1/340. Multinucleate-cell angiohistiocytoma occurring in a patient with mycosis fungoides.Multinucleate-cell angiohistiocytoma (MCAH) is a benign vascular proliferation of unknown etiology. Clinically, MCAH presents as grouped, erythematous, or violaceous papules on the extremities in older women. These lesions often resemble Kaposi's sarcoma. Histologic examination reveals characteristic bizarre-shaped, multinucleate giant cells, some of which contain three to six nuclei arranged in a ring-like or overlapping pattern, which stain positively for factor xiiia. In addition, there is a proliferation of dermal capillaries and venules with a mild lymphohistiocytic infiltrate. To our knowledge, this is the first reported case of MCAH occurring in a patient with mycosis fungoides.- - - - - - - - - - ranking = 1keywords = et (Clic here for more details about this article) |
2/340. Allergic contact dermatitis from transdermal clonidine in a patient with mycosis fungoides.Although therapy with transdermal clonidine is considered an effective method of hypertension control, this mode of delivery has been associated with localized dermal reactions in numerous patients. We present a patient with coexistent mycosis fungoides and allergic contact dermatitis from transdermal clonidine. The association of these two dermatologic processes has not been previously reported.- - - - - - - - - - ranking = 1keywords = et (Clic here for more details about this article) |
3/340. Granulomatous mycosis fungoides: report of a case with some histopathologic features of granulomatous slack skin.We describe a case of granulomatous mycosis fungoides, tumor stage, mimicking sarcoidosis in an 82-year-old man with a 2-year history of skin disease. The final diagnosis was established after one of seven biopsy specimens showed a nongranulomatous histologic picture of patch-stage mycosis fungoides. Monoclonality was proven for the lymphocytic population by T-cell-receptor rearrangement studies. The unusually extensive granulomatous inflammation with huge giant cells surrounded by CD1a-positive cells in the other six biopsy specimens was suggestive of the histopathology of granulomatous slack skin, another rare granulomatous cutaneous T-cell lymphoma. Because both a clinical and histologic overlap between granulomatous mycosis fungoides and granulomatous slack skin have been reported in the literature, we conclude that they may belong to the spectrum of a single disease.- - - - - - - - - - ranking = 1keywords = et (Clic here for more details about this article) |
4/340. Systemic sarcoidosis and cutaneous lymphoma: is the association fortuitous?The association of systemic sarcoidosis and malignant lymphoma is known as the 'sarcoidosis-lymphoma syndrome'. Cutaneous involvement is rare in this syndrome. We report a 52-year-old woman who was diagnosed as having tumour-stage mycosis fungoides. Complete remission was achieved by combination therapy consisting of isotretinoin, interferon (IFN) alpha, electron beam irradiation, photochemotherapy and topical corticosteroids. Three years later, the patient developed systemic sarcoidosis characterized by yellowish papules on the abdominal wall and the eyelids that histologically revealed non-caseating granulomas, multiple fine-nodular interstitial pulmonary infiltrates on chest X-ray, hilar lymphadenopathy, decreased vital capacity and increased lymphocyte count in bronchoalveloar lavage fluid. As opposed to most of the reported cases, in our patient the manifestation of cutaneous lymphoma preceded the diagnosis of systemic sarcoidosis. We review the cases reported in the literature and discuss a possible causal and temporal relationship as well as the role of IFN alpha in the development of sarcoidosis.- - - - - - - - - - ranking = 2keywords = et (Clic here for more details about this article) |
5/340. Ophthalmic abnormalities in patients with cutaneous T-cell lymphoma.PURPOSE: To determine the frequency of ophthalmic abnormalities in patients with cutaneous T-cell lymphoma (mycosis fungoides and sezary syndrome) and T-cell lymphoma involving the skin and to describe the clinical course of the disease with selected examples. methods: A computerized diagnostic retrieval system was used to identify all patients with T-cell lymphoma involving the skin who were examined at the Mayo Clinic (Rochester, minnesota) between January 1, 1976 and December 31, 1990. The medical records of affected patients were reviewed. RESULTS: During the 15-year interval from 1976 through 1990, cutaneous T-cell lymphoma was diagnosed in 2,155 patients. Of these 2,155 patients, 42 (1.95%; 26 male and 16 female) had at least 1 ophthalmic abnormality attributable to the disease. The diagnoses in these 42 patients were mycosis fungoides in 19, clinical variants of T-cell lymphoma of the skin (most commonly, peripheral T-cell lymphoma) in 11, and sezary syndrome in 12. Cicatricial eyelid ectropion was the most common finding, affecting 17 (40.4%) of the 42 patients. Thirty-seven patients had findings that, although probably not a direct consequence of cutaneous T-cell lymphoma, have been cataloged in previous studies. CONCLUSION: Although ophthalmic abnormalities in patients with cutaneous T-cell lymphoma are relatively uncommon, the manifestations of the disease are diverse and frequently difficult to treat.- - - - - - - - - - ranking = 3keywords = et (Clic here for more details about this article) |
6/340. Follicular mycosis fungoides.We describe a patient with follicular mycosis fungoides (MF), a rare folliculotropic variant of cutaneous T-cell lymphoma (CTCL). Follicular involvement in CTCL usually presents clinically as alopecia mucinosa associated histologically with follicular mucinosis. Follicular MF differs from alopecia mucinosa/follicular mucinosis associated with MF with regard to its clinical presentation, histology and, presumably, prognosis. Our patient presented with the characteristic findings of follicular MF, i.e. infiltrated plaques showing numerous enlarged, comedo-like follicular infundibula; histology was dominated by exclusive folliculotropism of atypical lymphocytes sometimes forming follicular Pautrier's microabscesses, and by lack of epidermotropism and follicular mucinosis. Despite photochemotherapy and treatment with oral retinoids and interferon alpha, the patient's follicular MF rapidly developed into a progressive CTCL with large tumorous lesions, but responded to electron beam therapy. The course of our patient's disease confirms the notion that follicular MF may be associated with a worse prognosis than classical MF. However, electron beam irradiation induced remission of follicular MF that was maintained by a combination therapy consisting of extracorporeal photopheresis and interferon alfa.- - - - - - - - - - ranking = 2keywords = et (Clic here for more details about this article) |
7/340. mycosis fungoides in patients under 20 years of age: report of 7 cases, review of the literature and study of the clinical course.BACKGROUND: mycosis fungoides (MF) is rare in young patients. Its clinical behavior is still uncertain, as some reports have suggested that it has a more aggressive course than does the adult-onset type. AIM: To ascertain if early-onset MF represents a heterogeneous group of cutaneous T cell lymphomas. MATERIALS AND methods: Clinical, immunohistopathological and follow-up data of early-onset (<20 years of age) MF cases reported in the literature (n = 42) plus 7 described herein were compared with those of a cohort of adult-onset MF patients (n = 252) diagnosed at our institution since 1975. RESULTS: The majority of the 49 early-onset MF patients had patch-plaque stage disease at diagnosis. Ten had hypopigmented lesions. The predominant phenotype was CD3 CD4 CD7-CD8-. Seven patients had a stage progression, 6 with extracutaneous involvement. Five- and 10-year survival rates were 93 and 74%, respectively. CONCLUSIONS: No statistically significant differences were found in the disease course between early- and adult-onset MF.- - - - - - - - - - ranking = 8keywords = et (Clic here for more details about this article) |
8/340. A novel Epstein-Barr virus-like virus, HV(MNE), in a macaca nemestrina with mycosis fungoides.Epstein-Barr virus (EBV) infection of humans has been associated with the development of lymphoid malignancies mainly of B-cell lineage, although occasionally T-cell lymphomas have been reported. We describe here the characterization of a novel EBV-like virus (HV(MNE)) isolated from a simian T-cell lymphotropic virus type I/II (STLV-I/II) seronegative pigtailed macaque (macaca nemestrina) with a cutaneous T-cell lymphoma. immunohistochemistry studies on the skin lesions demonstrated that the infiltrating cells were of the CD3( )/CD8( ) phenotype. Two primary transformed CD8( ) T-cell lines were obtained from cultures of peripheral blood mononuclear cells (PBMC) and skin, and, with time, both cell lines became interleukin-2-independent and acquired the constitutive activation of STAT proteins. polymerase chain reaction analysis of the dna from the cell lines and tissues from the lymphomatous animal demonstrated the presence of a 536-bp dna fragment that was 90% identical to EBV polymerase gene sequences, whereas the same dna was consistently negative for STLV-I/II sequences. Electron microscopy performed on both cell lines, after sodium butyrate treatment, showed the presence of a herpes-like virus that was designated HV(MNE) according to the existing nomenclature. in situ hybridization studies using EBV Epstein-Barr viral-encoded rna probes showed viral RNA expression in both CD8( ) T-cell lines as well as in the infiltrating CD8( ) T cells of skin-tissue biopsies. Phylogenetic analysis of a 465-bp fragment from the polymerase gene of HV(MNE) placed this virus within the lymphocryptovirus genus and demonstrated that HV(MNE) is a distinct virus, clearly related to human EBV and other EBV-like herpesviruses found in nonhuman primates.- - - - - - - - - - ranking = 1keywords = et (Clic here for more details about this article) |
9/340. Meningeal involvement by a transformed mycosis fungoides following Hodgkin's disease.A 58-year-old man had long-standing lesions of presumed large plaque parapsoriasis. Following treatment for nodal Hodgkin's disease (HD), these became more infiltrated, with a diagnosis of mycosis fungoides (MF). A few months later, nodules appeared on the right leg, which was lymphoedematous after inguinal irradiation for HD. Histopathological examination showed CD3 , CD30-, CD15- large pleomorphic lymphocytes, leading to the diagnosis of transformed MF. The cutaneous lesions were successfully treated with topical nitrogen mustard and interferon alfa-2b then methotrexate, but his general health worsened with depression and malaise, without specific neurological symptoms or extracutaneous spreading of the lymphoma. Cerebral computed tomographic scan revealed a cerebellar subdural collection, arachnoid cyst and quadriventricular hydrocephaly, initially considered to be non-specific. After a few weeks, clinical symptoms of intracranial hypertension appeared, and a cerebrospinal fluid (CSF) examination revealed meningeal involvement by the lymphoma. These cells were CD3-negative and the diagnosis was confirmed by polymerase chain reaction (PCR) study, which revealed an identical clonal rearrangement of the T-cell receptor gamma gene between cutaneous biopsies and the CSF. Repeated intrathecal injections of methotrexate and cranial irradiation were performed and the patient was still alive after 13 months. This case illustrates the possible meningeal involvement of MF that may be preceded by atypical and mild neurological or psychiatric symptoms, which may be dissociated from the evolution of the cutaneous lesions. Moreover, PCR study may be useful for both diagnosis and monitoring.- - - - - - - - - - ranking = 3keywords = et (Clic here for more details about this article) |
10/340. Regional lymphomatoid papulosis: a report of four cases.lymphomatoid papulosis (LyP) is a chronic self-healing cutaneous eruption which is clinically benign but histologically malignant. Lesions occur episodically over the trunk and limbs. We describe four patients with regional LyP. All were male, with a range in age at onset from 12 to 47 years. In all cases, lesions were confined to a segmental unilateral area. Two patients had type A and two type B LyP. We have long-term follow-up on one patient whose lesions were limited to the right buttock for more than 20 years before more widespread lesions developed. Another patient with lesions on the left flank had mycosis fungoides limited to the same region. Only one other case of LyP presenting in a regional distribution has previously been described.- - - - - - - - - - ranking = 1keywords = et (Clic here for more details about this article) |
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