P2704

Generalized subcutaneous panniculitis-like T-cell lymphoma following rituximab for hemolytic anemia in a patient with chronic lymphocytic leukemia Matthew Hall, MD, Mayo Clinic, Jacksonville, FL, United States; Jason Sluzevich, MD, Mayo Clinic, Jacksonville, FL, United States; John Snow, MD, Mayo Clinic, Jacksonville, FL, United States A 76-year-old man with a decade long history of chronic lymphocytic leukemia, recently complicated by Coombs-positive hemolytic anemia, was treated with four monthly infusions of rituximab (375mg/m2) in combination with cyclophosphamide (750mg/m2). The anemia resolved, but 2 months later the patient developed rapidly progressive, generalized, asymptomatic, red-brown indurated cutaneous nodules and plaques. Histopathology revealed infiltration of the subcutaneous fat with atypical lymphocytes that rimmed adipocytes, as well as zonal lobular fat necrosis. Immunohistochemical studies were positive for CD3, CD8, granzyme B, and beta F1, and negative for CD4, CD30, and CD56. In situ hybridization studies for Epstein-Barr virus were negative. T cell gene rearrangement studies by polymerase chain reaction revealed monoclonality. A diagnosis of subcutaneous panniculitis-like T-cell lymphoma (SPTCL) was rendered. The development of B-cell lymphoproliferative disorders in the setting of iatrogenic immunosuppression is well recognized. This case represents the unique development of a clonal cutaneous T-cell lymphoma in the setting of an immunosuppressive milieu characterized by systemic B cell depletion. To our knowledge, this is the first reported case of SPTCL associated with previous treatment with rituximab. Commercial support: None identified.

P2706
Topical carmustine in the treatment of mycosis fungoides: Efcacy and side effects nchez-Mateos, PhD, Hospital Universitario de La Princesa, Diana Santiago-et-Sa frica Jua rez, PhD, Hospital Department of Dermatology, Madrid, Spain; A Universitario de La Princesa, Department of Dermatology, Madrid, Spain; a-Diez, MD, Hospital Universitario de La Princesa, Department of Amaro Garc s, PhD, Hospital Universitario Dermatology, Madrid, Spain; Maximiliano Aragu e de La Princesa, Madrid, Spain; Rebeca Goiriz, PhD, Hospital Universitario de La Princesa, Department of Dermatology, Madrid, Spain Introduction: Carmustine (BCNU) is a nitrosourea alkilating agent topically used for mycosis fungoides (MF) for more than 3 decades. According to the EORTC consensus recommendations, it is included in the skin-directed therapy as a rstline recommendation for stages IA, IB, IIA, and III. Methods: Retrospective study of the patients diagnosed clinically and histologically with MF who have ever received topical BCNU at the Derpartment of Dermatology in Hospital Universitario de La Princesa from January of 1982 to February of 2009. It was used in alcoholic solution (2mg/mL) diluted in water once daily on involved areas, or undiluted if \3% of the body surface area was involved. Definitions of response are as follows: complete response (CR), disappearance of all clinical evidence of disease; partial response (PR), 50% or more improvement, both for at least 4 weeks; no response (NR), less than 50% of improvement. We have analyzed response, time of treatment to get CR, time of freedom from relapse, evolution and side effects. Results: This study includes 19 patients ranging in age from 40 to 83 years. The time from diagnosis to start of treatment ranged from \1 to 17 years. Fourteen patients were in initial stages (3 in IA, 10 in IB, and 1 in IIA) and ve were in advanced stages of the disease (1 in IIB, 1 in III, and 3 in IVA). The patients in IVA stage were receiving other systemic treatments. They were followed up for 7 to 142 months. Eighteen patients responded (94.7%): 13 patients achieved CR (72.2%) and ve patients got PR (27.7%). Only in one case this treatment was withdrawn for the acute cutaneous side effects. The CR was reached after a period of treatment from 2 to 10 months. Two patients have maintained the CR. The time of freedom from relapse ranged from 3 to 130 months. Although the majority of patients have suffered from relapses of the disease, they most have maintained a stable stage. Four patients have experienced progression of the disease. We have observed frequent cutaneous side effects, such as erythema after application and persistent poikilodermatous changes with striking telangiectasias. Neither hematologic side effects nor skin cancer were observed. Conclusions: Topical BCNU is an effective treatment for MF in initial stages and even in advanced stages when used as a coadjuvant therapy. It is a remarkable safe modality of treatment because the side effects observed are usually cutaneous without secondary malignancy. Commercial support: None identified.

P2705
Sarcoid-like granulomatous mycosis fungoides presenting with markedly hyperpigmented plaques Steven Nelson, MD, Mayo Clinic, Scottsdale, AZ, United States; David DiCaudo, MD, Mayo Clinic, Scottsdale, AZ, United States; Jennifer Ray, MD, Mayo Clinic, Scottsdale, AZ, United States A 69-year-old white woman presented with a 10-year history of asymptomatic, enlarging, hyperpigmented plaques on the lower extremities, upper extremities, and trunk. Cutaneous sarcoidosis was diagnosed 2 years earlier based on skin biopsy ndings, and hydroxychloroquine was started shortly after receiving the diagnosis. Minocycline was used for a 2-month period during the treatment course. Physical examination revealed multiple, large, well marginated, black patches and indurated plaques bilaterally on the lower legs. On the buttocks, trunk, and arms were violaceous plaques with overlying laxity and wrinkling of the skin. No skin changes were present in the axillae or groin. Incisional biopsy showed dense dermal and subcutaneous inltrate composed of small lymphocytes admixed with numerous histiocytes and large multinucleate giant cells. The inltrate expressed CD2, CD3, CD4, and CD5, with diminished expression of CD7, and absent expression of CD30 and CD56. Throughout the inltrate, numerous intracellular and extracellular pigment granules stained positively with the Fontana and iron stains. Clonal T-cell receptor gene rearrangement was detected by PCR analysis. The patient has discontinued the hydroxychloroquine and is scheduled to be seen in hematology to discuss treatment options. Sarcoid-like granulomas are present in approximately 2% of primary cutaneous lymphomas; granulomatous mycosis fungoides (GMF) is the most common lymphoma to demonstrate this feature. Sarcoidosis has been reported to precede the diagnosis of GMF. Many of these cases have been shown actually to be early GMF masked by the sarcoid-like reactions. The black hyperpigmentation makes this patients presentation unique. The hydroxychloroquine is suspected to have contributed to the impressive amount of both melanin and iron deposition. Although several of the plaques have overlying wrinkled, lax skin, the term granulomatous slack skin (GSS) is reserved for similar changes in the axillae and groin. GSS and GMF are differentiated by clinical features because they cannot reliably be distinguished histopathologically. Recent studies suggest that the prognosis of GMF is worse than that of classic mycosis fungoides. Commercial support: None identified.

P2707
zary syndrome and lung cancer association: Case report Se ria de Dermatologia, Hospital de Santa nica Universita Ana Rita Travassos, Cl ria de s Soares de Almeida, Cl nica Universita Maria, Lisboa, Portugal; Lu , Cl nica Dermatologia, Hospital de Santa Maria, Lisboa, Portugal; Marisa Andre ria de Dermatologia, Hospital de Santa Maria, Lisboa, Portugal; Paulo Universita ria de Dermatologia, Hospital de Santa Maria, nica Universita Leal Filipe, Cl Lisboa, Portugal Background: Cutaneous T-cell lymphoma (CTCL) has been associated with increased risk for secondary malignancies, namely other lymphomas and lung cancer. zary syndrome (SS) is a leukemic variant of CTCL and is classically dened by the Se zary cells in the triad of erythroderma, lymphadenopathy, and the presence of Se skin, lymph nodes, and peripheral blood. Case report: A 66-year-old white man, a heavy smoker, with a dermatosis characterized by erythematous patches and plaques conned to the lower limbs for 7 years, which were devalued. The patient was subsequently diagnosed of squamous cell carcinoma of the left lung (4 years later), and underwent neoadjuvant chemotherapy and lobectomy followed by chemotherapy. During this lung-guided treatment period, there was also a signicant improvement of the skin lesions. Thereafter, there was a progressive worsening of the dermatosis and the patient came to our outpatient unit with erythroderma and generalized pruritus. Laboratory examinazary cells tions showed peripheral leukocytosis with the presence of circulating Se (4600 cells/L); an expanded CD41 population and T-cell receptor (TCR) gene rearrangement was detected on peripheral blood and bone marrow aspirate. Skin punch biopsies were compatible with mycosis fungoides/CTCL. Computed tomography scan of chest, abdomen and pelvis revealed small bilateral axillary and inguinal enlarged lymph nodes. On the basis of these clinical, histopathologic, and molecular biologic findings, the patient fulfilled the WHO-EORTC criteria for SS. Conclusion: This clinical case reports the association of SS and lung cancer of the squamous cell carcinoma type. Although we do not know why there is an increased risk of lung cancer in CTCL patients, an appropriate monitoring for the early detection of second cancers might be warranted in patients with CTCL. Commercial support: None identified.

AB96

J AM ACAD DERMATOL

MARCH 2010