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The text is the summary of recent articles on Granulomatous Slack Skin at 75 thresold from National Library of Medicine (NLM). This information is subject to NCBI's Disclaimer and Copyright notice.
Skin Biopsy showed a loss of elastic fibers and an atypical granulomatous T-cell Infiltrate with epidermotropism, enabling a diagnosis of GSS to be made . 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 . lymphomatoid granulomatosis is a rare Epstein-Barr virus driven lymphoproliferative disorder . Within this array of clinical presentations, the World Health Organization classification recognizes 3 MF variants: folliculotropic MF, pagetoid reticulosis, and Granulomatous slack skin . Granulomatous mycosis fungoides is a very rare form of mycosis fungoides with histological resemblance to granulomatous slack skin .
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 . This article reviews its literature on the etiology, clinical findings, and treatment of Granulomatous Slack Skin Disease . 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 . Less common variants included unilesional MF, bullosa MF, ichthyosiform MF, granulomatous slack skin, and Pigmented purpura-like MF .
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 . 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) . The diagnosis was confirmed after sequential biopsies of Muscle, skin lesions, and lymph nodes, together with molecular genetic studies .
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 . Patients with these variants often also have classic mycosis fungoides at other sites of the body . Two patients developed areas of secondary anetoderma on Plaque stage lesions of mycosis fungoides . Elastic tissue stain demonstrated that elastic fibres were almost completely absent in the Dermis of the anetodermic lesions . Anetodermic mycosis fungoides should be added to the list of clinicopathological variants of mycosis fungoides and mycosis fungoides should also be considered as a possible disease causing secondary anetoderma .
It is characterized by pendulous skin folds with a predilection for Flexural areas . Histology shows an elastolytic granulomatous Infiltrate with Atypical lymphoid Cells . 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 . Diagnosis of MF can be difficult due to highly variable presentations and the sometimes nonspecific nature of histological findings . 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 . Recently, a new relapse has occurred which was again treated by extensive Surgery . No other manifestation of a lymphoproliferative disorder appeared . The diagnosis of Granulomatous mycosis fungoides versus early granulomatous slack skin was made .
References: 1 , 2 , 3 , 4 , 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 ,Press Refresh to fetch fresh content with references from pubmed. This content was cached on 2017-09-13 comments powered by Disqus
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