/S1040-0486(97)80009-9
[Show abstract][Hide abstract] ABSTRACT: A 60-year-old woman was referred to the Department of Dental Medicine at Long Island Jewish Medical Center for evaluation of multiple lesions of the tongue. She reported a long history of recurrent papular cutaneous eruptions that waxed and waned. A biopsy specimen of one of the cutaneous lesions was diagnosed as lymphomatoid papulosis. Sporadic, recurrent oral ulcers that resolved spontaneously were noted 10 to 14 days before the initial visit. These ulcers had recurred for the past 17 years. The most recent oral lesion was an erythematous, irregular, solitary ulcerated area on the middle third dorsum of tongue. The area quickly enlarged, ultimately developing extensive surface necrosis. Shortly after, a similar lesion on the posterior dorsum of the tongue developed. Biopsy specimens of the former lesion showed numerous, large, atypical, pleomorphic, and hyperchromatic cells with abundant mitoses. The large, atypical cells were immunohistochemically proven to be T lymphocytes. A diagnosis of lymphomatoid papulosis was made. Two weeks later, the tongue lesions had spontaneously and totally resolved. The clinical, histomorphologic, and immunohistochemical features, as well as gene rearrangement studies of this rare entity, are presented.
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