- Email: [email protected]
delta T-cell lymphoma mimicking lupus erythematosus profundus
P2109
P2111
Leukemia cutis and Sweet’s/pyoderma gangrenosum overlap in a 75-yearold man Olga Ulitsky, MD, University of Chicago, Chicago, IL, United States; Pedram Gerami, MD, Northwestern University, Chicago, IL, United States; Vesna PetronicRosic, MD, University of Chicago, Chicago, IL, United States
Pediatric mycosis fungoides with predominant CD8-positive T-cells Amanda Harper, MD, University of New Mexico, Albuquerque, NM, United States; Kenneth Harper, MD, MPH, University of New Mexico, Albuquerque, NM, United States; Charles Palmer, MD, University of New Mexico, Albuquerque, NM, United States; Barrett Zlotoff, MD, University of New Mexico, Albuquerque, NM, United States
A 75-year-old man with a history of myelodysplastic syndrome (09/03), which transformed into AML (09/04) per bone marrow biopsy, presented with rising white count and hemorrhagic skin lesions on his right arm and leg. The lesions were comprised of erythematous papules, plaques, and nodules, some of which were studded with pustules. Biopsy of a representative lesion showed papillary dermal edema and a mixed inflammatory infiltrate with numerous neutrophils and extravasated red blood cells in the papillary dermis. A folliculocentric neutrophilic abcess extended through the overlying epidermis. Also, large atypical lymphoid cells with folded nuclear membranes and prominent nucleoli consistent with leukemia cutis cells were seen. These cells stained with CD68 and CD14 monocytic protein markers. PAS, GMS, Gram, and Fite stains were all negative for micro-organisms. The diagnosis of leukemia cutis and Sweet’s syndrome/pyoderma gangrenosum overlap was made in a single lesion. The patient was started on prednisone and chemotherapy for AML (cytarabine, hydroxyurea). This resulted in improvement and eventual resolution of skin lesions. The differential diagnosis included histiocytoid Sweet’s syndrome, a recently described entity consisting of immature neutrophils with a histiocytoid appearance that can mimic leukemic blasts. However, based on our immunological monocytic markers, this possibility was ruled out. We want to emphasize the importance of doing immunohistochemistry to distinguish the lesions from histiocytoid Sweet’s syndrome because true leukemic involvement of the skin is an adverse prognostic factor for the patient.
We report a case of a 6-year-old boy with a 1-year history of multiple mildly pruritic oval, sharply demarcated, slightly scaly, erythematous patches on the sun-protected areas of the trunk, periaxillary areas, and upper legs. Skin biopsies showed a superficial perivascular bandlike infiltrate with epidermotropism. Intraepidermal lymphocytes displayed hyperchromatic, hyperconvoluted nuclei, consistent with mycoses fungoides (MF). Immunohistochemical study performed showed predominantly CD8-positive lymphocytes in the epidermis. T-cell gamma receptor (TCR) gene rearrangement studies did not reveal evidence of a monoclonal TCR gene rearrangement. The patient achieved a complete response with topical fluticasone propionate ointment, but has required maintenance therapy to prevent recurrence. MF is uncommon in childhood. Several recent series show that the cytotoxic (CD8positive) immunophenotype is over-represented in juvenile MF. Sixty to 65% of recently reported pediatric MF cases have shown a monoclonal TCR rearrangement (representing a dominant clone of T-cells); the remaining cases were diagnosed by characteristic histopathological features, even though TCR rearrangement studies were negative. Neither CD8-positive nor CD4-positive pediatric MF seems to have a worse prognosis than MF presenting in adulthood. Treatments for juvenile MF showing a 60% or better complete response include corticosteroids, topical nitrogen mustard, psoralen-UVA, and narrow-band UVB phototherapy. Most cases of MF presenting in childhood are limited to the early stage of IA or IB and are unlikely to progress to a more advanced stage.
Commercial support: None identified. Commercial support: None identified.
P2112
P2110 Cutaneous gamma/delta T-cell lymphoma mimicking lupus erythematosus profundus Paula Aguilera, MD, Hospital Clı´nic de Barcelona, Barcelona, Spain; Jose´ Manuel ´ , Jr, MD, PhD, Hospital Clı´nic de Barcelona, Barcelona, Spain; Teresa Mascaro Estrach, MD, PhD, Hospital Clı´nic de Barcelona, Barcelona, Spain In the new revised WHO-EORTC classification for cutaneous lymphomas, cutaneous gamma/delta T-cell lymphomas (CGD-TCL) have been included as a provisional entity. This type of lymphoma, when involving the subcutaneous fat, can mimick clinically and histologically other more indolent conditions such as subcutaneous panniculitic T-cell lymphomas (SPTCL) and lupus erythematosus profundus (LEP), and multiple biopsies may be needed to obtain a correct diagnosis. A good correlation of the clinical data with the histopathology and immunohistochemistry are required for diagnosis. Herein, we present a patient whose initial histopathologic findings resembled LEP, but presented an aggressive clinical course. A new biopsy was performed during the follow-up, and a final diagnosis of CGD-TCL was made. This case emphasizes the similarity of lymphomas involving the subcutaneous fat and LEP. The diagnosis should be based on the combination of clinicopathological, immunophenotypical, and molecular findings. Commercial support: None identified.
FEBRUARY 2007
Good response of mycosis fungoides treated with bexarotene Elena Roche, MD, Universitary General Hospital Valencia, Valencia, Spain; Marı´a Luisa Garcı´a-Melgares, MD, Universitary General Hospital Valencia, Valencia, Spain; Amparo Pe´rez-Ferriols, MD, Universitary General Hospital Valencia, Valencia, Spain; Juan Jose´ Vilata, MD, PhD, Universitary General Hospital Valencia, Valencia, Spain Introduction: Cutaneous T-cell lymphoma (CTCL) is a heterogeneous group of nonHodgkin’s lymphomas that manifest primarily in the skin. Mycosis fungoides and the leukaemic variant Sezary syndrome are the most common entities. No curative therapy exists and patients ultimately develop advanced or relapsed disease that is refractory to standard treatment options. To date, there is no curative treatment for this disease, and the objective is to control the symptoms and prevent the disease from progressing. Bexarotene, the first RXR-selective retinoid ‘‘rexinoid’’ approved for all stages of cutaneous T-cell lymphoma (CTCL) which are refractory to at least one prior systemic therapy, had a response rate (RR) of 45% at the optimal dose of 300 mg/m(2) per day in 2 multicenter trials. Patients and Methods: We carried out a descriptive study of 13 patients treated with bexarotene in our department. We analyzed the clinical characteristics of the patients and the efficacy of the treatment, and we collected data on the side effects that appeared. Results: There were 4 women and 9 men, aged between 28 and 79-years-old, with a half age of 59.53. Four out of 13 (38.5%) of the patients were in stage IB, 2/13 (15.4%) in IA, 3/13 (23%) were IIB, and 3/13 (23%) were IV. The overall response to the treatment was 53.9% (7/13). Four out of 13 (30.8%) patients had full remission, 3/13 (23.1%), had partial remission, 4/13 (30.8%) maintained stable and 2/13 (15.5%) progressed. Twenty-three-point-one percent of the patients were in monotherapy whereas 76.9% were in politherapy (topical or systemic). Tolerance to the treatment was good, and the most frequent side effects were hypertriglyceridemia (100%), hypercholesterolemia (92.3%), and central hypothyroidism (53.8%). Conclusions: The results that we obtained are similar to ones previously described. Bexarotene is an effective therapeutic option in this heterogeneous group of diseases. Commercial support: None identified.
J AM ACAD DERMATOL
AB141
P2111
Leukemia cutis and Sweet’s/pyoderma gangrenosum overlap in a 75-yearold man Olga Ulitsky, MD, University of Chicago, Chicago, IL, United States; Pedram Gerami, MD, Northwestern University, Chicago, IL, United States; Vesna PetronicRosic, MD, University of Chicago, Chicago, IL, United States
Pediatric mycosis fungoides with predominant CD8-positive T-cells Amanda Harper, MD, University of New Mexico, Albuquerque, NM, United States; Kenneth Harper, MD, MPH, University of New Mexico, Albuquerque, NM, United States; Charles Palmer, MD, University of New Mexico, Albuquerque, NM, United States; Barrett Zlotoff, MD, University of New Mexico, Albuquerque, NM, United States
A 75-year-old man with a history of myelodysplastic syndrome (09/03), which transformed into AML (09/04) per bone marrow biopsy, presented with rising white count and hemorrhagic skin lesions on his right arm and leg. The lesions were comprised of erythematous papules, plaques, and nodules, some of which were studded with pustules. Biopsy of a representative lesion showed papillary dermal edema and a mixed inflammatory infiltrate with numerous neutrophils and extravasated red blood cells in the papillary dermis. A folliculocentric neutrophilic abcess extended through the overlying epidermis. Also, large atypical lymphoid cells with folded nuclear membranes and prominent nucleoli consistent with leukemia cutis cells were seen. These cells stained with CD68 and CD14 monocytic protein markers. PAS, GMS, Gram, and Fite stains were all negative for micro-organisms. The diagnosis of leukemia cutis and Sweet’s syndrome/pyoderma gangrenosum overlap was made in a single lesion. The patient was started on prednisone and chemotherapy for AML (cytarabine, hydroxyurea). This resulted in improvement and eventual resolution of skin lesions. The differential diagnosis included histiocytoid Sweet’s syndrome, a recently described entity consisting of immature neutrophils with a histiocytoid appearance that can mimic leukemic blasts. However, based on our immunological monocytic markers, this possibility was ruled out. We want to emphasize the importance of doing immunohistochemistry to distinguish the lesions from histiocytoid Sweet’s syndrome because true leukemic involvement of the skin is an adverse prognostic factor for the patient.
We report a case of a 6-year-old boy with a 1-year history of multiple mildly pruritic oval, sharply demarcated, slightly scaly, erythematous patches on the sun-protected areas of the trunk, periaxillary areas, and upper legs. Skin biopsies showed a superficial perivascular bandlike infiltrate with epidermotropism. Intraepidermal lymphocytes displayed hyperchromatic, hyperconvoluted nuclei, consistent with mycoses fungoides (MF). Immunohistochemical study performed showed predominantly CD8-positive lymphocytes in the epidermis. T-cell gamma receptor (TCR) gene rearrangement studies did not reveal evidence of a monoclonal TCR gene rearrangement. The patient achieved a complete response with topical fluticasone propionate ointment, but has required maintenance therapy to prevent recurrence. MF is uncommon in childhood. Several recent series show that the cytotoxic (CD8positive) immunophenotype is over-represented in juvenile MF. Sixty to 65% of recently reported pediatric MF cases have shown a monoclonal TCR rearrangement (representing a dominant clone of T-cells); the remaining cases were diagnosed by characteristic histopathological features, even though TCR rearrangement studies were negative. Neither CD8-positive nor CD4-positive pediatric MF seems to have a worse prognosis than MF presenting in adulthood. Treatments for juvenile MF showing a 60% or better complete response include corticosteroids, topical nitrogen mustard, psoralen-UVA, and narrow-band UVB phototherapy. Most cases of MF presenting in childhood are limited to the early stage of IA or IB and are unlikely to progress to a more advanced stage.
Commercial support: None identified. Commercial support: None identified.
P2112
P2110 Cutaneous gamma/delta T-cell lymphoma mimicking lupus erythematosus profundus Paula Aguilera, MD, Hospital Clı´nic de Barcelona, Barcelona, Spain; Jose´ Manuel ´ , Jr, MD, PhD, Hospital Clı´nic de Barcelona, Barcelona, Spain; Teresa Mascaro Estrach, MD, PhD, Hospital Clı´nic de Barcelona, Barcelona, Spain In the new revised WHO-EORTC classification for cutaneous lymphomas, cutaneous gamma/delta T-cell lymphomas (CGD-TCL) have been included as a provisional entity. This type of lymphoma, when involving the subcutaneous fat, can mimick clinically and histologically other more indolent conditions such as subcutaneous panniculitic T-cell lymphomas (SPTCL) and lupus erythematosus profundus (LEP), and multiple biopsies may be needed to obtain a correct diagnosis. A good correlation of the clinical data with the histopathology and immunohistochemistry are required for diagnosis. Herein, we present a patient whose initial histopathologic findings resembled LEP, but presented an aggressive clinical course. A new biopsy was performed during the follow-up, and a final diagnosis of CGD-TCL was made. This case emphasizes the similarity of lymphomas involving the subcutaneous fat and LEP. The diagnosis should be based on the combination of clinicopathological, immunophenotypical, and molecular findings. Commercial support: None identified.
FEBRUARY 2007
Good response of mycosis fungoides treated with bexarotene Elena Roche, MD, Universitary General Hospital Valencia, Valencia, Spain; Marı´a Luisa Garcı´a-Melgares, MD, Universitary General Hospital Valencia, Valencia, Spain; Amparo Pe´rez-Ferriols, MD, Universitary General Hospital Valencia, Valencia, Spain; Juan Jose´ Vilata, MD, PhD, Universitary General Hospital Valencia, Valencia, Spain Introduction: Cutaneous T-cell lymphoma (CTCL) is a heterogeneous group of nonHodgkin’s lymphomas that manifest primarily in the skin. Mycosis fungoides and the leukaemic variant Sezary syndrome are the most common entities. No curative therapy exists and patients ultimately develop advanced or relapsed disease that is refractory to standard treatment options. To date, there is no curative treatment for this disease, and the objective is to control the symptoms and prevent the disease from progressing. Bexarotene, the first RXR-selective retinoid ‘‘rexinoid’’ approved for all stages of cutaneous T-cell lymphoma (CTCL) which are refractory to at least one prior systemic therapy, had a response rate (RR) of 45% at the optimal dose of 300 mg/m(2) per day in 2 multicenter trials. Patients and Methods: We carried out a descriptive study of 13 patients treated with bexarotene in our department. We analyzed the clinical characteristics of the patients and the efficacy of the treatment, and we collected data on the side effects that appeared. Results: There were 4 women and 9 men, aged between 28 and 79-years-old, with a half age of 59.53. Four out of 13 (38.5%) of the patients were in stage IB, 2/13 (15.4%) in IA, 3/13 (23%) were IIB, and 3/13 (23%) were IV. The overall response to the treatment was 53.9% (7/13). Four out of 13 (30.8%) patients had full remission, 3/13 (23.1%), had partial remission, 4/13 (30.8%) maintained stable and 2/13 (15.5%) progressed. Twenty-three-point-one percent of the patients were in monotherapy whereas 76.9% were in politherapy (topical or systemic). Tolerance to the treatment was good, and the most frequent side effects were hypertriglyceridemia (100%), hypercholesterolemia (92.3%), and central hypothyroidism (53.8%). Conclusions: The results that we obtained are similar to ones previously described. Bexarotene is an effective therapeutic option in this heterogeneous group of diseases. Commercial support: None identified.
J AM ACAD DERMATOL
AB141