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Granuloma annulare heralding relapse of angioimmunoblastic T cell non-Hodgkin lymphoma
P6207
P6618
Fluorescence diagnosis for follow-up of mycosis fungoides therapy Manal Bosseila, MD, Dermatology Department, Faculty of Medicine, Cairo, Egypt; Abeer Mostafa, MD, Clinical Pathology Department, National Cancer Institute, Cairo, Egypt; Dina Metwalli, MD, Dermatology Department, Faculty of Medicine, Cairo, Egypt; Doaa Mahgoub, MD, Dermatology Department, Faculty of Medicine, Cairo, Egypt; Fatma Salah ElDin, MBBCh, National Research Center, Cairo, Egypt; Marwa El-Shaer, MD, National Research Center, Cairo, Egypt
Ichthyosiform mycosis fungoides: Clinicopathologic, immunophenotypic, and molecular features Kee Suck Suh, MD, Department of Dermatology, Kosin University College of Medicine, Busan, South Korea; Dong Young Kang, MD, Department of Dermatology, Kosin University College of Medicine, Busan, South Korea; Jong Bin Park, MD, Department of Dermatology, Kosin University College of Medicine, Busan, South Korea; Min Soo Jang, MD, Department of Dermatology, Kosin University College of Medicine, Busan, South Korea; Sang Hwa Han, MD, Department of Dermatology, Kosin University College of Medicine, Busan, South Korea; Sang Tae Kim, MD, Department of Dermatology, Kosin University College of Medicine, Busan, Korea, North
Background: Response to therapy of mycosis fungoides (MF) cases is usually evaluated clinically and by repeated skin biopsies for routine histopathology or T cell receptor rearrangement. Fluorescence diagnosis (FD) represents a procedure for in vivo diagnosis of dysplastic or neoplastic tissue in a wide variety of tumors such as nonmelanoma skin cancer and follow-up of their therapy, especially photodynamic therapy. Objective: To evaluate the efficiency of FD as a noninvasive follow-up tool of therapy in cases of MF, in order to minimize the use of invasive skin biopsies. Methods: Twenty-five patients with MF stages I or II, confirmed clinically and histopathologically, were included in this study. The most affected patch/plaque at time of inclusion was subjected to clinical assessment, FD using 15% aminolevulinic acid cream, and skin biopsy for flowcytometric immunophenotyping for CD4+CD7e T lymphocytes. Patients were treated by various treatment modalities for 12 weeks, and the same patch/plaque was subjected again to FD and flowcytometric immunophenotyping. Quantification of fluorescence was enabled using digital image analysis and then compared to the gated percentage of MF cells (CD4+CD7e) infiltrating the skin. Ethical approval was attained from dermatology department research ethical committee. An informed consent for participation was obtained from patients. Results: Twenty-two patients completed the study, 14 (63.6%) of them showed clinically very good or good response to therapy. By FD of 22 evaluated lesions the mean accumulation factor, defined as the ratio of protoporphyrin IX (PpIX) fluorescence of tumor tissue to that of surrounding normal skin, was found to be 2.2 6 0.68 before therapy and 1.95 6 0.64 after therapy. On correlating the values for FD with flowcytometric immunophenotyping before and after therapy of skin lesions no statistically significant correlation was found. Conclusion: Fluorescence diagnosis, as a noninvasive tool for follow-up of treatment in cases of MF, shows promising results as indicator for disease improvement. Further verification of its role needs to be done on a larger scale of patients. Commercial support: None identified.
Background: Ichthyosiform mycosis fungoides (MF) is a rare variant of MF, characterized clinically as having an ichthyosiform skin eruption, and histologically as having mycosis fungoides and ichthyosis vulgaris (IV). It is relatively unknown compared to other variants. Previous studies on ichthyosiform MF have been mainly conducted in western countries, and only a few cases has been reported. Few studies have been conducted on Asians in a single institution on a large scale. Particularly, it is difficult to precisely define this disease because its clinicopathological findings vary depending on the study. In addition, the relationship between the occurrence of an ichthyosiform lesion and MF is also unknown. Methods: This study was conducted on 9 patients with an ichthyosiform lesion (4.2%) among 216 patients diagnosed with MF in the authors’ hospital. The patients underwent clinical, histopathological, and molecular examinations to determine the characteristics of ichthyosiform MF, and to obtain data that would be helpful for the diagnosis and treatment of this disease. Results: The patients’ ages ranged widely from 8 to 63 years. The mean age was 29.2 years including three patients aged 12 years or lower. They all had early-stage MF, of which the IB and IIA types were observed in 8 patients and 1 patient, respectively. The patients were classified according to their clinical features. There were 4 cases of typical MF and an ichthyosiform lesion, of which the ichthyosiform lesion occurred in 2 patients in the early stage of the disease, and in the remaining 2 patients after the occurrence of the typical MF lesion; one case of concurrent hypopigmented MF; 1 case of angiocentric MF with multiple ulcers; and 3 cases of only an ichthyosiform lesion. From a histologic perspective, 9 patients had both MF and IV in a single lesion, of whom 1 patient had concurrent vasculitis. As shown in IV, filaggrin expression decreased in the 9 patients. Two patients had a CD4/CD8 ratio [1. PCR detection of TCR g gene rearrangement showed monoclonality in 5 patients. Targeted phototherapy was primarily conducted on all the patients, and they showed either complete or incomplete responses. Conclusion: Ichthyosiform MF is a rare variant of MF, and seems to have relatively favorable prognoses. In the case of an ichthyosiform skin eruption, MF could be suspected. A biopsy is required to confirm ichthyosiform MF. Commercial support: None identified.
P6027 Granuloma annulare heralding relapse of angioimmunoblastic T cell nonHodgkin lymphoma Bassel Mahmoud, MD, PhD, Henry Ford Hospital, Detroit, MI, United States; Chauncey McHargue, MD, Henry Ford Hospital, Detroit, MI, United States Background: Granuloma annulare (GA) is a benign papular eruption seen in patients of all ages. The primary skin lesion is in the form of grouped papules in an enlarging annular shape, with color ranging from flesh-colored to mildly erythematous. The exact etiology of GA in unknown but studies have shown that cell-mediated delayedtype hypersensitivity reaction may be implicated. GA has been reported with solid organ tumors and as well as myeloproliferative disorders including leukemia and lymphoma. We present a rare case of GA heralding relapsed angioimmunoblastic T cell non-Hodgkin lymphoma (AITL).
P6120 Incidence of primary cutaneous T-cell lymphoma in Wales Rachel Abbott, MBBS, University Hospital of Wales, Cardiff, United Kingdom; Christian Aldridge, MBBCh, Prince Charles Hospital, Merthyr Tydfil, United Kingdom; Stefan Dojcinov, MD, University Hospital of Wales, Cardiff, United Kingdom; Vincent Piguet, MD, PhD, Cardiff University, Cardiff, United Kingdom
Case report: A 58-year-old white man with a history of AITL treated 3 years ago with 6 cycles of CHOP chemotherapy followed by autologous stem cell transplantation, after which he achieved complete remission. Patient started to develop a mildly itchy rash on the arms that spread to the trunk and lower extremities of few weeks duration. Examination showed widespread cutaneous and subcutaneous firm erythematous papules with no surface change. Differential diagnosis included cutaneous lymphoma, sarcoidosis and GA. Histopathologic examination of both cutaneous and facial lesions were consistent with GA. TCR gene rearrangement did not identify a clonal population of lymphocytes is the skin. A PET scan showed generalized adenopathy, and mediastinal node biopsy showed recurrent angioimmunoblastic T-cell lymphoma. Notably the recurrent lymphoma was surrounded by a caseating granulomatous infiltrate. Discussion: GA has been reported with malignant lymphoma including 1 case preceding the onset of AITL. The onset of GA ranges from 5 years before to 27 years after the diagnosis of lymphoma. Our case is a rare example of GA heralding relapsed AITL. The patient is currently being considered for allogeneic stem cell transplant and chemotherapy. GA in this case represents a paraneoplastic phenomenon. It remains to be seen whether a successful second remission results in resolution of the secondary skin GA lesions.
Primary cutaneous T-cell lymphoma is a rare disease. The incidence of this disease has not previously been studied in Wales, which has a population of 3 million. Our aim was to estimate the incidence rate of primary cutaneous T-cell lymphoma while conducting a clinical audit of the diagnosis and management of patients with primary cutaneous T-cell lymphoma. Patients with primary cutaneous T-cell lymphoma were identified through the All Wales Lymphoma Panel (a national pathology database), local health board pathology databases and dermatology outpatient clinics. Patients who were diagnosed between 01 August 2003 (after publication of the UK guidelines on the diagnosis and management of primary cutaneous T-cell lymphoma) and 31 October 2011 were included. In total, 120 patients diagnosed with primary cutaneous T-cell lymphoma between 01 July 2003 and 31 October 2011 were identified. The crude incidence rate over 8.3 years was 0.48 per 100,000 person-years. The age-adjusted (2000 US standard) incidence rate was 0.39 per 100,000 person years. The age-adjusted incidence rate of primary cutaneous T-cell lymphoma in Wales is similar to the reported standardized (1966 World standard population) incidence rate in Norway from 2000-2003 of 0.29 per 100,000 person years, however it is much lower than the reported age-adjusted (2000 US standard) incidence rate in the US from 2001-2005 of 7.7 per 100,000 person years. This discrepancy may be related to a true effect because of differences in population or because of other unexplained factors.
Commercial support: None identified.
Commercial support: None identified.
AB146
J AM ACAD DERMATOL
APRIL 2013
P6618
Fluorescence diagnosis for follow-up of mycosis fungoides therapy Manal Bosseila, MD, Dermatology Department, Faculty of Medicine, Cairo, Egypt; Abeer Mostafa, MD, Clinical Pathology Department, National Cancer Institute, Cairo, Egypt; Dina Metwalli, MD, Dermatology Department, Faculty of Medicine, Cairo, Egypt; Doaa Mahgoub, MD, Dermatology Department, Faculty of Medicine, Cairo, Egypt; Fatma Salah ElDin, MBBCh, National Research Center, Cairo, Egypt; Marwa El-Shaer, MD, National Research Center, Cairo, Egypt
Ichthyosiform mycosis fungoides: Clinicopathologic, immunophenotypic, and molecular features Kee Suck Suh, MD, Department of Dermatology, Kosin University College of Medicine, Busan, South Korea; Dong Young Kang, MD, Department of Dermatology, Kosin University College of Medicine, Busan, South Korea; Jong Bin Park, MD, Department of Dermatology, Kosin University College of Medicine, Busan, South Korea; Min Soo Jang, MD, Department of Dermatology, Kosin University College of Medicine, Busan, South Korea; Sang Hwa Han, MD, Department of Dermatology, Kosin University College of Medicine, Busan, South Korea; Sang Tae Kim, MD, Department of Dermatology, Kosin University College of Medicine, Busan, Korea, North
Background: Response to therapy of mycosis fungoides (MF) cases is usually evaluated clinically and by repeated skin biopsies for routine histopathology or T cell receptor rearrangement. Fluorescence diagnosis (FD) represents a procedure for in vivo diagnosis of dysplastic or neoplastic tissue in a wide variety of tumors such as nonmelanoma skin cancer and follow-up of their therapy, especially photodynamic therapy. Objective: To evaluate the efficiency of FD as a noninvasive follow-up tool of therapy in cases of MF, in order to minimize the use of invasive skin biopsies. Methods: Twenty-five patients with MF stages I or II, confirmed clinically and histopathologically, were included in this study. The most affected patch/plaque at time of inclusion was subjected to clinical assessment, FD using 15% aminolevulinic acid cream, and skin biopsy for flowcytometric immunophenotyping for CD4+CD7e T lymphocytes. Patients were treated by various treatment modalities for 12 weeks, and the same patch/plaque was subjected again to FD and flowcytometric immunophenotyping. Quantification of fluorescence was enabled using digital image analysis and then compared to the gated percentage of MF cells (CD4+CD7e) infiltrating the skin. Ethical approval was attained from dermatology department research ethical committee. An informed consent for participation was obtained from patients. Results: Twenty-two patients completed the study, 14 (63.6%) of them showed clinically very good or good response to therapy. By FD of 22 evaluated lesions the mean accumulation factor, defined as the ratio of protoporphyrin IX (PpIX) fluorescence of tumor tissue to that of surrounding normal skin, was found to be 2.2 6 0.68 before therapy and 1.95 6 0.64 after therapy. On correlating the values for FD with flowcytometric immunophenotyping before and after therapy of skin lesions no statistically significant correlation was found. Conclusion: Fluorescence diagnosis, as a noninvasive tool for follow-up of treatment in cases of MF, shows promising results as indicator for disease improvement. Further verification of its role needs to be done on a larger scale of patients. Commercial support: None identified.
Background: Ichthyosiform mycosis fungoides (MF) is a rare variant of MF, characterized clinically as having an ichthyosiform skin eruption, and histologically as having mycosis fungoides and ichthyosis vulgaris (IV). It is relatively unknown compared to other variants. Previous studies on ichthyosiform MF have been mainly conducted in western countries, and only a few cases has been reported. Few studies have been conducted on Asians in a single institution on a large scale. Particularly, it is difficult to precisely define this disease because its clinicopathological findings vary depending on the study. In addition, the relationship between the occurrence of an ichthyosiform lesion and MF is also unknown. Methods: This study was conducted on 9 patients with an ichthyosiform lesion (4.2%) among 216 patients diagnosed with MF in the authors’ hospital. The patients underwent clinical, histopathological, and molecular examinations to determine the characteristics of ichthyosiform MF, and to obtain data that would be helpful for the diagnosis and treatment of this disease. Results: The patients’ ages ranged widely from 8 to 63 years. The mean age was 29.2 years including three patients aged 12 years or lower. They all had early-stage MF, of which the IB and IIA types were observed in 8 patients and 1 patient, respectively. The patients were classified according to their clinical features. There were 4 cases of typical MF and an ichthyosiform lesion, of which the ichthyosiform lesion occurred in 2 patients in the early stage of the disease, and in the remaining 2 patients after the occurrence of the typical MF lesion; one case of concurrent hypopigmented MF; 1 case of angiocentric MF with multiple ulcers; and 3 cases of only an ichthyosiform lesion. From a histologic perspective, 9 patients had both MF and IV in a single lesion, of whom 1 patient had concurrent vasculitis. As shown in IV, filaggrin expression decreased in the 9 patients. Two patients had a CD4/CD8 ratio [1. PCR detection of TCR g gene rearrangement showed monoclonality in 5 patients. Targeted phototherapy was primarily conducted on all the patients, and they showed either complete or incomplete responses. Conclusion: Ichthyosiform MF is a rare variant of MF, and seems to have relatively favorable prognoses. In the case of an ichthyosiform skin eruption, MF could be suspected. A biopsy is required to confirm ichthyosiform MF. Commercial support: None identified.
P6027 Granuloma annulare heralding relapse of angioimmunoblastic T cell nonHodgkin lymphoma Bassel Mahmoud, MD, PhD, Henry Ford Hospital, Detroit, MI, United States; Chauncey McHargue, MD, Henry Ford Hospital, Detroit, MI, United States Background: Granuloma annulare (GA) is a benign papular eruption seen in patients of all ages. The primary skin lesion is in the form of grouped papules in an enlarging annular shape, with color ranging from flesh-colored to mildly erythematous. The exact etiology of GA in unknown but studies have shown that cell-mediated delayedtype hypersensitivity reaction may be implicated. GA has been reported with solid organ tumors and as well as myeloproliferative disorders including leukemia and lymphoma. We present a rare case of GA heralding relapsed angioimmunoblastic T cell non-Hodgkin lymphoma (AITL).
P6120 Incidence of primary cutaneous T-cell lymphoma in Wales Rachel Abbott, MBBS, University Hospital of Wales, Cardiff, United Kingdom; Christian Aldridge, MBBCh, Prince Charles Hospital, Merthyr Tydfil, United Kingdom; Stefan Dojcinov, MD, University Hospital of Wales, Cardiff, United Kingdom; Vincent Piguet, MD, PhD, Cardiff University, Cardiff, United Kingdom
Case report: A 58-year-old white man with a history of AITL treated 3 years ago with 6 cycles of CHOP chemotherapy followed by autologous stem cell transplantation, after which he achieved complete remission. Patient started to develop a mildly itchy rash on the arms that spread to the trunk and lower extremities of few weeks duration. Examination showed widespread cutaneous and subcutaneous firm erythematous papules with no surface change. Differential diagnosis included cutaneous lymphoma, sarcoidosis and GA. Histopathologic examination of both cutaneous and facial lesions were consistent with GA. TCR gene rearrangement did not identify a clonal population of lymphocytes is the skin. A PET scan showed generalized adenopathy, and mediastinal node biopsy showed recurrent angioimmunoblastic T-cell lymphoma. Notably the recurrent lymphoma was surrounded by a caseating granulomatous infiltrate. Discussion: GA has been reported with malignant lymphoma including 1 case preceding the onset of AITL. The onset of GA ranges from 5 years before to 27 years after the diagnosis of lymphoma. Our case is a rare example of GA heralding relapsed AITL. The patient is currently being considered for allogeneic stem cell transplant and chemotherapy. GA in this case represents a paraneoplastic phenomenon. It remains to be seen whether a successful second remission results in resolution of the secondary skin GA lesions.
Primary cutaneous T-cell lymphoma is a rare disease. The incidence of this disease has not previously been studied in Wales, which has a population of 3 million. Our aim was to estimate the incidence rate of primary cutaneous T-cell lymphoma while conducting a clinical audit of the diagnosis and management of patients with primary cutaneous T-cell lymphoma. Patients with primary cutaneous T-cell lymphoma were identified through the All Wales Lymphoma Panel (a national pathology database), local health board pathology databases and dermatology outpatient clinics. Patients who were diagnosed between 01 August 2003 (after publication of the UK guidelines on the diagnosis and management of primary cutaneous T-cell lymphoma) and 31 October 2011 were included. In total, 120 patients diagnosed with primary cutaneous T-cell lymphoma between 01 July 2003 and 31 October 2011 were identified. The crude incidence rate over 8.3 years was 0.48 per 100,000 person-years. The age-adjusted (2000 US standard) incidence rate was 0.39 per 100,000 person years. The age-adjusted incidence rate of primary cutaneous T-cell lymphoma in Wales is similar to the reported standardized (1966 World standard population) incidence rate in Norway from 2000-2003 of 0.29 per 100,000 person years, however it is much lower than the reported age-adjusted (2000 US standard) incidence rate in the US from 2001-2005 of 7.7 per 100,000 person years. This discrepancy may be related to a true effect because of differences in population or because of other unexplained factors.
Commercial support: None identified.
Commercial support: None identified.
AB146
J AM ACAD DERMATOL
APRIL 2013