Disseminated dermatophytic pseudomycetomas arising in an immunocompromised patient.
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Adult↗
Biomedical subjects
W Kempf
Publications and source records attributed to W Kempf.
Fludarabine: risk factor for aggressive behaviour of squamous cell carcinoma of the skin?
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Aged↗
Lyell syndrome revisited: analysis of 18 cases of severe bullous skin disease in a burns unit.
Over the last few years, understanding of the pathophysiology of toxic epidermal necrolysis (TEN), or Lyell's disease, has substantially increased. However, differentiation of severe bullous skin disease remains a challenge for the clinician, and one that is often complicated by late patient referral. We performed a retrospective analysis of all patients with severe bullous skin disease, admitted between 1997 and 2002 to the Burn Centre, which is an integrated part of the Division for Plastic, Hand- and Reconstructive Surgery at the University Hospital of Zurich, Switzerland. We present an overview of our strategies and of the diagnostic and therapeutic difficulties encountered. The final diagnoses of the 18 patients referred to the unit were as follows: eight cases of TEN, one case of staphylococcal scalded-skin syndrome (SSSS), two cases of generalised drug eruption, one case of acute generalised exanthematic pustulosis and one case of febrile ulceronecrotic pityriasis lichenoides et varioliformis acuta (PLEVA). In two cases, the diagnosis remained unclear. In three cases, paraneoplastic origins were suspected but not demonstrated. The overall mortality rate was 33% (six of 18 patients). Remarkably, all patients with histologically confirmed TEN survived. Six of these patients were successfully treated with intravenous immunoglobulins (IVIG). The most common single causative drug inducing TEN (four cases out of eight) was Phenytoin. Establishing an accurate diagnosis-based on a skin biopsy, harvested at an early stage-is more important than ever, because more specific and effective therapeutic modalities are available. As these potentially life-threatening bullous skin disorders are rare, we recommend, that care be provided by an experienced interdisciplinary team, comprising a dermatologist, or dermatopathologist, an intensive care specialist and a plastic surgeon.
Acute Disease↗
Paraneoplastic pityriasis lichenoides in cutaneous lymphoma: case report and review of the literature on paraneoplastic reactions of the skin in lymphoma and leukaemia.
Paraneoplastic dermatoses are non-neoplastic skin disorders which occur in the context of an underlying malignant neoplasm. The classic paraneoplastic dermatoses are mostly associated with solid internal malignancies. They only rarely occur in the context of nodal or primary cutaneous lymphomas. Apart from these classic paraneoplastic dermatoses, there are additional skin disorders reported to occur in close association with haematological and lymphoproliferative disorders which can thus be regarded as paraneoplastic manifestations. We report for the first time two patients with pityriasis lichenoides et varioliformis acuta in association with mycosis fungoides. In addition, we review the literature on paraneoplastic dermatoses of the skin which have been described in patients with leukaemias and primary cutaneous lymphomas.
Adult↗
HLA-G expression in basal cell carcinomas of the skin recurring after radiotherapy.
Basal cell carcinoma (BCC) of the skin represents the most common malignancy in the fair-skinned population worldwide. HLA-G is one of the molecules implicated in immunotolerance. To investigate the role of HLA-G in recurring BCCs, we constructed a tissue microarray containing 38 primary BCCs that underwent radiotherapy and 14 secondary BCCs recurring on the primary site after radiotherapy, and evaluated the HLA-G protein expression by immunohistochemistry. The HLA-G protein was most frequently expressed in aggressive sclerosing BCCs. Nodular BCC demonstrated the strongest HLA-G expression. Interestingly, tumor infiltrating mononuclear cells (TIMC) expressed the HLA-G molecule in BCCs that showed no recurrence. After comparing primary BCCs and BCCs relapsed after radiotherapy, we observed decreased HLA-G expression on tumor cells and the loss of HLA-G expression on TIMC in relapsed BCCs. After radiotherapy, immunobiology of BCC may change resulting in the down-regulation of HLA-G expression on tumor and on tumor-infiltrating cells.
Basal Cell Carcinoma↗
Atypical poorly differentiated cutaneous T-cell lymphoma with an angiocentric growth pattern presenting histologically as vasculitis.
We report the case of a 41-year-old patient with an aggressive cutaneous T-cell lymphoma (CTCL) initially presenting on histology as lymphocytic vasculitis. On repeated histological examination and by molecular biology work-up, the diagnosis of an unclassifiable CTCL could eventually be established. This atypical poorly differentiated lymphoma was of a CD4+ CD8- CD56- phenotype and followed an unfavourable course. The patient died despite chemo- and immunotherapy 7 months after the first consideration of lymphoma as diagnosis.
Adult↗
[Arthralgia, eosinophilia, proteinuria and diuretics-resistant leg edemas].
We report the case of a patient with eosinophilic fasciitis, a rare disease related to systemic sclerodermia and other fibrosing disorders. An involvement of the kidneys--like in this patient with proteinuria and hypoproteinemia--has been described in the literature but is very rare. We discuss diagnostic procedures and novel therapeutic options as combination therapies with steroids, methotrexate and ciclosporine A.
Adult↗
Tomato profilin Lyc e 1: IgE cross-reactivity and allergenic potency.
BACKGROUND: To date, very little data are available about the nature of tomato allergens. Immunoglobulin E (IgE) cross-reactive profilins have been suggested to account for allergic symptoms in patients suffering from tomato allergy. METHODS: The cDNA of tomato profilin was amplified by reversely transcribed polymerase chain reaction (RT-PCR) from total RNA extracted from ripe tomato fruit. The gene was cloned into the pET101D expression plasmid and the protein was produced in Escherichia coli BL21. Purification was performed via poly-l-proline (PLP) affinity chromatography. IgE reactivity of recombinant tomato profilin was investigated by immunoblot and enzyme-linked immunosorbent assay. IgE-inhibition studies were performed to analyse cross-reactivity with other profilins. To determine the allergenic activity of the recombinant protein, basophil histamine release assays using sera of patients with adverse reactions to tomato were performed. RESULTS: Profilin was identified as a new minor allergen in tomato fruits. The recombinant tomato profilin comprises 131 amino acids and high sequence identity to other allergenic food and pollen profilins. It was shown to be IgE-reactive with a prevalence of 22% (11/50) in tomato-allergic patients. In patients with tomato allergy and multiple sensitization to other foods and birch pollen, IgE directed against tomato profilin showed a strong cross-reactivity with profilins from plant food sources and birch pollen. The tomato profilin was able to induce mediator release from human basophils. CONCLUSION: The tomato profilin is a minor allergen in tomato fruit. Thus, it shows biological activity, as confirmed by in vitro histamine release assays with human basophils and thereby has the potential to account for clinical symptoms in tomato-allergic patients.
Adult↗
Baseline staging in cutaneous malignant melanoma.
BACKGROUND: Baseline staging in patients with primary cutaneous malignant melanoma (MM) is routine, but the diagnostic accuracy and the impact on clinical outcome are still unclear. OBJECTIVES: To evaluate the sensitivity and specificity of baseline staging in the early detection of regional lymph node metastases or distant metastases in patients with MM. METHODS: One hundred consecutive patients with MM of Breslow's tumour thickness over 1.0 mm were enrolled. All patients had an extensive baseline staging including physical examination, ultrasound (US) of the abdomen and regional lymph nodes, chest X-ray, whole-body positron emission tomography (PET) and sentinel lymph node biopsy. The sensitivity and specificity of detection of macroscopic or microscopic metastases in the regional lymph nodes or at distant sites were calculated for each method. RESULTS: Sentinel lymph node biopsy was positive in 26 patients. US detected two of 26 histologically tumour-positive sentinel nodes (sensitivity 8%, specificity 88%) and PET two of 26 (sensitivity 8%; specificity 100%). There were three lymph node metastases with a diameter > 4 mm. All of them were found suspect at physical examination. Two of them were detectable with US, two with PET, and all were identified with either US or PET. Nine patients had suspect findings at distant sites, which were all false positive on further investigation (specificity of the combined staging procedures 91%). At 18 (6-37) months' follow-up, five of 26 (19%) patients with a positive sentinel node and four of 74 (5%) of patients with a negative sentinel node had recurrent or progressive disease. CONCLUSIONS: The combination of physical examination and lymph node US detects the great majority of patients with macroscopic lymph node metastasis (approximately 3% of patients at baseline). Only 10% of patients who have a histologically tumour-positive sentinel node are macroscopically detectable. Altogether, approximately 25% of patients have a positive sentinel node biopsy, among 90% microscopic. The value of whole body staging at baseline remains limited, since distant metastases can hardly ever be detected. The survival benefit of baseline staging and surveillance in patients with cutaneous MM remains to be established by comparative prospective trials.
Abdomen↗
A randomized controlled clinical trial of topical photodynamic therapy with methyl aminolaevulinate in the treatment of actinic keratoses in transplant recipients.
BACKGROUND: Transplant recipients have an increased propensity to develop multiple actinic keratoses, which demonstrate an increased transformation rate into invasive squamous cell carcinoma. OBJECTIVE: To evaluate the efficacy and tolerability of topical photodynamic therapy with the new highly tumour-selective photosensitizer methyl aminolaevulinate vs. placebo in the treatment of actinic keratoses in transplant recipients. METHODS: Seventeen transplant recipients with a total number of 129 mild to moderate actinic keratoses were enrolled in a prospective, randomized, double-blind, placebo-controlled study. Two lesional areas within a patient were randomized for two consecutive treatments of topical photodynamic therapy 1 week apart using either methyl aminolaevulinate or placebo cream. Sites were illuminated with 75 J cm(-2) of visible light delivered at 80 mW cm(-2) by a noncoherent light source. Complete resolution and reduction in the number or size of actinic keratoses within the lesional area relative to the initial findings were evaluated at weeks 4, 8 and 16 after treatment. RESULTS: The lesional areas treated with methyl aminolaevulinate were clinically cleared in 13 of 17 patients at 16 weeks. A partial response was recorded in a further three. No reduction in the size or number of actinic keratoses was observed in one area treated with methyl aminolaevulinate and in all placebo-treated areas. Adverse events, such as erythema, oedema and crust formation, were mild to moderate, and treatment was well tolerated by all patients. CONCLUSION: Photodynamic therapy using methyl aminolaevulinate is a safe and effective treatment for actinic keratoses in transplant recipients. It may also reduce the risk of transformation of actinic keratoses to invasive, potentially fatal, squamous cell carcinoma.
Adult↗
Clinicopathological spectrum of mycosis fungoides.
Cutaneous lymphomas represent a heterogeneous group of T-, NK- and B-cell neoplasms, with mycosis fungoides (MF) being the most common subtype. MF has a plethora of clinicopathological manifestations. Many variants of this lymphoma differ substantially from the 'classical' Alibert-Bazin disease and are therefore sometimes referred to as 'atypical' forms of the disease. This review addresses the whole clinicopathological spectrum of mycosis fungoides with respect to epidemiology, clinical, histopathological, immunophenotypic and genotypic features and the clinical course and prognosis of its variants: classical, erythrodermic, follicular, syringotropic, bullous/vesicular, granulomatous, poikilodermic, hypo- and hyperpigmented, unilesional, palmoplantar, hyperkeratotic/verrucous, vegetating/papillomatous, ichthyosiform, pigmented purpura-like, pustular and mucosal involvement in MF.
Humans↗
Subcutaneous infection with Mycobacterium abscessus in a renal transplant recipient.
Transplant recipients show a high incidence of infections, often with atypical manifestations mainly because of drug-related immunosuppression. Mycobacterial infections can be life-threatening in immunosuppressed patients due to the risk of dissemination. There have been only few reports of cutaneous infections with Mycobacterium abscessus among immunosuppressed patients. We present a rare case with a sporotrichoid skin infection with M. abscessus in a renal recipient. The value of immunohistochemical detection of mycobacteria using an anti-BCG antibody in the diagnostic work-up skin biopsies is demonstrated. Long-term antibiotic treatment resulted in complete remission, although recurrences with atypical presentation were observed.
Aged↗
Combination of bexarotene and psoralen-UVA therapy in a patient with Mycosis fungoides.
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Anticarcinogenic Agents↗
The spectrum of mesenchymal skin neoplasms reflected by the new WHO classification.
Mesenchymal tumors are a heterogeneous group of tumors often arising in the skin and soft tissue. The tumors have been reclassified by the WHO in 2002. Benign mesenchymal tumors are about a hundred times more frequent than malignant mesenchymal tumors. Clinically, mesenchymal tumors often present as skin-colored nodes. Overall, elderly persons are more affected than younger individuals. The etiology is often unknown, sometimes there is an association with insults such as radiation, scars, or lymphedematous or venous stasis. Whereas some years ago a wide excision with a margin of 3-5 cm was performed for malignant variants, today micrographic surgery is preferred, as it can avoid mutilating procedures. Early detection and removal is critical since mesenchymal skin tumors often cannot be cured by radiation or chemotherapy.
Humans↗
Photodynamic therapy--a new treatment option for epithelial malignancies of the skin.
Photodynamic therapy (PDT) is based on the activation of a photosensitizer by illumination with visible light, leading to photochemical tissue destruction or immunomodulation. The greatest disadvantage of systemic administration of photosensitizers is cutaneous photosensitization of the patients, which lasts for some months. An alternative approach for epithelial precancerous lesions, such as actinic keratoses and Bowen's disease, as well as for superficial skin tumors, such as basal cell carcinomas, is the topical application of sensitizers, especially 5-aminolevulinic acid. Topical PDT causes selective tissue necrosis and tumor destruction and produces excellent cosmetic results. The following article summarizes the main principles of PDT and provides a synopsis of the present status of the use of PDT for epithelial skin malignancies.
Aminolevulinic Acid↗
[Acquiring images with very high resolution using a composing method].
Composing microscopic images of very high resolution from several parts posed some problems. One of them was the necessity to adjust the focusing level when moving from one part to another. Re-focusing lead to problems with joining the image parts, which did not correspond exactly, and the area of image fusion was noticeable. A computer program was developed to overcome these problems. Our program worked with all the image parts together to find their optimal order for image fusion. Individual image parts were joined using a steep gradient running along a randomly generated curve. This method gave good results even in images with background or holes in the tissue. The method of composing large images from individual parts was used for digitizing the skin lymphoma collection of the Institute of Dermatology, University Hospital, Zürich. This collection of digital images is a part of the 6th version of Hypertext atlas of Dermatopathology at www.muni.cz/atlases.
Image Processing, Computer-Assisted↗
Proteus syndrome.
A 34-year-old male patient was referred with a recalcitrant leg ulcer overlying an extensive vascular malformation, which had led several times to septic soft tissue infections. During his infancy he had been diagnosed to have Klippel-Trenaunay syndrome. Clinical examination revealed asymmetric hypertrophy of the lower extremities, an extensive portwine stain on the more severely affected left limb as well as prominent venous varicosities of both legs. Hands and feet showed striking cerebriform palmoplantar hypertrophy, and macrodactily with syndactily of several fingers. All toes had been amputated in early childhood due to extreme overgrowth and currently the patient walked on his forefeet in a prominent pes equinus deformity. Further symptoms consisted in several lipomas at both arms, another portwine stain at the left hemithorax and a single café-au-lait spot at the left scapula. Angio-magnetic resonance imaging scans of both legs showed an extensive venous-lymphatic vascular malformation involving the whole subcutis and infiltrating the muscle. The chronic wound was interpreted as venous stasis ulceration. Local percutaneous sclerotherapy of the dilated veins underneath the ulcer was discussed, but considered to carry a relevant risk of skin necrosis with consecutive progression of the wound. A conventional split-skin graft led to complete wound healing. Since, the patient consequently wears custom-made compression stockings and remained free from recurrences. The syndromatic constellation of palmoplantar overgrowth, multiple lipomas, giant fingers and toes, limb overgrowth, venous-lymphatic malformation and a café-au-lait spot led to the diagnosis of Proteus syndrome. The possible aetiology, clinical manifestations, differential diagnosis and management of this rare disorder are discussed.
Adult↗
Imiquimod in basal cell carcinoma: how does it work?
Imiquimod is a topical immune response modifier that binds to Toll-like receptor-7 and -8, inducing interferon-alpha. We treated superficial basal cell carcinomas (BCC) with imiquimod 5% cream daily for 5-8 days. The BCC lesions were biopsied before treatment and following imiquimod treatment, when the lesion showed the signs of erosion. We applied histology, immunohistochemistry and gene array technology (Affymetrix) to gain further insight into the mode of action of imiquimod. Our findings demonstrate that imiquimod-induced BCC regression is associated with a strong activity of the innate immune response, mediated by cells of macrophage-monocyte origin and is associated with the induction of apoptosis.
Adjuvants, Immunologic↗