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At least 19 recordsLinked to original sources
PubMed1981

Lymphoma-associated papulosis: lymphomatoid papulosis associated with lymphoma.

Lymphomatoid papulosis is a condition characterized by the continuing appearance of self-healing inflammatory papulonodules which histologically show atypical cells simulating a malignant process. We describe two patients who had features typical of lymphomatoid papulosis in whom a lymphoreticular malignancy developed. The first patient developed Hodgkin's disease 30 years after the onset of lesions that resembled lymphomatoid papulosis, and the second patient developed a lymphocytic lymphoma 11 months after the appearance of the self-healing papules. While most patients with lymphomatoid papulosis have a benign protracted course, "lymphoma-associated papulosis" represents the malignant end of this spectrum, in which lymphomatoid papulosis is associated with a lymphoma. All patients with lymphomatoid papulosis require close, long-term follow-up.

Biopsy↗
PubMed1985

Lymphomatoid papulosis.

Lymphomatoid papulosis is an unusual chronic skin disease characterized by the continuous appearance of papulonecrotic, nodular, and occasionally larger tumorous or plaque-like lesions showing histologic features suggestive of malignant lymphoma. The recognition of two histologic types of lymphomatoid papulosis, which are not distinct entities but represent the ends of a spectrum, has provided an explanation for the clinical and histologic variation and many of the current controversies in this disorder. These two types of lymphomatoid papulosis, called type A (or "histiocytic" type) and type B (or lymphocytic type), have been shown to be related to Hodgkin's disease and mycosis fungoides, respectively. Current evidence suggests that these conditions result from abnormal immune reactions to persistent and as yet unidentified antigens. This concept does not resolve the question of whether lymphomatoid papulosis is an inflammatory or malignant disease. In most patients, lymphomatoid papulosis runs a protracted but benign course. In others, however, it is associated with or develops into malignant lymphoma. Because reliable clinical and histologic criteria predictive of such a transition are lacking, all patients with lymphomatoid papulosis require close long-term follow-up.

Female↗
PubMed1995

[Lymphomatoid papulosis].

Lymphomatoid papulosis is a distinct entity in which recurring crops of haemorragic and necrotic papules display a cytologically malignant infiltrate. The aberrent cell is now generally accepted to be an active T helper phenotype. The expression of Ki-1 (CD30) on a significant portion of the infiltrating cells characterizes lymphomatoid papulosis and relates this disorder with Hodgkin's disease, mycosis fungoides and anaplasic T cell lymphoma which may be associated in 10 to 20% of lymphomatoid papulosis. The categorization of this disease as a benign disorder versus lymphoma remains controversial. Studies of T cell receptor gene rearrangement demonstrate clonality in many cases. So, this monoclonal population could have a malignant transformation induced by a triggering stimulus such as genetic translocation, or viral infection. Finally, recent opinions consider that lymphomatoid papulosis and Ki-1 (CD30) lymphomas are different parts of a clinical and histological spectrum constituted by cutaneous Ki-1 lymphoid infiltrates.

Antigens, CD↗
PubMed2003

Topical methotrexate for lymphomatoid papulosis.

Lymphomatoid papulosis is a lymphoproliferative disease characterized by recurrent papules, nodules, or plaques that spontaneously involute within several weeks. Despite having a histologically malignant-appearing infiltrate, patients with lymphomatoid papulosis have a clinically benign course. Several treatment options have been described for the management of lymphomatoid papulosis. We describe a patient who had complete resolution of lesions with the use of topical methotrexate.

Administration, Topical↗
PubMed1994

Lymphomatoid papulosis.

Lymphomatoid papulosis is a chronic disease of cutaneous lymphoid infiltration characterized clinically by involuting and recurring papules, plaques, and nodules. The intriguing combination of a usually benign clinical course, a cytologically malignant lymphoid infiltrate on histologic examination, and a clear, but sporadic association with extracutaneous lymphomas has stimulated significant investigation. Application of recent technical advances to research in lymphomatoid papulosis prompts this review.

Diagnosis, Differential↗
PubMed1989

Host response in lymphomatoid papulosis.

Lymphomatoid papulosis (LyP) is a papulonodular skin eruption characterized by spontaneous regression. In order to evaluate the role of host immune reactions in the phenomenon of regression, we analyzed skin biopsies from various stages of evolution in 16 patients with LyP using morphologic and immunoperoxidase techniques. Both rapidly regressing type A and more chronic type B lesions of LyP had modest perivascular and lesional infiltrates of small helper and suppressor T lymphocytes as well as histiocytes. Type A lesions in addition to numerous neutrophils and eosinophils had a more extensive lymphocytic infiltrate than type B lesions. Natural killer cells were not prominent in the skin lesions, although several patients had increased numbers of circulating natural killer cells. Our results demonstrate a correlation between the inflammatory components found in skin biopsies from patients with LyP and clinical behavior and suggest that cell-mediated immune reactions may play a role in the spontaneous regression of LyP.

Adult↗
PubMed1990

Hodgkin's disease followed by lymphomatoid papulosis. Immunophenotypic evidence for a close relationship between lymphomatoid papulosis and Hodgkin's disease.

The clinical association of lymphomatoid papulosis and Hodgkin's disease and the striking morphologic similarity of atypical cells in lymphomatoid papulosis to Reed-Sternberg cells in Hodgkin's disease suggest that lymphomatoid papulosis and Hodgkin's disease are related. To test this possibility we studied the antigenic profile of Reed-Sternberg cells in the lymph nodes and of atypical cells in cutaneous lesions of lymphomatoid papulosis in two patients with Hodgkin's disease and lymphomatoid papulosis. In paraffin sections both cell types expressed CD30, CD45 T cell-restricted antigens, and occasionally CD15 antigens. They were negative for CD45 B cell-restricted antigens and for lysozyme. In cutaneous lymphomatoid papulosis lesions a similar immunologic profile of the atypical cells was found; that is, they were positive for CD30, CD2, CD3, and CD25 but negative for B cell and macrophage antigens. The similarity of the immunophenotype of Reed-Sternberg cells in lymph nodes affected by Hodgkin's disease and the immunophenotype of atypical cells of lymphomatoid papulosis lesions in the same patients suggests that the malignant cells in both conditions are derived from activated T cells and that they are closely related if not identical.

Adult↗
PubMed1992

Epidemiology of lymphomatoid papulosis.

BACKGROUND: Lymphomatoid papulosis is a rare skin disease with malignant potential. Its epidemiology is largely unknown. METHODS: A case-control study of lymphomatoid papulosis was done to characterize the patient population and investigate the risk factors for its development. Fifty-seven patients with biopsy-proven lymphomatoid papulosis and 67 individually matched control subjects who were recruited among relatives and acquaintances of the patients answered a standard questionnaire over the telephone. RESULTS: Among patients with lymphomatoid papulosis, 3 had a history of Hodgkin disease, 3 had non-Hodgkin lymphoma, and 10 had mycosis fungoides; none of the control subjects reported such histories. No significant differences were observed between patients and control subjects in regard to residence or travel history or exposures to various physical, chemical, and biologic agents. A higher, although not statistically significant, percentage of patients than control subjects reported a history of radiation therapy and nonlymphoid malignant lesions. No differences were found between patients and control subjects in regard to other medical conditions or family medical history. CONCLUSIONS: Patients with lymphomatoid papulosis have a significantly increased frequency of prior or coexisting lymphoproliferative disorders, an increased frequency of nonlymphoid malignant lesions, and exposure to radiation therapy.

Adolescent↗
PubMed2000

Therapeutic use of interferon-alpha for lymphomatoid papulosis.

BACKGROUND: Lymphomatoid papulosis is a primary cutaneous, CD30 positive lymphoproliferative disorder with the potential to transform into systemic, malignant lymphoma. Therapeutic strategies for patients with lymphomatoid papulosis have been designed to prevent transformation but have proved to be either inefficacious or limited by side effects. METHODS: The authors compared the clinical, histologic, and immunohistochemical features from a group of five patients receiving interferon-alpha (IFN-alpha) subcutaneously three times per week with the same features from a group of six patients receiving conventional therapy, including photochemotherapy, antibiotics, topical corticosteroids, or surgery, in an open trial. RESULTS: In the IFN-alpha group, four patients showed a complete remission, and one patient showed a partial remission within a time period of 6 weeks. Two patients developed disease recurrences after discontinuation of short term IFN-alpha therapy (5-7 months). Thereof, one patient went into stable remission after long term IFN-alpha therapy (17 months), and one patient remains in partial remission. In the control group, one patient went into spontaneous remission, two patients showed partial remission, of which one patient developed progressive disease at a later time point, whereas three patients have recurrent disease despite of treatment. CONCLUSIONS: The current results indicate that the treatment with IFN-alpha of patients with lymphomatoid papulosis alters the clinical course of the disease with fewer side effects than previous regimens; however, short term treatment does not induce stable remission. Therefore, prolonged treatment appears to be warranted for these patients.

Adrenal Cortex Hormones↗
PubMed1983

Immunological, cytochemical and ultrastructural studies in lymphomatoid papulosis.

In lymphomatoid papulosis two histological types can be distinguished, i.e. type A and type B. In the present study various immunological, enzyme-histochemical and ultrastructural techniques were used to investigate the cellular infiltrate in both types of lymphomatoid papulosis. The type A lesions showed a predominance of large atypical cells, relatively few T cells and few or no Langerhans or related cells, as defined by a positive staining for OKT6 and NA I/34 antisera. The immunological, cytochemical and ultrastructural characteristics of these large atypical cells resembled those of the Langerhans cell/interdigitating reticulum cell series. The morphology and marker profile of these large cells resembled those of Reed-Sternberg cells, which suggests a relationship between lymphomatoid papulosis type A and Hodgkin's disease. The type B lesions showed a predominance of small, medium-sized and large cerebriform mononuclear cells with the phenotype of activated T helper cells, and numerous Langerhans and/or related cells. Their cellular composition was similar to that observed in the early stages of mycosis fungoides.

Antibodies, Monoclonal↗
PubMed2003

Bexarotene is a new treatment option for lymphomatoid papulosis.

BACKGROUND: Lymphomatoid papulosis (LyP) is a clonal T cell proliferation with large cell histology, a chronic course, and an increased risk of lymphoma. Bexarotene (Targretin) is an RXR-selective retinoid (rexinoid) approved for the cutaneous manifestations of cutaneous T cell lymphoma. OBJECTIVE: To determine whether bexarotene is effective in treating LyP. METHODS: Ten patients with chronic and symptomatic LyP were prospectively treated with oral (n = 3) or topical gel (n = 7) formulations of bexarotene. RESULTS: A favorable response to bexarotene treatment with decreased numbers or duration of lesions was seen in all with objective responses in 8 patients. CONCLUSIONS: Bexarotene may be an effective palliative treatment for LyP, warranting further controlled studies.

Administration, Oral↗
PubMed1999

Increased risk of lymphoid and nonlymphoid malignancies in patients with lymphomatoid papulosis.

BACKGROUND: Lymphomatoid papulosis (LyP) is a rare skin disease with malignant potential. The long term outcomes of patients with this disease have not been adequately assessed. METHODS: Fifty-seven patients with biopsy-proven LyP and 67 controls matched for age, gender, and race were followed prospectively from 1988 to 1996. Reported malignancies were confirmed by surgical pathology and/or autopsy reports. A search through the National Death Index through December 1995 was conducted to identify all deaths, and death certificates were procured. Expected numbers of malignancies based on SEER data were calculated for both the patient and the control groups. RESULTS: Six LyP patients (10.5%) and 1 control (1.5%) reported nonlymphoid malignancies (P = 0.047). Two patients and no controls developed lymphoid malignancies (mycosis fungoides and CD30(+) cutaneous lymphoma). The expected numbers of nonlymphoid and lymphoid malignancies in the LyP patient group, based on the SEER data, were 1.93 and 0.15, respectively, yielding a relative risk (with 95% confidence interval) of 3.11 (1.26-6.47) for nonlymphoid malignancies and 13.33 (2.24-44.05) for malignant lymphomas in the LyP patients. There was no significant difference between the observed and expected numbers of malignancies in the control group. Four LyP patients died during the follow-up, three due to malignancies; and one control died of a gunshot wound to the head (suicide). The difference in overall survival between the LyP patients and the controls was not statistically significant (P = 0. 12). CONCLUSIONS: Patients with LyP appear to have an increased risk of both lymphoid and nonlymphoid malignancies. The increased risk of nonlymphoid as well as lymphoid malignancies may suggest a basic underlying genetic defect leading to the development of malignancy in LyP patients.

Adolescent↗
PubMed1992

Immunophenotypic and genotypic characterization of lymphomatoid papulosis.

BACKGROUND: Lymphomatoid papulosis (LyP) is a chronic dermatosis that histologically resembles malignant lymphoma. Thus far, only a few cases of LyP have been characterized in detail with regard to immunophenotype, genotype, and karyotype. OBJECTIVE: Our purpose was to study seven patients with LyP and compare the results to those reported in the literature. METHODS: Skin biopsy specimens were analyzed by frozen section immunohistochemical and molecular biologic techniques. Cytogenetic analysis was also performed in three cases. RESULTS: The atypical lymphoid cells consisted of activated helper T cells; four of the seven patients had lesions with a detectable clonal T-cell population. A peripheral T-cell lymphoma developed in one patient before the emergence of a genotypically different LyP T-cell clone. Cytogenetic studies were abnormal in one case of LyP and normal in another, whereas the karyotype of the lymphoma was abnormal. CONCLUSION: LyP is a preneoplastic proliferation of activated helper T cells, which is often clonal and may regress and expand with the development of new LyP clones or lymphoma.

Adolescent↗
PubMed1995

Lymphomatoid papulosis in children.

BACKGROUND: Although lymphomatoid papulosis is well described in adults, the clinical course, prognosis, risk for lymphoma, and recommendations for follow-up have not been established in children. OBJECTIVE: Our aim was to analyze our data on six children with lymphomatoid papulosis and to analyze available information on reported cases from the literature to characterize better lymphomatoid papulosis in childhood and to compare it with adult-onset lymphomatoid papulosis. METHODS: Clinical records, laboratory studies, and histopathologic evaluation of skin biopsy specimens from six children with lymphomatoid papulosis were reviewed. A literature search was also performed and disclosed detailed information on 17 childhood cases. RESULTS: In most cases childhood lymphomatoid papulosis is clinically and histologically similar to lymphomatoid papulosis in adults, but three unusual patterns were identified in our children: first, after initial outbreak, dwindling outbreaks (both in frequency and number of lesions) until the eruption ceased completely; second, lymphomatoid papulosis localized to one area for years before generalizing, and third, presentation of lymphomatoid papulosis with hundreds of lesions. In our children and in those previously reported, response to systemic antibiotics and potent topical steroids was variable, as in adults. All our children to date have remained healthy; the longest period of follow-up is 9 years. However, in previously reported cases two patients with childhood-onset lymphomatoid papulosis had lymphoma as adults. CONCLUSION: Childhood lymphomatoid papulosis may be more likely to resolve spontaneously than adult lymphomatoid papulosis; nevertheless these children may still be at risk for lymphoma and thus need lifelong follow-up.

Adolescent↗
PubMed1996

Lymphomatoid papulosis and cutaneous CD30+ lymphoma.

Lymphomatoid papulosis and cutaneous CD30+ lymphoma are closely related conditions in which large atypical lymphocytes that have similar immunophenotypic features occur. In lymphomatoid papulosis, the lesions are papules and nodules that spontaneously involute. There are two polar histologic patterns, type A and B, in which the large atypical cells resemble those of Hodgkin's disease and mycosis fungoides, respectively, but in many cases, features of both types are present, either separately or in the same lesions. Variants of lymphomatoid papulosis include cases with a perifollicular distribution and those with lymphocytic vasculitis or dermal mucin deposits. Clinical lesions that tend to be stable, a monomorphous cellular composition, and in the case of immunocompromised patients, the presence of Epstein-Barr viral genome characterize cutaneous CD30+ lymphoma. A loss of response to transforming growth factor-beta, which normally dampens cellular proliferation, may differentiate CD30+ lymphoma from lymphomatoid papulosis.

Cell Division↗
PubMed1990

Follicular lymphomatoid papulosis.

A case of follicular lymphomatoid papulosis was studied by light- and electron-microscopic methods. The nature of atypical cells was analyzed with monoclonal antibodies. This case proved to be mainly a type B lymphomatoid papulosis with a predominance of T-helper markers in atypical lymphoid cells. All previously reported cases of this rare variant corresponded to type A lymphomatoid papulosis. Therefore we believe this to be the first case report of type B follicular lymphomatoid papulosis.

Adult↗
PubMed1993

[Lymphomatoid papulosis in a child].

We report a case of a 3-year-old boy who developed crops of papules and ulcerating nodules on the limbs in April 1992. Periodically, new lesions continue to erupt, while others resolve spontaneously. This course is characteristic for rhythmic paradoxical eruptions. This course and the clinical picture, supported by the histopathological and immunohistochemical findings, led to the diagnosis of lymphomatoid papulosis. Lymphomatoid papulosis is extremely rare in childhood. All published cases of lymphomatoid papulosis in children under 10 years of age are reviewed. The differential diagnosis of lymphomatoid papulosis in childhood includes arthropod assaults, pityriasis lichenoides et varioliformis acuta, primary cutaneous Hodgkin's disease, Ki-1 large cell anaplastic lymphoma and other lymphomas and pseudolymphomas in children.

Biopsy↗
PubMed1987

Lymphomatoid papulosis following Hodgkin's disease.

Lymphomatoid papulosis has occurred in association with Hodgkin's disease. In all cases previously described, Hodgkin's disease has developed after, or concurrently with, the onset of lymphomatoid papulosis. Two patients who developed lymphomatoid papulosis 1 and 10 years after the diagnosis and therapy for advanced Hodgkin's disease are reported. The purpose of this report is to document this unusual sequence.

Adult↗