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Tuesday, December 30, 2008

Primary cutaneous anaplastic large cell lymphoma of the face presenting as posttraumatic maxillary sinusitis.

Primary cutaneous anaplastic large cell lymphoma of the face presenting as posttraumatic maxillary sinusitis.
J Craniofac Surg. 2008 Nov

Chen RF, Chen CT, Liao HT, Chen CH, Chen YR.
Division of Trauma Plastic Surgery, Department of Plastic and Reconstruction Surgery, Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Chang Gung Institute of Technology, Taoyuan, Taiwan.


Primary cutaneous anaplastic large cell lymphoma (ALCL) is an uncommon disease. It has various presentations that may mislead the diagnosis and cause delay of treatment. We report a 12-year-old boy who experienced persistent swollen and painful right cheek after blunt facial injury. The patient was first treated for maxillary sinusitis according to clinical history, but the pathology turned out to be primary cutaneous ALCL. Symptoms improved after chemotherapy with complete remission. No recurrence was noted during the follow-up period. The presentations of primary cutaneous ALCL of the face may mimic posttraumatic maxillary sinusitis. Any patient with facial prolonged symptoms or nonhealing wound should be excluded from this entity.

Lippincott, Williams & Wilkins

Folliculotropic mycosis fungoides: an aggressive variant of cutaneous T-cell lymphoma.

Folliculotropic mycosis fungoides: an aggressive variant of cutaneous T-cell lymphoma.
Arch Dermatol. 2008 Jun

Gerami P, Rosen S, Kuzel T, Boone SL, Guitart J.
Department of Dermatology, Northwestern University Feinberg School of Medicine, and The Robert H. Lurie Comprehensive Cancer Center, Northwestern University, Chicago, Illinois, USA.


OBJECTIVES: To study the clinical features, therapeutic responses, and outcomes in patients with folliculotropic mycosis fungoides (FMF) and to compare our single-center experience of 43 patients with the findings from the Dutch Cutaneous Lymphoma Group.

SETTING: A single-center experience from the Northwestern University Multidisciplinary Cutaneous Lymphoma Group.

PATIENTS: Forty-three patients with FMF were included in the study and compared with 43 age- and stage-matched patients with classic epidermotropic mycosis fungoides (MF) with similar follow-up time.

RESULTS: Folliculotropic mycosis fungoides showed distinct clinical features, with 37 patients having facial involvement (86%) and only 6 having lesions limited to the torso (14%). The morphologic spectrum of lesions is broad and includes erythematous papules and plaques with follicular prominence with or without alopecia; comedonal, acneiform, and cystic lesions; alopecic patches with or without scarring; and nodular and prurigolike lesions. Sixty-five percent of patients had alopecia, which in 71% of cases involved the face. Severe pruritus was seen in 68% of patients. In general, patients responded poorly to skin-directed therapy and in almost all cases required systemic agents to induce even a partial remission, including patients with early-stage disease. Overall survival was poor. Patients with early-stage disease (< or ="IIA)"> or =IIB) had an outcome similar to those patients in the control group with conventional epidermotropic MF of a similar stage.

CONCLUSIONS: The morphologic spectrum of clinical presentation for FMF is broad and distinct from those in conventional MF. This is at least partially attributed to the ability of FMF to simulate a variety of inflammatory conditions afflicting the follicular unit. The disease course is aggressive, and many patients, including those with early disease, show a poor outcome particularly between 10 and 15 years after the initial onset of disease. Response to skin-directed therapy is poor even in early-stage disease, and our best results were seen with psoralen plus UV-A (PUVA) therapy with oral bexarotene or PUVA with interferon alfa. These findings corroborate those of the Dutch Cutaneous Lymphoma Group and further validate the classification of FMF as a distinct entity.

PubMed

A case of syringotropic cutaneous T-cell lymphoma treated with local radiotherapy.

A case of syringotropic cutaneous T-cell lymphoma treated with local radiotherapy.
J Am Acad Dermatol. 2009 Jan

Jacob R, Scala M, Fung MA.
Department of Radiation Oncology, University of California-Davis, Sacramento, California, USA.


Syringotropic cutaneous T-cell lymphoma (SCTCL) is a variant of mycosis fungoides that is characterized by the presence of lymphocytic infiltration of eccrine glandular structures and the absence of the classic features of MF, such as epidermotropism and Pautrier's microabscesses. We report a patient with SCTCL who presented with multiple hypopigmented patches and localized alopecia who was treated with local radiotherapy with excellent local control. In our experience, local electron radiation offers local control in early stage SCTCL, and it may be possible to reserve systemic therapy for more advanced stages of the disease.

PMID: 19103368 [PubMed - in process]

Primary cutaneous T-cell-rich B-cell lymphoma: a case report and literature review.

Primary cutaneous T-cell-rich B-cell lymphoma: a case report and literature review.
Acta Dermatovenerol Alp Panonica Adriat. 2008 Dec

Venizelos ID, Tatsiou ZA, Mandala E.
Pathology Department, Hippokration Hospital, 5 Exadactylou Street, 54635 Thessaloniki, Greece.
ivenizel@otenet.gr.


T-cell-rich B-cell lymphoma (TCRBCL) is a recently recognized B-cell lymphoma variant, characterized by a minor population of neoplastic B-cells existing in a background of predominant reactive T-lymphocytes. It is a rare entity, accounting for approximately 1 to 2% of all non-Hodgkin's lymphomas. It has both nodal and extranodal presentation. Primary cutaneous TCRBCL is an extremely rare lymphoma and only 16 cases have been documented thus far in the medical literature. We report the case of a 46- year-old man that presented with a slowly-growing, painless skin nodule on the left temporofrontal region of the scalp. A complete surgical excision was performed and histological examination revealed diffuse infiltration of the dermis by TCRBCL. A complete surgical excision of the skin lesion and systemic chemotherapy seems to have been effective because the patient is disease-free 2 years after the initial diagnosis was made. This study reports a very rare case of TCRBCL presented primarily in the skin. Because of its rarity, it is especially important to make the correct diagnosis using the appropriate immunohistochemical stains and apply the proper therapy.

PMID: 19104743 [PubMed - in process]