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ABSTRACT: Der Australier Steven Kossard beschrieb erstmals 1994 ein Krankheitsbild, das er “postmenopausal frontal fibrosing alopecia”
nannte. Bei 6 Frauen war es zu einem symmetrischen Zurückweichen des frontalen Haaransatzes gekommen. Histologisch lag das
Bild eines Lichen planopilaris vor. Die Augenbrauen waren häufig mit betroffen. Das restliche Integument war frei von Hautveränderungen
im Sinne eines Lichen ruber. Seit der Erstbeschreibung ist diese spezielle Form einer fibrosierenden Alopezie mehrfach beschrieben
worden. Bisher waren fast ausschließlich ältere Frauen betroffen. Wir stellen hier einen männlichen Patienten mit frontaler
fibrosierender Alopezie vom Typ Kossard vor.
In 1994 Steven Kossard described a new and peculiar type of hair loss that he named postmenopausal frontal fibrosing alopecia. In 6 elderly women he observed a symmetric regression of the frontal hair line. Often the eyebrows were also affected. Histology
showed lichen planopilaris. There were no clinical signs of lichen planus on the rest of the body. Since the original description
by Kossard, several cases of frontal fibrosing alopecia have been described – almost all of them in elderly women. We report
a man with frontal fibrosing alopecia of the Kossard type.
Der Hautarzt 04/2012; 53(6):409-411. · 0.58 Impact Factor
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ABSTRACT: Trotz ausgereifter diagnostischer Algorithmen findet die rein morphologische Diagnostik in der allgemeinen Pathologie und
auch in der Dermatohistologie insbesondere im Zug neuer Erkenntnisse aus der Grundlagenforschung ihre Grenzen. Die moderne
hochdifferenzierende histomorphologisch basierte Diagnostik stützt sich insbesondere in der Analyse lymphozytärer und mesenchymaler
Neoplasien zunehmend auf den Nachweis einer Expression von Oberflächenrezeptoren (beispielsweise CD3 als T-Lymphozyten-Oberflächenrezeptor),
von Signalproteinen (beispielsweise CyclinD in der Zellzyklussteuerung) oder Strukturproteinen in den Tumorzellen (beispielsweise
Actin in myogenen Zellen). Unter molekularbiologischen Methoden versteht man im weiteren Sinn Techniken, mit denen es möglich
ist, über die morphologische Betrachtung hinaus zelluläre und extrazelluläre Makromoleküle wie Proteine und Nukleinsäuren
nachzuweisen. Auf Proteinebene ist die selektive Darstellung von Proteinen mittels immunhistochemischer Methoden am Schnittpräparat
weitverbreitet und unverzichtbarer Bestandteil in der modernen pathologisch-anatomischen Diagnostik.
Despite sophisticated diagnostic algorithms, pure morphologic diagnosis has reached its limits in many areas of general and
dermatologic pathologiy, especially in the wake of advances in basic sciences. Modern microscopic diagnosis, especially when
evaluating lymphocytic and mesenchymal tumors, depends greatly on identifying the expression of surface markers (for example
CD3 as T-cell surface receptor), signal proteins (cyclin D in cell cycle control) or structural proteins in tumor cells (actin
in myogenous cells). Molecular biological methods include those techniques which make it possible to identify cellular and
extracellular macro-molecules such as proteins and nucleic acids. At the protein level, the selective identification of proteins
on sections via immunohistochemical methods is a widely used and essential component of modern pathologic-anatomic diagnosis.
Der Hautarzt 04/2012; 58(9):753-759. · 0.58 Impact Factor
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ABSTRACT: Despite sophisticated diagnostic algorithms, pure morphologic diagnosis has reached its limits in many areas of general and dermatologic pathology, especially in the wake of advances in basic sciences. Modern microscopic diagnosis, especially when evaluating lymphocytic and mesenchymal tumors, depends greatly on identifying the expression of surface markers (for example CD3 as T-cell surface receptor), signal proteins (cyclin D in cell cycle control) or structural proteins in tumor cells (actin in myogenous cells). Molecular biological methods include those techniques which make it possible to identify cellular and extracellular macro-molecules such as proteins and nucleic acids. At the protein level, the selective identification of proteins on sections via immunohistochemical methods is a widely used and essential component of modern pathologic-anatomic diagnosis.
Der Hautarzt 10/2007; 58(9):753-9. · 0.58 Impact Factor
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ABSTRACT: Although survival in patients with thin melanomas (tumour thickness < or = 0.75 mm) is usually excellent, thin melanomas have the potential to metastasize.
To determine risk factors for the development of disease progression in patients with thin cutaneous melanomas.
A retrospective study was performed between 1977 and 1998 to identify risk factors for the development of disease progression in 2302 patients with cutaneous melanoma with tumour thickness < or = 0.75 mm, diagnosed and treated at the Department of Dermatology and Allergology, Ludwig-Maximilians University, Munich, Germany. The Kaplan-Meier method was used to estimate the influence of different clinical characteristics for the occurrence of first progression during 10 years of follow-up.
An analysis of the data from 6298 patients with cutaneous melanoma identified 2302 patients (37%) who presented with cutaneous melanoma with a tumour thickness < or = 0.75 mm, without clinical signs of metastasis at initial diagnosis (clinical stage Ia). A small subgroup of our patients (77 of 2302) developed metastatic disease during the follow-up period. The estimated rate of occurrence of metastasis after 10 years of follow-up was 4.7%. The mean follow-up time was 62 months (median 46). Of these 77 patients, 16 experienced progression at the primary tumour site and 32 presented with regional lymph node metastases. Twenty-eight patients primarily developed systemic metastases (seven patients with and 20 without regional lymph node metastases, one patient with regional lymph node metastases and local recurrence). In one patient the primary site of metastatic disease was not reported. Clinical characteristics included age, sex of the patient and different subtypes of cutaneous melanoma: superficial spreading melanoma, nodular melanoma, acrolentiginous melanoma (ALM) and lentigo maligna melanoma (LMM). Male patients and patients with LMM or ALM were significantly over-represented (P = 0.02 and P = 0.002). In the group of 77 patients with thin melanomas (< or = 0.75 mm), local recurrence was over-represented as compared with those with melanomas > 0.75 mm. No difference in group was found for overall survival after the occurrence of lymph node metastasis as the first manifestation of disease progression.
Thorough follow-up and skin examination is recommended for a subgroup of patients with thin tumours, which consists of male patients with LMM or ALM located in the head and neck region.
British Journal of Dermatology 10/2003; 149(4):788-93. · 3.67 Impact Factor
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ABSTRACT: Laser capture microdissection (LCM) is a powerful tool to isolate morphologically defined cell populations from histological slides. It is an easy and fast method to be applied on both cryosections and formalin-fixed, paraffin-embedded tissues. The main problem studying pigmentary skin lesions is the heterogeneity of the biopsy material, consisting of a mixture of melanocytes, keratinocytes, subcutaneous tissue, collagenous fibers and skin appendages. LCM enables the researcher to easily isolate pure tumor cells from surrounding cells. Microdissection is used to separately study chromosomal aberrations or mutations in malignant melanoma (MM) and a pre-existing nevus. Other studies examined the microsatellite instability or loss of heterozygosity (LOH) in microdissected nevi or melanoma lesions. In our laboratory, we used LCM to screen microdissected nevi and melanomas and melanoma metastases for the presence of the BRAF-mutation. Currently we are using immunomicrodissection, the combination of LCM and immunohistochemistry to selective study the BRAF mutation in cells activated by phosphorylation due to the RAS/RAF/MAPK-pathway. There is growing interest in combining laser-capture microdissection with gene-expression analysis, such as gene-expression microarrays. The ongoing research regarding gene-expression and laser-microdissection nourishes the hopes to introduce these two techniques into the routine diagnostic of pigmentary skin lesions.
Pigment Cell Research 09/2003; 16(5):581-581. · 4.29 Impact Factor
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ABSTRACT: Mutational acivation of the RAS/RAF/MAPK pathway has a central role in proliferation of melanocytes. A single point mutation in exon 15 of the BRAF gene has recently been reported in a high percentage in cultured melanoma cells and in 6 of 9 primary melanomas examined. The high frequency of BRAF mutations was considered to be a possible target for therapy of advanced stages of malignant melanoma. To clarify the role of the T1796A BRAF mutation in tumorigenesis of malignant melanoma and to evaluate its presence in benign melanocytic lesions, we screened primary malignant melanomas, malignant melanoma in situ, various types of melanocytic nevi (e.g. congenital, dysplastic, intradermal), Spitz nevi and malignant melanoma with an underlying nevus by single-strand conformational polymorphism (SSCP) and direct DNA sequencing for the presence of mutations of the BRAF gene. The frequency of the specific T1796A mutation in malignant melanoma was with 29% (28/97) considerably low. Melanoma metastases showed a similar rate (21%) as the primary melanomas (3/14) Moreover we could detect the mutation in several non-malignant melanocytic skin lesions (39/187). Spitz nevi (0/63), which histologically can resemble malignant melanoma, and blue nevi (0/19) did not show any mutated cases, while papillomatous nevi (20/27) showed the highest frequency of mutations (74%). In melanomas with an underlying nevus the mutation was either present in both the laser-capture microdissected nevus cells and the microdissected melanoma cells or both lesions were negative for the BRAF mutation except one case, supporting the theory of the nevus progressing into a malignant melanoma. We screened 312 (including 103 microdissected tissue samples) melanocytic skin lesions for the presence of the specific BRAF mutation. As the mutation could be found in similar frequency in both malignant and benign lesions, we conclude that the T1796A mutation of the BRAF gene is non-specific for progression of a nevus to a melanoma. Other so far unknown cofactors seem to be of importance and further studies have to be undertaken to clarify the role of T1796A for the RAS/RAF/MAPK pathway.
Pigment Cell Research 09/2003; 16(5):580-580. · 4.29 Impact Factor
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Der Hautarzt 07/2003; 54(6):544-6. · 0.58 Impact Factor
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ABSTRACT: Recent reports indicate that patients with malignant melanoma might be at higher risk for developing a non-cutaneous unrelated second malignancy. We describe the case of a 46-year-old woman who had a malignant melanoma on her right shoulder that was treated in 1998 by surgical excision combined with axillary lymph node dissection. In 1999, ultrasound examination of peripheral lymph nodes revealed one suspicious echopoor structure in the woman's right axilla that was not palpable. Diagnostic excision and histopathological examination revealed a small B-cell lymphocytic lymphoma, and further investigations led to a diagnosis of chronic lymphocytic B-cell leukaemia (B-CLL). We would like to point out the value of high-resolution ultrasound examination in the follow-up of patients with malignant melanoma; this examination can detect early metastasis as well as other unrelated malignancies.
Journal of the European Academy of Dermatology and Venereology 10/2002; 16(5):491-3. · 2.98 Impact Factor
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ABSTRACT: In 1994 Steven Kossard described a new and peculiar type of hair loss that he named postmenopausal frontal fibrosing alopecia. In 6 elderly women he observed a symmetric regression of the frontal hair line. Often the eyebrows were also affected. Histology showed lichen planopilaris. There were no clinical signs of lichen planus on the rest of the body. Since the original description by Kossard, several cases of frontal fibrosing alopecia have been described--almost all of them in elderly women. We report a man with frontal fibrosing alopecia of the Kossard type.
Der Hautarzt 07/2002; 53(6):409-11. · 0.58 Impact Factor
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Journal of Oral and Maxillofacial Surgery 02/2002; 60(1):117-20. · 1.64 Impact Factor
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ABSTRACT: Diagnosis of scabies infection can be difficult as in many cases only few mites are present on an infected person, and in some cases the skin manifestations can be subtle or atypical. We describe the use of polymerase chain reaction (PCR) to amplify Sarcoptes scabiei DNA in a patient presenting with clinically atypical eczema. Cutaneous scales were PCR positive for S. scabiei DNA before, and negative 2 weeks after, therapy. This method facilitates fast and very sensitive diagnosis of clinically atypical or inapparent scabies infection and therapy control in severely affected patients and may help to identify previously unrecognized scabies cases.
British Journal of Dermatology 12/2001; 144(3):614 - 618. · 3.67 Impact Factor
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ABSTRACT: The spectrum of mycosis fungoides is exceedingly broad. Many different variants have been described, based on both clinical appearance and histological pattern. A rare form which shows preferential infiltration of hair follicles by malignant lymphocytes is follicular mycosis fungoides.
We reviewed our experience with nine cases of follicular mycosis fungoides.
The unifying feature was infiltration of the hair follicle epithelium by atypical lymphocytes causing varying degrees of damage to the hair follicles. In some specimens the lymphocytes displayed only minor atypia leading to a misinterpretation as pseudolymphoma. Gene rearrangement studies were particularly helpful for establishing a diagnosis of malignant lymphoma. Additionally, epidermotropism of lymphocytes, eosinophils and mucin deposition were present to varying degrees. Mucin makes the distinction from mycosis fungoides-associated follicular mucinosis difficult. We found both dermal mucin and a follicular mucinosis pattern present at different stages of disease in the same patient.
We suggest the term mycosis fungoides-associated follicular mucinosis should be replaced by follicular mycosis fungoides in future lymphoma classification schemes.
Journal of Cutaneous Pathology 12/2001; 28(10):525-30. · 1.56 Impact Factor
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ABSTRACT: Tuberous sclerosis complex (TSC) is an autosomal dominantly inherited disorder associated with an alteration of the TSC2 tumor suppressor gene which encodes for the protein product tuberin. The disease is characterized by the development of hamartomas, e.g. cutaneous angiofibromas which consist of vascular cells, interstitial cells, and normal components of the skin. The Eker rat model, an animal model of inherited cancer, has been shown to carry a mutation of TSC2.
Immunohistochemical analyses of human angiofibromas were performed using antibodies directed against tuberin and angiogenic growth factors. Proliferation of human dermal microvascular endothelial cells (HDMEC) was determined after incubation with the supernatants of TSC2 (+/+) and TSC2 (-/-) rat embryonic fibroblasts (REF) that were derived from the Eker strain.
Loss of the expression of tuberin was observed in the interstitial cells of 13 of 39 angiofibromas. The expression of tuberin was retained in the vascular cells. In all analyzed angiofibromas, the angiogenic factors bFGF, PD-ECGF, VEGF and angiogenin were detected in the interstitial cells and/or vascular cells. Expression of PDGF-B and TGF-beta1 was weak. Tissue culture supernatants from TSC2 (-/-) REF stimulated the growth of HDMEC significantly more than supernatants from TSC2 (+/+) REF.
A functional loss of tuberin may stimulate vascular growth.
Journal of Cutaneous Pathology 11/2001; 28(9):470-5. · 1.56 Impact Factor
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ABSTRACT: Cutaneous lymphomas are a heterogeneous group of lymphomas that show variations in histology, immunophenotype, and prognosis. At the time of presentation, cutaneous lymphomas may be primary or may involve the skin as a secondary site of involvement. Primary cutaneous lymphomas, in many instances, are distinct from morphologically similar lymphomas arising in lymph nodes. Their natural history is often more indolent than nodal lymphomas, and for that reason, they often require different therapeutic approaches. A classification scheme should recognize those lymphomas that are unique to the skin, as well as those arising in other sites. The mode of presentation of a lymphoma is often an indication of underlying biological distinctions. However, organ-specific classification systems undermine communication among medical specialists. The World Health Organization classification of hematopoietic and lymphoid malignancies offers a comprehensive approach and proposes that lymphomas should be viewed as a list of individual diseases and that each disease can be defined by a constellation of morphological, biological, and clinical features. The current review will focus on the spectrum of primary and secondary cutaneous lymphomas, emphasizing those features of importance to the clinical oncologist.
Clinical lymphoma 10/2001; 2(2):86-100; discussion 101-2. · 3.11 Impact Factor
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ABSTRACT: The peripheral blood mononuclear cells (PBMC) of individuals with nickel contact allergy are reported to proliferate to a varying degree upon nickel stimulation in vitro. Different phenotypes of the T cells involved are described. With regard to preferential use of the T-cell receptor (TCR), analysis of the several families of the TCR-gamma gene allows rearrangement evaluation of all T cells regardless of predominant surface expression of TCR alpha/beta.
The PBMC of 10 nickel-allergic and five nonallergic individuals were cultured for 4 days in the presence of either medium, PHA, NiSO4, or tetanus toxoid (TT). Proliferation was measured by radioactive thymidine uptake and expressed as stimulation index (SI). T-cell clonality was assessed by analysis of the TCR-gamma chain gene, including the use of PCR with a primer combination covering the four main groups (Vgamma1-8, Vgamma9, Vgamma10, and Vgamma11) of the variable region of the TCR-beta chain gene.
In the allergic individuals, proliferation to NiSO4 was significantly (P<0.05) higher than in nonallergics (mean SI: 18.05/17.87 vs 0.67/2.27). In unstimulated and PHA-stimulated cultures, there was a random TCR spectrum in both groups. In contrast, in nickel-allergic individuals or individuals with recent TT-booster, oligoclonality could be observed in the correspondingly stimulated cultures.
In addition to proliferation assay, analysis of T-cell clonality may be a further means to characterize clinical hypersensitivity reactions on the basis of antigen-dependent oligoclonal T-cell expansion, as in the case of tissue-infiltrating lymphocytes.
Allergy 08/2001; 56(8):767-70. · 6.27 Impact Factor
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Der Hautarzt 07/2001; 52(6):551-3. · 0.58 Impact Factor
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ABSTRACT: A 45-year-old man was admitted with generalized itchy papules. He was originally from the Carribean island of Grenada. The disease had started two years before and was diagnosed as lupus erythematosus, polymorphic light eruption and atopic eczema. Physical examination showed skin-colored papules all over the integument, sebostasis and enlarged supraclavicular and inguinal lymph nodes.
Lymphocyte count was elevated with 58% as well as LDH (322 U/l) and gamma GT (133 U/l). In a blood smear characteristic pleomorphic lymphoid cells (flower cells) could be obtained. Histopathologic evaluation demonstrated a subepidermally located infiltrate of pleomorphic lymphocytes with epidermal involvement. HTLV-I/II serology (ELISA-screening test) was positive. Polymerase chain reaction analysis revealed HTLV-specific sequences.
Diagnosis of adult T-cell lymphoma/leukemia was obtained. Treatment consisted of interferon alpha 2b and zidovudine which resulted in a rapid response. However, as a result of an increased loss of weight (12 kg) this therapy was stopped. Two weeks later generalized lymphadenopathy and disseminated skin lesions were observed. Chemotherapy (CHOP-scheme) was initiated. The patient deceased with signs of an acute leukemia after a short period.
Adult T-cell lymphoma/leukemia is a rare disease in Europe. However, in patients with skin rashes, and lymphadenopathy, which are originally from HTLV-I endemic areas, adult T-cell lymphoma/leukemia should be considered.
DMW - Deutsche Medizinische Wochenschrift 05/2001; 126(14):389-92. · 0.53 Impact Factor
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Der Hautarzt 04/2001; 52(6):551-553. · 0.58 Impact Factor
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ABSTRACT: Diagnosis of scabies infection can be difficult as in many cases only few mites are present on an infected person, and in some cases the skin manifestations can be subtle or atypical. We describe the use of polymerase chain reaction (PCR) to amplify Sarcoptes scabiei DNA in a patient presenting with clinically atypical eczema. Cutaneous scales were PCR positive for S. scabiei DNA before, and negative 2 weeks after, therapy. This method facilitates fast and very sensitive diagnosis of clinically atypical or inapparent scabies infection and therapy control in severely affected patients and may help to identify previously unrecognized scabies cases.
British Journal of Dermatology 04/2001; 144(3):614-8. · 3.67 Impact Factor
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ABSTRACT: Killer inhibitory receptors (KIR) have a modulating effect on the cytotoxic functions of natural killer (NK) cells and T cells. Because lymphoma cells often have the same receptors as their non-neoplastic counterparts, this study investigated the expression of KIR on well defined groups of NK and T cell lymphomas, with and without a cytotoxic phenotype, from different sites of origin.
Nine CD56+/CD3- NK cell lymphomas, 29 CD3+/CD56- T cell lymphomas with a cytotoxic phenotype, and 19 T cell lymphomas without a cytotoxic phenotype were stained for KIR using monoclonal antibodies specific for CD94, CD158a, and CD158b. In addition, the expression of KIR was studied on normal lymphoid tissues.
KIR expression was seen in five of nine true NK cell lymphomas including three of four nasal, one of four cutaneous, and one of one intestinal lymphoma nasal type. Double staining for CD56 and CD94 in normal lymphoid tissues revealed that KIR was predominantly expressed by CD56+ NK cells and sporadically on CD8+ T cells. Moreover, enteropathy-type T cell lymphomas with a cytotoxic phenotype showed KIR expression (three cases expressing CD94 and one case expressing CD158a). All nodal and extranodal nonintestinal T cell lymphomas with or without a cytotoxic phenotype lacked expression of KIR.
These results show that KIR expression is restricted to CD56+/CD3- true NK cell lymphomas originating from the nose, gut, and skin, as well as in a subset of extranodal T cell lymphomas originating from the small intestine, which possessed a cytotoxic phenotype. Thus, the presence of KIR on NK/T cell lymphomas seems to mimic the distribution of KIR found on NK and T cells in normal lymphoid tissue.
Journal of Clinical Pathology 04/2001; 54(3):224-8. · 2.31 Impact Factor
