Cases reported "Lymphomatoid Papulosis"

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1/7. Regional lymphomatoid papulosis: a report of four cases.

    lymphomatoid papulosis (LyP) is a chronic self-healing cutaneous eruption which is clinically benign but histologically malignant. Lesions occur episodically over the trunk and limbs. We describe four patients with regional LyP. All were male, with a range in age at onset from 12 to 47 years. In all cases, lesions were confined to a segmental unilateral area. Two patients had type A and two type B LyP. We have long-term follow-up on one patient whose lesions were limited to the right buttock for more than 20 years before more widespread lesions developed. Another patient with lesions on the left flank had mycosis fungoides limited to the same region. Only one other case of LyP presenting in a regional distribution has previously been described.
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ranking = 1
keywords = mycosis fungoides, fungoides, mycosis
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2/7. lymphomatoid papulosis associated with recurrent cutaneous T-cell lymphoma.

    INTRODUCTION: lymphomatoid papulosis is a chronic benign disease which may be associated with malignant lymphomas. This case illustrates the relapsing and remitting nature of both lymphomatoid papulosis and its potential of developing cutaneous T-cell lymphoma and narrow-band ultraviolet B (NB-UVB) phototherapy as a new modality of treatment of early-stage mycosis fungoides in these patients. CLINICAL PICTURE: A 44-year-old woman has had recurrent crops of papules and nodules of lymphomatoid papulosis on the limbs for 15 years. Histological features are consistent with the type B lesions of lymphomatoid papulosis. Eight years after the initial onset of these lesions she developed cutaneous T-cell lymphoma (mycosis fungoides). Since then, she has had recurrence of mycosis fungoides following the cessation of phototherapy, but had no evidence of systemic involvement. TREATMENT: The lesions of lymphomatoid papulosis responded to intermittent courses of oral methotrexate. mycosis fungoides was treated with oral psoralen and ultraviolet A phototherapy with good response. Unfortunately, the lesions relapsed, whenever phototherapy was discontinued. The most recent recurrence of mycosis fungoides was treated with NB-UVB therapy. OUTCOME: The papules of lymphomatoid papulosis continue to appear but she remains free of lesions of mycosis fungoides, 10 months after cessation of NB-UVB therapy. CONCLUSION: Long-term surveillance is essential in all cases of lymphomatoid papulosis as accurate predictors for the development of malignant lymphoma in these individuals are still lacking.
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ranking = 5.2143818109645
keywords = mycosis fungoides, fungoides, mycosis
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3/7. lymphomatoid papulosis in an hiv-positive man.

    lymphomatoid papulosis (LyP) is a rare cutaneous lymphoproliferative condition characterized by a chronic, recurrent eruption of papules and nodules that undergo spontaneous regression. The disorder is usually clinically benign; with a minority of cases progressing to malignant lymphoma. LyP is divided into two subtypes based on histologic appearance. Type A resembles Hodgkin's disease with up to 20% of large CD30 lymphocytes. Type B resembles mycosis fungoides showing an infiltrate of CD4 lymphocytes and scattered CD30 cells. Clinically LyP often resembles pityriasis lichenoides et varioliformis acuta but has a strikingly different histological appearance. Histologically, LyP resembles lymphoma (anaplastic T-cell or Hodgkin's) but is distinguished by its benign course. Here we present a case of LyP in a severely immune-repressed hiv-positive patient. This patient presented with pruritic papules involving the upper extremities and a CD4 T-cell count of 4. Histopathologic examination showed a dense superficial dermal infiltrate comprising normal-sized lymphocytes admixed with larger lymphocytes. immunophenotyping showed most of the lymphocytes to be CD3 (T cells). The scattered larger cells were CD30 . The smaller lymphocytes were CD8 rather than CD4 as expected for non-hiv-appointed LyP. This may be because of the immune disregulation of hiv disease and the absolute and relative paucity of CD4 T cells relative to CD8 T cells.
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keywords = mycosis fungoides, fungoides, mycosis
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4/7. lymphomatoid papulosis in a patient with Waldenstrom's macroglobulinemia.

    lymphomatoid papulosis (LyP) is a rare skin disease with a benign course but a malignant histologic appearance based on atypical lymphocytes. Waldenstrom's macroglobulinemia (WM) is a diffuse infiltration of the bone marrow by cells that synthesize IgM immunoglobulins. A 46-year-old female presented with a two year history of weakness, fatigue, anemia, and recurring multiple erythematous papules and nodules in the skin. The skin biopsy showed pleomorphic lymphoid cells with atypical mitoses permeated by a diffuse dermal infiltrate of normal appearing lymphocytes. The neoplastic cells were positive for CD30, CD3, and CD5 but negative for CD20 and EMA. The bone marrow was hypercellular due to a diffuse infiltration by lymphocytes, plasma cells, and plasmacytoid lymphocytes. LyP is a lymphoproliferative disorder of CD30-positive T cells that may be associated with other lymphoid malignancies, particularly Hodgkin's disease, mycosis fungoides, and anaplastic T cell lymphomas. To our knowledge, this is the first report of a LyP appearing in a patient with WM.
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keywords = mycosis fungoides, fungoides, mycosis
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5/7. Three cases of lymphomatoid papulosis with a CD56 immunophenotype.

    We report 3 cases of lymphomatoid papulosis (LyP) with a CD56 , cytotoxic immunophenotype. All 3 patients presented with clinical histories typical of LyP, with one patient having associated mycosis fungoides. Histologically, two cases were type A LyP and one was type B. All 3 cases demonstrated a T-cell receptor clone in lesional skin without evidence of blood involvement. The atypical lymphocytes in each of the 3 cases expressed cytotoxic granules (T-cell intracellular antigen-1 and granzyme B ) and were CD8 and CD56 . Expression of CD56 is associated with a poor prognosis in subcutaneous panniculitis-like T-cell lymphoma and blastic natural killer cell lymphoma. However, the two cases of CD56 LyP previously reported and the 3 cases in this series all appear to be pursuing an indolent course with no evidence of systemic disease.
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keywords = mycosis fungoides, fungoides, mycosis
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6/7. lymphomatoid papulosis associated with Ki-1-positive anaplastic large cell lymphoma. A report of two cases and a review of the literature.

    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, 10%-20% 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.
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ranking = 1
keywords = mycosis fungoides, fungoides, mycosis
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7/7. Follicular lymphomatoid papulosis.

    A case of follicular lymphomatoid papulosis (LyP) is reported. The patient was a 60-year-old Japanese woman. Clinically, cutaneous eruptions were reddish, centrally depressed, dome-shaped papules on the extensor aspect of the forearm. Histologically, they exhibited features that fulfilled the disease criteria described by Pierard, et al., i.e., (Am J Dermatopathol 1980;2:173-80), mixed cellular infiltrates including atypical Reed-Sternberg cell-like type-A cells and mycosis cell-like type-B cells surrounding hyperplastic follicular epithelia. The patient also showed many typical nonfollicular LyP papules, i.e., rhythmically recurrent papules which underwent spontaneous involution within a few weeks, over a 10-year period. The coincidental occurrence of a rare variant of follicular LyP and typical LyP in the same individual further suggests that follicular LyP is merely a histological pattern of LyP involving epithelial adnexae.
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ranking = 0.073287674231018
keywords = mycosis
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