Presentation
Similar presentations have been described as a verrucous and hyperplastic variant of mycosis fungoides due to the presence of prominent epidermal hyperplasia. [ncbi.nlm.nih.gov]
Entire Body System
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Thyroid Nodule
Investigations were within normal apart from thyroid nodule, enlarged cervical and axillary lymph nodes by ultrasound with distorted shape and lost hilum. [pulsus.com]
Skin
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Thin Eyebrows
Note bilateral dermatochalasis, thinned eyebrows, reddish, rugged skin on forehead and slight thickening of the right upper eyelid compared to the left. [nature.com]
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Honey-Colored Crust
The term extensive secondary bacterial infection was used for patients with widespread honey-colored crusted lesions at first presentation ( Figure 1 D). All statistical analyses were performed using the SPSS statistical software (IBM Corp). [jamanetwork.com]
Workup
(See Pathophysiology, Etiology, Presentation, and Workup.) Patch-stage mycosis fungoides. Plaque-stage mycosis fungoides. Hypopigmented cutaneous T-cell lymphoma. Courtesy of Jeffrey Meffert, MD. [emedicine.medscape.com]
CME/SAM Upon completion of this activity you will be able to: define and classify precancerous/lymphomatous cutaneous T-cell dyscrasias. outline the immunohistochemical workup and the clinical/pathologic features that permit distinction among folliculotropic [academic.oup.com]
Serum
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Thrombocytosis
Three patients had a coexistent hematological disorder: 1 patient had B-cell chronic lymphocytic leukemia, 1 patient had myelodysplastic syndrome, and 1 patient had essential thrombocytosis. [jamanetwork.com]
Colonoscopy
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Multiple Ulcerations
We report a case of folliculotropic mycosis fungoides (FMF) presenting with multiple ulcerative nodular lesions and persistent eosinophilia. Severe facial lesions resulted in a leonine appearance. [ncbi.nlm.nih.gov]
HLA Type
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HLA-DR5
HLA-DR5 and DQB1*03 class II alleles are associated with cutaneous T-cell lymphoma. J Invest Dermatol. 1996; 107 :373–6. [ PubMed ] [ Google Scholar ] 14. Welsh EA, Clark HH, Epstein SZ, Reveille JD, Duvic M. [ncbi.nlm.nih.gov]
HLA-DR5 and DQB1*03 class II alleles are associated with cutaneous T-cell lymphoma. J Invest Dermatol 1996;107:373-6. [ PUBMED ] 14. Welsh EA, Clark HH, Epstein SZ, Reveille JD, Duvic M. [e-ijd.org]
Biopsy
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Liver Biopsy
Diagnosis of AIH was established based on the liver biopsy (highly active autoimmune hepatitis) and the exclusion of viral etiology, drug-induced hepatotoxicity, and inherited metabolic disorders of the liver. [ncbi.nlm.nih.gov]
Treatment
Folliculotropic mycosis fungoides (MF) is an uncommon subtype of MF which has a more aggressive natural history and is more resistant to treatment than other forms of MF. In this article, an innovative method of treatment is presented. [ncbi.nlm.nih.gov]
Prognosis
The prognosis of folliculotropic MF is best estimated using the TNM staging criteria. Many clinicians feel that this variant of MF portends a worse prognosis; however, there are no studies to support this idea. [ncbi.nlm.nih.gov]
Etiology
Autoimmune hepatitis (AIH) is still an unclear progressive liver disease of unknown etiology which features hypergammaglobulinemia, detectable autoantibodies, and interface hepatitis (5). [ncbi.nlm.nih.gov]
Etiology The etiology remains unknown. Diagnostic methods The diagnosis is based on clinical presentation and should be confirmed by a skin biopsy. [orpha.net]
When lesions that share an etiologic factor are classified on the basis of histologic findings, the diagnosis is usually superficial EAC, as the deep form is rare. In keeping with 3 318 J.J. [healthdocbox.com]
[…] a good term since nonspecific as to histological type) Epidemiology Most common type of cutaneous T cell lymphoma, almost 50% of all primary cutaneous lymphomas Adults / elderly but also children and adolescents M:F ratio 2:1, black:white ratio 1.7 Etiology [pathologyoutlines.com]
Epidemiology
Summary Epidemiology The annual incidence of MF and its variants is estimated at between 1/350,000 and 1/110,000, with folliculotropic MF accounting for about 5% of MF cases. Males are more frequently affected than females. [orpha.net]
[…] on MCMN 2017 27 Clinical Staging and Prognostic Factors in Folliculotropic Mycosis Fungoides. ( 27276223 ) van Santen S....Willemze R. 2016 28 Folliculotropic mycosis fungoides: clinical and epidemiological evaluation in a single center in Brazil. ( 26711732 [malacards.org]
"Mycosis fungoides: review of epidemiological observations". Dermatology. 201 (1): 21–28. doi : 10.1159/000018423. ISSN 1018-8665. PMID 10971054. Korgavkar, Kaveri; Xiong, Michael; Weinstock, Martin (2013-11-01). [en.wikipedia.org]
Synonyms Epidemiology Mycosis fungoides accounts for only 0.5% of all non-Hodgkin lymphomas, but is the most common cutaneous T-cell lymphoma. [e-immunohistochemistry.info]
Pathophysiology
understanding pathophysiology and clinical relevance. J Clin Aesthet Dermatol 2010;3:32–8 Zachou K, Muratori P, Koukoulis GK, Granito A, Gatselis N, Fabbri A, et al. Review article: autoimmune hepatitis -- current management and challenges. [adc.mef.hr]
"Mycosis fungoides: pathophysiology and emerging therapies". Seminars in Oncology. 34 (6 Suppl 5): S21–8. doi : 10.1053/j.seminoncol.2007.11.006. PMID 18086343. [en.wikipedia.org]
(See Pathophysiology, Etiology, Presentation, and Workup.) Patch-stage mycosis fungoides. Plaque-stage mycosis fungoides. Hypopigmented cutaneous T-cell lymphoma. Courtesy of Jeffrey Meffert, MD. [emedicine.medscape.com]
Ana Bolaños.Mycosis fungoides Author: Doctor Lorenzo Cerroni1 Creation date: October 2003 Scientific editor: Professor Benvenuto Giannotti 1Department of Dermatology, University.Hypopigmented mycosis fungoides: a review of its clinical features and pathophysiology [camosaxomidegucamus.xpg.com.br]
] and segmental glomerular sclerosis. [9] Among eight reported cases of CTCL associated with kidney disease, all except one were late-stage CTCL, and three patients died of progressive disease. [10] However, it is unclear whether this association is pathophysiological [ijdvl.com]
Prevention
CONCLUSION: The awareness and the identification of the various histopathological presentations of FMF by pathologists, as well as by clinicians, are imperative to prevent diagnostic errors. 2014 John Wiley & Sons A/S. [ncbi.nlm.nih.gov]
Treatment is challenging due to the need for immunosuppression to prevent graft rejection, with fine adjustments to prevent worsening of his lymphoproliferative disease. [pediatrics.aappublications.org]
Eliminating the variable risk factors may help in preventing the condition. [dovemed.com]