[Multiple erythema].
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Administration, Oral↗
Biomedical subjects
M J Flaig
Publications and source records attributed to M J Flaig.
[Adult T-cell lymphoma/leukemia: a very rare disease in Europe].
HISTORY AND CLINICAL FINDINGS: 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. INVESTIGATIONS: 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, TREATMENT AND COURSE: 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. CONCLUSIONS: 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.
Antibodies, Viral↗
[Figurated plaque on actinically damaged skin. Actinic O'Brien granuloma].
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Aged↗
[Bullous scabies and scabies-triggered bullous pemphigoid].
Two patients were admitted to our hospital with tense blisters on an erythematous base, typical for bullous pemphigoid. In both patients an infestation with Sarcoptes scabiei was diagnosed by dermatoscopy as well as histological examination. In one patient the clinical diagnosis of bullous pemphigoid could be confirmed by immunofluorescence microscopy, histopathology and a clinical relapse of bullous pemphigoid without scabies infestation. In the other patient no evidence for an autoantibody-mediated autoimmune blistering disease was found. We postulate that bullous scabies could develop after long persistency of the parasites leading to a specific immune response with activation of T helper type 2 (Th2) cells causing high levels of the cytokine interleukin 5 and then consecutively eosinophilia. Secretion of proteolytic enzymes near the basal membrane zone might explain the development of intraepidermal, often suprabasal blisters. In contrast, in the first patient the scabies infestation might have triggered a flare up of the underlying autoimmune disease. Comparison of our two patients demonstrates two entities: bullous pemphigoid triggered by scabies as a Koebner phenomenon and a bullous subtype of scabies mimicking bullous pemphigoid. Therefore both, scabies infestation triggering bullous pemphigoid and bullous pemphigoid-like scabies should be included in the differential diagnosis of vesicles, tense blisters and erythema, especially at an early clinical stage.
Administration, Oral↗
Follicular mycosis fungoides. A histopathologic analysis of nine cases.
BACKGROUND: 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. METHODS: We reviewed our experience with nine cases of follicular mycosis fungoides. RESULTS: 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. CONCLUSIONS: We suggest the term mycosis fungoides-associated follicular mucinosis should be replaced by follicular mycosis fungoides in future lymphoma classification schemes.
Aged↗
Expression of killer cell inhibitory receptors is restricted to true NK cell lymphomas and a subset of intestinal enteropathy-type T cell lymphomas with a cytotoxic phenotype.
BACKGROUND/AIMS: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.
CD3 Complex↗
Cutaneous manifestations of lymphoma: a clinical guide based on the WHO classification. World Health Organization.
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.
Female↗
[Diagnosis of cutaneous B-cell lymphomas].
Skin is the second most common site of extranodal lymphoma. The significance of cutaneous B-cell lymphomas has been underestimated due to the lack of monoclonal antibodies and molecular genetics in former years. Cutaneous B-cell lymphomas represent a heterogeneous group of entities which show variation in histology, immunophenotype and in prognosis. The most common entities presenting in skin are follicular lymphomas, marginal zone B-cell lymphomas, and diffuse large B-cell lymphomas. The majority of primary cutaneous B-cell lymphomas have an excellent prognosis. Whereas in B-cell lymphomas with secondary spread to the skin the clinical course is dependent on the prognosis of the primary lymphoma. It is evident that primary cutaneous lymphomas are distinct from nodal lymphomas and not extranodal manifestations of their lymph-node-based counterparts. In the current review the morphologic spectrum of cutaneous B-cell lymphomas comprising primary and secondary cutaneous B-cell lymphomas is discussed according to the WHO classification scheme.
Diagnosis, Differential↗
Detection of clonal T cells in lichen planus.
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Base Sequence↗
Impact of molecular analysis in the diagnosis of cutaneous lymphoid infiltrates.
The diagnosis and classification of cutaneous lymphomas is a challenge for the dermatopathologist. This is particularly true for determining the distinction between a malignant lymphoma and a benign reactive infiltrate (pseudolymphoma). Recent advances in molecular genetics, as the determination of clonality of lymphoid infiltrates, have emerged as an important tool to overcome these diagnostic dilemmas. In our experience, more than 90% of cutaneous T-cell lymphomas have a rearrangement of the T-cell receptor gamma chain gene, whereas clonal rearrangements in cutaneous T-cell pseudolymphomas could not be found. However, the demonstration of clonality does not necessarily indicate malignancy. There have been several reports that have identified clonal lymphoid proliferations in pityriasis lichenoides et varioliformis acuta, pseudolymphomas, and lichen planus. For this reason one must carefully evaluate the information that is provided by these techniques. The results should always correlate with clinical, histologic, and immunophenotypic data to achieve the correct diagnosis.
Blotting, Southern↗
Large plaque parapsoriasis: clinical and genotypic correlations.
Twelve patients with large plaque parapsoriasis (LPP) were investigated for the presence of predominant T-cell clones, analyzing the T-cell receptor (TCR) gamma-chain gene. The diagnostic and prognostic significance of TCR gene rearrangement status was assessed by a correlation with the long-term clinical follow-up. Six out of 12 patients showed a clonal T-cell population. Clinically, among the patients with clonal disease one developed clearcut mycosis fungoides (MF) after a follow-up of 8 years, in the other 5 patients no such diagnosis could be made after follow-up of 2-21 years (median: 9 years). In patients with polyclonal infiltrates the lesions remained virtually unchanged. These findings indicate that in LPP TCR gene rearrangement status has no prognostic significance and does not allow distinction of LPP and early MF. Both conditions show a clonal T-cell infiltrate with similar frequency, are very similar in clinical and histologic presentation and according to recent studies share the same low risk to develop overt MF. Therefore both terms refer to the identical clinical situation. This should be designated as early MF and efforts should concentrate on identifying those patients that are at risk to develop aggressive disease.
Adult↗
Livedoid vasculopathy in a patient with factor V mutation (Leiden).
BACKGROUND: Frequently, no underlying disease can be detected in patients with livedoid vasculopathy. For these forms, an unknown vaso-occlusive or thrombogenic process has been accused to play a role. Thus, a patient with livedoid vasculopathy was examined for different parameters which can be involved in coagulopathies. METHODS: Laboratory studies for different autoantigen reactive immunoglobulins, cryoglobulins, and circulating immune complexes were carried out. Besides dermatopathologic examination, a biopsy specimen was analyzed by direct immunofluorescence for immunoglobulin (Ig) and complement deposits. Furthermore, hemostaseological function tests including activated protein C (APC) resistance were undertaken. RESULTS: Positive only at very low titres were antinuclear antibodies and c-ANCA, all other parameters were within normal ranges or negative. Direct immunofluorescence revealed IgM, C3 and fibrogen deposits. Hemostaseological function tests demonstrated a pathologic activated protein c resistance and PCR analysis a heterozygous defect of the factor V (Leiden). CONCLUSIONS: The diagnosis of livedoid vasculopathy associated with factor V mutation (Leiden) was made. Since the underlying cause for livedoid vasculopathy often remains unknown, we suggest that hemostaseological function tests including APC resistance and factor V gene mutation analysis should be carried out. Further studies have to follow in order to elucidate the role of mutant factor V in livedoid vasculopathy and in cutaneous ulcerations.
Activated Protein C Resistance↗
Lack of detection of human herpesvirus (HHV)-8 DNA in lesional skin of German pemphigus vulgaris and pemphigus foliaceus patients.
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DNA, Viral↗
Cutaneous lymphomas: a proposal for a unified approach to classification using the R.E.A.L./WHO Classification.
BACKGROUND: The classification of cutaneous lymphomas has been controversial. The EORTC has proposed that conventional classification schemes are not suitable for cutaneous lymphomas, and that a unique classification system is required. DESIGN: The authors review the suitability of the R.E.A.L. Classification for cutaneous lymphomas, and compare it with the newly proposed EORTC system. The principles of the R.E.A.L. Classification have been adopted by the WHO committees for the classification of hematopoietic and lymphoid neoplasms. Each disease is defined as a distinct entity based on an integration of morphology, immunophenotypic and genetic features, clinical presentation and course, and normal cellular counterpart. If either primary or secondary involvement of the skin is a constant factor, this aspect is considered integral to disease definition. RESULTS: Organ-specific classification schemes may impede the recognition of common features of diseases involving multiple anatomic sites. For example, cutaneous marginal zone B-cell lymphomas (formerly designated cutaneous immunocytomas) mirror the features of MALT lymphomas in other anatomic sites. While the EORTC Classification for cutaneous lymphomas attempts to emphasize certain aspects of these neoplasms of importance to dermatologists, the use of multiple classification systems is a step backward, and may lead to confusion among hematologists/oncologists, and dermatologists. Nevertheless, cutaneous lymphomas often have a more indolent natural history than nodal lymphomas, and may require different therapeutic approaches. Clinical features are an important prognostic factor and should be utilized in guiding therapy. For cutaneous lymphomas the presence or absence of systemic spread is particularly important. Additionally, the site of origin is often important in the definition of disease entities. CONCLUSIONS: Organ-specific classification schemes, such as the EORTC Classification for cutaneous lymphomas, are not required, and indeed may impede the recognition of common features of diseases involving multiple anatomic sites. A common classification system, such as the R.E.A.L./WHO Classification, should be utilized for all lymphomas, regardless of the site of origin.
Europe↗
Morphologic spectrum of cutaneous B-cell lymphomas.
Cutaneous B-cell lymphomas represent a heterogeneous group of entities which show variation in histology, immunophenotype, and in prognosis. In the current review the morphologic spectrum of cutaneous B-cell lymphomas comprising primary and secondary cutaneous B-cell lymphomas is discussed according to the REAL classification scheme.
Diagnosis, Differential↗
The revised European-American Classification of Lymphoid Neoplasms (REAL): a preferred approach for the classification of cutaneous lymphomas.
The Revised European-American Classification of Lymphoid Neoplasms (REAL) classification is based on the principle that each type of lymphoma is a distinct disease defined by morphology, immunophenotypic and genetic features, clinical presentation, and course. If either primary or secondary involvement of the skin is a constant factor, this aspect is considered integral to disease definition. Organ-specific classification schemes, such as that proposed by the European Organization for Research and Treatment of Cancer (EORTC) for cutaneous lymphomas, are not required, and indeed may impede the recognition of common features of diseases involving multiple anatomic sites. The use of multiple classification systems is a step backward, and may lead to confusion among hematologists/oncologists and dermatologists. Nevertheless, cutaneous lymphomas in many instances are distinct. Their natural history is often more indolent than nodal lymphomas, and for that reason they often require different therapeutic approaches. We agree with the efforts of the EORTC classification to emphasize the unique clinical aspects of many cutaneous lymphomas, as this recognition is essential for appropriate clinical management. As has been learned for nodal lymphomas, clinical features play an important role in prognosis and should be used in guiding therapy. For cutaneous lymphomas, the presence or absence of systemic spread is particularly important.
Hodgkin Disease↗