LYMPHOMA, CUTANEOUS/MYCOSIS FUNGOIDES

P5984CD8-positive primary cutaneous T-cell lymphoma with peripheral andcentral nervous system involvement

Rosa Ballester S�anchez, MD, Hospital General Universitario de Valencia, Valencia,Spain; Amparo P�erez Ferriols, MD, PhD, Hospital General Universitario deValencia, Valencia, Spain; Blanca de Unamuno Bustos, MD, Hospital GeneralUniversitario de Valencia, Valencia, Spain; V�ıctor Alegre de Miquel, MD, PhD,Hospital General Universitario de Valencia, Valencia, Spain

Background: CD8-positive T-cell lymphomas involving the skin are relatively rareand have recently been of significant interest. Some cases clinically present mycosisfungoides, while others have an aggressive clinical course with frequent extracuta-neous dissemination. Four distinct subtypes of CD8+ cutaneous T-cell lymphomas(CTCLs) have been identified to date, but there are several cases that remainunspecified. However, to our knowledge, there are no published reports of patientswith a CD8-CTCL with peripheral and central nervous involvement. We report anexample of such a case.

Observations: We describe a patient with a CD8+ cutaneous T-cell lymphoma withan initially indolent course who developed peripheral and central nervous systeminvolvement. The lymphoma exhibited rapid and aggressive behavior and noresponse to aggressive treatment.

Conclusions: Several cutaneous T-cell lymphomas can express the CD8 phenotype,but correlations between clinical and pathologic data are necessary to reach adiagnosis. The WHO/EORTC classification for primary cutaneous lymphomasfacilitates more uniformity in diagnosis and provides a useful distinction betweenindolent and more aggressive types of primary cutaneous lymphoma. In cases withextracutaneous involvement, it is important to use aggressive treatment methods.

APRIL 20

cial support: None identified.

Commer

P6312A case of primary cutaneous anaplastic large cell lymphoma presentingwith numerous patches, papules, and nodules in a child

Young Lip Park, MD, PhD, Soonchunhyang University Hospital, Bucheon,Bucheon, Gyenggi-do, South Korea; Han Eul Lee, MD, SoonchunhyangUniversity Hospital, Cheonan, Cheonan, Chungcheongnam-do, South Korea; JiHoon Sim, MD, Soonchunhyang University Hospital, Bucheon, Bucheon,Gyenggi-do, South Korea; Kyu Uang Whang, MD, PhD, SoonchunhyangUniversity Hospital, Seoul, South Korea; Seung Il Choi, MD, SoonchunhyangUniversity Hospital, Seoul, South Korea; Sung Yul Lee, MD, PhD, SoonchunhyangUniversity Hospital, Cheonan, Cheonan, Chungcheongnam-do, South Korea; YouIn Bae, MD, Soonchunhyang University Hospital, Bucheon, Bucheon, Gyenggi-do, South Korea

Primary cutaneous anaplastic large cell lymphoma is a well-defined CD30-positivelymphoproliferative disorder with relatively good prognosis and response totreatment. PCALCL is characterized by a solitary or localized skin tumor and usuallyoccurs in adults and rarely in children and adolescents. Herein we report a 10-year-old boy with disseminated hyperpigmented patches, papules and nodules on histrunk, buttocks and upper and lower extremites. Histologic and immunohisto-chemical examination revealed CD30-positive large cell lymphoma. There was nohistory of concurrent lymphomatic papulosis, mucosis fungoides, or other lym-phoma. Also there was no evidence of systemic involvement. Therefore, wediagnosed our case as primary cutaneous anaplastic large cell lymphoma.

cial support: None identified.

Commer

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P6952A diagnostic challenge: Mycosis fungoides or psoriasis

Jorga Fialova, MD, Department of Dermatovenereology 2nd Medical SchoolCharles University and Bulovka Hospital, Praha, Czech Republic; Jana Hercogova,MD, Department of Dermatovenereology 2nd Medical School Charles Universityand Bulovka Hospital, Praha, Czech Republic; Nadezda Vojackova, MD,Department of Dermatovenereology 2nd Medical School Charles Universityand Bulovka Hospital, Praha, Czech Republic

Mycosis fungoides (MF) is the most common cutaneous lymphoma arising in mid- tolate adulthood with an incidence rate of 0.41/100,000 person years. Path, plaqueand tumor stage are distinguished. The diagnosis is based on a combination ofclinical manifestations, histopathology, and T cell monoclonality. In the early stages,the diagnosis of MF is a problem. Repeated and multiple biopsies may not confirmMF for months to years. Psoriasis is taken to account in differential diagnosis, mainlyin plaques stage. Therapy is stage-related. In the stage, in which the histopathologicdiagnosis is not confirmed, PUVA photochemotherapy is the most effectivetreatment. Next treatment options are oral isotretinoin, bexarotene, subcutaneousinterferon-alfa, and extracorporeal PUVA. The prognosis of MF depends on the age atpresentations and stage of the disease. General pustular psoriasis (GPP), a rare formof psoriasis first described in 1910 by Leo Ritter von Zumbusch, is characterized byan abrupt onset of widespread erythematous lesions followed by superficialyellowish, usually confluent sterile pustules, accompanied by constitutional symp-toms such as fatigue, fever, generalizedweakness or leukocytosis. Further diagnosticcriteria include the presence of spongioform pustules histiopathologically. Relapsesand remission occur, flares are induced by triggers factors similar those in plaquepsoriasis, such infections, emotional stress, vaccinations, sun exposure. Life-threatening complications such a bacterial superinfection, sepsis, metabolical orhemodynamic failure are seen predominantly in adults. Special types, such asimpetigo herpetiformis, annular type, psoriasis cum pustulatione, and acroderma-titis continua of Hallopeau, have been described. All types of GPP may follow or befollowed by psoriasis. Treatment option of the systemic retinoids, corticosteroids,cyclosporine, methotrexate or biologics depends on the severity of disease, patient’sage, sex or underlying risk factor. Our case describes the 66-year-old woman with aclinical diagnosis of MF. The histopathologic exminations did not confirm thediagnosis despite repeated biopsies. GPP induced by PUVA photochemotherapy aswell as clinical lesions of MF was successfully treated with acitretin.

cial support: None identified.

Commer

P6697Acute scleritis and nonerythrodermic skin rash: An unusual presentationof S�ezary syndrome

M. Chattopadhyay, Addenbrooke’s Hospital, Cambridge University Hospitals NHSFoundation Trust, Cambridge, United Kingdom; E. Rytina, Addenbrooke’sHospital, Cambridge University Hospitals NHS Foundation Trust, Cambridge,United Kingdom; G. Follows, Addenbrooke’s Hospital, Cambridge UniversityHospitals NHS Foundation Trust, Cambridge, United Kingdom; G. Meligonis,Addenbrooke’s Hospital, Cambridge University Hospitals NHS Foundation Trust,Cambridge, United Kingdom; P. Meyer, Addenbrooke’s Hospital, CambridgeUniversity Hospitals NHS Foundation Trust, Cambridge, United Kingdom; P.Norris, Addenbrooke’s Hospital, Cambridge University Hospitals NHSFoundation Trust, Cambridge, United Kingdom

A 74-year-old man presented with an 8-day history of red sore eyes with cleardischarge, blurred vision and photophobia. A nonpruritic rash had developed fromhis waist downward 6 days previously. Skin examination revealed erythematous,slightly scaly papules on his buttocks and lower extremities. Bilateral inguinallymphadenopathy was also noted. Ophthalmic examination showed bilaterallyreduced visual acuities, white cells in the tear film, dilatation of conjunctival andepiscleral vessels, scleral thickening, anterior uveitis and immensely raised intraoc-ular pressure. Full blood count revealed an isolated lymphocytosis. A blood filmshowed these were cells with cerebriform nuclei and immunophenotypingdemonstrated CD4 positive cells with coexpression of CD2, 3 and 5 but no CD7expression and CD4:CD8 ratio much greater than 10. Bonemarrow aspirate showedsimilar lymphocyte phenotype as blood. Histology from a skin biopsy revealedperivascular and nodular infiltrate of large, atypical T cells, immunoreactive for CD2,3, 4 and 5 but negative for CD7. There was clonal TCRG and TCRB generearrangement with the same T cell clone populating the skin, blood and bonemarrow. CT scan showed small volume lymph nodes above and below thediaphragm. Based on these above laboratory and clinical findings, a diagnosis ofS�ezary syndrome was made, although not all features were typical for this disease.His eye symptoms improved dramatically and his skin rash resolved after the firstcycle of gemcitabine/CVP chemotherapy. He has now completed 6 cycles ofchemotherapy and is currently in remission. The initial presentation of this casewith bilateral hypertensive scleritis was secondary to lymphomatous infiltration ofthe episclera and conjunctiva. Ophthalmic manifestations of cutaneous T-celllymphomas are rare and generally occur in the advanced stages of the disease.Although our patient did not have a skin rash characteristic of erythrodermic S�ezarysyndrome at presentation, this was only 6 days from the onset of cutaneous features;his skinmay have progressed onto erythroderma if left untreated. This case is uniquebecause of the initial acute oculocutaneous presentation, to the best of ourknowledge not described in the literature so far.

cial support: None identified.

Commer

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