RESULTS: In all, 34 patients presented with follicle-based patch/flat plaques, keratosis pilaris-like lesions, and/or acneiform lesions, defined clinically as early stage (IA, IB), and 15 presented with follicle-based infiltrated plaques and/or tumors [ncbi.nlm.nih.gov]

• Anemia

  […] side effects Liposomal doxorubicin Infection, anemia, bleeding HDAC inhibitors (vorinostat, romidepsin) Infection, anemia, leukopenia, pulmonary embolism Gemcitabine Anemia, leukopenia, flu-like symptoms Deoxycoformycin Infection, potential to induce [dermatologyadvisor.com]

  Hodgkin Lymphoma Presenting with Various Immunologic Abnormalities, Including Autoimmune Hepatitis, Hashimoto’s Thyroiditis, Autoimmune Hemolytic Anemia, and Immune Thrombocytopenia. [adc.mef.hr]

  33%), fatigue (31%), diarrhea (28%), drug eruption (24%), upper respiratory tract infection (22%), musculoskeletal pain (22%), skin infection (19%), pyrexia (17%), edema (16%), nausea (16%), headache (14%), thrombocytopenia (14%), constipation (13%), anemia [poteligeohcp.com]

  If other parts of the body are also affected, widespread symptoms, such as weight loss, fatigue, anemia, fever, and night sweats, can also be present Folliculotropic Mycosis Fungoides is usually diagnosed with a biopsy of the affected skin or taking a [dovemed.com]

• Weight Loss

  A small number of people with a CTCL have more general symptoms such as unexplained weight loss, fevers or night sweats. [lymphoma-action.org.uk]

  If other parts of the body are also affected, widespread symptoms, such as weight loss, fatigue, anemia, fever, and night sweats, can also be present Folliculotropic Mycosis Fungoides is usually diagnosed with a biopsy of the affected skin or taking a [dovemed.com]

  These lesions are frequently accompanied by systemic symptoms such as fever and weight loss, and an associated hemophagocytic syndrome may be observed. [emedicine.medscape.com]

• Asymptomatic

  A 72-year-old man presented with a 4-year history of asymptomatic erythematous plaques on his face, neck, and scalp. He had no systemic symptoms or lymphadenopathy. [ncbi.nlm.nih.gov]

  Clinical synopsis A 72-year-old man presented to NYU Dermatologic Associates in January 2005 with a 4-year history of asymptomatic, erythematous plaques on his face. [escholarship.org]

  D, Large asymptomatic hyperpigmented patch with numerous overlying comedones on the left chest. [pediatrics.aappublications.org]

  Hypopigmented MF is often seen in young, dark-skinned patients of Indian or African-American origin who present with asymptomatic or slightly pruritic, non-scaly patches with irregular borders (Fig. 7) (22-28). [mussenhealth.us]

• Falling

  Cutaneous and subcutaneous hematomas associated with falls in a geriatric hospital. Toutous Trellu L., Herrmann F. R., Assir N., Kaya A., Cheretakis A., Kaya G. [dermatologicahelvetica.com]

  The hair sometimes falls out in the affected skin. If the face is involved, the facial features can change. Tumors: tumors can originate from plaques, red skin, or normal skin. They are usually reddish brown or purple. [encyclopedia.com]

  Shin et al26 recently identified 4 of 7 cases of FMF with a gene expression profile associated with a poor outcome similar to CTCL cases with aggressive tumor stage.26 In our study, the Kaplan-Meier curve in early FMF falls between the curves for conventional [jamanetwork.com]

• Visual Impairment

  impairment If the gastrointestinal (GI) tract is involved, then the following GI tract symptoms may be observed: Ulcers Diarrhea Inflammation GI bleeding Liver and spleen enlargement Joint inflammation and fluid buildup (edema) can occur, if the joints [dovemed.com]

• Alopecia

  Conclusions: Alopecia was observed in 5 (3.18%) patients with MF, which makes it a rare finding, which included alopecia areata-like patchy loss in 2 and alopecia within MF lesions in 3. [ncbi.nlm.nih.gov]

• Papule

  […] and patches Patches 48 Conventional MF Ichthyosiform MF PCALCL T 2 N 0 M 0 /IB PUVA, MTX, IFN-α 54 PR - 57 41 11 32/M Face Erythematous papules and ill-defined patches - 24 - - T 1 N 0 M 0 /IA PDT 2 CR - 48.5 47 12 46/F Face Erythematous papules and [synapse.koreamed.org]

  There was an overall 10-year survival rate of 71% in the early plaque-stage FMF group compared to a rate of 80% in the patches and follicular papules group. [healio.com]

  Grouped follicular papules and patch/plaque lesions with follicular prominence were the most frequent clinical findings. Folliculocentric lesions such as milia, cysts and acneiform lesions, alopecia, loss of hair or eyebrows were also seen. [ncbi.nlm.nih.gov]

  Interestingly, all 5 patients presented with widespread follicular papules and patches, and none of them presented with plaques. [jamanetwork.com]

• Skin Lesion

  A spectrum of skin lesions, both typical and atypical, such as leonine facies, acneiform lesions, psoriasiform plaques, purulent ulcerations and cystic milia-like lesions for mycosis fungoides, were seen. [ncbi.nlm.nih.gov]

  Overall, 174 patients (86%) presented with generalized skin lesions involving multiple body regions, while 9 (4%) and 20 (10%) patients had presented with solitary or localized skin lesions, respectively. [jamanetwork.com]

  It is usually initially seen by dermatologists with patients presenting with skin lesions. Patients often have a history of several years of eczematous or dermatitic skin lesions before the diagnosis is finally established. [radiopaedia.org]

  FMF typically presents with hair loss, comedones, and cystic skin lesions. Syringotropic MF is defined by the eccrine gland infiltration by atypical T cells. [semanticscholar.org]

• Pruritus

  Systematic review of 186 additional patients confirmed male predominance (ratio of men to women, 3.2:1.0), prevalent pruritus (73%), frequent follicular mucinosis (69%) and epidermotropism (37%) microscopically, and common head, neck, and trunk involvement [ncbi.nlm.nih.gov]

  Pruritus is often severe. Most cases present with mucinous degeneration of the hair follicles: these cases are referred to as MF-associated follicular mucinosis. Secondary bacterial infections are frequent. [orpha.net]

• Dermatitis

  T cell lymphomatoid contact dermatitis: a challenging case and review of the literature. Contact Dermatitis. 2014;72(2):65-74. 23. Whittaker SJ, Smith NP, Jones RR, Luzzatto L. [content.sciendo.com]

  […] mucinosis, the histologic features of follicular mycosis fungoides may include a granulomatous reaction, cystic and comedonal changes, an eosinophilic folliculitis pattern and basaloid folliculolymphoid hyperplasia as well as pustular changes, interface dermatitis [ncbi.nlm.nih.gov]

  Con infiltrado neutrofílico-esinofílico incluimos: psoriasis anular, vasculitis, dermatitis IgA lineal, dermatitis eosinofílica, eritema marginado reumático y eritemas anulares de la infancia. [healthdocbox.com]

  Irritant dermatitis, chronic radiation dermatitis TSEB For patients with Stage IB or IIA disease with progression or refractory disease Irritant dermatitis, chronic radiation dermatitis Second-Line Oral bexarotene Can be combined with multiple other modalities [dermatologyadvisor.com]

• Aggressive Behavior

  Sézary syndrome; a variant of mycosis fungoides with a leukaemic component and much more aggressive behavior. [e-immunohistochemistry.info]

  Conversely, an alternative explanation for the aggressive behavior of FMF may be related to the intrinsic gene expression of the tumor cells. [jamanetwork.com]

  Although a small percentage of patients may undergo spontaneous remission, a more aggressive behavior is more likely. Staging for systemic lymphoma and multiagent chemotherapy is recommended. [emedicine.medscape.com]

(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]

Type and results of initial treatment were retrieved from the Dutch Registry. [rug.nl]

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]

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]

However, recent studies described a subgroup of patients with FMF with a more favorable prognosis. [e-cancer.fr]

Etiology The etiology remains unknown. Diagnostic methods The diagnosis is based on clinical presentation and should be confirmed by a skin biopsy. [orpha.net]

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]

The etiology and pathogenesis of MF remain unclear; however, the various clinical presentations and the typical progression from patch to plaque to tumor stage have been described. [escholarship.org]

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]

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]

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]

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]

(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]

] 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]

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]