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Biomedical subjects

H Trau

Publications and source records attributed to H Trau.

At least 19 recordsLinked to original sources
PubMed2006

Mycosis fungoides associated with B-cell malignancies.

BACKGROUND: The coexistence of mycosis fungoides, a peripheral T-cell lymphoma, and B-cell malignancies or Hodgkin's lymphoma in the same patient is unusual. Most descriptions are isolated case reports and case series are strikingly sparse. OBJECTIVES: To detect cases of mycosis fungoides associated with B-cell malignancies or Hodgkin's lymphoma and to analyse the characteristics of and the interplay between the lymphoproliferative neoplasms. METHODS: Patients with mycosis fungoides who had B-cell malignancies or Hodgkin's lymphoma were selected from among 398 patients either treated or followed up in two tertiary medical centres during a 7-year period. RESULTS: Eleven patients with mycosis fungoides and B-cell malignancy were detected (seven of non-Hodgkin's lymphoma, three of chronic lymphocytic leukaemia, one of multiple myeloma). No case of Hodgkin's lymphoma was found. In seven patients the mycosis fungoides preceded the B-cell malignancy whereas in four it was the B-cell malignancy which occurred first. The time elapsed between onset of the two malignancies ranged from 4 to 22 years (average: 12 years). Patients who had mycosis fungoides as the first neoplasm presented with earlier stages of mycosis fungoides (four of seven: IA, three of seven: IB) than those who had mycosis fungoides as their second neoplasm (of four, one: IB, one: folliculotropic, two: IIB). Among the four patients in whom the appearance of mycosis fungoides followed the B-cell malignancy, three had been treated with multiagent chemotherapy. Two patients who presented with early-stage mycosis fungoides (IA) as the first lymphoma developed mycosis fungoides tumours after becoming immunosuppressed. In two patients infiltrates composed of both malignant T- and B-cell populations were found in a single biopsy. One showed two distinct populations of the malignant cells in the skin tumour, thus constituting a classical composite lymphoma of mycosis fungoides and chronic lymphocytic leukaemia, while in the other patient the two malignant populations of marginal B-cell lymphoma and mycosis fungoides (as evidenced by both phenotypic and genotypic findings) were intermingled. CONCLUSIONS: This case series indicates that while the coexistence of Hodgkin's lymphoma and mycosis fungoides is extremely rare, the association of mycosis fungoides and B-cell malignancies is not as rare as reflected in the literature, with non-Hodgkin's lymphoma constituting the most common associated B-cell malignancy. In this series as well as in the cases reported in the literature mycosis fungoides usually preceded the development of B-cell malignancies, which may be in accordance with previous reports of an increased risk of developing a second haematological neoplasm. The importance of a competent immune system for patients with mycosis fungoides is well demonstrated in these cases. It is suggested that for greater precision the criteria for diagnosis of composite lymphoma of the skin should include both phenotypic and genotypic features.

Adult↗
PubMed2006

UVB therapy of pityriasis lichenoides--our experience with 29 patients.

BACKGROUND: Pityriasis lichenoides comprises a clinical and pathological spectrum of disorders. So far no highly effective treatment has been reported. Previous small studies have suggested that ultraviolet B (UVB) is a good alternative. METHODS: This is a retrospective analysis of 29 pityriasis lichenoides patients treated in our institution with broad- or narrow-band UVB during the period 1996-2002. Twenty-one of these patients had one or more previous unsuccessful treatments. RESULTS: Complete response was achieved in 93.1% in both treatment groups, with 73% of them still relapse free after a mean follow-up of 58 and 38 months in broad- and narrow-band UVB treatment groups, respectively. Mild side-effects were observed in about one-third of the patients. CONCLUSION: We believe both forms of UVB are a good option for pityriasis lichenoides and should be considered as the first line in generalized cases interested in treatment.

Adult↗
PubMed2006

The role of IVIg treatment in severe pemphigus vulgaris.

BACKGROUND: High-dose intravenous immunoglobulin (IVIg) has become a part of the treatment armentarium in pemphigus vulgaris (PV). Some consider IVIg as an adjuvant steroid sparing agent in PV, while others as disease modifying that can be used as monotherapy. METHODS: We report our experience with a series of 12 PV patients with severe disease treated with IVIg as an adjuvant therapy. RESULTS: Ten of 12 patients (83%) showed response to six cycles of IVIg, six (50%) having complete remission and four (33%) having a partial response. This response rate is concordant with previous reports. The therapy was well tolerated. In all 12 patients, treatment with IVIg allowed a gradual reduction of prednisone dose compared with baseline levels. CONCLUSION: IVIg treatment was beneficial as a steroid sparing agent in our series of patients with severe PV.

Adult↗
PubMed2006

UVB in the management of early stage mycosis fungoides.

BACKGROUND: Several options for treatment of early mycosis fungoides (MF) offer similar success rates. Previous small studies have shown UVB to be at least as effective as PUVA. OBJECTIVE: To summarize our experience with UVB treatment of early MF. METHODS: A retrospective analysis of early-stage MF patients treated by narrow band (NB) or broad band (BB) UVB in our institution between 1996 and 2002. Most patients achieving complete response (CR) were put on maintenance until natural sun exposure was possible and followed up every 3-6 months. The results were compared to those previously reported regarding PUVA. RESULTS: Sixty-eight and 43 patients were treated by NB and BB UVB, respectively. Eighty-six per cent (84 and 89% in NB and BB UVB groups, respectively) of IA and 71% (78 and 44% in NB and BB UVB groups, respectively) of IB patients achieved CR within a mean of 12.8 and 10.6 weeks, respectively. When maintenance was stopped, 65 and 30% had not relapsed after an average follow up of 27 and 222 weeks, respectively. Non-relapse rate was 33 and 48% for those having had vs. those not having had maintenance, respectively. CONCLUSIONS: Our results are comparable to all previously reported for skin-targeted treatments, including PUVA and, to our belief, reflect the nature of early MF, in which CR can probably be achieved in most of the patients. Among the responding patients there is no relapse during prolonged follow-up in about one third of the cases. Thus, we believe treatment should be stopped completely following first CR induction and maintenance treatment should be considered for relapsing patients only. Both broad and narrow UVB options are good and future choices should be made on the basis of short- and long-term side-effects.

Adolescent↗
PubMed2004

Pulsed versus continuous terbinafine dosing in the treatment of dermatophyte onychomycosis.

BACKGROUND: The accepted regimen for terbinafine, one of the most effective treatments for dermatophyte onychomycosis, is continuous administration of 250 mg/day over 16 weeks. A few small studies, however, have raised the possibility of an alternative regimen: pulsed administration of 500 mg/day for 1 week, every 4 weeks (over 16 weeks), without decreasing treatment efficacy. OBJECTIVE: Our aim was to compare the efficacy and safety of both regimens in a large group of patients. METHODS: Retrospective analysis of 260 patients with culture proven dermatophyte onychomycosis treated in seven outpatient clinics run by two dermatologists using one of the terbinafine protocols on a chronological basis: 105 patients were treated using the continuous regimen during 1998/1999 and 155 patients were treated using the pulsed regimen during 1999/2002. Mycological and clinical cure were assessed 2 and 3 months, respectively, after completion of the last therapeutic course. Side effects were documented for the pulse regimen group only and compared with historical data previously published for the continuous protocol. RESULTS: The mycological, clinical and complete (mycological and clinical) cure rates of the toenails were 72.1%, 53.5% and 47.1% in the pulse regimen versus 82%, 35% and 34% in the continuous regimen, respectively (p=0.091, 0.0002 and 0.047, respectively). The mycological, clinical and complete cure rates of the fingernails were 91.7%, 83.3% and 79.2% respectively in the pulse group versus 100% (all parameters) in the continuous group (no significant difference). In general, both regimens were well tolerated and few side effects were reported. CONCLUSION: The pulsed regimen is at least as effective as continuous dosing and thus, at 50% less cost and more convenience, is preferable to a continuous regimen.

Adolescent↗
PubMed2004

Cutaneous manifestations in the cardiofaciocutaneous syndrome, a variant of the classical Noonan syndrome. Report of a case and review of the literature.

We report one patient with cardiofaciocutaneous (CFC) syndrome. He presented with clinical findings characteristic of this condition such as: cutaneous abnormalities, including ichthyosis, widespread keratosis pilaris, a peculiar craniofacial appearance with sparse, curly hair and low-set posteriorly rotated ears; congenital heart defects; and mild mental and motor retardation. We submit a comprehensive review of previously published articles regarding the dermatological findings in CFC syndrome (recently shown to be a variant of Noonan syndrome) emphasizing diagnostic criteria and its differentiation from the Costello syndrome.

Abnormalities, Multiple↗
PubMed2003

Imported mucosal leishmaniasis in a traveler.

We report a case of mucosal leishmaniasis in a traveler returning from South America. The traveler developed nasal symptoms 2 months after the appearance of cutaneous ulcers. Diagnosis of mucosal Leishmania viannia braziliensis infection was made 5 years later. The clinical presentation and diagnosis for the patient are reported, and previous cases in travelers are reviewed. We recommend that mucosal examination should be part of follow-up for L. viannia braziliensis infection in travelers. Mucosal leishmaniasis should be part of the differential diagnosis of mucosal lesions in patients with a history of travel to South America, however remote the likelihood of infection.

Adult↗
PubMed2002

The Koebner phenomenon: review of the literature.

First described in 1877 as the appearance of psoriatic lesions in the uninvolved skin of psoriatic patients as a consequence of trauma, the Koebner phenomenon has since been described in numerous diseases. Other authors have tried to implicate either infections or parasitic causes as the pathogenesis of this phenomenon. Subsequent research by many authors have contributed to our poor understanding of this reaction in the hope of understanding the pathogensis of psoriasis. We present a review of the literature covering the following topics as they relate to the Koebner phenomenon: diseases that koebnerize and their possible causes, predisposing and provoking factors, type, site, depth and degree of trauma, the all or none phenomenon, time lag, site preference, medications, inhibition of koebnerization and reverse koebnerization.

Humans↗
PubMed2001

Wolf's isotopic response: a case of zosteriform lichen planus on the site of healed herpes zoster.

Based on histological findings, the patient had lichen planus. The clinical picture suggests Wolf's isotopic response on the site of healed herpes zoster. These conditions taken into account lead to the diagnosis of zosteriform lichen planus. Lichenoid papules in a dermatomal arrangement are extremely rare. The clinical, dermoscopic and histological features of this case are described.

Adult↗
PubMed2001

The hypereosinophilic syndrome associated with CD4+CD3- helper type 2 (Th2) lymphocytes.

We describe herein the clinical and laboratory manifestations of a unique group of patients (pts) presenting with hypereosinophilic syndrome (HES) who were treated in our medical centers for 4-13 years. Skin biopsies, flow cytometry of peripheral blood mononuclear cells (PBMC), assays for cytokines and immunoglobulin (Ig) production in vitro, and Southern blots of T-cell receptor (TCR) genes were performed. All four pts had a persistent hypereosinophilia (> 1.9 x 10(9)/L) and chronic skin rash. Three of four had elevated IgE, thrombotic manifestations and lung involvement (asthma and/or infiltrates), and one had deforming sero-negative arthritis of the hands. 66-95% of their peripheral T-cells expressed CD4 but not CD3 or TCR molecules on the cell surface membrane. Activated CD4+CD3- cells secreted interleukin (IL)-4 and/or 5, and were required for maximal IgE secretion by autologous B-cells. Two pts had evidence of rearrangement of TCR genes of the CD4+CD3- cells, one of whom died of anaplastic lymphoma. In conclusion, HES with CD4+CD3- lymphocytosis may be associated with high serum IgE, dermatological, pulmonary, thrombotic and rheumatic manifestations which may be due to Th2 effects of CD4+CD3- cells migrating to end organs. Fatal systemic lymphoid malignancy may also develop in some pts with monoclonal expansion of the CD4+CD3- T-cells.

Adult↗
PubMed2000

Photodynamic therapy of cutaneous lymphoma using 5-aminolevulinic acid topical application.

BACKGROUND: Photodynamic therapy (PDT) with topical application of 5-aminolevulinic acid (ALA) is a new and effective modality for treatment of superficial basal and squamous cell carcinomas. OBJECTIVE: We present the kinetics of ALA-induced protoporphyrin IX (PP) accumulation and the results of ALA PDT treatment on two patients with different stages (stage I and stage III) of mycosis fungoides (MF)-type cutaneous T-cell lymphoma (CTCL). METHODS: ALA-Decoderm cream was applied to the lesions for 16 hours. Spectrofluorescence measurements of PP accumulation were carried out before, during, and 1 hour after photoirradiation (580-720 nm) using the VersaLight system. RESULTS: Different patterns of PP fluorescence kinetics were observed in patients with early and advanced stages of the disease. During photoirradiation the intensity of fluorescence decreased depending on the lesion thickness. One hour after the photoirradiation procedure no PP fluorescence was observed in the stage I MF lesion, while in the thick stage III MF lesions, PP fluorescence reappeared; after an additional 10-15 minutes of irradiation PP fluorescence disappeared. Complete response with excellent cosmetic results was observed in the stage I lesion after a single irradiation with a light dose of 170 J/cm2; in five stage III lesions, complete response was achieved after fractionated irradiation with a total light dose of 380 J/cm2 (follow-up at 27 and 24 months, respectively). CONCLUSION: The results showed a high response of both stage I and stage III MF lesions to ALA PDT. This modality appears to be very effective and can be used successfully for MF treatment.

Abdomen↗
PubMed2000

Erysipelas-like erythema of familial Mediterranean fever: clinicopathologic correlation.

BACKGROUND: Familial Mediterranean fever (FMF) is an autosomal recessive disease that tends to affect certain ethnic groups. It is characterized by recurrent, self-limited attacks of peritonitis, pleuritis, and synovitis. Erysipelas-like erythema (ELE) is the pathognomonic skin manifestation. Lesions are characterized by tender erythematous plaques, usually located on the lower legs. They may be triggered by physical effort and subside spontaneously within 48 to 72 hours of bed rest. Fever and leukocytosis may accompany this condition. OBJECTIVE: The purpose of this study was to describe the histology and the immunofluorescence findings in ELE and to discuss these observations in relation to the clinical findings in FMF. METHODS: We studied 7 patients with FMF in whom ELE developed. In all patients a biopsy was performed within 18 hours from onset of the lesion. In addition to routine hematoxylin and eosin stains, immunohistochemistry to evaluate the infiltrate and direct immunofluorescence were performed. Patients were followed up for their ELE lesions. RESULTS: Histologic examination revealed edema of the superficial dermis and sparse perivascular infiltrate composed of a few lymphocytes, neutrophils, and nuclear dust. Vasculitis was not observed. Direct immunofluorescence showed, in all cases, deposits of C3 in the wall of the small vessels of the superficial vascular plexus. In some cases fibrinogen and IgM were also observed. CONCLUSION: These findings are in accordance both with those found previously in the erysipelas-like phenomenon and those in the peritoneum of patients with FMF. The sparse infiltrate and the deposition of C3 also are compatible with the clinical picture of self-resolving lesions of short duration. It also suggests that erysipelas-like erythema belongs to the spectrum of neutrophilic dermatoses and supports a pathogenesis that involves abnormal inhibition of the inflammatory cascade.

Adult↗
PubMed1999

Insect bite-like reaction in patients with hematologic malignant neoplasms.

BACKGROUND: Exaggerated reaction to insect bites, mainly to mosquitoes, is infrequently described in patients with chronic lymphocytic leukemia. Skin lesions usually appear months to years after the diagnosis of leukemia and are unrelated to laboratory findings, disease course, or therapy. OBSERVATIONS: We describe 8 patients with various hematologic disorders (chronic lymphocytic leukemia, acute lymphoblastic leukemia, acute monocytic leukemia, mantle-cell lymphoma, large-cell lymphoma, and myelofibrosis) who developed insect bite-like reaction. Although the clinical picture and the histological characteristics of the lesions were typical for insect bites, none of the patients actually had a history, course, or response to treatment suggestive of arthropod assaults. In 2 patients, the eruption preceded the diagnosis of the malignant neoplasm. The rash persisted for months to years and was resistant to therapies other than systemic corticosteroids. The 3 patients with chronic lymphocytic leukemia seemed to have a worse prognosis than expected for their disease. In 1, the polymerase chain reaction detected leukemic cells in the infiltrate. CONCLUSIONS: Insect bite-like reaction is an infrequent, disturbing, and difficult-to-treat nonspecific phenomenon in patients with hematologic malignant neoplasms. Since it may precede the hematologic disorder, oriented evaluation is warranted. We speculate that immunodeficiency plays a role in its pathogenesis; however, the exact pathogenesis and its prognostic implications await further studies.

Adolescent↗