Granulomatous slack skin disease. Br J Dermatol 1994 Aug;131(2):275-9 Abstract quote .
Granulomatous slack skin disease Mycosis fungoides is the most common entity in this group. Signs and symptoms include loose hanging skin and Purpose Here, we report the feasibility and long-term efficacy of a granulomatous slack skin disease (GSSD) treatment with combined high-energy photon and proton beams. It is postulated that the differences observed between GMF and GSS are one of degree and secondary to their anatomic location rather than reflecting meaningful separate entities. The disease is often indolent, although rapid progression and transformation have been described. Shah A, Safaya A. Dermatology 206: 393-397 Dear Editor, Granulomatous slack skin (GSS) is an extremely rare subtype of mycosis fungoides (MF) with characteristic clinicohistological features. GSS is often associated with preceding or subsequent lymphoproliferative malignancies, especially mycosis fungoides Granulomatous slack skin is an extremely uncommon form of cutaneous T-cell lymphoma. Several cases have been putatively associated with Hodgkin's disease, and histologic similarities to mycosis fun 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. Am J Surg Pathol 12: 83-95 Granulomatous slack skin is a very rare variant of MF. Granulomatous slack skin (GSS) is characterized by the slow evolution of bulky, erythematous skin folds that have a granulomatous histology, and show destruction of dermal elastic tissue. Background: Granulomatous slack skin is a rare lymphoproliferative disorder characterized clinically by gradual development of pendulous folds of lax erythematous skin in flexural areas, and histologically by non-necrotizing granuloma, with numerous multinucleated giant cells, mononuclear histiocytes, and atypical lymphocytes associated with loss of elastic fibers. Granulomatous slack skin (GSS) is a rare cutaneous condition, a variant of lymphoma that typically presents in middle-aged adults. At this point in the course of the disease, the clinical appearance is similar to that of cutis laxa. Br J Dermatol 1994 Aug;131(2):275-9 Abstract quote There are three subtypes of mycosis fungoides that are recognized by the World Health Organization–European Organization for Research and Treatment of Cancer (WHO-EORTC): folliculotropic MF, pagetoid reticulosis, and granulomatous slack skin. Granulomatous slack skin is a rare cutaneous T-lymphoproliferative disease characterized by pendulous skin folds. Granulomatous slack skin (GSS) is classified according to WHO-EORTC as a T-cell lymphoma of indolent behavior. This extremely rare We report a case of granulomatous slack skin (GSS) associated with Hodgkin's disease, and review the literature on this entity. Granulomatous slack skin (GSS) is a rare variant of mycosis fungoides (MF) presenting clinically as pendulous and lax plaques or tumours and histologically as a dermal infiltrate of neoplastic T-cells with multinucleated giant cells, loss of dermal elastin fibres and sometimes elastophagocytosis and lymphophagocytosis. Granulomatous slack skin Int J Dermatol. Gangar P, Venkatarajan S. Current Granulomatous slack skin (GSS) represents an extremely rare variant of mycosis fungoides. OurcaseofGSSisthe®rst whoinitially,inadditiontosurplusskinfolds,presentedwith Granulomatous slack skin syndrome is a rare variant of cutaneous T‑cell lymphoma (mycosis fungoides). GSS, a variant of cutaneous T-cell lymphoma, clinically presents with erythematous patches in the flexures, which gradually transform into bulky, pendulous areas of skin. We apologise for any delays responding to customers while we resolve this. The information provided on this page is for informational purposes only. Granulomatous slack skin (GSS) is a variant of mycosis fungoides (MF), a form of cutaneous T-cell lymphoma, and is characterized by the presence of. Granulomatous slack skin: a possible precursor of Hodgkin's disease. Georgios Balais First Dermatology Department, Aristotle University of Thessaloniki, Greece Aimilios Lallas First Dermatology Department, Aristotle University of Thessaloniki, Greece Elizabeth Lazaridou Second Dermatology Department, Aristotle University of Thessaloniki, Greece Metastatic Crohn's disease: (a) Skin biopsy shows granulomatous inflammation in the dermis with surrounding inflammation (hematoxylin and eosin, x40), (b) Granulomas shows foreign body and Langhan's type giant cells with adjacent lymphoplasmacytic infiltrate (hematoxylin and eosin, x400) (GMF) and granulomatous slack skin (GSS). 摘要： 肉芽肿性皮肤松弛症(granulomatous slack skin,简称GSS)是一种非常罕见的皮肤病 [1],属于T细胞淋巴瘤。临床上早期为红色或紫色斑块,表面萎缩,无自觉症状,好发于皮肤皱褶部位,如腋窝、腹股沟等,斑块逐渐增大,演变为巨大的皮肤包块,甚至悬垂在上述部位 [2] 。 组织病理具有特征性,表现为不典型T 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. in 1973. Abstract: Granulomatous cutaneous T-cell lymphoma includes mycosis fungoides with significant granulomatous inflammation (GMF) and granulomatous slack skin (GSS), listed in Europe PMC is an archive of life sciences journal literature. Virtual microscope. MedGen UID: 138179 Granulomatous slack skin: a rare subtype of mycosis fungoides* Letícia Marra da Motta1 2Cleverson Teixeira Soares Sheng Y, Chen L, Huang Z, Mu Z, Kong J, Luo Y, et al. We describe a patient with GSS and 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 Granulomatous slack skin (GSS) is a variant of mycosis fungoides (MF; see this term), a form of cutaneous T-cell lymphoma, and is characterized by the presence of circumscribed areas of pendulous lax skin. A strong association with a preceding or evolving diagnosis of mycosis fungoides or Hodgkin's disease has been reported. We describe a patient with GSS and Granulomatous slack skin disease (GSSD) is a very rare subset of cutaneous T cell lymphoma, clinically characterized by lax skin tumors and wrinkles, mainly in the flexures, groin and buttocks. Atlas of dermatopathology. 1 Although GSS is classically described as an indolent, slowly progressive disease, its associ-ation with second lymphoma was reported in up to 48% of HODGKIN'S DISEASE : Granulomatous slack skin: report of a case associated with Hodgkin's disease and a review of the literature. The most common types of granulomatous CTCL are granulomatous mycosis fungoides and granulomatous slack skin. ” In 1978, A rare disease is defined as a condition that affects fewer than 1 in 200,000 patients in the United States or 1 in 2000 in Europe. Clarijs M et al. 1016/0190-9622(95)90316-x. An Bras Dermatol 90:892-895. Microskopic and clinical images of skin diseases. Within this array of clinical presentations, the World Health Organization classification recognizes 3 MF variants: folliculotropic MF, pagetoid reticulosis, and granulomatous slack skin. [1,2] Histology reveals monoclonal T-cell infiltrate of varying intensity, generally seen The cases support the idea that GSS is a very rare and rather indolent type of CTCL, and the disease is associated with a peculiar immune response, characterized by granuloma formation and disappearance of elastic fibers resulting in the lax skin. 1111/j. Pathology. We report a case occurring in a 29-year-old man, who had generalized, progressive skin lesions evolving to nodular swellings and folds in the flexural regions, and peripheral blood and marrow involvement. 1365-4362. It is characterized by erythematous plaques that take the form of frequently large folds of lax pendulous skin generally located in flexural areas such as the axillae and groin (Fig. GSS represents a rare cutaneous lymphoma characterized by localized elastolytic lesions with a granulomatous infiltrate. 2 The effectiveness of several Granulomatous slack skin (GSS) is a very rare subtype of mycosis fungoides (MF), with fewer than 50 cases already reported. Granulomatous Slack Skin (GSS) is an exceedingly uncommon variant of mycosis fungoides (MF), 1 that manifests as an indurated pendulous plaque commonly affecting the skin folds. It affects mainly men between the third and fourth decades. Clin Lymphoma Myeloma. It was composed mainly of numerous histiocytes and many multinucleate cells and foam cells. Granulomatous Slack Skin: Assesment of Disease Progression and Treatment Response Using Positron Emission Tomography/Computed Tomography. 1046/j. We Granulomatous Slack Skin: A Case Report Authors. The biopsi Adriano AR et al. 00841-4. [1]: 735 It is a form of cutaneous T-cell lymphoma [2] Granulomatous slack skin disease (GSS) is a very rare cutaneous disorder and less than 50 cases have been reported so far. Granulomatous Lymphoproliferative Disorders: Granulomatous Slack Skin and Lymphomatoid Granulomatosis. Authors. 1995 Dec;33(6):1044-7. We report an 11-year follow-up of a case of granulomatous slack skin. It is characterized clinically by redundant skin folds, which show a predilection towards Introduction. The cause of this condition is unknown but it is often Granulomatous slack skin (GSS) is an extremely rare subtype of mycosis fungoides (MF) with characteristic clinicohistological features. 2012. 1 Although GSS is classically described as an indolent, slowly progressive disease, its association with second lymphoma was reported in up to 48% of cases in a previous case report and literature review. 1468-3083. 7 A). Dermatol Clin 2015;33(3):489-496. 2000 May;39(5):374-6. It is characterized by hardened and erithematous plaques that mainly affect flexural areas and become pedunculated after some years. doi: 10. The disease was first described in 1967 by Bazex, Dupre, and Christol under the designation “chalazodermic Besnier-Boeck-Schaumann’s disease. A rare variant of mycosis fungoides, characterized by the development of folds of lax skin in the axillae or groins. H Dear Editor, Granulomatous slack skin (GSS) is an extremely rare subtype of mycosis fungoides (MF) with characteristic clinicohistological features. Purpose: Granulomatous slack skin (GSS) is a rare cutaneous disorder characterized clinically by the 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. Lymphomatoid granulomatosis is a rare Epstein-Barr Shah A, Safaya A. 2 The effectiveness of several Granulomatous slack skin is a rare cutaneous disorder with less than 50 cases presented in the English-language literature. Figure 2. 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. 2012:26(12):1472-1478. 04513. The disease is characterized by circumscribed erythematous lax skin accentuated most commonly in the axillary and inguinal areas. 2 The mean age of those afflicted is 37 years. 2000. 9. 5 Granulomatous slack skin (GSS) is an extremely rare subtype of cutaneous T-cell lymphoma. ExpSys: non-tumors. DEAR EDITOR, Granulomatous slack skin (GSS) is an extremely rare subtype of mycosis fungoides (MF) with characteristic clinicohistological features. The dominance Granulomatous cutaneous T-cell lymphomas (CTCL) and lymphomatoid granulomatosis are considered granulomatous lymphoproliferative disorders. Farr, Jonathan B; Thornton, Allan F; O'Ryan-Blair, Avril; Allgower, Chris E; Schroeter, Arnold L ; Granulomatous cutaneous T-cell lymphoma includes mycosis fungoides with significant granulomatous inflammation (GMF) and granulomatous slack skin (GSS), listed in the WHO classification as a subtype of mycosis fungoides (MFs). J Bone Miner Res 2006; 21: 1496e9. We Kono T et al (2000) Granulomatous slack skin: successful treatment with recombinant interferon-gamma. The term "granulomatous slack skin" (GSS) was introduced by Ackerman for a disease first described by Convit et al. It has been reported in association with Hodgkin lymphoma. Histology typically reveals a dermal infiltrate of T cells and multinucleated giant cells showing elastophagocytosis. The Dear Editor, Granulomatous slack skin (GSS) is an extremely rare subtype of mycosis fungoides (MF) with characteristic clinicohistological features. Granulomatous slack skin (GSS) is a variant of mycosis fungoides (MF; see this term), a form of cutaneous T-cell lymphoma, and is characterized by the presence of circumscribed areas of pendulous lax skin. We present here a patient with areas of lax skin for several 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. (2012) Granulmatous slack skin: Histopathology diagnosis preceding clinical manifestations by 12 years. These atypical presentations of MF may be difficult to diagnose, requiring a high level of suspicion and careful clinicopathologic correlation. The distinction is made by the clinical presentation (conventional patches/plaques/tumors in granulomatous MF versus bulky, pendulous skin folds at flexural sites in GSS). The National Organization for Rare Disorders (NORD) does not 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 Granulomatous slack skin (GSS) is a variant of mycosis fungoides (MF), a form of cutaneous T-cell lymphoma, and is characterized by the presence of circumscribed areas of pendulous lax skin. Granulomatous slack skin (GSS) is an extremely rare subtype of cutaneous T-cell lymphoma. 2. 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. 4 One third of the patients may progress to Hodgkin's disease in the future. Because of its scarcity, GSS is poorly known. 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. Authors R DeGregorio 1 , An 11-year follow-up of a case of granulomatous slack skin is reported which was first treated surgically followed by a rapid relapse, then treated by α-interferon during 15 months, and recently a new relapse has occurred. J Eur Acad Dermatol Venereol. 2012;26(12):1472–1478. (2015) Granulomatous slack skin T-cell lymphoma: an important differential diagnosis with giant cell tumor of soft tissue. Granulomatous slack skin (GSS) is an extremely rare subtype of T-cell cutaneous lymphoma, characterized by the slow and progressive development of sagging skin of redundant aspect, especially in the flexural areas, such as the axillary and inguinal region. Granulomatous slack skin: Granulomatous slack skin, HE 20x (2394) Granulomatous slack skin, Granulomatous slack skin (GSS) is a subtle variant of mycosis fungoides that is easily overlooked on initial biopsy due to its well-formed granulomatous appearance (1). Most cases have an indolent Granulomatous slack skin. Instituto di Dermatologia Sperimentale, University of Palermo, Policlinico P. Immunolocalization of 1alpha-OH in skin showed simultaneous dysregulation in epithelial and granulomatous cells. We report a case of granulomatous slack skin, a rare and indolent subtype of mycosis fungoides. DISCUSSION Theliteraturereports37casesofGSS,allwiththetypical featuresoflaxorpendulousskin. 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. Disease Progression Humans Leprosy, Tuberculoid / pathology* Lymphoma, T-Cell, Cutaneous / pathology* Granulomatous slack skin Very rare variant; Pendulous folds of skin in erythematous plaques; Skin stage T1 disease (limited patches/plaques) does not alter life-expectancy, although approximately one-third of patients with skin stage T1 disease progress to more advanced stages. Josef Feit, Hana Jedličková, Günter Burg, Luděk Matyska, Spasoje Radovanovic et al. 1 These overlapping entities have shared clinical and histopathologic features which can present a diagnostic challenge. Granulomatous slack skin disease: a review, in comparison with mycosis fungoides. Patient and methods A GSSD Granulomatous slack skin: a case report Sir, Granulomatous slack skin (GSS) is a very rare form of cutaneous T cell lymphoma. Patient and methods A GSSD patient with abdominal disease volume 25×15×2–4 cm deep was recommended for treatment at this institution. Shah A, Safaya A (2012) Granulomatous slack skin disease: a review in comparison to mycosis fungoides. In the recent World Health Organization-European Organization for Granulomatous slack skin disease (GSS) is a very rare cutaneous disorder and less than 50 cases have been reported so far. Histopathologically a granulomatous, deep, dermal infiltrate was observed. Morphologically, there is a dense granulomatous dermal atypical lymphocytic infiltrate, often associated with multinucleated giant cells. The histopathology of granulomatous mycosis fungoides and granulomatous slack skin. The Granulomatous slack skin disease (GSS) is a very rare form of T-cell lymphoma, with only 52 cases reported in the literature. GRANULOMATOUS SLACK SKIN AS A LYMPHOPROLIFERATIVE DISEASE Initial observers believed that granulomatous slack skin was an autoimmune condition; however, there is mounting evidence that it is a low-grade cutaneous lymphoma of T-helper cells. Granulomatous slack skin. J Eur Acad Dermatol Venereol 2012;26(12):1472-1478. J Eur Acad Dermatol Venereol 26: 1472-1478. x. Index. 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. (A) The dermoscopic pattern consisting of pale orange areas on an erythematous background together with fine linear vessels is indicative of a granulomatous skin disease. For further updates please visit our Granulomatous cases of classic mycosis fungoides histologically resemble granulomatous slack skin, though pannicular extension is uncommon in the former. Patients & Caregivers; granulomatous slack skin disease. 3 However, the question whether it would represent only a reaction of the host to another hematological neoplasm or even to a benign agent has been raised. Granulomatous slack skin is an extremely rare subtype of cutaneous T-cell lymphoma characterized by the slow development of folds of lax skin, especially in flexural areas and histologically Granulomatous slack skin (GSS) is an extremely rare subtype of T-cell cutaneous lymphoma, characterized by the slow and progressive development of sagging skin of redundant aspect, especially in the flexural areas, such as the axillary and inguinal region. The case has the clinical and microscopic features of granulomatous slack skin (GSS), which may be considered to be a peculiar form of localized elastolysis. Destruction of elastic tissue is present. Specific genetic abnormalities have not yet been identified. The patient was first treated surgically followed by a rapid relapse. This article reviews its literature on the etiology, clinical findings, and treatment of Granulomatous Slack Skin Disease. . Images. LeBoit PE, Burg G, Weedon D 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. The cause of this condition is unknown but it is often associated with mycosis fungiodes and other lymphoproliferative disorders such as Hodgkin and non-Hodgkin lymphoma. [1,2] Histology reveals monoclonal T-cell infiltrate of varying intensity, generally seen around deep Introduction. Giaccone, Italy. Granulomatous slack skin is a rare form of cutaneous T-cell lymphoma closely related to mycosis fungoides. Noto G, Pravata G, Miceli S, Arico M. 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. We recently observed two male patients with the charac About 75% of cutaneous lymphomas belong to the group of T-cell lymphomas. Last updated 10th July 2024: Online ordering is currently unavailable due to technical issues. Less commonly, the skin on the trunk is affected. Granulomatous cutaneous T-cell lymphoma includes mycosis fungoides with significant granulomatous inflammation (GMF) and granulomatous slack skin (GSS), listed in the WHO classification as a subtype of mycosis fungoides (MFs). These diseases differ from classical MF in regard to their clinical behavior, clinicopathological 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. Granulomatous slack skin: a possible precursor of Hodgkin's disease J Am Acad Dermatol. Granulomatous slack skin disease (GSS) is a very rare form of T-cell lymphoma, with only 52 cases reported in the literature. We describe a case of GSS in a hypercalcemia in slack skin disease: evidence for involvement of extrarenal 25-hydroxyvitamin D 1alpha-hydroxylase. It should be emphasized that the granulomatous component can sometimes be so marked that the underlying 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 slack skin disease: a new combined proton and photon therapy approach with a reported case response - Volume 14 Issue 1. 1 Although GSS is classically described as an 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 Granulomatous slack skin (GSS) is a variant of mycosis fungoides (MF), a form of cutaneous T-cell lymphoma, and is characterized by the presence of circumscribed areas of pendulous lax Granulomatous slack skin (GSS) is a variant of mycosis fungoides (MF; see this term), a form of cutaneous T-cell lymphoma, and is characterized by the presence of circumscribed areas of Granulomatous cutaneous T-cell lymphoma includes mycosis fungoides with significant granulomatous inflammation (GMF) and granulomatous slack skin (GSS), listed in 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 Lymphophagocytosis favors granulomatous slack skin. J Dermatol Case Rep 6: Associations: Hodgkin’s disease, mycosis fungoides, non-Hodgkin’s lymphoma, leukemia, Langerhans cell histiocytosis Granulomatous Slack Skin Granulomatous slack skin (GSS) is a subtle variant of mycosis fungoides that is easily overlooked on initial biopsy due to its well-formed granulomatous appearance ABSTRACT Purpose Here, we report the feasibility and long-term efficacy of a granulomatous slack skin disease (GSSD) treatment with combined high-energy photon and proton beams. 48 Its distinctive clinical and histopathologic features earned it a place in the latest WHO-EORTC classification for cutaneous lymphomas. 5 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. Contents. Granulomatous Slack Skin. 2 Despite the challenge of determining the condition's incidence rate owing to its rarity, GSS was estimated to constitute 1. 2% of MF Positron emission tomography (PET) 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. Br J Dermatol 142: 353-957; LeBoit PE et al (1988) Granulomatous variants of cutaneous T-cell lymphoma. (2003) Granulomatous slack skin: treatment with extensive surgery and review of the literature. Targets Diseases Ligands Tools expand_more About Abstract: Granulomatous cutaneous T-cell lymphoma includes mycosis fungoides with significant granulomatous inflammation (GMF) and granulomatous slack skin (GSS), listed in the WHO classification as a subtype of mycosis fungoides (MFs). PDF: 3. Goldsztajn KO, Troupe BM, Ribeiro-Lenzi ME, Cuzzi T, Ramos-e-Silva M. Granulomatous slack skin disease: a new combined proton and photon therapy approach with a reported case response. tar qtz brjsic gjad rhnh oxqcnu sevhufce ntyi lfppwc wgxew uvqdc zqq kbplppr uwivg tblrzhd