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Biomedical subjects

H Kerl

Publications and source records attributed to H Kerl.

At least 55 records · Page 3Linked to original sources
PubMed2001

Cutaneous follicle center cell lymphoma, follicular type.

This article discusses the clinicopathologic and molecular features of primary cutaneous follicle center cell lymphoma, follicular type. Synthesis of morphologic, immunohistochemical, and molecular studies have clearly characterized this peculiar morphological variant of the cutaneous B-cell lymphomas. Although local recurrences can be frequently observed, the overall prognosis of these patients is very good and extracutaneous dissemination is very rare.

Humans↗
PubMed2001

Grover's disease associated with Sarcoptes scabiei.

An 83-year-old man presented with a 4-month history of discrete, itchy papules mainly distributed on the trunk and upper extremities. Histopathologic examination of two biopsies from lesions on the trunk revealed mainly focal suprabasal acantholysis and an inflammatory infiltrate composed mainly of lymphocytes with a few eosinophils. The overall clinical and histopathologic features were consistent with Grover's disease. However, scrapings taken from the skin lesions showed numerous mites of Sarcoptes scabiei. Subsequent treatment with an antiscabies cream led to a rapid complete cure, and no skin lesions have been observed during a 6-month follow-up. A review of the literature revealed 2 other cases of cutaneous lesions fulfilling the clinical and histopathologic features of Grover's disease in which mites of S. scabiei were demonstrated. Our observation further highlights the unusual association of Grover's disease with S. scabiei mites and emphasises the importance of excluding this easily treatable skin infestation in all patients with Grover's disease.

Acantholysis↗
PubMed2001

Collision of squamous-cell carcinoma with melanoma in situ in a child with xeroderma pigmentosum.

Coexistence (collision) of two different neoplasms in the same lesion has previously been documented by several authors. In this report, we describe a 13-year-old boy with xeroderma pigmentosum presenting with squamous-cell carcinoma and melanoma arising at the same site on the nose. Histopathologically, the melanoma component of the lesion was located mainly eccentrically to the squamous-cell carcinoma component. Immunohistochemical stains confirmed the histopathologic findings. Mutations for p53 assessed using single-strand conformation polymorphism, and sequencing analysis revealed a CC-to-TT transition at codon 159 of the p53 gene in the squamous-cell component but not in the melanoma component. This finding suggests a possible role for UV in the pathogenesis of at least the squamous-cell component of the tumor. To the best of our knowledge, this is the first report of a collision tumor comprising squamous-cell carcinoma and melanoma arising in childhood.

Adolescent↗
PubMed2001

High mutation frequency at Ha-ras exons 1-4 in squamous cell carcinomas from PUVA-treated psoriasis patients.

Clinical follow-up studies have revealed that PUVA-treated patients are at increased risk of skin cancer, particularly squamous cell carcinoma (SCC). However, since psoralen and UVA (PUVA) is not only a potent mutagen and carcinogen but also an immunosuppressor, and since other (co)carcinogenic factors often exist in psoriasis patients, the exact causes and mechanisms of PUVA-associated SCC are still not completely understood. In order to fill this gap the tools of molecular epidemiology are being used to study the SCC mutational spectra of p53 and Ha-ras, two of the most commonly mutated genes in human cancers. A previous mutation analysis revealed that SCC in PUVA-treated patients often carried mutated p53 genes and that many of the mutations had the UV fingerprint (i.e. C-->T or CC-->TT transitions at dipyrimidine sites). In the present study DNA-sequencing analysis revealed a total of 18 Ha-ras missense or nonsense mutations at exons 1-4 in 13 of 17 SCC (76%) from 8 of 11 (73%) PUVA-treated psoriasis patients. Six of the 18 mutations (33%) were of UV-fingerprint type (C-->T transitions), five (28%) were at 5'-TpG sites (i.e. potential psoralen-binding sites and thus potentially caused by PUVA) and seven were of other type (39%), including six G:C-->T:A transversions at hotspot codon 12. In addition, in the case of 6 of the 11 subjects (55%) both tumor and normal skin samples contained a T:A-->C:G base change at codon 27 (a 5'-ATT site), a change previously hypothesized to be a possible silent Ha-ras polymorphism at one allele. When we compared the present Ha-ras mutation spectrum with the p53 mutation spectrum from a previous study of the samples, we found that approximately half of the tumors harbored mutations in both Ha-ras and p53. Together, our results indicate that Ha-ras mutations are present in a large proportion of PUVA-associated SCC and that UVB, PUVA and other agents may induce Ha-ras mutations and act together with p53 in the formation of SCC in psoriasis patients.

Base Sequence↗
PubMed2001

Diagnostic criteria of primary cutaneous B-cell lymphomas and pseudolymphomas.

Primary B-cell lymphomas of the skin are defined as malignant B-cell proliferations presenting with cutaneous involvement alone and no evidence of extracutaneous manifestations over a period of at least six months when complete staging has been performed. The major subtypes are follicle center-cell lymphoma, marginal zone lymphoma and large B-cell lymphoma of the leg (EORTC classification 1997). Primary B-cell lymphomas of the skin differ significantly from nodal lymphomas especially with respect to their clinical behavior. Pseudolymphomas of the skin are inflammatory diseases that simulate malignant lymphomas either clinically, histopathologically, or both. Particular pseudolymphomas may mimic cutaneous B-cell lymphomas. The most important examples are: lymphomatoid drug reactions, lymphocytoma (borrelia burgdorferi as causative agent), arthropod reactions, pseudolymphomas associated with vaccinations or tattoos and inflammatory pseudotumors. In recent years, new immunohistological and molecular techniques have added important criteria for the differentiation of cutaneous lymphomas from pseudolymphomas.

Diagnosis, Differential↗
PubMed2000

Primary cutaneous follicle center cell lymphoma with follicular growth pattern.

Cutaneous B-cell infiltrates showing a prominent follicular growth pattern with germinal centers are thought by some authors to represent either marginal zone lymphomas with reactive germinal centers or pseudolymphomas. To establish whether a true primary cutaneous follicular lymphoma exists, we studied biopsies from 15 patients with skin lesions characterized histopathologically by the presence of B-cell infiltrates with follicular pattern. Staging investigations, including bone marrow biopsy, were negative in all patients. All were negative for bcl-2 protein expression and did not present the t(14;18). In all biopsy specimens neoplastic follicles showed 1 or more morphologic or immunophenotypic criteria of malignancy (presence of a reduced mantle zone, absence of tingible body macrophages, reduced proliferation rate). In 9 specimens a monoclonal rearrangement of J(H) genes could be detected by polymerase chain reaction analysis. After laser beam microdissection, a band of the same length could be observed in 6 probes from different follicles from the same specimen, indicating the presence of the same monoclonal population of follicle center cells. Follow-up examinations in all patients revealed no evidence of extracutaneous spread (mean follow-up, 48.7 months). Our study demonstrates that primary cutaneous follicular lymphoma represents a distinct entity of the cutaneous B-cell lymphomas. (Blood. 2000;95:3922-3928)

Adult↗
PubMed2000

Granulomatous cheilitis and Borrelia burgdorferi: polymerase chain reaction and serologic studies in a retrospective case series of 12 patients.

BACKGROUND: Granulomatous cheilitis (GC) is a chronic granulomatous inflammation of the lips of unknown etiology, which may be associated with peripheral facial nerve paralysis and/or lingua plicata (Melkersson-Rosenthal syndrome [MRS]). Borrelia burgdorferi is a spirochete that causes Lyme borreliosis, a multisystemic infectious disease with frequent occurrence of facial nerve paralysis. An etiologic role of B burgdorferi in various granulomatous diseases has been suggested. The present study was performed to examine a possible causative role of B burgdorferi for GC/MRS by B burgdorferi-specific polymerase chain reaction analysis of biopsy specimens from affected lip tissue and determination of B burgdorferi IgG and IgM serum antibodies using enzyme-linked immunosorbent assay and immunoblot tests. OBSERVATIONS: We examined a retrospective case series of 12 patients with GC/MRS from a Lyme borreliosis endemic area (median duration of disease, 8 months [range, 3-348 months]). Borrelia burgdorferi-specific DNA could not be amplified by polymerase chain reaction in any of the 12 patients. One (13%) of 8 patients tested had a serum B burgdorferi IgG response on enzyme-linked immunosorbent assay, and 2 patients (25%) had an IgM response, but immunoblot testing yielded negative results in all 8 patients. CONCLUSION: The results of the present study do not indicate that B burgdorferi has an etiologic role in GC/MRS.

Adult↗
PubMed2000

[Risk factors for local complications in erysipelas].

BACKGROUND AND OBJECTIVE: A complicated course of erysipelas is not uncommon. Bullous, haemorrhagic, necrotic and purulent lesions may be encountered. Today no reliable data exist as to which constitutional factors renders a patient at risk for developing complicated erysipelas though several risk factors, particularly diabetes mellitus, are often suggested. Based on the analysis of patients with erysipelas at the Department of Dermatology in Graz, factors determining the risk for complicated erysipelas should be identified. PATIENTS/METHODS: In a retrospective case-control study clinical data sheets of 766 in- patients treated at the department were evaluated with respect to the course of the erysipelas and with respect to potential risk factors. RESULTS: General risk factors for local complications were location at the lower extremities, pre-existing hepatic or renal disease, hyperuricaemia, and diabetes mellitus. Hepatic and renal disease and - to a lesser extent - diabetes particularly predisposed for bullous and haemorrhagic lesions, while vascular occlusive disease enhanced the risk for necrotic lesions. CONCLUSIONS: Location and hepatic and renal disease are the most important risk factors, while diabetes is probably of less significance than previously suggested.

Adult↗
PubMed2000

[Therapy of primary cutaneous B-cell lymphomas].

BACKGROUND AND OBJECTIVE: Primary cutaneous B-cell lymphomas (PCBCL) represent a unique type of extranodal B-cell lymphomas. Recently, the "European Organization for Research and Treatment of Cancer (EORTC)-Cutaneous Lymphoma Study Group" classified PCBCL into two major groups: one with low-grade malignancy and excellent prognosis (follicle center cell lymphoma, immunocytoma/marginal zone B-cell lymphoma) and the other with intermediate malignancy and worse prognosis (large B-cell lymphoma of the leg). The clinical course and the prognosis of both groups clearly distinguish them from nodal lymphomas with similar morphological aspects, thus underlying the need for different treatment modalities. PATIENTS/METHODS: We investigated retrospectively the therapeutic data from 51 patients with PCBCL (40 low grade lymphomas, 11 large B-cell lymphomas). Several treatment modalities were used: total excision, radiotherapy, polychemotherapy, systemic corticosteroids, systemic antibiotics, as well as a variety of combination treatments. RESULTS: Recurrence, dissemination and/or death of the patients were not significantly related to any single treatment modality. CONCLUSIONS: In our opinion, the choice of treatment for PCBCL depends on the histologic classification, the number, spread and localization of the infiltrates, and on the general condition of the patient.

Adult↗
PubMed2000

[Squamous epithelial carcinoma in erythema ab igne].

Erythema ab igne (EAI) is a red-brown net-like hyperpigmentation due to repetitive exposure to submaximal heat over a long period of time. This rash is typically seen on the anterior lower legs of people who sit in front of open fireplaces or coal stoves and on the trunk of patients with chronic disorders who seek pain relief by long-term use of hot-water bottles or heating pads. EAI can not only be a diagnostic sign for underlying internal diseases including malignant tumors but can also give rise to cutaneous malignancies. We report on a 75-year-old female patient who developed several thermal keratoses and squamous cell carcinomas at the site of erythema ab igne caused by chronic heat exposure over several years.

Abdominal Neoplasms↗
PubMed2000

[The spectrum of cytotoxic lymphomas of the skin].

Cytotoxic lymphomas are peripheral T- and natural killer-cell lymphomas with primary or secondary skin manifestations. They constitute a heterogeneous group of lymphoproliferative disease. They are characterized by expression of cytotoxic proteins and are frequently associated with an aggressive clinical course. A brief introduction to cytotoxic lymphocytes and proteins is followed by a detailed description of clinical, histological, immunohistochemical and genetic characteristics of cutaneous cytotoxic lymphomas.

Humans↗
PubMed2000

[Cutaneous angiosarcoma].

BACKGROUND AND OBJECTIVES: Angiosarcomas of the skin arise almost exclusively in the following clinical settings: 1. the face and scalp, usually in elderly individuals, 2. lymphedematous regions (lymphedema-associated angiosarcomas), and 3. skin that has been previously irradiated (post-radiation angiosarcomas). Clinical and histopathologic diagnosis of angiosarcoma is difficult often resulting in great delay that obviates against early and possibly successful treatment of these very aggressive neoplasms. Diagnostic problems are described, and prognostic factors as well as the effect of different forms of treatment on the outcome are discussed. PATIENTS/METHODS: Retrospective study of 11 patients with cutaneous angiosarcomas. Clinical presentation, histopathology, therapy and survival time are analysed. RESULTS: Only 1 of 11 cases cutaneous angiosarcoma was clinically identified. Survival time was 1-24 months. Three patients who received radical surgery have not developed metastases and are still alive. CONCLUSIONS: Clinical and histopathologic diagnosis of cutaneous angiosarcomas is often very difficult. Prognosis is very bad; radical surgery seems to be the best therapeutical option.

Adult↗
PubMed2000

The clinicopathologic spectrum of cytotoxic lymphomas of the skin.

Cytotoxic lymphomas of the skin comprise a spectrum of peripheral T cell and natural killer cell lymphomas with primary or secondary skin manifestations. They comprise a broad, heterogeneous group of lymphoproliferative disorders, and are characterized by expression of cytotoxic proteins (TIA-1, granzyme A and B, and perforin). These lymphomas often show an aggressive clinical course. In this article a detailed description of clinical, histopathologic, immunohistochemical, and molecular characteristics of cutaneous cytotoxic lymphomas follows a brief introduction on cytotoxic lymphocytes.

Diagnosis, Differential↗
PubMed2000

Controversies in cutaneous lymphomas.

Recently great advances were achieved in the recognition and classification of primary cutaneous lymphomas. With this increased knowledge, one must realize that we must deal with new concepts that are sometimes confusing and controversial. The following controversial subjects are presented in this article: (1) Classification of cutaneous lymphomas; (2) Cutaneous T-cell lymphoma with small/medium-sized pleomorphic cells as a distinct entity; (3) Primary cutaneous follicle center lymphoma and marginal zone lymphoma/ immunocytoma.

Humans↗
PubMed2000

Basaloid neoplasms in nevus sebaceus.

BACKGROUND: Nevus sebaceus (NS) (organoider nevus) may frequently be associated with the development of a number of benign and malignant neoplasms among which basaloid neoplasms are the most common. Histopathologic criteria for diagnosis and classification of basaloid proliferations arising in NS are still debated. Most previous investigators have considered them to represent mainly basal cell carcinomas (BCCs). On the contrary, a number of recent authors have proposed that most basaloid neoplasms in NS exhibit predominantly morphologic features implying benignancy, thus representing trichoblastomas (TBs). In this study, we attempted to characterize better the histopathologic features of basaloid neoplasms in NS in a large series based on current morphologic criteria. METHODS: Three-hundred and sixteen cases of NS seen over 19 years were consecutively sampled and reviewed for basaloid neoplasms. Twenty-four cases of basaloid neoplasms in NS were identified and categorized based on current histopathologic criteria either as TB or BCC. For comparison of histopathologic features, 37 solitary TB were also studied. RESULTS: Following histopathologic analysis, 22 cases were categorized as TB (91.6%, 10 males, 12 females; mean age 40.8 years, range 19-78 years) and 2 cases as BCC (8.4%, 1 male, 1 female; 32 years and 40 years). Clinical features in both groups were generally similar. The lesions presented exclusively on the head and neck as skin colored to pigmented papules or nodules within NS (scalp in 19 TB cases and 1 BCC case; face in 2 TB cases and 1 BCC case; neck in 1 TB case). Histopathologically, TB in NS were characterized by smooth-bordered basaloid aggregations with either a nodular and/or a superficial pattern, abundant fibrous stroma with focal clefts within the stroma, and prominent features of limited follicular differentiation (rudimentary follicular germs in concert with papillae). In contrast, BCC in NS showed basaloid aggregations that vary markedly in size and shape, scant fibrous stroma, focal mucinous clefts between basaloid aggregations and surrounding stroma, and lack of prominent rudimentary follicular germs in concert with papillae. Remarkably, sections in a few cases of TB showed features occasionally found in BCCs but presently widely considered to be unspecific (e.g., ulceration, cystic degeneration, and focal clefts between basaloid aggregations and surrounding stroma). Two cases of TB in NS were associated with a sebaceoma and 1 case with a desmoplastic trichilemmoma. Follow-up data in 14 TB cases and 2 BCC cases (mean follow-up 28.8 months; range 1 to 160 months) revealed no local recurrences or distant metastases. CONCLUSION: Our study confirms that the vast majority of the basaloid neoplasms arising in NS show clear-cut morphologic criteria for TB, whereas only a few cases display histopathologic features consistent with BCC. In a minority of cases, basaloid neoplasms with overall morphologic features of TB may present problems in diagnosis when they exhibit a few histopathologic features traditionally associated with BCC or when they occur in combination with other adnexal neoplasms.

Adolescent↗
PubMed2000

Extended extracorporeal photochemotherapy with extracorporeal administration of 8-methoxypsoralen in systemic sclerosis. An Austrian single-center study.

BACKGROUND: Extracorporeal photochemotherapy (EXP) is an immunomodulating therapy that has been used in a limited number of patients with systemic sclerosis (SSC) with controversial results. The present study was performed to evaluate the efficacy and safety of extended EXP with extracorporeal application of liquid 8-methoxypsoralen (8-MOP) in the treatment of SSC. METHODS: Eleven women with progressive SSC of recent onset were treated for a period of 16-57 months. Skin changes, physical performance, extracutaneous manifestations, and quality of life were evaluated before initiation of EXP and at regular intervals thereafter. RESULTS: From the start to the last set of EXP, we observed an overall improvement and/or stabilization of skin changes and physical performance in 5 of 11 patients (45%). Extracutaneous manifestations deteriorated in 10 of 11 patients (91%) (P<0.05), and quality of life deteriorated in 9 of 11 patients (82%) from a mean score of 10 before, to 17 at the last set of EXP (P<0.05). No major side effects were noted. CONCLUSION: Extended EXP with extracorporeal administration of 8-MOP is a safe and well tolerated treatment modality. However, it provides only (minor) improvement of skin changes of a subset of SSC patients and does not beneficially influence extracutaneous manifestations and quality of life.

Adult↗