- Email: [email protected]
Case of mycosis fungoides with atypical immunophenotype
P2708
P2710
Primary cutaneous aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma showing less pronounced epidermotropism Sook Kyung Lee, MD, Department of Dermatology, Maryknoll Hospital, Busan, South Korea; Hyun Soo Sim, MD, Department of Dermatology, Busan Baik Hospital, Busan, South Korea; Jong-Keun Seo, MD, Department of Dermatology, Busan Baik Hospital, Busan, South Korea; Young Seok Lee, MD, Department of Dermatology, Maryknoll Hospital, Busan, South Korea Primary cutaneous aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma is a rare and provisional entity, characterized by a proliferation of epidermotropic CD8+ cytotoxic T cells and an aggressive clinical behavior. We report a case of 92-year-old man who presented with multiple erythematous patches and ulceronecrotic plaques with less pronounced epidermotropism and dense perivascular and periappendageal atypical lymphocytes with large cell morphology, extending to subcutis. The tumor cells expressed CD2, CD3, CD8, granzyme B, TIA-a, ßF1 and were negative for CD4, CD7, CD30, CD56, CD45RO, CD45RA, and Epstein-Barr virus stains. A monoclonal TCR-g gene rearrangement was present. He had a 1-year history of seborrheic dermatitis, exfoliative dermatitis, and was recently treated for the several papules under the diagnosis of lichenoid drug eruption at other clinic, but he showed no improvement and visited our hospital. The clinical course showed rapid progression from papules to generalized erythematous patches, nodules, and ulceronecrotic plaques within only 1 month before visit. Despite symptomatical treatment during hospitalization, there was no improvement of the skin lesions, then he died because of multiple organ failure 1 month later. In our case, the aggressive clinical behavior and appearance of skin lesions were consistent with primary cutaneous aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma, but it showed less pronounced epidermotropism, which was different from classically described type.
Case of mycosis fungoides with atypical immunophenotype Husein Husein-ElAhmed, MD, San Cecilio Clinical Hospital, Department of Dermatology, Granada, Spain; Jose Aneiros-Cachaza, MD, PhD, San Cecilio Clinical Hospital, Department of Pathology, Granada, Spain; Jose AneirosFernandez, MD, San Cecilio Clinical Hospital, Department of Pathology, Granada, Spain; Marıa Antonia Fernandez-Pugnaire, MD, San Cecilio Clinical Hospital, Department of Dermatology, Granada, Spain; Salvador Arias-Santiago, MD, San Cecilio Clinical Hospital, Department of Dermatology, Granada, Spain Background: Fewer than 5% of cases of mycosis fungoides (MF) present with a cytotoxic/suppressor CD8+ phenotype which, despite immunophenotypic similarities with CD8+ aggressive lymphomas, is regarded as a phenotypic variant of MF. We report a case of woman who presented with this atypical immunophenotype of MF. Case report: A 75-year-old white woman with a history of arterial hypertension in 2000 complained of a rash for 13 to 15 years, which initially did not bother her. It started as an erythematous, nonscaly rash, which was nonpruritic except after a warm shower. Gradually over the years the rash progressed to indurated, scaly, erythematous patches which worsened during the summer. Upon presentation to our clinic the rash covered about 45% of patient’s body, principally on the trunk, hands, and upper legs. It had become more pruritic. A skin biopsy revealed a CD3+, CD8+, CD5+, and CD4e atypical lymphocytes invading the epidermis. There was no evidence for lymph node or organ involvement by computerized tomography or of peripheral blood involvement by flow cytometry. Therefore, the patient was deemed to have stage IB CD8+ mycosis fungoides. The patient was started on topical steroid but his skin disease progressed. Psoralen plus ultraviolet A was added to her regimen. Within weeks she began to respond with a decrease of pruritus, epithelial desquamation and obvious skin lesions. By 3 months of treatment, she had achieved a complete response.
Commercial support: None identified.
Conclusion: MF showing a CD8+ phenotype present with a long-standing disease which has been reported to have a nonaggressive clinical behavior. In our case, a good response to psoralen plus ultraviolet A treatment was achieved and the time period between disease onset and diagnosis was long. This highlights the complexities in the diagnosis and treatment of these disorders and underscores the need for further research regarding etiology and pathogenesis. Commercial support: None identified.
P2711 P2709 Childhood primary cutaneous CD30-positive anaplastic large cell lymphoma: A child case with spontaneous regression. Ritsuko Kobayashi, MD, St. Lukes International Hospital, Tokyo, Japan; Hikaru Eto, MD, St. Lukes International Hospital, Tokyo, Japan; Tomoe Kajiwara, St. Lukes International Hospital, Tokyo, Japan; Toshiaki Nakano, MD, St. Lukes International Hospital, Tokyo, Japan; Yohko Momose, MD, St. Lukes International Hospital, Tokyo, Japan Primary cutaneous CD30-positive anaplastic large cell lymphoma (C-ALCL) is in one of the spectrum of CD30-positive primary cutaneous lymphoproliferative disorders including lymphomatoid papulosis (LyP). LyP generally tend to show spontaneous regression and therefore classified as a benign tumor. Primary C-ALCL is classified as malignant tumor, normally affects adults, which prognosis is relatively well. The majority of primary C-ALCL cases published in the literature do not show spontaneous regression. A 5-year-old girl presented with an asymptomatic indurated erythematous nodule on her left lower eyelid. It became considerably enlarged and doubled in size within a month. Histological findings of biopsy revealed that atypical large lymphocytes were massively infiltrated in the dermis and fat tissue. In immunostaining, marker profiles of the atypical cells were CD4+, CD30+, anaplastic lymphoma kinase (ALK)e. From these data, we diagnosed her as primary C-ALCL. After biopsy, the tumor gradually became reduced in size without any treatment. Primary C-ALCL has only rarely been described in the pediatric population. This case seems to be a rare primary C-ALCL child case who showed spontaneous regression. Commercial support: None identified.
AB116
J AM ACAD DERMATOL
Erythroderma and pruritus of 1 year of evolution Jacinto Orgaz-Molina, MD, Hospital Clınico San Cecilio, Granada, Spain; Husein Husein-Elhamed, MD, Hospital Clınico San Cecilio, Granada, Spain; Marisa Soriano-Hernandez, MD, Hospital Clınico San Cecilio, Granada, Spain; Ram on Naranjo-Sintes, MD, Hospital Clınico San Cecilio, Granada, Spain Background: Sezary syndrome is the leukemic variant of mycosis fungoides. It is classically described by the triad of erythroderma, widespread adenopathies, and circulating cerebriform nuclear contour cells (Sezary cells). Cutaneous erythroderma is a diagnostic challenge in dermatology. Case report: A 64 year-old was referred to our clinic with a 1-year history of progressive skin lesions associated with widespread intense pruritus and multiples cervical and inguinal adenopathies. Medical history showed no drug administration, systemic, nor dermatologic diseases. Before attending to our center, two biopsies were performed in another clinic with no significant findings. We performed a cutaneous biopsy, fine needle aspiration of an inguinal adenopathy and peripheral blood test; these medical tests confirmed the diagnosis of Sezary syndrome. Extension study was negative. Treatment with bexaroten and phototherapy was iniciated. Discussion: Sezary syndrome is a low-incidence disease. Diagnosis is often delayed because of nonspecific histologic manifestations in the early stages. Morever, the presence of Sezary cells is not a pathognomonic finding or the classic triad is quite unspecific. Sezary syndrome is a clinical entity that requires the association of complementary criteria. As our case, the evidence by PCR test to demonstrate the clonal nature of Sezary cells in peripheral blood is a specific criterion. Commercial support: None identified.
FEBRUARY 2011
P2710
Primary cutaneous aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma showing less pronounced epidermotropism Sook Kyung Lee, MD, Department of Dermatology, Maryknoll Hospital, Busan, South Korea; Hyun Soo Sim, MD, Department of Dermatology, Busan Baik Hospital, Busan, South Korea; Jong-Keun Seo, MD, Department of Dermatology, Busan Baik Hospital, Busan, South Korea; Young Seok Lee, MD, Department of Dermatology, Maryknoll Hospital, Busan, South Korea Primary cutaneous aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma is a rare and provisional entity, characterized by a proliferation of epidermotropic CD8+ cytotoxic T cells and an aggressive clinical behavior. We report a case of 92-year-old man who presented with multiple erythematous patches and ulceronecrotic plaques with less pronounced epidermotropism and dense perivascular and periappendageal atypical lymphocytes with large cell morphology, extending to subcutis. The tumor cells expressed CD2, CD3, CD8, granzyme B, TIA-a, ßF1 and were negative for CD4, CD7, CD30, CD56, CD45RO, CD45RA, and Epstein-Barr virus stains. A monoclonal TCR-g gene rearrangement was present. He had a 1-year history of seborrheic dermatitis, exfoliative dermatitis, and was recently treated for the several papules under the diagnosis of lichenoid drug eruption at other clinic, but he showed no improvement and visited our hospital. The clinical course showed rapid progression from papules to generalized erythematous patches, nodules, and ulceronecrotic plaques within only 1 month before visit. Despite symptomatical treatment during hospitalization, there was no improvement of the skin lesions, then he died because of multiple organ failure 1 month later. In our case, the aggressive clinical behavior and appearance of skin lesions were consistent with primary cutaneous aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma, but it showed less pronounced epidermotropism, which was different from classically described type.
Case of mycosis fungoides with atypical immunophenotype Husein Husein-ElAhmed, MD, San Cecilio Clinical Hospital, Department of Dermatology, Granada, Spain; Jose Aneiros-Cachaza, MD, PhD, San Cecilio Clinical Hospital, Department of Pathology, Granada, Spain; Jose AneirosFernandez, MD, San Cecilio Clinical Hospital, Department of Pathology, Granada, Spain; Marıa Antonia Fernandez-Pugnaire, MD, San Cecilio Clinical Hospital, Department of Dermatology, Granada, Spain; Salvador Arias-Santiago, MD, San Cecilio Clinical Hospital, Department of Dermatology, Granada, Spain Background: Fewer than 5% of cases of mycosis fungoides (MF) present with a cytotoxic/suppressor CD8+ phenotype which, despite immunophenotypic similarities with CD8+ aggressive lymphomas, is regarded as a phenotypic variant of MF. We report a case of woman who presented with this atypical immunophenotype of MF. Case report: A 75-year-old white woman with a history of arterial hypertension in 2000 complained of a rash for 13 to 15 years, which initially did not bother her. It started as an erythematous, nonscaly rash, which was nonpruritic except after a warm shower. Gradually over the years the rash progressed to indurated, scaly, erythematous patches which worsened during the summer. Upon presentation to our clinic the rash covered about 45% of patient’s body, principally on the trunk, hands, and upper legs. It had become more pruritic. A skin biopsy revealed a CD3+, CD8+, CD5+, and CD4e atypical lymphocytes invading the epidermis. There was no evidence for lymph node or organ involvement by computerized tomography or of peripheral blood involvement by flow cytometry. Therefore, the patient was deemed to have stage IB CD8+ mycosis fungoides. The patient was started on topical steroid but his skin disease progressed. Psoralen plus ultraviolet A was added to her regimen. Within weeks she began to respond with a decrease of pruritus, epithelial desquamation and obvious skin lesions. By 3 months of treatment, she had achieved a complete response.
Commercial support: None identified.
Conclusion: MF showing a CD8+ phenotype present with a long-standing disease which has been reported to have a nonaggressive clinical behavior. In our case, a good response to psoralen plus ultraviolet A treatment was achieved and the time period between disease onset and diagnosis was long. This highlights the complexities in the diagnosis and treatment of these disorders and underscores the need for further research regarding etiology and pathogenesis. Commercial support: None identified.
P2711 P2709 Childhood primary cutaneous CD30-positive anaplastic large cell lymphoma: A child case with spontaneous regression. Ritsuko Kobayashi, MD, St. Lukes International Hospital, Tokyo, Japan; Hikaru Eto, MD, St. Lukes International Hospital, Tokyo, Japan; Tomoe Kajiwara, St. Lukes International Hospital, Tokyo, Japan; Toshiaki Nakano, MD, St. Lukes International Hospital, Tokyo, Japan; Yohko Momose, MD, St. Lukes International Hospital, Tokyo, Japan Primary cutaneous CD30-positive anaplastic large cell lymphoma (C-ALCL) is in one of the spectrum of CD30-positive primary cutaneous lymphoproliferative disorders including lymphomatoid papulosis (LyP). LyP generally tend to show spontaneous regression and therefore classified as a benign tumor. Primary C-ALCL is classified as malignant tumor, normally affects adults, which prognosis is relatively well. The majority of primary C-ALCL cases published in the literature do not show spontaneous regression. A 5-year-old girl presented with an asymptomatic indurated erythematous nodule on her left lower eyelid. It became considerably enlarged and doubled in size within a month. Histological findings of biopsy revealed that atypical large lymphocytes were massively infiltrated in the dermis and fat tissue. In immunostaining, marker profiles of the atypical cells were CD4+, CD30+, anaplastic lymphoma kinase (ALK)e. From these data, we diagnosed her as primary C-ALCL. After biopsy, the tumor gradually became reduced in size without any treatment. Primary C-ALCL has only rarely been described in the pediatric population. This case seems to be a rare primary C-ALCL child case who showed spontaneous regression. Commercial support: None identified.
AB116
J AM ACAD DERMATOL
Erythroderma and pruritus of 1 year of evolution Jacinto Orgaz-Molina, MD, Hospital Clınico San Cecilio, Granada, Spain; Husein Husein-Elhamed, MD, Hospital Clınico San Cecilio, Granada, Spain; Marisa Soriano-Hernandez, MD, Hospital Clınico San Cecilio, Granada, Spain; Ram on Naranjo-Sintes, MD, Hospital Clınico San Cecilio, Granada, Spain Background: Sezary syndrome is the leukemic variant of mycosis fungoides. It is classically described by the triad of erythroderma, widespread adenopathies, and circulating cerebriform nuclear contour cells (Sezary cells). Cutaneous erythroderma is a diagnostic challenge in dermatology. Case report: A 64 year-old was referred to our clinic with a 1-year history of progressive skin lesions associated with widespread intense pruritus and multiples cervical and inguinal adenopathies. Medical history showed no drug administration, systemic, nor dermatologic diseases. Before attending to our center, two biopsies were performed in another clinic with no significant findings. We performed a cutaneous biopsy, fine needle aspiration of an inguinal adenopathy and peripheral blood test; these medical tests confirmed the diagnosis of Sezary syndrome. Extension study was negative. Treatment with bexaroten and phototherapy was iniciated. Discussion: Sezary syndrome is a low-incidence disease. Diagnosis is often delayed because of nonspecific histologic manifestations in the early stages. Morever, the presence of Sezary cells is not a pathognomonic finding or the classic triad is quite unspecific. Sezary syndrome is a clinical entity that requires the association of complementary criteria. As our case, the evidence by PCR test to demonstrate the clonal nature of Sezary cells in peripheral blood is a specific criterion. Commercial support: None identified.
FEBRUARY 2011