P6207Fluorescence 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 ofMedicine, Cairo, Egypt; Fatma Salah ElDin, MBBCh, National Research Center,Cairo, Egypt; Marwa El-Shaer, MD, National Research Center, Cairo, Egypt

Background: Response to therapy of mycosis fungoides (MF) cases is usuallyevaluated clinically and by repeated skin biopsies for routine histopathology or Tcell receptor rearrangement. Fluorescence diagnosis (FD) represents a procedurefor in vivo diagnosis of dysplastic or neoplastic tissue in a wide variety of tumorssuch as nonmelanoma skin cancer and follow-up of their therapy, especiallyphotodynamic therapy.

Objective: To evaluate the efficiency of FD as a noninvasive follow-up tool of therapyin 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 andhistopathologically, were included in this study. The most affected patch/plaque attime of inclusion was subjected to clinical assessment, FD using 15% aminolevulinicacid 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 flowcytometricimmunophenotyping. Quantification of fluorescence was enabled using digitalimage analysis and then compared to the gated percentage of MF cells (CD4+CD7e)infiltrating the skin. Ethical approval was attained from dermatology departmentresearch ethical committee. An informed consent for participation was obtainedfrom patients.

Results: Twenty-two patients completed the study, 14 (63.6%) of them showedclinically very good or good response to therapy. By FD of 22 evaluated lesions themean accumulation factor, defined as the ratio of protoporphyrin IX (PpIX)fluorescence of tumor tissue to that of surrounding normal skin, was found to be2.2 6 0.68 before therapy and 1.95 6 0.64 after therapy. On correlating the valuesfor FD with flowcytometric immunophenotyping before and after therapy of skinlesions no statistically significant correlation was found.

Conclusion: Fluorescence diagnosis, as a noninvasive tool for follow-up of treatmentin 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.

AB146

cial support: None identified.

Commer

P6027Granuloma annulare heralding relapse of angioimmunoblastic T cell non-Hodgkin 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 patientsof all ages. The primary skin lesion is in the form of grouped papules in an enlargingannular shape, with color ranging from flesh-colored to mildly erythematous. Theexact etiology of GA in unknown but studies have shown that cell-mediated delayed-type hypersensitivity reaction may be implicated. GA has been reported with solidorgan tumors and as well as myeloproliferative disorders including leukemia andlymphoma. We present a rare case of GA heralding relapsed angioimmunoblastic Tcell non-Hodgkin lymphoma (AITL).

Case report: A 58-year-old white man with a history of AITL treated 3 years ago with6 cycles of CHOP chemotherapy followed by autologous stem cell transplantation,after which he achieved complete remission. Patient started to develop a mildlyitchy rash on the arms that spread to the trunk and lower extremities of few weeksduration. Examination showed widespread cutaneous and subcutaneous firmerythematous papules with no surface change. Differential diagnosis includedcutaneous lymphoma, sarcoidosis and GA. Histopathologic examination of bothcutaneous and facial lesions were consistent with GA. TCR gene rearrangement didnot identify a clonal population of lymphocytes is the skin. A PET scan showedgeneralized adenopathy, and mediastinal node biopsy showed recurrent angioim-munoblastic T-cell lymphoma. Notably the recurrent lymphoma was surrounded bya caseating granulomatous infiltrate.

Discussion: GA has been reported with malignant lymphoma including 1 casepreceding the onset of AITL. The onset of GA ranges from 5 years before to 27 yearsafter the diagnosis of lymphoma. Our case is a rare example of GA heralding relapsedAITL. The patient is currently being considered for allogeneic stem cell transplantand chemotherapy. GA in this case represents a paraneoplastic phenomenon. Itremains to be seen whether a successful second remission results in resolution ofthe secondary skin GA lesions.

cial support: None identified.

Commer

J AM ACAD DERMATOL

P6618Ichthyosiformmycosis fungoides: Clinicopathologic, immunophenotypic,and molecular features

Kee Suck Suh, MD, Department of Dermatology, Kosin University College ofMedicine, Busan, South Korea; Dong Young Kang, MD, Department ofDermatology, Kosin University College of Medicine, Busan, South Korea; JongBin Park, MD, Department of Dermatology, Kosin University College of Medicine,Busan, South Korea; Min Soo Jang, MD, Department of Dermatology, KosinUniversity 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 UniversityCollege of Medicine, Busan, Korea, North

Background: Ichthyosiform mycosis fungoides (MF) is a rare variant of MF, charac-terized clinically as having an ichthyosiform skin eruption, and histologically ashaving mycosis fungoides and ichthyosis vulgaris (IV). It is relatively unknowncompared to other variants. Previous studies on ichthyosiformMF have been mainlyconducted in western countries, and only a few cases has been reported. Fewstudies have been conducted on Asians in a single institution on a large scale.Particularly, it is difficult to precisely define this disease because its clinicopatho-logical findings vary depending on the study. In addition, the relationship betweenthe 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 patientsunderwent clinical, histopathological, andmolecular examinations to determine thecharacteristics of ichthyosiform MF, and to obtain data that would be helpful for thediagnosis and treatment of this disease.

Results: The patients’ ages ranged widely from 8 to 63 years. The mean age was 29.2years including three patients aged 12 years or lower. They all had early-stage MF, ofwhich 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 casesof typical MF and an ichthyosiform lesion, of which the ichthyosiform lesionoccurred in 2 patients in the early stage of the disease, and in the remaining 2patients after the occurrence of the typical MF lesion; one case of concurrenthypopigmented MF; 1 case of angiocentric MF with multiple ulcers; and 3 cases ofonly an ichthyosiform lesion. From a histologic perspective, 9 patients had both MFand IV in a single lesion, of whom 1 patient had concurrent vasculitis. As shown inIV, filaggrin expression decreased in the 9 patients. Two patients had a CD4/CD8ratio[1. PCR detection of TCR g gene rearrangement showed monoclonality in 5patients. Targeted phototherapy was primarily conducted on all the patients, andthey showed either complete or incomplete responses.

Conclusion: Ichthyosiform MF is a rare variant of MF, and seems to have relativelyfavorable prognoses. In the case of an ichthyosiform skin eruption, MF could besuspected. A biopsy is required to confirm ichthyosiform MF.

cial support: None identified.

Commer

P6120Incidence 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, UnitedKingdom; Stefan Dojcinov, MD, University Hospital of Wales, Cardiff, UnitedKingdom; Vincent Piguet, MD, PhD, Cardiff University, Cardiff, United Kingdom

Primary cutaneous T-cell lymphoma is a rare disease. The incidence of this diseasehas not previously been studied in Wales, which has a population of 3 million. Ouraim was to estimate the incidence rate of primary cutaneous T-cell lymphoma whileconducting a clinical audit of the diagnosis and management of patients withprimary cutaneous T-cell lymphoma. Patients with primary cutaneous T-celllymphoma were identified through the All Wales Lymphoma Panel (a nationalpathology database), local health board pathology databases and dermatologyoutpatient clinics. Patients who were diagnosed between 01 August 2003 (afterpublication of the UK guidelines on the diagnosis and management of primarycutaneous T-cell lymphoma) and 31 October 2011 were included. In total, 120patients diagnosed with primary cutaneous T-cell lymphoma between 01 July 2003and 31 October 2011 were identified. The crude incidence rate over 8.3 years was0.48 per 100,000 person-years. The age-adjusted (2000 US standard) incidence ratewas 0.39 per 100,000 person years. The age-adjusted incidence rate of primarycutaneous T-cell lymphoma in Wales is similar to the reported standardized (1966World standard population) incidence rate in Norway from 2000-2003 of 0.29 per100,000 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 100,000person years. This discrepancy may be related to a true effect because of differencesin population or because of other unexplained factors.

cial support: None identified.

Commer

APRIL 2013