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1/9. 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.
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keywords = skin disease
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2/9. A case of primary cutaneous CD30 T-cell lymphoproliferative disorder with features of granulomatous slack skin disease.

    We present a patient with primary CD30 cutaneous T-cell lymphoma whose histological and clinical features overlapped with those of granulomatous slack skin disease (GSSD). A 26-year-old woman had infiltrative erythema on the abdominal wall and an incurable ulcerative lesion on the left knee. Her skin progressively became atrophic and pendulous, showing a hyperpigmented appearance over almost the whole body. Histopathologically, a dense lymphoid cell infiltrate accompanying numerous macrophages and multinucleated giant cells (MGC) extended into the subcutaneous tissue. Most lymphoid cells were small and positive for T-cell markers. Some relatively large atypical cells were scattered in the lesion, most of which (60%) were positive for CD30. T-cell receptor-beta gene rearrangement was confirmed in the abdominal lesion. MGC infiltrated more dominantly into a deeeper layer of the skin with the elastic fibres there almost completely disappearing. Immunoreactivity for CD30 of MGC was negative and overexpression of elastolytic metalloproteinases was observed. The association between primary cutaneous CD30 lym- phoproliferative disorders and GSSD has not previously been reported. Overexpression of elastolytic metalloproteinases in MGC contributes to the disappearance of the elastic fibres and enhances the severity of the clinical course.
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keywords = skin disease
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3/9. Monitoring the decrease of circulating malignant T cells in cutaneous T-cell lymphoma during photopheresis and interferon therapy.

    BACKGROUND: The prognosis of patients with stage IV cutaneous T-cell lymphoma (CTCL) is grim and therapeutic options are limited. Treatment of advanced-stage CTCL is aimed at suppressing the dominant T-cell clone, which is typically present in the skin, peripheral blood, and lymph nodes. OBSERVATIONS: We detected the expansion of 1 T-cell clone expressing the T-cell receptor V beta 14 in the peripheral blood of a patient with stage IVA CTCL. Before initiation of combination therapy with photopheresis and low-dose interferon alpha, the dominant T-cell clone represented 84% of the total T-cell population. After successful therapy, this clone showed a dramatic decrease to 6% of the T-cell population after 6 months of treatment. This reduction in the percentage of the malignant T-cell population in response to therapy was paralleled by clinical skin improvement from initial generalized erythroderma to undetectable skin disease. CONCLUSIONS: This case demonstrates that response to combination treatment with photopheresis and low-dose interferon alpha in patients with advanced CTCL may be accurately and quantitatively followed up by monitoring the percentage of the malignant T-cell clone (when identifiable) within the total circulating T-cell population by flow cytometry.
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ranking = 1
keywords = skin disease
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4/9. Granulomatous slack skin disease--disease features and response to pentostatin.

    Granulomatous slack skin disease (GSSD) is a rare condition characterized clinically by redundant skin folds, which show a predilection towards flexural areas, and histologically by a granulomatous T-cell infiltrate and loss of elastic fibres. The disease is often indolent, although rapid progression and transformation have been described. There is much debate as to whether this condition is a subset of mycosis fungoides or a separate disease entity in itself. We describe a case of GSSD with unique manifestations including granulomatous bone marrow involvement and hypercalcaemia. The patient has twice achieved a good response to pentostatin after failure of combination chemotherapy. This is the first report of the successful use of the purine analogue pentostatin in the management of GSSD.
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ranking = 5
keywords = skin disease
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5/9. vitamin d-mediated hypercalcemia in slack skin disease: evidence for involvement of extrarenal 25-hydroxyvitamin D 1alpha-hydroxylase.

    A case of granulomatous slack skin disease is presented in which we studied the possible involvement of extrarenal 1,25(OH)2D in the pathogenesis of the patient's hypercalcemia. Immunolocalization of 1alpha-OH in skin showed simultaneous dysregulation in epithelial and granulomatous cells. INTRODUCTION: granuloma-forming diseases such as sarcoidosis are associated with extrarenal synthesis of active 1,25-dihydroxyvitamin D [1,25(OH)2D]. Here we describe a case of granulomatous slack skin disease in which we have studied the possible involvement of extrarenal synthesis of 1,25(OH)2D in the pathogenesis of the patient's hypercalcemia. The aim of the study was to clarify the etiology of hypercalcemia in this patient. MATERIALS AND methods: This was a case study of a 19-year-old man with a T-cell lymphoproliferative disorder diagnosed as granulomatous slack skin disease who presented with hypercalcemia and raised serum 1,25(OH)2D. Analysis of expression of the enzyme 25-hydroxyvitamin D 1alpha-hydroxylase (1alpha-hydroxylase), which catalyzes synthesis of 1,25(OH)2D, was carried out by immunohistochemical analysis of involved and uninvolved skin. Approval was granted by the Mayo Foundation Institutional review Board and Biospecimens Subcommittee. RESULTS: In uninvolved skin, expression of 1alpha-hydroxylase was confined to the basal layer of the epidermis, whereas slack skin showed overexpression of the enzyme in dermal granulomata and basal cells of the epidermis. CONCLUSIONS: hypercalcemia associated with granulomatous slack skin syndrome seems to be caused by dysregulation of 1alpha-hydroxylase expression in both epidermal and dermal granulomatous cells. This contrasts with psoriasis and sarcoidosis of the skin, in which overexpression of the enzyme is restricted to keratinocytes and granulomata, respectively.
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keywords = skin disease
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6/9. Cutaneous T-cell lymphoma arising from parakeratosis variegata: long-term observation with monitoring of T-cell receptor gene rearrangements.

    BACKGROUND: parakeratosis variegata is a rare skin disease first described in 1890. Even today, the disease entity remains confusing because various names indicating similar skin conditions have been used. Several cases of parakeratosis variegata have been reported to develop into cutaneous T-cell lymphoma, but there have been no reports describing the occurrence of lymphoma after a long-term follow-up period nor have T-cell receptor gene rearrangements been monitored in this disease. OBJECTIVE: Our purpose was to determine whether parakeratosis variegata (long-standing premycotic condition) can develop into cutaneous T-cell lymphoma. methods: We analyzed skin specimens from a patient with a 33-year history of parakeratosis variegata by Southern blotting using a T-cell receptor gene probe. RESULTS: We could detect apparent rearranged bands of T-cell receptor gene in the skin specimens taken in 1993 in contrast to the dna analysis in 1988 which featured no such rearranged band. CONCLUSION: This case represents a critical stage of parakeratosis variegata converting to lymphoma. Our results indicate that parakeratosis variegata generated monoclonality of T cells in its chronic course.
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keywords = skin disease
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7/9. Cutaneous T-cell lymphoma associated with granulomatous slack skin.

    Granulomatous slack skin disease (GSS) is a rare disorder characterized by bulky cutaneous lesions and epithelioid and giant cell granulomas with destruction of the dermal elastic tissue. We detail the observation of a 29-year-old man with clinical and histological features of GSS. Pendulous skin tumors were associated with typical clinical and immunohistochemical aspects of mycosis fungoides and with clonal rearrangement of the V gamma T-cell receptor gene in lesional skin. This case report supports cutaneous T-cell lymphoma as a cause of GSS.
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keywords = skin disease
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8/9. Granulomatous slack skin in childhood.

    Granulomatous slack skin is an uncommon cutaneous T-helper cell lymphoma closely related to mycosis fungoides. To the best of our knowledge this disease has not been previously described in children. We report on an 11-year-old boy who presented with painless slack skin masses in the neck, right axilla and arm, anterior wall of the abdomen, both inguinal regions, and the malleolar and dorsal aspects of the feet. The disease started 3 years earlier with erythematous lesions on the neck and wrists. Histologic examination of a specimen from the abdominal mass revealed an extensive lymphoid infiltrate with scattered multinucleated giant cells extending from the papillary dermis to the subcutis. The lymphoid cells showed the following immunophenotype: CD43 (MT1), CD45 , CD45RO , CD20-. The phenotype of the giant cells was lysozyme positive, CD68 and Mac387-. The tumoral lymphoid cells had clonal rearrangement for the gene of the beta chain of the T-cell receptor (C beta TCR). The disease could be controlled with systemic glucocorticoids. Due to the presence of many histiocytes arranged in aggregates in the papillary and mid-dermis, this case was initially considered to be a cutaneous form of histiocytosis. We recommend deep and extensive biopsies in patients with slack skin disease.
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keywords = skin disease
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9/9. Granulomatous slack skin. Report of three patients with an updated review of the literature.

    PURPOSE: Granulomatous slack skin (GSS) is a rare cutaneous disorder characterized clinically by the evolution of circumscribed erythematous lax skin masses, especially in the body folds, and histologically by a granulomatous T-cell infiltrate and loss of elastic fibers. GSS is often associated with preceding or subsequent lymphoproliferative malignancies, especially mycosis fungoides (MF) and Hodgkin's disease (HD). No effective treatment is known yet. Whether this entity is a benign disorder, a peculiar host reaction to a malignant lymphoma, a precursor of malignant lymphoma or an indolent cutaneous T-cell lymphoma (CTCL) in itself is still a matter of debate. patients AND methods: The results of the patients with GSS from the netherlands are compared with the cases reported in the world literature. RESULTS: A female patient had had GSS for 8 years without developing a secondary malignancy. In a second female patient with a histologically confirmed diagnosis of MF, GSS developed 18 years later in the axillary and inguinal folds which had previously been affected by plaque-stage MF lesions. A third male patient with a 6-year history of erythematosquamous skin disease diagnosed as CTCL developed GSS. Moreover, granuloma formation was also found in a facial basal cell carcinoma, in a cervical lymph node and the spleen. Clonal rearrangements of the T-cell receptor beta genes were found in the 2 female patients; the male patient could not be tested. CONCLUSION: GSS is a rare clinicopathological entity. Only 34 patients have been described so far. The development of GSS within plaque MF lesions has not been reported before. Our third case developed very extensive skin lesions and showed a strong propensity to develop granulomas as compared to cases reported before. The presence of a clonal T-cell population was demonstrated in all cases tested. Our cases support the idea that GSS is a very rare and rather indolent type of CTCL. Apparently, the disease is associated with a peculiar immune response, characterized by granuloma formation and disappearance of elastic fibers resulting in the lax skin. The relationship between GSS and other preexisting or subsequent lymphoproliferative diseases (diagnosed in approximately 50% of the cases) warrants a life-long follow-up.
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keywords = skin disease
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