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Mycosis fungoides is a potentially fatal skin condition whose variable clinical appearance may mimic a variety of benign, inflammatory dermatoses, making it a diagnostic challenge. Three patients with mycosis fungoides, treated with multiple approved topical and systemic therapies, presented with acquired verrucous lesions on the extremities. The verrucous presentation of mycosis fungoides is one of the many atypical forms of the disease, with a paucity of documented cases in the medical literature. Although rare, verrucous and hyperkeratotic lesions in mycosis fungoides may increase the risk of considerable diagnostic delay if clinicians do not maintain a high degree of suspicion in cases of acquired verrucous lesions in the appropriate clinical setting., (© 2015 The International Society of Dermatology.)

Background: Lymphomatoid papulosis (LyP) is a CD30(+) lymphoproliferative disorder, with a self-regressing clinical course and malignant histopathology., Objective: The aim of this study was to evaluate characteristics, risk factors, associated malignancies, long-term outcome, and treatment of LyP in a large cohort representing the experience of the MD Anderson Cancer Center., Methods: Patient charts and clinical and histopathologic data of 180 patients with LyP were retrospectively assessed., Results: A total of 56.7% of patients was men. Histologic subtype A was found in 47.2%, type B in 17.2%, type C in 22.8%, type D in 7.8%, type E in 0.6%, and mixed subtype in 4.4% of the patients. One hundred fourteen lymphomas were observed in 93 patients, with mycosis fungoides (61.4%) and anaplastic large cell lymphoma (26.3%) being the most common forms. Risk factors for development of lymphoma included sex and histologic subtype. Number of lesions and symptom severity were not associated with lymphoma development. Patients with type D were less likely to have lymphomas. Treatment provided symptomatic relief but did not prevent progression to lymphoma., Limitations: The limitation of this study is the retrospective study design., Conclusion: Patients with LyP are at increased risk of associated lymphomas. Thorough patient counseling is needed and long follow-up periods are required to detect and treat secondary lymphomas., (Copyright © 2015 American Academy of Dermatology, Inc. All rights reserved.)

Background: Mycosis fungoides (MF) is the most frequent type of primary cutaneous natural killer and T-cell lymphoma. MF-required immunosuppressive therapies and MF-related immunosuppressive characteristics render patients with MF more prone to infections., Aim: To describe the clinical features of cutaneous infections observed in MF patients., Materials and Methods: A series of 56 MF patients were followed prospectively over 3 years and screened for cutaneous infections., Type of Study: Prospective observational study., Results: Four herpes simplex virus type-I (HSV-I), 2 staphylococcal (S. aureus) impetiginizations and 2 Malassezia infections were detected in single isolated plaque/patch stage MF as well as 1 varicella zoster virus infection (herpes zoster, HZ) and 2 cases of cellulitis in 10 patients. All patients presented advanced MF. All the diagnoses were delayed due to atypical clinical presentations., Conclusions: Patients with advanced MF should be particularly monitored for skin infections, especially by HSV and S. aureus. Unexplained exacerbation or the sudden appearance of oozing or ulcerations in MF lesions should initiate a search for viral or bacterial agents. Cellulitis and HZ can be severer and prolonged in MF patients., (© 2017 S. Karger AG, Basel.)

Background: Extracorporeal photopheresis (ECP) is recommended for the erythrodermic mycosis fungoides (MF) and Sezary syndrome (SS) alone or in combination with other therapies. The possibility of a differential response in the blood and skin has hardly been addressed in the literature., Objectives: To evaluate the clinical response rate of patients with erythrodermic MF and SS to ECP as part of a multimodality approach and to compare the kinetics of the blood and skin responses in the presence of leukaemic involvement., Methods: Twenty patients were treated with ECP and other modalities at a tertiary medical centre in 2003-2013. Ten patients had SS, 1 CD8-positive patch-stage MF with leukaemic involvement and nine erythrodermic MF. Clinical and outcome data were collected retrospectively from the medical files. Response was evaluated overall and for blood and skin separately., Results: Adjunctive therapies were interferon-α, narrow-band ultraviolet B, psoralen and ultraviolet A, isotretinoin, acitretin, methotrexate, prednisone, topical nitrogen mustard and total skin or localized hands/feet electron beam radiotherapy. Overall response was documented in 13 patients (65%)--complete 30%, partial 35%--and maintained for >2 years in 38.5%. In patients with leukaemic involvement (n = 11), the blood response occurred earlier than skin response (P = 0.008) and was maintained longer (P = 0.03). In three of the patients with a complete blood response, the skin response was partial (n = 2) or absent (n = 1)., Conclusion: Extracorporeal photopheresis as part of a multimodality approach yields a high durable clinical response in patients with erythrodremic MF and SS. The kinetics of the response differ between the blood and skin. The blood response occurs earlier and lasts longer; it does not necessarily predict the clinical skin response. Further studies are needed to determine if there is a survival advantage to a blood response in the absence of a skin response., (© 2015 European Academy of Dermatology and Venereology.)

Mycosis fungoides (MF) represents almost 50% of all primary cutaneous lymphomas and more than 70% of cutaneous T-cell lymphomas (CTCL). Arising from preferentially skin-homing lymphocytes with genetic instability, MF evolves through stages (IA-IVB), producing inconspicuous inflammatory features in the beginning and finally resulting in a proliferation of cytomorphologic, phenotypic, and genotypic abnormal tumor cells. Over the past 200 years, there has been much confusion in the classification of lymphomas due to semantic disagreements (MF, CTCL, parapsoriasis, lymphosarcoma, reticulum cell sarcoma, and many other terms), lack of diagnostic standard criteria, and new molecular diagnostic methods. Studies on extracutaneous involvement in early stages (IA-IIA) are almost completely lacking. In advanced stages of MF (IIB-IVB), discovery of extracutaneous involvement is dependent on the methods used (physical examination, technology, molecular diagnostics, autopsy, and laparoscopy) and reveals a wide range of results. Due to the inflammation-simulating features in the beginning of the disease, early diagnosis is very difficult to assess. Extracutaneous involvement has previously been documented in more than 70% of autopsies. More recent studies give much lower figures. Like all lymphomas, MF is a systemic disease from the very beginning, with distinct homing preferences in tumor cells. Organs most commonly involved during the lengthy course of the disease are, in descending frequency, lymph node/peripheral blood, liver, spleen, lung, bone marrow, GI tract, pancreas, and kidney., (Copyright © 2015. Published by Elsevier Inc.)

Diffuse large B-cell lymphoma (DLBCL) is the most common subtype of non-Hodgkin lymphoma with diverse clinical, pathological and genetic features. An 80-year-old woman was diagnosed with a stage IV-X-A (Ann Arbor staging system) low grade systemic follicular lymphoma (FL). Four months after the diagnosis, she developed asymptomatic, indurated, annular erythematous plaques with centrifugal growth on the abdomen, arms and neck. The skin biopsy revealed a dermal infiltration compatible with diffuse large B-cell lymphoma. Light chain restriction by flow cytometry was demonstrated. The variable, diverse and joining genes of immunoglobulin G heavy chains were sequenced and cloned, and showed the same pattern for both the initial follicular lymphoma and the skin infiltration. Translocation t (14;18) was present in both samples. Based on these findings, a diagnosis of transformation of follicular lymphoma into diffuse large B cell lymphoma was made. Although other hematological disorders such as primary cutaneous diffuse large B cell lymphoma, mycosis fungoides and the cutaneous infiltration of chronic juvenile myeloid leukemia can present as annular lesions, we were unable to find any previous reports of these as a manifestation of cutaneous infiltration by systemic non-Hodgkin lymphoma.

Acanthosis nigricans maligna (ANM) is a paraneoplastic phenomenon most commonly associated with visceral adenocarcinoma, in particular, gastric adenocarcinoma. Clinically, AMN is characterized by rapidly progressive symmetrical skin thickening and hyperpigmentation of the intertriginous areas with peripheral acrochorda. The diagnosis is made by a detailed medical work-up for occult malignancies with particular emphasis on endocrinological diseases. We report a 67-year-old man that presented clinically with acanthosis nigricans, in which a subsequent diagnosis of mycosis fungoides was made.

Lymphomatoid papulosis (LyP) lies within the spectrum of primary cutaneous CD30-positive lymphoproliferative disorders. Approximately 10% to 15% of patients with LyP develop other lymphomas, most commonly mycosis fungoides (MF), suggesting a biological relationship between these distinctive diseases. Here, we describe the clinical and histopathologic features of 11 patients who had both LyP and MF, including a total of 30 biopsy specimens (14 LyP and 16 MF). Clinically, LyP lesions were characterized by clustered papules undergoing spontaneous regression and were classified as type A (n = 11), type C (n = 2), or type D (n = 1). All cases of MF were characterized clinically by patch/plaque disease, were stage I or II at the time of diagnosis, and consisted of a CD4-predominant epidermotropic T-cell infiltrate. We used polymerase chain reaction-based methods to assess the TCR-β chain (TCRB) and TCR-γ chain (TCRG) in both LyP and MF lesions of all patients. Monoclonal TCR gene rearrangements were detected in 13 LyP lesions from 10 of 11 patients and in 14 MF lesions from 10 of 11 patients. All 10 patients in whom their skin lesions carried monoclonal TCR gene rearrangements exhibited overlapping clones in both their LyP and MF lesions; additional non-overlapping clones were identified in 3 LyP lesions from 2 patients and 1 MF lesion from another patient. The demonstration of shared monoclonal T-cell receptor gene rearrangements in LyP and MF lesions in almost all patients suggests a common origin between these distinctive clinicopathological diseases., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Mycosis fungoides (MF) and Sézary syndrome (SS) belong to the group of primary cutaneous T-cell lymphomas (CTCL). Regardless of the stage of the disease, patients with MF and SS can suffer from chronic pruritus. The aim of the study was to investigate the correlation between the interleukin 31 (IL-31) serum level, the degree of pruritus and CTCL severity; and to compare the frequency of IL-31 gene polymorphisms between patients and the control group, and between patients at different stages of the disease. Pruritus affected 67.7% of patients with MF and SS in our study. The IL-31 serum level was significantly higher in CTCL patients than in the control group but there were no positive correlation between IL-31 serum level and pruritus. A statistically significant difference in allele frequencies for IL-31 IVS2+12 gene polymorphisms between early and advanced stages was detected; GAG haplotype was more frequent and AGA was less frequent in stage IA patients compared with patients in the other stages of the disease.

Mycosis fungoides is the most common subtype of primary cutaneous lymphoma and has several clinical variants. We report a 74-year-old man presenting with an acquired palmoplantar keratoderma initially diagnosed and treated as psoriasis with suboptimal improvement. Several months later the patient developed patches and plaques that were histologically consistent with mycosis fungoides. These lesions were ameliorated with the treatment of the underlying mycosis fungoides and the palmoplantar keratoderma resolved promptly with radiotherapy. This case highlights the importance of considering mycosis fungoides as an infrequent but serious cause of acquired palmoplantar keratoderma., (© 2014 The Australasian College of Dermatologists.)

In this chapter, we will discuss the most common alopecias due to drugs and other skin and systemic disorders. The following hair disorders will be analyzed: telogen effluvium (acute and chronic); anagen effluvium; folliculotropic mycosis fungoides; and folliculitis due to bacteria, fungi, parasites, human immunodeficiency virus disease, lupus erythematosus, and sarcoidosis. We will cover topics including the epidemiology, etiology, clinical picture, and diagnosis of and current treatments for each disease., (© 2015 S. Karger AG, Basel.)

Background: Mycosis fungoides, the most common subtype of cutaneous T-cell lymphoma, is more common in patients aged 45-55., Objective: Cardiovascular risk factors have been investigated in several skin diseases. However, the relation between cardiovascular diseases and mycosis fungoides remains unclear. Therefore, the aim of this study was to assess cardiovascular risk factors in patients with mycosis fungoides., Methods: 32 patients with mycosis fungoides and 26 healthy controls were enrolled in the study. Glucose, total cholesterol, high-density lipoprotein cholesterol, triglyceride, homocysteine, high sensitivity C-reactive protein, low-density lipoprotein-cholesterol, were measured in the sera of patients., Results: Patients had significantly higher high-sensitivity C-reactive protein, homocysteine, low-density lipoprotein-cholesterol, total cholesterol (p= 0.032) (p< 0.001) (p= 0.001) (p< 0.001). There was a positive correlation between the levels of homo-cysteine and total cholesterol (p= 0.001, r = +0.431). Additionally, a significantly positive correlation was found between the levels of high-sensitivity C-reactive protein and low-density lipoprotein-cholesterol (p= 0.014, r = +0.320) in patient group., Conclusions: Patients with mycosis fungoides had significantly higher levels of total-cholesterol, low-density lipoprotein-cholesterol, homocysteine and high-sensitivity C-reactive protein than healthy subjects. The present study has demonstrated an increased rate of cardiovascular risk in patients with mycosis fungoides. Even though the etiology of these associations is elusive, dermatologists should be sensitized to investigate metabolic derangements in patients with mycosis fungoides, in order to lessen mortality and comorbidity with a multidisciplinary approach.

Background: Few reports have described vitiligo developing in patients with cutaneous T-cell lymphoma (CTCL)., Objective: We sought to identify possible factors that might predispose patients with CTCL to vitiligo., Methods: Patient demographics, CTCL disease characteristics and treatments were analyzed in 25 patients with CTCL who developed vitiligo. Cox proportional hazards modeling was used to identify associations of risk factors with the development of vitiligo., Results: Younger age, later CTCL disease stage (stages IIB-IV) and presence of a CD8+CD4- mycosis fungoides phenotype were associated with the development of vitiligo. After adjusting for disease stage, increased risk of vitiligo was associated with methotrexate and CD4 antibody therapies (although the total number of patients with these was small), while decreased risk was associated with nitrogen mustard and PUVA therapies., Conclusions: No single feature was common to all of our patients, suggesting that multiple factors may contribute to the development of vitiligo in a patient-specific fashion., (© 2015 S. Karger AG, Basel.)

Mycosis fungoides (MF) is the most common type of cutaneous lymphoma and accounts for approximately 50% of all lymphomas arising primarily in the skin. The three types of MF lesions are patches, plaques, and tumors, according to which the disease is traditionally divided into three clinical stages. The clinical course can be protracted and take years or decades. In the final stage, MF evolves to a systemic form of the disease. Nodular prurigo (NP) is still a condition of unknown etiology characterized by papulonodular eruption and intense pruritus. Multiple diseases, including dermatological, systemic, and psychiatric diseases, have been assumed to cause NP. Pruritic skin lesions have been known to precede clinically evident B and T cell lymphomas for years. In the literature, pruritus and NP have been reported in patients affected by systemic Hodgkin and non-Hodgkin lymphomas. Only two cases of cutaneous lymphoma as underlying disease in patients with PN have been reported in the literature. We report a rare case of a patient with concomitant non-Hodgkin skin lymphoma - MF and NP. Our female patient with a 10-year history of MF stage IIb during the last three years had been presenting for regular check-up with itchy, newly formed, rarely disseminated nodules 5-8 mm in diameter on the forearms and lower legs. Sharply limited erythematosquamous, slightly infiltrated foci (as part of MF as the underlying disease) were visible on the trunk and extremities. Extracutaneous involvement of MF was excluded. We performed a biopsy on a nodule from the lower leg to rule out tumor stage MF; the biopsy confirmed NP. We conclude that prurigo nodules should not be confused with tumor stage MF. NP is a therapeutic challenge for any dermatologist. Any underlying diseases should be treated first.

Background: Mycosis fungoides (MF) and pseudo-MF (or MF simulant) can be associated with B-cell malignancies, but distinction between a true neoplasm and a reactive process may be difficult., Objectives: To report seven patients with B-cell malignancy and folliculotropic MF or pseudo-MF and emphasize on criteria allowing distinction between the two conditions., Methods: We retrospectively and prospectively included seven patients with B-cell malignancy who presented skin lesions histologically consisting in a folliculotropic T-cell infiltrate and reviewed the literature on the topic., Results: Four men and three women had a chronic lymphocytic leukaemia (n = 6) or a MALT-type lymphoma (n = 1). Five patients had localized papules, and two had patches and plaques. Histological examination showed in all cases a diffuse dermal T-cell infiltrate with folliculotropic involvement and follicular mucinosis associated with clusters of the B-cell lymphoma, without significant expression of follicular helper T-cell markers. T-cell rearrangement studies showed a polyclonal pattern in the patients with papules and a monoclonal pattern in the cases of patches and plaques. Papular lesions had an indolent evolution, whereas patches and plaques persisted or worsened into transformed MF., Conclusion: Folliculotropic T-cell infiltrates associated with B-cell malignancies can be either a true folliculotropic MF or a pseudo-MF. The distinction between both conditions cannot rely only on the histopathological aspect, but needs both a clinical pathological correlation and the search for a dominant T-cell clone. Whether the neoplastic T and B cells derive from a common ancestor or the T-cell proliferation is promoted by the underlying B-cell lymphoma remains unsolved, but interaction between B and T cell in the skin does not appear to be dependent on a TFH differentiation of the T-cell infiltrate., (© 2014 European Academy of Dermatology and Venereology.)

We report the case of a healthy 17-year-old adolescent boy with an unremarkable medical history who presented with an asymptomatic fixed rash on the abdomen, buttocks, and legs. The rash initially developed in a small area on the right leg 2 years prior and had progressed slowly. Prior biopsies were consistent with pigmented purpura. Clinical examination revealed multiple annular purpuric patches on the abdomen, buttocks, and legs covering approximately 20% of the body surface area without lymphadenopathy or hepatosplenomegaly. Additional biopsies demonstrated changes consistent with mycosis fungoides (MF). T-cell receptor g gene rearrangements demonstrated clonality. The patient was diagnosed with stage IB MF of the pigmented purpura-like variant. The patient responded well to psoralen plus UVA therapy. It has been proposed that pigmented purpuric dermatosis (PPD) is a form of cutaneous T-cell lymphoid dyscrasia and that T-cell gene rearrangement studies should be obtained for prognostic evaluation in patients with widespread disease. In our patient, the clinical appearance of the lesions, pathologic findings, and gene rearrangement studies led to the diagnosis of MF. Until the potential for evolution of PPD to malignant disease is better understood, further evaluation of MF in patients with an unusual presentation of pigmented purpura is warranted.

Background: The purpose of this study was to determine the prevalence of vitamin D deficiency in CTCL patients and whether supplementation corrects vitamin D deficiency or treatment outcome., Patients and Methods: Three hundred eleven CTCL patients including 27/311 (8.7%) with Sézary syndrome (SS), 169 cancer controls, and 69 normal controls from the M.D. Anderson clinics had 25(OH)D3 levels determined and categorized as deficient (< 20 ng/mL),insufficient (20-29 ng/mL), or sufficient (≥ 30 ng/mL). Clinical response was determined according to a change in percent body surface area involvement., Results: Low 25(OH)D3 (< 30 ng/mL) levels were present in 76.9% of mycosis fungoides/SS patients, 75.2% of cancer controls, and 66.7% of healthy controls (P ¼ .05, .07) and in 30% to 39% of historical normal controls. Correction of deficiency was successful in 35% or 55 of 156 patients who were given dealer's choice of either vitamin D2 at 50,000 IU orally (p.o.) biweekly or D3 1000 IU p.o. daily. Correction of vitamin D levels was noted in 27 of 100 (27%) patients given D3 and 28 of 56 (50%) given D2. Responses to standard CTCL therapy was similar among patients with corrected and persistently low levels (P ¼ .51)., Conclusion: To our knowledge,this is the first study of vitamin D status in CTCL patients. Vitamin D deficiency was present in CTCL and other cancer patients compared with normal and historical controls. Correction of vitamin D deficiency and type of vitamin D supplementation used did not affect the overall clinical disease response.

Background: A rare variant of mycosis fungoides (MF), syringotropic MF (STMF) is characterized by a particular tropism of the lymphocytic infiltrate for the eccrine structures, and included in the follicular subtype of MF in the World Health Organization-European Organization for Research and Treatment of Cancer classification of cutaneous lymphomas., Objective: We sought to determine the clinicopathologic features and disease course of patients with STMF., Methods: A retrospective study was conducted to identify patients with STMF from 1998 to 2013., Results: Nineteen patients were included: 15 men and 4 women, mean age 55 years (range, 24-86). Most had multiple lesions (n=16, 84%) with associated alopecia (n=12, 63%) and/or punctuated aspect (n=12, 63%). Palms or soles were involved in 10 cases (53%). Folliculotropism was found in 13 cases (68%). After a median follow-up of 70 months (range, 2-140), 3 patients died, 1 from disease-related death. The 5-year overall and disease-specific survival were 100%. The disease-specific survival was significantly higher than in 54 patients with folliculotropic MF without syringotropism (5-year disease-specific survival, 74%; 95% confidence interval, 58%-94%, P=.02)., Limitations: Retrospective setting is a limitation., Conclusions: In the spectrum of adnexotropic MF, STMF appears as a distinct entity from follicular MF, with peculiar clinical characteristics and natural history., (Copyright © 2014 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.)

Background: Little is known about the immunological milieu of the skin-draining lymph nodes (LNs) in mycosis fungoides (MF)., Objectives: We studied dendritic cell (DC) subsets in the dermatopathic lymphadenitis of MF patients., Methods: We immunohistochemically examined DC subsets and their distribution in 16 LN samples from 14 patients with MF (N1 LN, eight patients; N2, four; and N3, four), and we compared them with non-metastatic sentinel LNs from eight patients with melanoma., Results: The number of S-100 protein+ DCs was markedly increased in the LNs from the MF patients and the major component was DC-LAMP+ mature DCs in the outer and paracortex areas, where DC-SIGN+ immature DCs were relatively decreased in proportion. In contrast, DC-SIGN+ cells were relatively increased in proportion compared to DC-LAMP+ cells in the medulla. Although no significant difference was observed in the proportions of CD1a+ or Langerin+ DCs among the N1, N2, and N3 nodes, CD163+ M2-type macrophages were increased in number in the N2 and N3 nodes., Conclusions: Our observations indicate that mature DCs accumulate in the outer and paracortex areas in dermatopathic lymphadenitis and M2-type macrophages might increase in number during disease progression.

Background: Interferon (IFN) is a treatment option for both mycosis fungoides (MF) and hepatitis C virus (HCV) infection. Chemotherapy and anti-HCV treatment are generally not administered concurrently for fear of overlapping side effects., Objective: Herein, we report on a subset of patients who received IFN-containing therapy for MF and HCV infection simultaneously. We aimed to evaluate whether concomitant treatment for MF and HCV is effective and well tolerated., Methods: We reviewed the records of patients who were seen at MD Anderson Cancer Center from 2008 to 2013 with histologically confirmed MF and chronic HCV infection, and were treated with simultaneous focus on both diseases., Results: Six HCV-infected patients with MF received simultaneous anti-HCV and anti-MF treatment with IFN-containing therapy. Two patients achieved sustained virological response (regarded as virological cure). They both received antiviral combination therapy with ribavirin. All patients experienced some improvement of their cutaneous lesions, with two of them achieving complete MF remission. All six patients developed side effects while receiving treatment; two of them had grade 4 toxic effects requiring treatment discontinuation., Conclusion: IFN-based therapy can be administered for MF and HCV infection concurrently to provide not only virological but also oncological benefits to chronically HCV-infected MF patients. However, this regimen is poorly tolerated. Further studies are warranted in this patient population, using different treatment combinations with improved efficacy, safety, and tolerability.

Pruritus is a symptom that significantly affects the patient's quality of life in cutaneous T cell lymphoma (CTCL). The most effective treatments are those that address the condition itself; however, it is often not possible to control this symptom. Lymphoma-related pruritus normally becomes more severe as CTCL progresses, constituting an important factor for quality of life in these patients. Substance P is a neuromodulator which appears to play a key role in pruritus. Aprepitant is a neurokinin-1 receptor antagonist affecting the substance P receptor. So far, several cases have been documented with an antipruritic response to the drug aprepitant in advanced-stage mycosis fungoides (MF). In this paper, we describe an excellent response to aprepitant in a female patient with severe pruritus secondary to hypopigmented stage I MF. We would also like to stress the absence of nausea and vomiting of this combined therapy of interferon and aprepitant. Aprepitant could improve tolerance to interferon., (© 2013 Wiley Periodicals, Inc.)

We report the case of a patient from Brazil with a bloodstream infection caused by a strain of methicillin-resistant Staphylococcus aureus (MRSA) that was susceptible to vancomycin (designated BR-VSSA) but that acquired the vanA gene cluster during antibiotic therapy and became resistant to vancomycin (designated BR-VRSA). Both strains belong to the sequence type (ST) 8 community-associated genetic lineage that carries the staphylococcal chromosomal cassette mec (SCCmec) type IVa and the S. aureus protein A gene (spa) type t292 and are phylogenetically related to MRSA lineage USA300. A conjugative plasmid of 55,706 bp (pBRZ01) carrying the vanA cluster was identified and readily transferred to other staphylococci. The pBRZ01 plasmid harbors DNA sequences that are typical of the plasmid-associated replication genes rep24 or rep21 described in community-associated MRSA strains from Australia (pWBG745). The presence and dissemination of community-associated MRSA containing vanA could become a serious public health concern.

Nodular regenerative hyperplasia of the liver -a type of noncirrhotic portal hypertension- is a rare condition of unknown etiopathogenesis that has been associated with multiple disorders, including diverse types of hematologic disease. We report the case of a 36-year-old female patient diagnosed with cutaneous T-cell lymphoma of the mycosis fungoides variety, staged as T2N0M0B0, where a transjugular liver biopsy demonstrated the presence of nodular regenerative hyperplasia with a hepatic venous pressure gradient of 15 mm Hg. The study was triggered by the incidental radiologic finding of hepatomegaly with indirect evidence of portal hypertension. We are not aware of any previous reports on the association of nodular regenerative hyperplasia with mycosis fungoides in the medical literature.

We describe a 34-year-old Japanese man with syringotropic CD8+ mycosis fungoides (MF) accompanied by hypohidrosis who was treated with vorinostat and retinoids. Interestingly, immunohistochemical staining for dermcidin revealed a decrease of sweat in the eccrine glands, and a sweat test by the iodine starch method proved hypohidrosis in the MF-affected areas. Six months after treatment with this combination therapy, the patient's advanced MF was under control., (© 2012 The Authors Australasian Journal of Dermatology © 2012 The Australasian College of Dermatologists.)

Mycosis fungoides usually follows an indolent clinical course. We report here a rapidly progressive case of mycosis fungoides with peculiar clinical and histological features, presenting as a haematoma-like mass on the thigh accompanied by multiple reddish brown erythematous lesions on the trunk and extremities. Histopathologically, the erythematous lesions showed epidermotropism of atypical T lymphocytes expressing CD4 and prominent syringotropism without syringometaplasia. The haematoma-like lesion consisted of diffuse and dense infiltrates of medium-to-large-sized pleomorphic lymphocytic cells expressing CD30, suggesting that CD30+ large-cell transformation had occurred. Mycosis fungoides presenting as a haematoma-like lesion is rare and may be a poor prognostic sign.

Follicular mucinosis (FM) is an uncommon reaction pattern in which the accumulation of mucin in the follicular epithelium is the main pathologic finding. FM may be idiopathic (primary follicular mucinosis [PFM]), in association with mycosis fungoides or cutaneous T-cell lymphoma, or in association with other neoplastic and inflammatory conditions. Herein we report a case of PFM with identical T-cell clone rearrangement at anatomically distinct sites, supporting the idea that some authors have proposed, that FM may represent a low-grade lymphoproliferative disease related to mycoses fungoides with favorable prognosis., (© 2013 Wiley Periodicals, Inc.)

Background: Large-cell transformation in mycosis fungoides [MF] is associated with an aggressive clinical course and poor survival. In addition to tumour progression, infectious complications may be the cause of death. We report a case of aseptic pustular dermatosis in transformed MF that very quickly became aggressive and was followed by rapid death of the patient., Patients and Methods: A 47-year-old man presented with disseminated CD30+ transformed MF skin tumours refractory to chemotherapy for nine consecutive months. Ten days after a first course of dexamethasone, cytarabine and carboplatin, he was hospitalized for febrile neutropenia. Following this episode of neutropenia, he developed a febrile pustular rash disseminated on the tumours and on plaques, associated with marked neutrophilic leucocytosis. Bacteriological and mycological skin and blood samples were negative. Tumour biopsies showed a dermal infiltrate of CD30+ T-cells corresponding to the transformed MF, mixed with neutrophils. The pustule biopsies revealed intra-epidermal pustules and dermal-epidermal infiltration of neutrophils. The pustular eruption regressed with corticosteroids. Despite a last line of treatment with romidepsin, the patient died 6 weeks after the "neutrophilic" event., Discussion: Episodes of neutrophilic dermatosis [ND] are frequently seen in haematological disease and some substances can induce a variant form of eccrine hidradenitis. However, our patient's presentation was different, without the appearance of "classic" ND but rather diffuse pustular lesions carrying a poor prognosis, which was followed by rapid death. Once the hypothesis of infectious complications has been ruled out, treatment is based on systemic corticosteroids associated with specific treatment of the lymphoma., (Copyright © 2013 Elsevier Masson SAS. All rights reserved.)

Vesiculobullous eruptions in mycosis fungoides (MF) are extremely rare. Here, we report a case of a 62-year-old woman presenting with erythematous patches and plaques of 2 years in duration, who had recently developed vesicles on erythematous MF plaques. Histopathological examination showed intra-subepidermal blisters, and infiltration of the epidermis by atypical lymphoid cells, forming Pautrier's microabscesses. Negative immunofluorescence excluded autoimmune blistering diseases. Immunohistochemistry revealed a CD4⁺ T-cell phenotype and gene rearrangement study confirmed a clonal T-cell proliferation. Kaposi's varicelliform eruption (KVE) developed in the patient 1 week after initiation of systemic corticosteroids and immunotherapy. Cluster of vesicles and erosions arising on the pre-existing plaque and a positive immunofluorescence test for Herpes simplex virus and histopathological examination confirmed the diagnosis of cutaneous herpes infection. This is the first case report on bullous MF complicated by KVE in the published work., (© 2013 Japanese Dermatological Association.)

Background: Granulomatous mycosis fungoides (GMF) is an uncommon variant of mycosis fungoides (MF)., Objective: We sought to analyze the relative frequency, clinicopathologic characteristics, prognosis, and therapeutic responses of GMF., Methods: We conducted a retrospective case-control study of patients with GMF and age- and stage-matched patients with classic MF between 1981 and 2012., Results: A total of 27 patients with GMF were identified, representing 6.3% of all patients with MF at our center. Skin manifestations were similar to classic MF having an atypical lichenoid CD4(+) CD8(-) lymphocytic infiltrate with interstitial histiocytes and/or perivascular granulomas with giant cells. Fewer patients with GMF achieved a partial response or complete response with topical (57% vs 83%; P = .002) or ultraviolet light (62% vs 90%; P = .006) therapy. The 5- and 10-year progression-free survival rates were significantly lower in patients with GMF (59% and 33%) compared with patients with classic MF (84% and 56%; P = .02), but overall survival was similar between groups (86% and 72% vs 85% and 85%; P = .54)., Limitations: The retrospective methodology may underestimate the frequency of GMF. The median follow-up time may be too short to detect possible differences in overall survival., Conclusion: More frequent disease progression and poorer response to skin-directed therapies were observed in patients with GMF. Our findings may be helpful in selecting the most appropriate treatment for these patients., (Copyright © 2013 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.)

A 53-year-old Japanese male was diagnosed with atopic dermatitis and initiated treatment with a local dermatologist in February 2001. A routine medical check-up revealed proteinuria, microscopic hematuria and slight elevation of serum creatinine level in spring 2006. He was referred to our hospital for nephrology consultation and followup.In December 2009, he developed a sudden high fever of greater than 39 °C with sore throat. In addition, his serum creatinine level greatly increased to 4.6 mg/dl. His prevalent renal illness was thought to be rapidly progressive glomerulonephritis (RPGN). Soon after admission, kidney and skin biopsies were performed. The kidney specimens showed that the glomeruli had proliferative mesangial cells strongly positive for anti-IgA antibody, 75% of which manifested fibrocellular crescentic formation surrounding the Bowman's capsules. His skin disease was pathologically proven to be mycosis fungoides(MF). It is likely that his kidney disease was exacerbated by the upper respiratory tract infection and converted to RPGN. Ash is kidney function rapidly declined, hemodialysis therapy was initiated and he remains on chronic dialysis therapy. This is a serious case of IgA nephropathy associated with MF,which developed into RPGN and subsequent end-stage renal disease.

The etiology of status asthmaticus (SA), a complication of severe asthma, is unknown. Fungal exposure, as measured by fungal atopy, is a major risk factor for developing asthma, but the relationship of fungi in SA per se has not previously been reported. In this five patient retrospective case series study, lower respiratory tract cultures were performed on bronchoalveolar lavage or tracheal aspirate fluid, comparing standard clinical laboratory cultures with a specialized technique in which respiratory mucus was removed prior to culture. We show that mucolytic treatment allows an increased detection of fungal growth, especially yeast, from the lower airways of all SA patients. We also demonstrate that inhalation of the yeast Candida albicans readily induces asthma-like disease in mice. Our observations suggest that SA may represent a fungal infectious process, and support additional prospective studies utilizing anti-fungal therapy to supplement conventional therapy, broad-spectrum antibiotics and high-dose glucocorticoids, which can promote fungal overgrowth., (Copyright © 2012 Elsevier Inc. All rights reserved.)