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Granulomatous slack skin disease

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
Karen O Goldsztajn, Beatriz Moritz Trope, Maria Elisa Ribeiro Lenzi, Tullia Cuzzi, Marcia Ramos-E-Silva
BACKGROUND: Granulomatous slack skin is a very rare subtype of T-cell cutaneous lymphoma, characterized by the slow development of cutaneous sagging, especially on flexural areas. Its behavior is indolent and the treatment, in the majority of cases, disappointing. MAIN OBSERVATION: We report a 54-year-old black patient with granulomatous slack skin, who at the beginning of the investigation showed intense xeroderma and generalized lymph node enlargement. The diagnosis was established based on histopathologic findings long before the disease's characteristic clinical presentation appeared...
December 31, 2012: Journal of Dermatological Case Reports
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
Alejandra Abeldaño, Mariana Arias, Adriana Benedetti, Karina Ochoa, Matías Maskin, Graciela Pellerano, María Cristina Kien, Edgardo Chouela
Unusual variants of mycosis fungoides (MF) differ substantially from the classical presentation, and most of them resemble other dermatologic diseases. The authors reviewed files of patients with MF who consulted our clinic between November 1995 and June 2010 to evaluate the relative frequency and clinical behavior of these variants. Among 98 patients with MF, 32 (32.65%) had unusual variants. The most common types included follicular MF (31.25%), hypopigmented MF (18.75%), poiquilodermic MF (15.6%), and erythrodermic MF (12...
July 2011: Skinmed
Youyu Sheng, Lianjun Chen, Zhemin Huang, Zhanglei Mu, Jincheng Kong, Yan Luo, Qinping Yang
Granulomatous slack skin (GSS) is an extremely rare subtype of cutaneous T-cell lymphoma. A 14-year-old boy had suffered from progressive infiltrative erythema and plaques that gradually evolved into lax masses and pendulous skin on his axilla, anterior wall of the abdomen, bilateral inguinal region, and thighs. Histopathologic examination of the skin lesion and inguinal lymph node demonstrated granulomatous infiltration with multinucleated giant cells. Positron emission tomography (PET)/computed tomography (CT) scan was performed after acute exacerbation and exhibited slightly high fluorodeoxyglucose (FDG) distribution of skin lesions, without any evidence of abnormality in the metabolism of FDG in lymph nodes or other extralymphatic organs...
December 2009: Clinical Lymphoma & Myeloma
Chao-long Peng, Mu-shui Fang
No abstract text is available yet for this article.
April 2009: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
Michael Tronnier
Presented in part at the XXVIII Symposium of the International Society of Dermatopathology, Paris, November 14-17, 2007. A 13-year-old boy developed a bulky mass close to the right axillary region and an infiltrated plaque on the abdominal skin. Two years earlier, the diagnosis of follicular mycosis fungoides has been established. Biopsies of both areas revealed typical features of granulomatous slack skin (GSS). Within some of the giant cells, doubly refractile material of unknown origin was found. It may be speculated that in the reported case of follicular mycosis fungoides in childhood, GSS developed as a specific reaction pattern of the disease because of foreign bodies...
May 2009: Journal of Cutaneous Pathology
Pa-Fan Hsiao, Cheng-Hsiang Hsiao, Tsen-Fang Tsai, Ming Yao, Shiou-Hwa Jee
We describe a case of granulomatous slack skin in a 31-year-old woman with an unusual presentation of acquired ichthyosis and muscular masses involving four limbs over 3 years. Vesicles and ulcerative skin nodules first appeared only 3 months prior to diagnosis. The diagnosis was confirmed after sequential biopsies of muscle, skin lesions, and lymph nodes, together with molecular genetic studies. The patient responded poorly to various therapies, including thalidomide, and died of doxorubicin-related cardiomyopathy...
2009: American Journal of Clinical Dermatology
Werner Kempf, Sonja Ostheeren-Michaelis, Marco Paulli, Marco Lucioni, Janine Wechsler, Heike Audring, Chalid Assaf, Thomas Rüdiger, Rein Willemze, Chris J L M Meijer, Emilio Berti, Lorenzo Cerroni, Marco Santucci, Christian Hallermann, Mark Berneburg, Sergio Chimenti, Alistair Robson, Martà Marschalko, Dmitry V Kazakov, Tony Petrella, Sylvie Fraitag, Agnes Carlotti, Philippe Courville, Hubert Laeng, Robert Knobler, Philippa Golling, Reinhard Dummer, Günter Burg
BACKGROUND: Granulomatous cutaneous T-cell lymphomas (CTCLs) are rare and represent a diagnostic challenge. Only limited data on the clinicopathological and prognostic features of granulomatous CTCLs are available. We studied 19 patients with granulomatous CTCLs to further characterize the clinicopathological, therapeutic, and prognostic features. OBSERVATIONS: The group included 15 patients with granulomatous mycosis fungoides (GMF) and 4 with granulomatous slack skin (GSS) defined according to the World Health Organization-European Organization for Research and Treatment of Cancer classification for cutaneous lymphomas...
December 2008: Archives of Dermatology
A Farooque, C Moss, D Zehnder, M Hewison, N J Shaw
BACKGROUND: The most serious complication of subcutaneous fat necrosis (SCFN), a rare condition of the newborn characterized by indurated purple nodules, is hypercalcaemia. However, the mechanism for this hypercalcaemia remains unclear. OBJECTIVES: To determine whether the hypercalcaemia associated with SCFN involves expression of the vitamin D-activating enzyme 25-hydroxyvitamin D(3)-1alpha-hydroxylase (1alpha-hydroxylase) in affected tissue. METHODS: Skin biopsies from two male patients with SCFN and hypercalcaemia were taken...
February 2009: British Journal of Dermatology
Emma Clare Benton, Stephen L Morris, Alistair Robson, Sean J Whittaker
Granulomatous slack skin disease (GSS) is a very rare form of T-cell lymphoma, with only 52 cases reported in the literature. In the recent World Health Organization-European Organization for Research and Treatment of Cancer consensus classification GSS is considered to be a variant of mycosis fungoides. We describe a patient with GSS and histologic evidence of necrobiosis, which has not been previously reported.
October 2008: American Journal of Dermatopathology
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
L Requena, E González-Guerra, J Angulo, A E DeVore, O P Sangueza
Mycosis fungoides is the most common type of primary cutaneous T-cell lymphoma. Several rare clinicopathological variants of mycosis fungoides have been described. Patients with these variants often also have classic mycosis fungoides at other sites of the body. Anetoderma is a cutaneous disorder in which multiple, oval lesions or atrophic plaques with wrinkled surface develop progressively due to loss of the dermal elastic tissue. Primary anetoderma occurs when there is no underlying associated disease and it arises on clinically normal skin, whereas secondary anetoderma appears in the same site as a previous specific skin lesion...
January 2008: British Journal of Dermatology
Gonca Gokdemir, Andaç Argon, Damlanur Sakiz, Dilek Argon, Adem Köşlü
Granulomatous slack skin (GSS) is a rare form of cutaneous T-cell lymphoma, closely related to mycosis fungoides. It is characterized by pendulous skin folds with a predilection for flexural areas. Histology shows an elastolytic granulomatous infiltrate with atypical lymphoid cells. Granulomatous mycosis fungoides is an important histologic differential diagnosis to be considered. We present a 19-year-old man with a gradually enlarging erythematous, and bulky lesions on his body. Histologically, a dense atypical lymphoid cell infiltration with numerous multinucleated giant cells and elastolysis was observed...
2008: Medical Oncology
Ida Münster Ikonomou, Hege Vangstein Aamot, Sverre Heim, Alexander Fosså, Jan Delabie
Granulomatous slack skin is a rare cutaneous T-lymphoproliferative disease characterized by pendulous skin folds. Histology typically reveals a dermal infiltrate of T cells and multinucleated giant cells showing elastophagocytosis. Specific genetic abnormalities have not yet been identified. Currently, granulomatous slack skin is classified according to the World Health Organization classification as a variant of mycosis fungoides although supporting genetic evidence is yet lacking. We present a well-documented case of a 46-year-old man with the typical histologic and clinical findings of granulomatous slack skin...
May 2007: American Journal of Surgical Pathology
D Nashan, D Faulhaber, S Ständer, T A Luger, R Stadler
Mycosis fungoides (MF), a low-grade lymphoproliferative disorder, is the most common type of cutaneous T-cell lymphoma. Typically, neoplastic T cells localize to the skin and produce patches, plaques, tumours or erythroderma. Diagnosis of MF can be difficult due to highly variable presentations and the sometimes nonspecific nature of histological findings. Molecular biology has improved the diagnostic accuracy. Nevertheless, clinical experience is of substantial importance as MF can resemble a wide variety of skin diseases...
January 2007: British Journal of 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
Nnenna Osuji, Louise Fearfield, Estella Matutes, Andrew C Wotherspoon, Christopher Bunker, Daniel Catovsky
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...
October 2003: British Journal of Haematology
Magali Clarijs, Françoise Poot, Andoni Laka, Christian Pirard, André Bourlond
We report an 11-year follow-up of a case of granulomatous slack skin. The patient was first treated surgically followed by a rapid relapse. Then he was treated by alpha-interferon during 15 months. When this treatment was stopped, the disease relapsed again. Extensive surgery was undertaken. Recently, a new relapse has occurred which was again treated by extensive surgery. No other manifestation of a lymphoproliferative disorder appeared. The clinical, histological, immunohistochemical, ultrastructural, molecular biological and genetic studies with a review of 39 other cases described in the literature are presented...
2003: Dermatology: International Journal for Clinical and Investigative Dermatology
K Wada, C Maesawa, T Satoh, T Akasaka, T Masuda
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...
November 2002: British Journal of Dermatology
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