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Granulomatous slack skin syndrome

S Uma Maheswari, V Sampath, A Ramesh
Granulomatous slack skin syndrome is a rare variant of cutaneous T-cell lymphoma (mycosis fungoides). It is characterized clinically by redundant skin folds, which show a predilection towards flexural areas such as the axilla and the groin. Histologically, it shows a granulomatous T-cell infiltrate and loss of elastic tissue. It has an indolent but progressive course; and is usually refractory to treatment. We report a unique case of slack skin syndrome, sparing the classical sites with rapid and unusual involvement of non-intertriginous areas...
2018: Indian Journal of Dermatology, Venereology and Leprology
Begoña Echeverria, Magalys Vitiello, Clara Milikowski, Francisco Kerdel
Granulomatous Slack Skin (GSS) is a very rare condition that has been described as a variant of Mycosis Fungoides. It is characterized by the development of bulky and pendulous skin folds in flexural areas that are histologically formed by atypical T lymphocytes, histiocytes and giant cells. We report the case of a 37-year-old African American female with history of Sézary Syndrome that while on treatment for the disease and in a space of one month developed exorbitant slack folds in the axillae and cervical area mimicking GSS...
August 12, 2015: Journal of Cutaneous Pathology
Pamela Gangar, Sangeetha Venkatarajan
Granulomatous cutaneous T-cell lymphomas (CTCL) and lymphomatoid granulomatosis are considered granulomatous lymphoproliferative disorders. The most common types of granulomatous CTCL are granulomatous mycosis fungoides and granulomatous slack skin. Lymphomatoid granulomatosis is a rare Epstein-Barr virus driven lymphoproliferative disorder. This article reviews the etiopathogenesis, clinical presentation, systemic associations, and management of both granulomatous slack skin syndrome and lymphomatoid granulomatosis...
July 2015: Dermatologic Clinics
A Shah, A Safaya
Granulomatous slack skin (GSS) is a rare cutaneous disorder characterized by the evolution of circumscribed erythematous loose skin masses, especially in the body folds, and histologically by a loss of elastic fibers and granulomatous T-cell infiltrates. This disease is often associated with preceding or successive lymphoproliferative malignancies, especially Mycosis Fungoides (MF) and Hodgkin's Disease (HD). Whether Granulomatous Slack Skin Disease is a benign disorder, an unusual host reaction or a precursor of malignant lymphoma or an indolent Cutaneous T-cell Lymphoma (CTCL) in itself, is still a controversy...
December 2012: Journal of the European Academy of Dermatology and Venereology: JEADV
Martine Bagot
Mycosis fungoides is the most frequent cutaneous T cell lymphoma. Mycosis fungoides-related cutaneous T cell lymphomas include folliculotropic mycosis fungoides, with or without mucinosis, pagetoid reticulosis and granulomatous slack skin. Sezary syndrome is characterized by pruriginous erythroderma, adenopathies, atypical lymphocytes in the blood and a more aggressive course. B cell lymphomas represent 25 % of cutaneous lymphomas. Primary cutaneous follicle center cell lymphomas are the most frequent. They are often located on the head and trunk and have an indolent course...
October 2010: Bulletin de L'Académie Nationale de Médecine
F Gallardo, M P García-Muret, O Servitje, T Estrach, I Bielsa, A Salar, E Abella, C Barranco, R M Pujol
BACKGROUND: The presence of a prominent granulomatous tissue reaction in skin biopsies from primary cutaneous or systemic malignant lymphomas with secondary cutaneous involvement is a rare but well-known phenomenon. OBJECTIVE: This paper aims to characterize and study a series of cutaneous lymphomas showing a prominent granulomatous component. PATIENTS AND METHODS: The clinical, histopathological and evolutive features of granulomatous variants of mycosis fungoides (5 patients, 2 of them associating 'granulomatous slack skin' features), Sézary syndrome (1 patient), CD30(+) cutaneous T-cell lymphoma (2 patients), CD4(+) small/medium pleomorphic cutaneous T-cell lymphoma (1 patient), primary cutaneous B-cell lymphoma (3 patients) and peripheral T-cell lymphoma with secondary epithelioid granulomatous cutaneous involvement (4 patients) were reviewed...
June 2009: Journal of the European Academy of Dermatology and Venereology: JEADV
J C Moreno-Giménez, R Jiménez-Puya, M Galán-Gutiérrez, C Pérez-Seoane, F M Camacho
Granulomatous slack skin syndrome is a rare clinical and pathologic disorder. Only 42 patients have been reported, one of whom we described in 1997--the only child so far reported. We now describe the evolution of this patient and the transformation of the disease into a peripheral T-cell lymphoma, and the complications resulting in the child's death.
November 2007: Pediatric Dermatology
Helen Karakelides, Jordan L Geller, Arnold L Schroeter, Hong Chen, Philip S Behn, John S Adams, Martin Hewison, Robert A Wermers
UNLABELLED: 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...
September 2006: Journal of Bone and Mineral Research: the Official Journal of the American Society for Bone and Mineral Research
L B Ostezan, V C Fabré, J P Callen
No abstract text is available yet for this article.
February 1997: Archives of Dermatology
M A Wasik, R Sackstein, D Novick, J R Butmarc, Q Zhang, E C Vonderheid, M E Kadin
We report the case of a patient with a clinically aggressive large cell lymphoma (LCL) which expressed several T-lymphocyte markers and, in addition, CD56 and, to a lesser degree, CD68 antigens. A marked increase in serum concentration of interleukin (IL)-2 was found (490 and 167 pg/0.1 mL in two serum samples collected 6 months apart). This increase in IL-2 appeared unique to this lymphoma because serum concentration of IL-2 was not increased in any of the cases of various types of cutaneous lymphoproliferative disorders tested: mycosis fungoides-related cutaneous T-cell lymphoma (CTCL: 28 patients), granulomatous slack-skin syndrome (GS-SS: 1 patient), anaplastic large cell lymphoma (ALCL: 2 patients), subcutaneous gamma/delta T-cell lymphoma (gamma/delta-TCL: 1 patient), adult-type leukemia/lymphoma (ATLL: 1 patient), and lymphomatoid papulosis (LyP: 4 patients)...
July 1996: Human Pathology
P von den Driesch, V Mielke, M Simon, G Staib, J Tacke, W Sterry
The term "granulomatous slack skin" (GSS) was introduced by Ackerman for a disease first described by Convit et al. in 1973. GSS represents a rare cutaneous lymphoma characterized by localized elastolytic lesions with a granulomatous infiltrate. We recently observed two male patients with the characteristic features of this disease. Both patients responded well to therapy with interferon-alpha 2b. In one patient clinical remission was stable under long-term treatment with clofazimine. We report on common features of these two patients and give a review of the cases published in the literature...
December 1994: Der Hautarzt; Zeitschrift Für Dermatologie, Venerologie, und Verwandte Gebiete
G Burg, A Häffner, R Böni, S Dommann, R Dummer
Cutaneous T cell lymphomas (CTCL) are lymphoproliferative disorders, which can be classified by an adaptation of the Kiel classification. The most common CTCL, Mycosis fungoides (MF) and Sézary syndrome (SS), are monoclonal T helper memory lymphomas. They belong to the group of peripheral T cell lymphomas. However, CTCL includes other disease entities such as granulomatous slack skin, pagetoid reticulosis, and lipotropic CTCL. Polymerase chain reaction of the T cell receptor (TCR)-gamma and screening of the PCR products for sequence-specific mobility in acrylamide gels has increased the detection limit for clonal T cells in the skin...
1995: Recent Results in Cancer Research
B D Zelickson, M S Peters, M R Pittelkow
Current uses for gene rearrangement analysis in clinical dermatology are listed in Table 3. This technique is useful for determining the existence of clonal populations within a background of polyclonal lymphoid cells; therefore, it is helpful in the diagnosis and staging of patients with CTCL and PTCL. Although dual genotypes do occur, this technique is usually capable of determining lineage in a clonal lymphoid infiltrate and elucidating and characterizing the etiopathogenesis of certain neoplasms. On the basis of this review of the literature and our own experience, we conclude that gene rearrangement analysis shows great promise for monitoring response to therapy and detecting progression or relapse in patients with CTCL and PTCL...
April 1991: Clinics in Dermatology
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