1/14. Granulomatous slack skin: a distinct disorder or a variant of mycosis fungoides?About 75% of cutaneous lymphomas belong to the group of T-cell lymphomas. mycosis fungoides is the most common entity in this group. Granulomatous slack skin is a rare form of cutaneous T-cell lymphoma closely related to mycosis fungoides. We present here a patient with areas of lax skin for several years who developed a generalized erythroderma with associated immunoactivation and a deterioration in his general condition. This report discusses clinically and histologically the differential diagnoses, namely granulomatous slack skin and granulomatous mycosis fungoides, and suggests that these 2 disorders are only variants in the broad spectrum of a single disease.- - - - - - - - - - ranking = 1keywords = erythroderma (Clic here for more details about this article) |
2/14. Transient CD30 nodal transformation of cutaneous T-cell lymphoma associated with cyclosporine treatment.BACKGROUND: mycosis fungoides (MF) may evolve from pre-existing chronic atopic or psoriasiform dermatitis and the histology can be equivocal. Early patch and plaque lesions of MF may evolve into tumors, disseminate to lymph nodes, bone marrow, and internal organs, and/or undergo transformation to a large cell size. methods: A patient with a history of "atopic dermatitis" followed by "psoriasis" rapidly developed exfoliative erythroderma and axillary lymphadenopathy following treatment with cyclosporine. At presentation, biopsy specimens of skin lesions and lymph nodes and staging were obtained. We present the treatment and follow-up of this patient and review the medical literature for similar cases. RESULTS: Multiple skin biopsy specimens from lesions revealed changes consistent with low-grade, cutaneous, T-cell lymphoma (MF) without evidence of large cell transformation and psoriasiform epidermal hyperplasia. CD30 large cell transformation was present in the lymph node. Adenopathy and erythroderma resolved without systemic therapy following discontinuation of cyclosporine and treatment with psoralen/ultraviolet A (PUVA), isotretinoin, interferon-alpha, and antimicrobials. CONCLUSIONS: This case documents a close relationship between atopy, psoriasis, and the development of cutaneous T-cell lymphoma, and illustrates that an immunosuppressive agent, cyclosporine, can dramatically alter the course of the disease.- - - - - - - - - - ranking = 2keywords = erythroderma (Clic here for more details about this article) |
3/14. Bilateral breast involvement in sezary syndrome.Cutaneous T-cell lymphoma is a term used for mycosis fungoides and sezary syndrome, the distinct clinical entities where the skin is the primary organ of involvement. sezary syndrome is the leukemic variant of mycosis fungoides, presenting with generalized erythroderma, lymphadenopathy, and atypical cells (the Sezary cells) in the peripheral blood and bone marrow. The dissemination of cutaneous T-cell lymphoma may occur with no exception of the organs; however, no prior report exists about the sezary syndrome secondarily involving the breasts. We report the clinical and radiological findings of bilateral breast involvement in a case of sezary syndrome.- - - - - - - - - - ranking = 1keywords = erythroderma (Clic here for more details about this article) |
4/14. Composite mycosis fungoides and B-cell chronic lymphocytic leukemia.Concordant or composite mycosis fungoides and B-cell chronic lymphocytic leukemia (B-CLL) is exceedingly rare, with only 10 cases previously described to our knowledge. We report a case of a 64-year-old woman who developed generalized erythroderma 5 years after the diagnosis of early stage B-CLL. Over the next 6 years of her clinical course multiple sequential samples of skin, peripheral blood, and one enlarged lymph node were studied in detail by flow cytometry, immunohistochemistry, molecular diagnostics, and electron microscopy. The progressive cutaneous infiltrates were initially characterized as leukemia cutis, infiltration by B-CLL. Three years later, when she developed worsening skin disease and lymphadenopathy, the cutaneous infiltrates were characterized as cutaneous T-cell lymphoma. At that point, a biopsy of an enlarged lymph node revealed a composite lymphoma of both B-CLL and cutaneous T-cell lymphoma, and the peripheral blood also contained circulating cells of both neoplasms. Herein we summarize the literature on concordant cutaneous T-cell lymphoma and B-CLL, and the literature on concordant T- and B-cell neoplasms in general, with a review of the postulated relationships between these neoplasms.- - - - - - - - - - ranking = 1keywords = erythroderma (Clic here for more details about this article) |
5/14. mycosis fungoides presenting as Ofuji's papuloerythroderma.We report three patients presented with clinical features of Ofuji's papuloerythroderma (pruritic erythematous papules and extensive erythema sparing all skin folds), however, showing histopathological findings of mycosis fungoides (Pautrier's microabscess, haloed lymphocytes, disproportionate epidermotropism, and wiry collagen bundles). One case was associated with plaque stage of mycosis fungoides and follicular mucinosis. T-cell receptor (TCR) gene rearrangement analysis in the lesional skin tissue demonstrated rearrangement of the gamma chain in all cases. HTLV-1 serology was negative for two patients who conducted HTLV-1 test.We think that Ofuji's papuloerythroderma might be a variant of early mycosis fungoides rather than secondary skin manifestations to certain cutaneous inflammatory diseases.- - - - - - - - - - ranking = 6keywords = erythroderma (Clic here for more details about this article) |
6/14. Unilesional mycosis fungoides mimicking bowen's disease.Cutaneous T-cell lymphoma includes a heterogeneous group of lymphomas that share the unique feature of T lymphocytes which are tropic to the skin. Clinically, the presentation of this disease is protean and can range from a single lesion to generalized erythroderma. Unilesional mycosis fungoides, a rare variant, is presented here. Its morphology mimicked squamous cell carcinoma in situ, and was refractory to conventional therapies. A review of the treatment of this subset is also presented.- - - - - - - - - - ranking = 1keywords = erythroderma (Clic here for more details about this article) |
7/14. Full clinical recovery after topical acyclovir treatment of Epstein-Barr virus associated cutaneous B-cell lymphoma in patient with mycosis fungoides.Primary cutaneous T- and B-cell lymphomas are a heterogeneous group of diseases with varied clinical presentations and prognosis. The use of new molecular, histological, and clinical criteria has improved their recognition. Cutaneous B-cell and T-cell lymphomas are seldom found together in the same patient. Here we report a rare case of mycosis fungoides variant of a cutaneous T-cell lymphoma (CTCL) which later developed Epstein-Barr virus (EBV) associated cutaneous B-cell lymphoproliferative disorder. The patient initially presented with generalized erythroderma, extensive plaques, and axillary lymphadenopathy. Histopathology and immunophenotyping of her tumor from the right breast nodule revealed a T-cell lymphoma consistent with mycosis fungoides. She was initially treated with pentostatin, followed by topical mechlorethamine and topical steroids. After progression of her mycosis fungoides with worsening diffuse skin lesions on this regimen, her treatments were changed to oral bexarotene with an initial partial response followed by stable disease. Three years from her initial presentation, she developed ulcerated cauliflower-like nodules on her forehead. biopsy of these lesions revealed EBV-positive large- and medium-sized pleomorphic B-cells consistent with EBV-driven B-cell lymphoproliferative disorder. She was treated with topical acyclovir cream on the involved skin areas while continuing with oral bexarotene for mycosis fungoides. Skin lesions gradually diminished and totally disappeared after four weeks of topical acyclovir treatment. Bexarotene treatment was continued for another year until the mycosis fungoides progressed and became wide spread causing her death four and a half years after the initial diagnosis. The coexistence of two cutaneous non-Hodgkin lymphomas of different lineage in the same patient and the complete clinical response of EBV-related B-cell cutaneous component to topical acyclovir makes this rare case particularly interesting.- - - - - - - - - - ranking = 1keywords = erythroderma (Clic here for more details about this article) |
8/14. carbamazepine-induced eruption histologically mimicking mycosis fungoides.carbamazepine is an important drug used in the management of seizures, trigeminal neuralgia, and chronic pain syndromes. It has been associated with a variety of adverse skin reactions including urticaria, lichenoid eruptions, erythroderma, erythema multiforme, stevens-johnson syndrome, and toxic epidermal necrolysis. A 39-year-old white male had been started on carbamazepine for intractable pain which resulted from a right foot crush injury. Approximately 3 months after the start of therapy, the patient had developed a generalized skin eruption following an entire day of sun exposure. Skin biopsies revealed an atypical lymphoid infiltrate in the dermis with collections of the atypical lymphocytes within spongiotic vesicles in the epidermis, suggestive of mycosis fungoides. The patient was treated with systemic prednisone. Subsequent biopsies failed to reveal atypical lymphocytes. Previous reports have described spongiotic eruptions with foci of atypical lymphocytes in contact dermatitis and in patients treated with phenytoin. To the best of our knowledge, this is the first reported case of a carbamazepine-induced eruption simulating mycosis fungoides histologically.- - - - - - - - - - ranking = 1keywords = erythroderma (Clic here for more details about this article) |
9/14. Coexistence of pemphigus foliaceus and mycosis fungoides.mycosis fungoides was documented in a patient two years after pemphigus foliaceus had been diagnosed and treated with corticosteroids. Eight years later, the patient was found to have stage IV lymphomatous disease with generalized erythroderma and palpable, histologically positive lymph nodes. hematoxylin-eosin staining of a specimen of erythroderma revealed mycosis fungoides, while direct immunofluorescence of this tissue revealed intercellular IgG deposits diagnostic of pemphigus foliaceus.- - - - - - - - - - ranking = 2keywords = erythroderma (Clic here for more details about this article) |
10/14. sezary syndrome presenting as a follicular dermatosis.A patient with erythroderma initially characterized by follicular papular dermatosis was diagnosed clinically and histologically as having probable pityriasis rubra pilaris. In addition the patient had an elevated WBC with absolute lymphocytosis. Ninety percent of the lymphocytes had the features of Sezary cells by light and electron microscopy. He was diagnosed as having Sezary's syndrome despite the noncharacteristic findings in the skin biopsy. This case illustrates that Sezary's syndrome can present initially as a follicular dermatosis.- - - - - - - - - - ranking = 1keywords = erythroderma (Clic here for more details about this article) |
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