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PubMed2002

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.

Aged↗

PubMedDate not supplied

Mycosis fungoides presenting as pigmented purpuric eruption.

A case of pigmented purpuric eruptions evolving to mycosis fungoides during the 4-year follow-up period is described. Clinical manifestation was characterized by petechial lesions with irregular shaped, diffusely pigmented plaques partly sharing morphological similarities with chronic pigmented purpura. Histologically, lymphocytes infiltrated around the capillaries of the superficial dermis with extravasated erythrocytes as well as into the epidermis to form Pautrier microabscesses. Whereas CD4+ cells were observed in the epidermis and upper dermis, CD8+ cells tended to be distributed around the capillaries. Notably, the Rumpel-Leede test revealed extensive punctuate purpura limited to the lesional skin. The aggregation response of platelets was not impaired. Either CD4+ tumor lymphocytes or CD8+ reactive lymphocytes appeared to induce capillary damage resulting in the formation of petechial lesions. Pigmented purpuric eruptions, such as atypical chronic pigmented purpura, is thus an important initial clinical manifestation of mycosis fungoides.

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PubMed1998

Mycosis fungoides palmaris et plantaris or acral pagetoid reticulosis?

There has been ongoing debate about the nature of Woringer-Kolopp disease (unilesional pagetoid reticulosis). Despite the histologic resemblance to mycosis fungoides, these lesions are typically solitary and indolent. Recently, cutaneous plaques of epidermotropic lymphocytes restricted to acral sites resembling Woringer-Kolopp disease were reported to show T-cell clonality, leading to the designation mycosis fungoides palmaris et plantaris. We describe a similar case of recurrent plaques on palms and soles of a 45-year-old man that persisted for >14 years without other cutaneous or systemic disease. Histologically, the lesions were comprised of epidermotropic atypical lymphocytes with sparse dermal infiltrates. Immunohistochemically, the majority of intraepidermal lymphocytes labeled as CD8-positive suppressor/cytotoxic T cells and expressed alphaE beta7 (CD103), an integrin associated with epitheliotropism. Polymerase chain reaction studies revealed similar clonal gene rearrangements of T-cell receptors beta and gamma in tissue from both palm and sole. In view of these findings, the diagnosis of mycosis fungoides palmaris et plantaris may be appropriate. To date, however, the lesions have remained localized and continue to resolve spontaneously. As such, the behavior is similar to what has been described as pagetoid reticulosis. Long-term follow-up will be necessary to determine the biologic potential of this disease.

Antigens, CD↗

PubMed1992

Xanthomatized atypical T cells in a patient with mycosis fungoides and hyperlipidemia.

BACKGROUND: Lipoprotein-T-cell interactions are being reported with increasing frequency, and there is evidence that lipoproteins play a role in immunoregulation. We describe a patient with mycosis fungoides and hyperlipidemia who developed xanthomatization in one preexisting plaque. The case is unique in that some of the lipidized cells were atypical T cells. In previously reported cases of mycosis fungoides with dystrophic xanthomatosis, the lipid-containing cells have been identified only as histiocytes. OBSERVATIONS: Immunopathologic features, electron microscopy, and lipid stains of the xanthomatized plaque demonstrated that some of the lipid-laden cells were atypical T cells. CONCLUSIONS: In mycosis fungoides, malignant T cells may be intimately involved in processing of tissue lipids. We suggest that low-density lipoprotein receptors on activated T cells facilitated the cytoplasmic lipidization in this case.

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PubMed2006

[Imiquimod in treatment of palpebral mycosis fungoides].

CLINICAL CASE: Our patient was a 37-year-old male with palpebral mycosis fungoides, the most common T-cell cutaneous lymphoma. He had been treated previously with topical corticosteroids. We treated him with Imiquimod cream (5%) three times a week. DISCUSSION: A clinical improvement was obtained with this drug, so we believe immuno-modulating agents need to be considered in the treatment of palpebral mycosis fungoides, especially because of its capacity of being a reflection of leukaemia.

Adult↗

PubMed1994

Cutaneous necrosis associated with the antiphospholipid syndrome and mycosis fungoides.

The development of extensive cutaneous necrosis in a patient with tumour-stage mycosis fungoides is described. Skin biopsies showed a lymphomatous infiltrate, and thrombosis of dermal blood vessels. Investigation revealed the presence of anticardiolipin antibodies, a lupus anticoagulant, and low free protein S, which contributed to a prothrombotic state. Antiphospholipid antibodies have been detected in non-Hodgkin's lymphoma, but clinical manifestations are uncommon. Such autoantibodies may be produced by neoplastic lymphoid cells. The frequency with which antiphospholipid antibodies occur in mycosis fungoides is currently unknown.

Antiphospholipid Syndrome↗

PubMed1985

Topical nitrogen mustard in mycosis fungoides.

A twelve-year experience in thirty-three patients suffering from early mycosis fungoides in the plaque stage confirmed the effectiveness of topical nitrogen mustard (chlormethine) therapy. Fourteen patients were in complete remission at the latest time of observation and seven in partial remission. The probability of freedom from relapse was approximately 50% after 6 and 12 years. Three deaths attributable to mycosis fungoides were recorded. Another three patients had to discontinue treatment due to contact dermatitis to nitrogen mustard. Specific precautions were undertaken in order to protect personnel handling the drug. No damaging or toxic effects were observed among staff and no haematological side-effects were observed among the patients. The treatment as a whole was well tolerated.

Administration, Topical↗

PubMed1999