Cases reported "Precancerous Conditions"

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1/5. A case of pre-sezary syndrome preceded by hand lesions.

    Pre-sezary syndrome is an erythroderma with a chronic course, clinical findings of sezary syndrome, lymphocytic subepidermal band infiltration at times, and repeated cycles of circulating Sezary cells of less than 1,000 cells/mm3. Duration of the pre-existing skin diseases preceding pre-Sezary erythroderma varies from a few weeks to 20 years. Before the erythroderma develops, these patients are diagnosed with contact dermatitis, neurodermatitis, chronic dermatitis, atopic dermatitis, or asteatotic eczema. hand lesion also precedes the pre-Sezary erythroderma. This condition has been controlled by three cycles of chemotherapy consisting of vincristine, cytoxan, doxorubicin, and prednisolone. We describe a case of pre-sezary syndrome preceded by hand lesion and treated with chemotherapy.
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2/5. A paraneoplastic mixed bullous skin disease: breakdown in tolerance to multiple epidermal antigens.

    We report a new type of paraneoplastic mixed bullous skin disease in a patient with a B-cell lymphoma associated with monoclonal IgM kappa paraproteinaemia. The patient's clinical and histological features were reminiscent of bullous pemphigoid. Characterization of antiepidermal antibodies by immunoblotting and indirect immunoelectron microscopy demonstrated a novel pattern of reactivity: IgG antibodies were directed against desmoplakins I-II and BPAG2, and were associated with antidesmoglein 3 polyclonal IgM antibodies. Such an autoreactive pattern involving antidesmoplakins, anti-BPAG2 and antidesmoglein 3 antibodies has not been previously reported. It reflects the breakdown of tolerance to multiple epidermal antigens observed in some patients with malignancy.
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keywords = skin disease
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3/5. Hereditary non-polyposis colorectal cancer associated with disseminated superficial porokeratosis. microsatellite instability in skin tumours.

    A 73-year-old man presented with typical lesions of disseminated superficial porokeratosis (DSP) and multiple seborrhoeic keratoses on his face, trunk and extremities, and later developed a keratoacanthoma on his lip. He belonged to a cancer-prone pedigree susceptible to colonic, uterine and other internal cancers, and had a personal history of early gastric cancer and advanced adenocarcinoma of the descending colon without adenomatous polyps at age 59 years. polymerase chain reaction amplification of skin samples for seven separate microsatellite polymorphisms revealed microsatellite instability (MSI) at multiple loci in five of six seborrhoeic keratoses and the keratoacanthoma, strongly suggesting underlying defects in dna mismatch repair. Although no germline mutations in two mismatch repair genes hMSH2 and hMLH1 were found, our patient was recognized as having hereditary non-polyposis colorectal cancer (HNPCC) based on the family history and the findings of the microsatellite analysis of skin tumours. This confirmed the usefulness of detection of MSI in prevalent and readily accessible skin lesions, including non-sebaceous non-dysplastic tumours such as seborrhoeic keratosis in the screening of HNPCC families. Although DSP may also be inherited as an autosomal dominant condition, this particular skin disease appeared to be sporadic in our patient and, to our knowledge, no association of DSP or other forms of porokeratosis with HNPCC has previously been reported. In contrast to the seborrhoeic keratoses and keratoacanthoma, no MSI was observed in two samples from DSP lesional epidermis examined.
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4/5. Atypical manifestations of pityriasis lichenoides chronica: development into paraneoplasia and non-Hodgkin lymphomas of the skin.

    Three patients with atypical courses and manifestations of pityriasis lichenoides chronica (PLC) are presented. The first patient is a 21-year-old white woman who showed a good response of her PLC lesions as well as her reactive oligoarthritis to repeated PUVA treatments combined with oral prednisone during 1 year. The effect of the treatment then decreased. The patient developed a low-grade malignant lymphoma of the lung. When the lymphoma of the lung improved after chemotherapy, the PLC eruptions improved, too. The second patient is a 41-year-old man, whose Hodgkin's disease stage IVa was successfully treated by chemotherapy and radiotherapy in 1984. In 1987 he showed PLC lesions which responded well to puva therapy, later also in combination with etretinate. Until 1988 repeated skin biopsies revealed a non-specific eczematous pattern. In 1989 the recalcitrant PLC eruptions finally revealed a pleomorphic non-Hodgkin lymphoma of the skin with medium-sized cells. The third patient had a PLC for about 9 years when Hodgkin's disease stage Ia was diagnosed. At the beginning the skin biopsy showed an eczematous pattern, but 2 years later, in 1990, skin infiltrations of a large-cell, anaplastic non-Hodgkin lymphoma were seen. These cases show that PLC in rare cases may either represent a paraneoplastic skin disease or may itself develop into cutaneous lymphomas.
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5/5. Follicular mucinosis associated with scarring alopecia, oligoclonal T-cell receptor V beta expansion, and staphylococcus aureus: when does follicular mucinosis become mycosis fungoides?

    A diagnosis of alopecia mucinosa, occurring as a single scalp lesion, was made in a 40-year-old white woman who had a history of trauma. Follicular mucinosis, staphylococcus aureus, and oligoclonal expansion of the T-cell receptor V beta chain genes 6 and 7 were present in the skin. Epidermotropic T-cell skin diseases with oligoclonal T-cell proliferations may be the result of HLA- and cytokine-determined reaction patterns to persistent antigens.
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