P5927Beware of the mottled red leg

Sivanie Vivehanantha, Dermatology Department, University Hospital Coventry,Coventry, United Kingdom; Anand Lokare, Haematology Department, UniversityHospital Coventry, Coventry, United Kingdom; Andrew Ilchyshyn, DermatologyDepartment, University Hospital Coventry, Coventry, United Kingdom; DavidSnead, Histopathology Department, University Hospital Coventry, Coventry,United Kingdom; Imtiaz Ahmed, Dermatology Department, University HospitalCoventry, Coventry, United Kingdom; Ure Eke, Dermatology Department,University Hospital Coventry, Coventry, United Kingdom

Primary cutaneous diffuse large B-cell lymphoma, leg type (DLBCLLT) is typically amore aggressive form of primary cutaneous B-cell lymphoma which occurs almostexclusively in elderly women as solitary or clustered red or red-brown nodules onthe distal aspect of one leg. We report an intriguing case presenting as a mottled, redleg, initially mis-diagnosed as cellulitis. An 82-year-old woman was admitted tohospital by her general practitioner for treatment of right leg cellulitis which wasunresponsive to oral antibiotics in the community. She had also been treatedunsuccessfully with furosemide to reduce the associated leg oedema. She had abackground history of chronic obstructive pulmonary disease, bronchiectasis andprevious breast cancer. The admitting medical team commenced intravenousantibiotics to treat the presumed right lower leg cellulitis. A dermatology reviewwas requested 6 days later as the erythematous appearance extended to her thighs.A detailed history revealed that her right leg became red, hot and swollen 6 weeksbefore hospitalization and 3 weeks later induration was noted. There was no historyof fever or chills to support a diagnosis of cellulitis. On examination, there werediscrete erythematous, mottled, indurated plaques, with a striking livedoid patternof infiltration between her right ankle and upper thigh. Islands of normal skin werenoted between the plaques, her right leg was larger in diameter than the left leg andthe patient was noticeably cachectic. There was a purplish discoloration of her leftankle and a livedoid appearance on the medial aspect of her left leg. A skin biopsywas performed from the right thigh and a doppler ultrasound excluded a deep veinthrombosis. The skin biopsy demonstrated diffuse infiltration by high grademalignant CD20 and Bcl-2 positive lymphoid tumor with Ki-67 nearly 100%,confirming an aggressive non-Hodgkin lymphoma. Following a discussion at thelymphoma multidisciplinary team meeting, a diagnosis of presumed DLBCLLT wasmade based on the clinical and histologic findings. A palliative approach wasadopted given her comorbidities, frail condition, and the aggressive nature of thedisease. She was started on a reducing dose of prednisolone and managed in thecommunity. She died a month after initial presentation to hospital. To ourknowledge, such an unusual presentation of DLBCLLT has never been previouslyreported.

AB144

cial support: None identified.

Commer

P6972Cutaneous NK/T-cell lymphoma presenting as targetoid lesions

Silke Heinisch, University of Pittsburgh Department of Dermatology, Pittsburgh,PA, United States; Larisa J. Geskin, MD, University of Pittsburgh Department ofDermatology, Pittsburgh, PA, United States

A 63-year-old white woman with a 2-year history of extranodal NK/T-cell lymphoma,nasal type treated in the past with cisplatin/radiation, who was currently receivingsteroid (dexamethasone), methotrexate, ifosfamide, L-asparaginase, and etoposide(SMILE) regimen with good clinical response, presented to the hospital for hersecond SMILE therapy administration. On admission, her skin examination revealedmultiple, asymptomatic targetoid lesions which were present for 2 weeks.Dermatology inpatient consultation was obtained. Physical examination revealedan ill-appearing white female with generalized, nontender violaceous targetoidplaques with surrounding erythema, some with central bulla and ulceration.Differential diagnosis included bullous erythema multiforme, fixed drug reaction,erythema multiformeelike drug reaction, vasculitis, deep fungal infection, parane-oplastic blistering diseases and cutaneous lymphoma. Skin biopsy was obtained, andit revealed a dense atypical lymphoid dermal infiltrate with emperipolesis, necrosisand erythrocyte extravasation. Atypical lymphocytes stained with CD2, CD3, CD4,CD56, TIA-1, and Granzyme B. Ki-67 stained 60% of the infiltrate, and EBV wasidentified by in situ hybridization. A diagnosis of secondary cutaneous involvementof extranodal NK/T-cell lymphoma, nasal type was made. The patient experiencedrapid demise despite SMILE therapy and subsequent romidepsin therapy, and shedied within 3 months of development of her skin lesions. In patients with cytotoxiclymphomas, secondary cutaneous involvement may show unusual features, such asthe targetoid lesions present in our patient. Biopsy needs to be obtained to rule outcutaneousmetastasis of the underlying lymphoma. Extranodal NK/T-cell lymphoma,nasal type development of cutaneous lesions is a poor prognostic marker and isassociated with systemic dissemination of the disease and resistance to therapy.

cial support: None identified.

Commer

J AM ACAD DERMATOL

P6298Cutaneous T-cell lymphoma in patients with chronic lymphocyticleukemia

Michael Chang, MD, Mayo Clinic, Rochester, MN, United States; Amy Weaver, MS,Mayo Clinic, Rochester, MN, United States; Jerry Brewer, MD, Mayo Clinic,Rochester, MN, United States

Background: An association between cutaneous T-cell lymphoma (CTCL) andchronic lymphocytic leukemia (CLL) exists.

Objective: To further understand the course of CTCL in patients with CLL.

Methods: A search was conducted of the master diagnosis index at our institution toidentify patients with both CLL and CTCL from 1980 to 2010. A retrospective chartreview was then conducted.

Results: Of the 14 patientswith CTCL and CLL, 8 hadmycosis fungoides (MF) (2withpatch stage, 2 with plaque stage, 2 with tumor stage, and 2 with erythrodermic stageMF), 4 had S�ezary syndrome, 1 had natural killer cell lymphoma involving the skin,and 1 had peripheral T-cell lymphoma involving the skin. Eight had concurrentdiagnoses, 5 received a CLL diagnosis first, and 1 received a CTCL diagnosis first. Tenpatients were deceased at the time of data abstraction due to unknown causes (n ¼4), lymphoma (n¼ 2), pneumonia (n¼ 2), MF (n¼ 1), or respiratory failure (n¼ 1).Of the 9 patients with concurrent or prior CTCL, 7 were deceased with a mediantime to death of 10.2 months (range, 6 to 89 months). Of the 5 patients with CLLbefore CTCL, 3 were deceased at 18, 27, and 47 months following their CTCLdiagnosis. The median survival for the two groups was 12 and 47 months,respectively.

Limitations: Retrospective small case series.

Conclusion: Patients with CTCL concurrent or before CLL have a worse overallsurvival than patients with CLL who then develop CTCL later. Larger studies areneeded.

cial support: None identified.

Commer

P6965Diffuse large B-cell lymphoma leg type secondary to testicular diffuselarge cell-B lymphoma

Ana Pamp�ın Franco, Hospital Universitario Fundaci�on Alcorc�on, Alcorc�on, Spain;Enrique G�omez de la Fuente, Hospital Universitario Fundaci�on Alcorc�on,Alcorc�on, Spain; Fernando Javier Pinedo Moraleda, Hospital UniversitarioFundaci�on Alcorc�on, Alcorc�on, Spain; Jos�e Luis L�opez Estebaranz, HospitalUniversitario Fundaci�on Alcorc�on, Alcorc�on, Spain; Jos�e Luis Rodr�ıguez Peralto,Hospital Universitario Doce de Octubre, Madrid, Spain; Pablo Ortiz Romero,Hospital Universitario Doce de Octubre, Madrid, Spain; Rosa Adelaida FeltesOchoa, Hospital Universitario Fundaci�on Alcorc�on, Alcorc�on, Spain

Diffuse large B-cell lymphoma (DLBCL) is the most common testicular lymphoma inadults. It usually occurs in men[60 years of age, and it is an aggressive tumor withintermediate prognosis. It can disseminate to diverse extranodal sites, and rarely tothe skin. We present the case of a patient with secondary cutaneous involvement oftesticular DLBCL, presenting identical features than primary cutaneous DLBCL legtype (PCDLBCL LT). An 80 year-old man presented with one month history ofmultiple infiltrated violaceous nodules on his left leg. The patient was diagnosed ashaving a testicular DLBCL. He was treated with orchidectomy, 4 chemotherapycourses with rituximab, cyclophosphamide, doxorubicin and prednisone (R-CHOP), central nervous system (CNS) prophylaxis intratecal chemotherapy andconsolidation local radiotherapy with complete clinical response. Two years aftertreatment cutaneous lesions appeared. A biopsy was performed and demonstrated adiffuse dermal infiltration of atypical large lymphocitic cells with predominance ofimmunoblasts and centroblasts. Immunohistochemical markers showed diffuse andstrong positivity for CD20, CD45, Bcl-2 and MUM, and negativity for Bcl-6, CD3 andCD10. Those findings were identical to those showed in the previous testicularlymphoma. Molecular analysis revealed monoclonal rearrangement of the immuno-globulin heavy chain (Jh) gene by the same clone in testis and skin. Therefore, thediagnosis of secondary cutaneous involvement of DLBCL LT was established. Todate, the patient is receiving a new chemotherapy cycle with rituximab andbendamustine. PCDLBCL LT is characterized by nodular lesions generally on thelegs. Histologic and immunophenotypical features consist of infiltration by large Bcells, predominantly of immunoblasts and centroblasts with expression of B-cellmarkers (CD19, CD20, and CD22) and Bcl-2, MUM1 and FOXP1. The most commonsites of secondary dissemination are lymph nodes and CNS. There are few cases inthe literature of testicular DLBCL with secondary cutaneous involvement. To ourknowledge, our case is the unique in which monoclonal rearrangement of theimmunoglobulin heavy chain (Jh) gene by the same clone was demonstrated bymolecular analysis. In conclusion, we present the case of DLBCL LT secondary totesticular DLBCL. We want to emphasize the importance of clinical examination ofboth skin and testis, because of the possibility to infiltrate both locations.

cial support: None identified.

Commer

APRIL 2013