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Cutaneous plasmablastic lymphoma in an HIV-positive female
P2003
P2005
An unusual case of ‘‘recurrent impetigo’’ Katherine Panting, MBBCh, Broadgreen Hospital, Liverpool, United Kingdom; Niamh Leonard, MBChB, Royal Liverpool University Hospital, Liverpool, United Kingdom; Richard Azurdia, MBChB, Broadgreen Hospital, Liverpool, United Kingdom We report a 32-year-old female with a 3-year history of intermittent weeping, crusting, and swelling to the bridge of the nose that had become persistent over the preceding 8 weeks before dermatology review. She had been treated with antibiotics for suspected impetigo on a number of occasions with partial benefit. She was systemically very well with no symptoms of weight loss, night sweats or pruritus. There was a history of allergic contact dermatitis to nickel, but there was no other history of note. On examination, she had an erythematous plaque to the bridge of the nose and inner cheeks with overlying hyperkeratosis, ulceration and induration measuring 8 cm 3 4 cm. There was no lymphadenopathy. The initial differential diagnosis was wide, including lupus erythematosus, infection (including fungal or mycobacterial infection), granuloma faciale and a neoplastic lesion. A number of investigations were requested which showed a positive speckled anti-nuclear antigen (ANA) of 1:640, negative endonuclear antibody (ENA) and mildly elevated erythrocyte sedimentation rate (ESR) of 26. Full blood count (FBC), renal function, liver function, syphilis serology, ANCAs, and a chest radiograph were all normal or negative. An urgent incisional biopsy was obtained which showed surface ulceration, a dense dermal infiltrate of lymphocytes and occasional lymphocytes in the epidermis. Immunohistochemical staining showed CD56-positivity, and the findings were felt to be consistent with a natural killer (NK)/T-cell lymphoma of nasal type (no EBV studies were undertaken in our sample). Further staging investigations were performed, bone marrow aspirate and trephine showed no evidence of involvement and a CT scan demonstrated multiple small nodes within the chest, axilla and abdomen and mucosal thickening within the sinuses. Lymph node biopsy was not performed. The patient was treated with 6 cycles of systemic chemotherapy in the form of cyclophosphamide 750 mg/m2, doxorubicin 50 mg/m2, vincristine 1.4mg/m2, and prednisolone 100 mg daily (days 1-5) followed by involved field radiotherapy to the nose and sinuses (40 Gy in 20 fractions). There was excellent clinical resolution following treatment and she is now 11 months postchemotherapy and under close follow-up. NK/T-cell lymphoma is an aggressive, rare subtype of noneHodgkin’s lymphoma commonly presenting as an ulcerative, destructive midline facial lesion. It has a worse prognosis than many other types of lymphoma and is often resistant to standard chemotherapy regimes.1 Estimated 3-year survival is around 60% following CHOP chemotherapy and adjuvant radiotherapy; however, the prognosis is worse if the patient displays advanced disease stage, poor performance status, high LDH, or is aged above 60 years of age.2 It is often associated with EpsteineBarr virus (EBV) and usually displays CD56, CD2, CD45RO, and CD43 positivity on immunohistochemistry. The case highlights the importance of including NK/T-cell lymphoma in the differential diagnosis for suspected infective midline facial lesions unresponsive to antibiotic therapy. References 1. Kin WS, et al. CHOP followed by involved field radiation: is it optimal for localized nasal natural killer T-cell lymphoma? Ann Oncol 2001;12:349-52. 2. Lee J, et al. Nasal-type NK/T-cell lymphoma: clinical features and treatment outcome. Br J Cancer 2005;92:1226-30.
Epidemiologic study of primary cutaneous lymphomas along 20 years at the Department of Dermatology of the University General Hospital of Valencia, Spain Elena Roche-Gamo´n, MD, University General Hospital of Valencia, Valencia, Spain; Maria Luisa Garcia-Melgares, MD, University General Hospital of Valencia, Valencia, Spain; Amparo Pe´rez-Ferriols, MD, PhD, University General Hospital of Valencia, Valencia, Spain; Vı´ctor Alegre de Miquel, MD, PhD, University General Hospital of Valencia, Valencia, Spain Introduction: The term primary cutaneous lymphoma (PCL) refers to cutaneous Tcell lymphomas (CTCL) and cutaneous B-cell lymphomas (CBCL) that present in the skin with no evidence of extracutaneous disease at the time of diagnosis. Materials and methods: We conducted a retrospective and prospective review of all patients with PCL presenting to the dermatology department at University General Hospital of Valencia, Spain, between May 1987 and May 2007. Results: A whole of 357 patients with PLC were diagnosed during the period of study. Three hundred and one (84.3%) of them belong CTCL and 56 (15.7%) CBCL. The most important anatomopathologic diagnoses were mycosis fungoides (MF) in 209 (58.5%), lymphomatoid papulosis (LP) in 60 (16.8%), others CTCL in 32 (9%), primary cutaneous marginal-zone B-cell lymphoma (PCMZL) in 29 (8.1%), folliclecenter lymphoma (FCL) in 18 (5%) and finally diffuse large B-cell lymphoma, leg type (DLBCL) in 9 (2.5%). In the group of CTCL 69.4% (209/301) were MF, 19.9% (60/301) PL, primary cutaneous CD41 small/medium peripheral T-cell lymphoma in 5.3% (16/301), unspecified T-cell lymphoma in 1.9% (6/301), primary cutaneous anaplastic large-cell lymphoma in 1.3% (4/301), extranodal NK/T-cell lymphoma, nasal-type, in 0.7% (2/301), primary cutaneous peripheral T-cell lymphoma, unspecified in 0.7% (2/301), and primary cutaneous angioinmunoblastic T-cell lymphoma in 0.7% (2/301). In the group of CBCL the percentages of each type were PCMZL in 51.8 % (29/56), FCL in 32.1 % (18/56), and DLBCL in 16.1% (9/56). Finally, we compare the epidemiologic characteristics (age and sexes) of CBCL and the CTCL. Commercial support: None identified.
Commercial support: None identified.
P2004 Three cases of nasal type NK/T-cell lymphoma Lucı´a Martı´nez Casimiro, Hospital General Universitario, Valencia, Spain; Violeta Zaragoza Ninet, Hospital General Universitario, Valencia, Spain; Maria Luisa Garcı´a Melgares Linares, Hospital General Universitario, Valencia, Spain; Vı´ctor Alegre de Miquel, Hospital General Universitario, Valencia, Spain Nasal type NK-T-cell lymphoma, previously known as lethal midline granuloma, is a rare type of noneHodkin’s lymphoma associated with the EpsteineBarr virus (EBV). Here we show three cases with nasal-type NK-T-cell lymphoma seen in our hospital. Case 1: A 57-year-old female presented in September 1989 with several-month history of nasal congestion and purulent rhinorrhea. A biopsy of an intranasal mass was diagnosed as idiopathic lethal midline granuloma and treated with radiotherapy. In April 1994, she experienced erythema and edema that involved the nasal bridge, malar and right periorbital area. A skin nasal biopsy revealed necrosis and reactive inflammation. CT scan showed midline destruction. She was treated with radiotherapy, chemotherapy, and surgical debridement. In August 1996, multiple red plaques appeared on her right leg. Skin biopsy showed diffuse infiltration of atypical lymphoid cells positive for CD3 and CD56 within the entire dermis and subcutaneous tissue. She died in a month. Case 2: A 42-year-old female, HIV positive, had in October 2006 a 2-month history of nasal obstruction and swelling and erythema of the nose. Nasal mucosa PAAF biopsy showed necrosis and reactive cells. She developed crusted plaques on her nose. A skin biopsy of the border showed a dense infiltrate of pleomorfic lymphocytes positive for CD3, CD56, and EBV within the entire dermis and necrosis. TC scan showed a mass in cavum and multiple lymph nodes. She was treated with CHOP chemotherapy but died within 3 months. Case 3: A 66-year-old female presented in February 2007 with a 6-month history of red plaques and nodules on her legs and left breast. Skin biopsies showed a diffuse infiltration of atypical lymphoid cells positive for CD3, CD56, and EBV throughout the dermis and subcutaneous tissue. TC scan showed a mass in the right nasal cavity. She is being treated with CHOP chemotherapy. Nasal NK-T cell lymphomas arise in the nasal cavity and paranasal sinuses. Patients present with nasal obstruction or extensive midfacial destruction. Other presentations include skin nodules. The prognosis is very poor independent on the treatment. Histology reveals a diffuse proliferation of atypical lymphocytes positive for CD2, CD56 and cytoplasmic CD3 with angiocentricity involving the dermis and often the subcutaneous tissue, and necrosis. This lymphoma is very rare in HIV and has worse prognosis. Commercial support: None identified.
FEBRUARY 2008
P2006 Cutaneous plasmablastic lymphoma in an HIV-positive female Violeta Zaragoza Ninet, MD, Consorcio Hospital General Universitario De Valencia, Valencia, Spain; Lucı´a Martı´nez Casimiro, MD, Consorcio Hospital General Universitario, Valencia, Spain; Vı´ctor Alegre De Miguel, Consorcio Hospital General Universitario, Valencia, Spain; Juan Jose´ Vilata Corell, Consorcio Hospital General Universitario, Valencia, Spain Introduction: Plasmablastic lymphoma (PBL) is an aggressive, uncommon and recently described variant of diffuse large B-cell lymphoma (DLBCL). It typically occurs in the setting of HIV infection, presents in the oral cavity and 60% of cases are said to be EBV-related. Case report: A 43-year-old female presented with painful, fast growing, solid and livid nodules on her trunk and both legs. The patient had a known B3 HIV infection under antiretroviral therapy. The biopsy consisted of a normal epidermis overlying a dermis that was largely effaced by a diffuse infiltrate of monomorphic, partly immunoblastic, partly plasmacytoid cells, with a high proliferation index. The immunohistochemical staining was negative for CD20, CD3, TDT, bcl-6, and HHV-8, but positive for the plasma cell marker CD138. In situ hybridization for EBV-encoded RNA (EBER) was positive. A diagnosis of PBL was made. The patient was staged IV-A with subsequent examinations. Chemotherapy, including the CHOP regimen and intrathecal therapy, was started in combination with her antiretroviral treatment. The patient underwent just 1 round of CHOP and died because of chemotherapy complications. Conclusion: Since its first description, there are only few additional reports describing this relatively new variant of DLBCL. This case illustrates morphologic and immunohistochemical criteria for PBL, its clinical features, and poor prognosis. Furthemore, our case shows an inusual presentation arising at a cutaneous site. Commercial support: None identified.
J AM ACAD DERMATOL
AB101
P2005
An unusual case of ‘‘recurrent impetigo’’ Katherine Panting, MBBCh, Broadgreen Hospital, Liverpool, United Kingdom; Niamh Leonard, MBChB, Royal Liverpool University Hospital, Liverpool, United Kingdom; Richard Azurdia, MBChB, Broadgreen Hospital, Liverpool, United Kingdom We report a 32-year-old female with a 3-year history of intermittent weeping, crusting, and swelling to the bridge of the nose that had become persistent over the preceding 8 weeks before dermatology review. She had been treated with antibiotics for suspected impetigo on a number of occasions with partial benefit. She was systemically very well with no symptoms of weight loss, night sweats or pruritus. There was a history of allergic contact dermatitis to nickel, but there was no other history of note. On examination, she had an erythematous plaque to the bridge of the nose and inner cheeks with overlying hyperkeratosis, ulceration and induration measuring 8 cm 3 4 cm. There was no lymphadenopathy. The initial differential diagnosis was wide, including lupus erythematosus, infection (including fungal or mycobacterial infection), granuloma faciale and a neoplastic lesion. A number of investigations were requested which showed a positive speckled anti-nuclear antigen (ANA) of 1:640, negative endonuclear antibody (ENA) and mildly elevated erythrocyte sedimentation rate (ESR) of 26. Full blood count (FBC), renal function, liver function, syphilis serology, ANCAs, and a chest radiograph were all normal or negative. An urgent incisional biopsy was obtained which showed surface ulceration, a dense dermal infiltrate of lymphocytes and occasional lymphocytes in the epidermis. Immunohistochemical staining showed CD56-positivity, and the findings were felt to be consistent with a natural killer (NK)/T-cell lymphoma of nasal type (no EBV studies were undertaken in our sample). Further staging investigations were performed, bone marrow aspirate and trephine showed no evidence of involvement and a CT scan demonstrated multiple small nodes within the chest, axilla and abdomen and mucosal thickening within the sinuses. Lymph node biopsy was not performed. The patient was treated with 6 cycles of systemic chemotherapy in the form of cyclophosphamide 750 mg/m2, doxorubicin 50 mg/m2, vincristine 1.4mg/m2, and prednisolone 100 mg daily (days 1-5) followed by involved field radiotherapy to the nose and sinuses (40 Gy in 20 fractions). There was excellent clinical resolution following treatment and she is now 11 months postchemotherapy and under close follow-up. NK/T-cell lymphoma is an aggressive, rare subtype of noneHodgkin’s lymphoma commonly presenting as an ulcerative, destructive midline facial lesion. It has a worse prognosis than many other types of lymphoma and is often resistant to standard chemotherapy regimes.1 Estimated 3-year survival is around 60% following CHOP chemotherapy and adjuvant radiotherapy; however, the prognosis is worse if the patient displays advanced disease stage, poor performance status, high LDH, or is aged above 60 years of age.2 It is often associated with EpsteineBarr virus (EBV) and usually displays CD56, CD2, CD45RO, and CD43 positivity on immunohistochemistry. The case highlights the importance of including NK/T-cell lymphoma in the differential diagnosis for suspected infective midline facial lesions unresponsive to antibiotic therapy. References 1. Kin WS, et al. CHOP followed by involved field radiation: is it optimal for localized nasal natural killer T-cell lymphoma? Ann Oncol 2001;12:349-52. 2. Lee J, et al. Nasal-type NK/T-cell lymphoma: clinical features and treatment outcome. Br J Cancer 2005;92:1226-30.
Epidemiologic study of primary cutaneous lymphomas along 20 years at the Department of Dermatology of the University General Hospital of Valencia, Spain Elena Roche-Gamo´n, MD, University General Hospital of Valencia, Valencia, Spain; Maria Luisa Garcia-Melgares, MD, University General Hospital of Valencia, Valencia, Spain; Amparo Pe´rez-Ferriols, MD, PhD, University General Hospital of Valencia, Valencia, Spain; Vı´ctor Alegre de Miquel, MD, PhD, University General Hospital of Valencia, Valencia, Spain Introduction: The term primary cutaneous lymphoma (PCL) refers to cutaneous Tcell lymphomas (CTCL) and cutaneous B-cell lymphomas (CBCL) that present in the skin with no evidence of extracutaneous disease at the time of diagnosis. Materials and methods: We conducted a retrospective and prospective review of all patients with PCL presenting to the dermatology department at University General Hospital of Valencia, Spain, between May 1987 and May 2007. Results: A whole of 357 patients with PLC were diagnosed during the period of study. Three hundred and one (84.3%) of them belong CTCL and 56 (15.7%) CBCL. The most important anatomopathologic diagnoses were mycosis fungoides (MF) in 209 (58.5%), lymphomatoid papulosis (LP) in 60 (16.8%), others CTCL in 32 (9%), primary cutaneous marginal-zone B-cell lymphoma (PCMZL) in 29 (8.1%), folliclecenter lymphoma (FCL) in 18 (5%) and finally diffuse large B-cell lymphoma, leg type (DLBCL) in 9 (2.5%). In the group of CTCL 69.4% (209/301) were MF, 19.9% (60/301) PL, primary cutaneous CD41 small/medium peripheral T-cell lymphoma in 5.3% (16/301), unspecified T-cell lymphoma in 1.9% (6/301), primary cutaneous anaplastic large-cell lymphoma in 1.3% (4/301), extranodal NK/T-cell lymphoma, nasal-type, in 0.7% (2/301), primary cutaneous peripheral T-cell lymphoma, unspecified in 0.7% (2/301), and primary cutaneous angioinmunoblastic T-cell lymphoma in 0.7% (2/301). In the group of CBCL the percentages of each type were PCMZL in 51.8 % (29/56), FCL in 32.1 % (18/56), and DLBCL in 16.1% (9/56). Finally, we compare the epidemiologic characteristics (age and sexes) of CBCL and the CTCL. Commercial support: None identified.
Commercial support: None identified.
P2004 Three cases of nasal type NK/T-cell lymphoma Lucı´a Martı´nez Casimiro, Hospital General Universitario, Valencia, Spain; Violeta Zaragoza Ninet, Hospital General Universitario, Valencia, Spain; Maria Luisa Garcı´a Melgares Linares, Hospital General Universitario, Valencia, Spain; Vı´ctor Alegre de Miquel, Hospital General Universitario, Valencia, Spain Nasal type NK-T-cell lymphoma, previously known as lethal midline granuloma, is a rare type of noneHodkin’s lymphoma associated with the EpsteineBarr virus (EBV). Here we show three cases with nasal-type NK-T-cell lymphoma seen in our hospital. Case 1: A 57-year-old female presented in September 1989 with several-month history of nasal congestion and purulent rhinorrhea. A biopsy of an intranasal mass was diagnosed as idiopathic lethal midline granuloma and treated with radiotherapy. In April 1994, she experienced erythema and edema that involved the nasal bridge, malar and right periorbital area. A skin nasal biopsy revealed necrosis and reactive inflammation. CT scan showed midline destruction. She was treated with radiotherapy, chemotherapy, and surgical debridement. In August 1996, multiple red plaques appeared on her right leg. Skin biopsy showed diffuse infiltration of atypical lymphoid cells positive for CD3 and CD56 within the entire dermis and subcutaneous tissue. She died in a month. Case 2: A 42-year-old female, HIV positive, had in October 2006 a 2-month history of nasal obstruction and swelling and erythema of the nose. Nasal mucosa PAAF biopsy showed necrosis and reactive cells. She developed crusted plaques on her nose. A skin biopsy of the border showed a dense infiltrate of pleomorfic lymphocytes positive for CD3, CD56, and EBV within the entire dermis and necrosis. TC scan showed a mass in cavum and multiple lymph nodes. She was treated with CHOP chemotherapy but died within 3 months. Case 3: A 66-year-old female presented in February 2007 with a 6-month history of red plaques and nodules on her legs and left breast. Skin biopsies showed a diffuse infiltration of atypical lymphoid cells positive for CD3, CD56, and EBV throughout the dermis and subcutaneous tissue. TC scan showed a mass in the right nasal cavity. She is being treated with CHOP chemotherapy. Nasal NK-T cell lymphomas arise in the nasal cavity and paranasal sinuses. Patients present with nasal obstruction or extensive midfacial destruction. Other presentations include skin nodules. The prognosis is very poor independent on the treatment. Histology reveals a diffuse proliferation of atypical lymphocytes positive for CD2, CD56 and cytoplasmic CD3 with angiocentricity involving the dermis and often the subcutaneous tissue, and necrosis. This lymphoma is very rare in HIV and has worse prognosis. Commercial support: None identified.
FEBRUARY 2008
P2006 Cutaneous plasmablastic lymphoma in an HIV-positive female Violeta Zaragoza Ninet, MD, Consorcio Hospital General Universitario De Valencia, Valencia, Spain; Lucı´a Martı´nez Casimiro, MD, Consorcio Hospital General Universitario, Valencia, Spain; Vı´ctor Alegre De Miguel, Consorcio Hospital General Universitario, Valencia, Spain; Juan Jose´ Vilata Corell, Consorcio Hospital General Universitario, Valencia, Spain Introduction: Plasmablastic lymphoma (PBL) is an aggressive, uncommon and recently described variant of diffuse large B-cell lymphoma (DLBCL). It typically occurs in the setting of HIV infection, presents in the oral cavity and 60% of cases are said to be EBV-related. Case report: A 43-year-old female presented with painful, fast growing, solid and livid nodules on her trunk and both legs. The patient had a known B3 HIV infection under antiretroviral therapy. The biopsy consisted of a normal epidermis overlying a dermis that was largely effaced by a diffuse infiltrate of monomorphic, partly immunoblastic, partly plasmacytoid cells, with a high proliferation index. The immunohistochemical staining was negative for CD20, CD3, TDT, bcl-6, and HHV-8, but positive for the plasma cell marker CD138. In situ hybridization for EBV-encoded RNA (EBER) was positive. A diagnosis of PBL was made. The patient was staged IV-A with subsequent examinations. Chemotherapy, including the CHOP regimen and intrathecal therapy, was started in combination with her antiretroviral treatment. The patient underwent just 1 round of CHOP and died because of chemotherapy complications. Conclusion: Since its first description, there are only few additional reports describing this relatively new variant of DLBCL. This case illustrates morphologic and immunohistochemical criteria for PBL, its clinical features, and poor prognosis. Furthemore, our case shows an inusual presentation arising at a cutaneous site. Commercial support: None identified.
J AM ACAD DERMATOL
AB101