Background: Mycosis fungoides palmaris et plantaris (MFPP), characterized by hyperkeratotic patches or plaques confined to the palms and soles, is rare and easy to misdiagnose because of the clinical similarity to pso...Background: Mycosis fungoides palmaris et plantaris (MFPP), characterized by hyperkeratotic patches or plaques confined to the palms and soles, is rare and easy to misdiagnose because of the clinical similarity to psoriasis, cutaneous inflammatory dermatoses, and dermatophytic infections. The literature about MFPP mostly consists of case reports with short-term follow-up. Objective: Our purpose was to evaluate the clinicopathologic features, T-cell receptor (TCR) gene rearrangement findings, and prognosis of MFPP. Patients and methods: This retrospective study has been reviewed in the clinicopathologic, TCR γ gene rearrangement findings and follow-up study of 12 patients with MFPP. Results: The duration of diseases ranged from 9 months to 25 years with a mean duration of 5.3 years. Clinically, hyperkeratotic patches and plaques were observed in all cases, with 6 cases having developed on the palms and soles and 6 cases on the palms only. In TNM classifications, all cases were confined to T1N0M0 (stage IA) showing an early stage of mycosis fungoides (MF). Histopathologic findings revealed marked hyperkeratosis, parakeratosis with plasma, epidermotropism, convoluted lymphocytes, haloed lymphocytes, dense infiltrate of lymphocytes in all 12 cases (100% ), Pautrier’ s microabscess in 9 cases (75% ), a wiry bundle of collagen in 11 cases (91.7% ) and basilar epidermotropism in 3 cases (25% ). TCR γ gene rearrangement was performed except for one case and monoclonality was detected in 10 of 11 cases. In the comparison group with cutaneous inflammatory dermatoses, all cases showed polyclonality. Treatment was done with Re-PUVA (acitretin and PUVA), ultraviolet A1, as well as systemic acitretin and methotrexate. Most patients showed a good response. In the follow-up study of 9 cases for a mean period of 47.6 months, only one patient’ s skin lesions were extended to the trunk and face, but the other patients had no sign of extracutaneous involvement. Limitations: These results were obtained from patients with MFPP in Korea. A cooperative study with other ethnic groups will be helpful. Conclusions: If a patient has recalcitrant palmoplantar dermatosis, MFPP should be suspected and histopathologic studies with TCR gene rearrangement should be done for early diagnosis of MFPP.展开更多
Objective: To extend previous observations regarding the prognostic value of analyzing lymph node DNA from patients with cutaneous T- cell lymphoma for the presence of a monoclonal T- cell population by Southern blot ...Objective: To extend previous observations regarding the prognostic value of analyzing lymph node DNA from patients with cutaneous T- cell lymphoma for the presence of a monoclonal T- cell population by Southern blot vs polymerase chain reaction (PCR) methods. Design: Inception cohort study from 1982 to 1998. Recruitment of new patients ended in 1994. Setting: A tertiary care referral center in Seattle, Wash. Patients: Fifty- five uniformly staged patients with the diagnosis of cutaneous T- cell lymphoma who underwent a lymph node biopsy, 21 with clinically abnormal nodes and 34 with normal nodes. Interventions: Lymph nodes were evaluated for T- cell receptor (TCR) γ - chain gene rearrangement by 2 PCR methods: capillary electrophoresis and denaturing gradient gel electrophoresis. The same lymph nodes were evaluated by Southern blot analysis for TCR β - chain gene rearrangement and examined histopathologically on the basis of the National Cancer Institute lymph node classification system. Patients were observed clinically for a mean of 9.5 years. Main Outcome Measures: Skin stage, clinical lymph node examination, lymph node histologic examination, Southern blot analysis, and PCR analyses were evaluated as potential prognostic predictors by univariate and multivariate analyses. The statistical association of TCR analysis and clinical outcome was determined among all patients. Hazard ratios (HRs) by Cox proportional hazards regression analysis were used to estimate the risk of a poor clinical outcome. Cumulative survival rates were analyzed by the Kaplan- Meier method. Results: A skin stage of T3 (tumors) or T4 (erythroderma) was the most powerful predictor of a poor clinical outcome (HR, 31.3 vs T1; P< .001). Patients with detectable TCRγ - chain gene rearrangement in lymph node DNA by PCR also were more likely to have a poor outcome (HR, 5.1; P< .001), but it was a less powerful predictor than skin stage. Even when the skin stage, presence or absence of lymphadenopathy, and histologic lymph node score were known for the patient, Southern blot analysis still added to prediction of a poor outcome (HR, 9.3; P=.007), whereas PCR provided no statistically significant additional information on outcome. Conclusions: Detection of a monoclonal T- cell population by PCR in lymph nodes of patients with cutaneous T- cell lymphoma does not enhance prediction of clinical outcome and probability of survival beyond what can be determined from clinical examination and histologic lymph node scores. Skin stage and the presence or absence of lymphadenopathy remain the most important determinants of clinical outcome.展开更多
文摘Background: Mycosis fungoides palmaris et plantaris (MFPP), characterized by hyperkeratotic patches or plaques confined to the palms and soles, is rare and easy to misdiagnose because of the clinical similarity to psoriasis, cutaneous inflammatory dermatoses, and dermatophytic infections. The literature about MFPP mostly consists of case reports with short-term follow-up. Objective: Our purpose was to evaluate the clinicopathologic features, T-cell receptor (TCR) gene rearrangement findings, and prognosis of MFPP. Patients and methods: This retrospective study has been reviewed in the clinicopathologic, TCR γ gene rearrangement findings and follow-up study of 12 patients with MFPP. Results: The duration of diseases ranged from 9 months to 25 years with a mean duration of 5.3 years. Clinically, hyperkeratotic patches and plaques were observed in all cases, with 6 cases having developed on the palms and soles and 6 cases on the palms only. In TNM classifications, all cases were confined to T1N0M0 (stage IA) showing an early stage of mycosis fungoides (MF). Histopathologic findings revealed marked hyperkeratosis, parakeratosis with plasma, epidermotropism, convoluted lymphocytes, haloed lymphocytes, dense infiltrate of lymphocytes in all 12 cases (100% ), Pautrier’ s microabscess in 9 cases (75% ), a wiry bundle of collagen in 11 cases (91.7% ) and basilar epidermotropism in 3 cases (25% ). TCR γ gene rearrangement was performed except for one case and monoclonality was detected in 10 of 11 cases. In the comparison group with cutaneous inflammatory dermatoses, all cases showed polyclonality. Treatment was done with Re-PUVA (acitretin and PUVA), ultraviolet A1, as well as systemic acitretin and methotrexate. Most patients showed a good response. In the follow-up study of 9 cases for a mean period of 47.6 months, only one patient’ s skin lesions were extended to the trunk and face, but the other patients had no sign of extracutaneous involvement. Limitations: These results were obtained from patients with MFPP in Korea. A cooperative study with other ethnic groups will be helpful. Conclusions: If a patient has recalcitrant palmoplantar dermatosis, MFPP should be suspected and histopathologic studies with TCR gene rearrangement should be done for early diagnosis of MFPP.
文摘Objective: To extend previous observations regarding the prognostic value of analyzing lymph node DNA from patients with cutaneous T- cell lymphoma for the presence of a monoclonal T- cell population by Southern blot vs polymerase chain reaction (PCR) methods. Design: Inception cohort study from 1982 to 1998. Recruitment of new patients ended in 1994. Setting: A tertiary care referral center in Seattle, Wash. Patients: Fifty- five uniformly staged patients with the diagnosis of cutaneous T- cell lymphoma who underwent a lymph node biopsy, 21 with clinically abnormal nodes and 34 with normal nodes. Interventions: Lymph nodes were evaluated for T- cell receptor (TCR) γ - chain gene rearrangement by 2 PCR methods: capillary electrophoresis and denaturing gradient gel electrophoresis. The same lymph nodes were evaluated by Southern blot analysis for TCR β - chain gene rearrangement and examined histopathologically on the basis of the National Cancer Institute lymph node classification system. Patients were observed clinically for a mean of 9.5 years. Main Outcome Measures: Skin stage, clinical lymph node examination, lymph node histologic examination, Southern blot analysis, and PCR analyses were evaluated as potential prognostic predictors by univariate and multivariate analyses. The statistical association of TCR analysis and clinical outcome was determined among all patients. Hazard ratios (HRs) by Cox proportional hazards regression analysis were used to estimate the risk of a poor clinical outcome. Cumulative survival rates were analyzed by the Kaplan- Meier method. Results: A skin stage of T3 (tumors) or T4 (erythroderma) was the most powerful predictor of a poor clinical outcome (HR, 31.3 vs T1; P< .001). Patients with detectable TCRγ - chain gene rearrangement in lymph node DNA by PCR also were more likely to have a poor outcome (HR, 5.1; P< .001), but it was a less powerful predictor than skin stage. Even when the skin stage, presence or absence of lymphadenopathy, and histologic lymph node score were known for the patient, Southern blot analysis still added to prediction of a poor outcome (HR, 9.3; P=.007), whereas PCR provided no statistically significant additional information on outcome. Conclusions: Detection of a monoclonal T- cell population by PCR in lymph nodes of patients with cutaneous T- cell lymphoma does not enhance prediction of clinical outcome and probability of survival beyond what can be determined from clinical examination and histologic lymph node scores. Skin stage and the presence or absence of lymphadenopathy remain the most important determinants of clinical outcome.
