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Pemphigus vegetans of Hallopeau: immunofluorescent studies.

Immunofluorescent techniques were used in the study of tissue from a patient with pemphigus vegetans of Hallopeau. The findings were typical of pemphigus, confirming the propriety of classifying this entity as a benign variant of pemphigus, rather than a disease of purely microbial etiology. The question is raised whether other types of vegetating pyodermas actually are separate diseases or represent a spectrum of pemphigus with the clinical appearance dependent on the patient's immunity to his disease. Immunofluorescent studies are proposed as a basis for classification.

Aged↗

[Psychogenic depressions in forensic psychiatric practice].

Analyzes the problems of diagnosis, classification, and assessment of psychogenic depression in forensic psychiatric practice. Clinical manifestations of such depressions are variable and numerous; the range of psychogenic depressions includes the borderline and psychotic forms each of which may be a separate entity or represent a stage in the development of a reactive depression. Distinguishing of these variants may be reasonable to assess the severity of a depressive disorder when deciding expert problems and for assessing the capacity of the subject exposed to expert evaluation to take advantage of his or her legal rights.

Adjustment Disorders↗

[Criteria of the evaluation of tubal function in patients with secretory otitis media].

Tubular function was assessed in patients with secretory otitis media before and after surgery (an original version of anterior tympanostomy). Preoperative tubular function was measured by impedance tympanometry. After tympanostoma was placed the acoustic tube was subjected to direct manometry. Perioperative measurements were made for tube patency for liquid (hydrocortisone). The tests of pressure adjustment implied routine spirit manometry and updated impedancemetry. Technical specifications of modern impedance meters met the criteria of clinical fitness for post-tympanostomy patients. The criteria of assessment and correlating quantitative values of the draining and ventilation capacities of the acoustic tube were determined. This was the basis for an original classification of tubular function grades. It was established that in secretory otitis media drainage dysfunction of the acoustic tube was registered in two thirds of the patients (66%), ventilatory dysfunction occurred in 100% of patients. There was a correlation between these functions. This fact was used for design of a simple screening variant of ear manometry i.e. registration of the threshold of acoustic tube passive opening. Screening manometry is convenient for following up postoperative changes in the tubular function and defining duration of carrying tympanostomy in secretory otitis.

Acoustic Impedance Tests↗

Cutaneous squamous cell carcinoma: a comprehensive clinicopathologic classification--part two.

Cutaneous squamous cell carcinoma (SCC) includes many subtypes with widely varying clinical behaviors, ranging from indolent to aggressive tumors with significant metastatic potential. However, the tendency for pathologists and clinicians alike is to refer to all squamoid neoplasms as generic SCC. No definitive, comprehensive clinicopathological system dividing cutaneous SCCs into categories based upon their aggressiveness has yet been promulgated. Therefore, we have proposed the following based upon the malignant potential of SCC variants, separating them into categories of low (< or = 2% metastatic rate), intermediate (3-10%), high (greater than 10%), and indeterminate behavior. Low-risk SCCs include SCC arising in actinic keratosis, HPV-associated SCC, tricholemmal carcinoma, and spindle cell SCC (unassociated with radiation). Intermediate-risk SCCs include adenoid (acantholytic) SCC, intraepidermal epithelioma with invasion, and lymphoepithelioma-like carcinoma of the skin. High-risk subtypes include de novo SCC, SCC arising in association with predisposing factors (radiation, burn scars, and immunosuppression), invasive Bowen's disease, adenosquamous carcinoma, and malignant proliferating pilar tumors. The indeterminate category includes signet ring cell SCC, follicular SCC, papillary SCC, SCC arising in adnexal cysts, squamoid eccrine ductal carcinoma, and clear-cell SCC. Subclassification of SCC into these risk-based categories, along with enumeration of other factors including tumor size, differentiation, depth of invasion, and perineural invasion will provide prognostically relevant information and facilitate the most optimal treatment for patients.

Carcinoma, Squamous Cell↗

Cutaneous squamous cell carcinoma: a comprehensive clinicopathologic classification. Part one.

Cutaneous squamous cell carcinoma (SCC) includes many subtypes with widely varying clinical behaviors, ranging from indolent to aggressive tumors with significant metastatic potential. However, the tendency for pathologists and clinicians alike is to refer to all squamoid neoplasms as generic SCC. No definitive, comprehensive clinicopathological system dividing cutaneous SCCs into categories based upon their aggressiveness has yet been promulgated. Therefore, we have proposed the following based upon the malignant potential of SCC variants, separating them into categories of low (< or = 2% metastatic rate), intermediate (3-10%), high (greater than 10%), and indeterminate behavior. Low-risk SCCs include SCC arising in actinic keratosis, HPV-associated SCC, tricholemmal carcinoma, and spindle cell SCC (unassociated with radiation). Intermediate-risk SCCs include adenoid (acantholytic) SCC, intraepidermal epithelioma with invasion, and lymphoepithelioma-like carcinoma of the skin. High-risk subtypes include de novo SCC, SCC arising in association with predisposing factors (radiation, burn scars, and immunosuppression), invasive Bowen's disease, adenosquamous carcinoma, and malignant proliferating pilar tumors. The indeterminate category includes signet ring cell SCC, follicular SCC, papillary SCC, SCC arising in adnexal cysts, squamoid eccrine ductal carcinoma, and clear-cell SCC. Subclassification of SCC into these risk-based categories, along with enumeration of other factors including tumor size, differentiation, depth of invasion, and perineural invasion will provide prognostically relevant information and facilitate the most optimal treatment for patients.

Carcinoma, Squamous Cell↗

Acute monocytic leukemias.

Normal monocytes and macrophages are characterized by peculiar ultrastructural and cytochemical features and, in addition, show characteristic membrane properties, a variety of special functional capacities, and important secretory activities. Almost all these cytological features can also be detected in leukemic monocytic cells, and it is quite conceivable that these peculiar features may influence or determine the clinical syndrome associated with the leukemic accumulation of monocytic cells. The morphological identification of monocytic leukemias is a very intriguing diagnostic problem and some controversies still exist concerning their cytological classification. For clinical as well as scientific purposes the diagnosis of monocytic leukemias should rely on the demonstration of specific monocytic features of the leukemic cells. Clinical findings frequently associated with acute monocytic leukemia include increased frequency of leukemic tissue infiltrations as well as increased tendency to hypokalemia and - especially in the "immature" variants - to disorders of hemostasis.

Cell Adhesion↗

[Lipomatous tumors of the skin and soft tissue. New entities and concepts].

Mesenchymal neoplasms of lipogenic differentiation are the most common soft-tissue neoplasms in adults. In recent years a number of "new" entities and variants have been described. Their recognition is important to avoid diagnostic pitfalls and inappropriate therapy. Chondroid lipoma, myolipoma, and purely cutaneous spindle-cell/pleomorphic lipoma are biologically benign neoplasms which may mimic sarcomas morphologically. Despite high-grade morphology cutaneous liposarcomas carry a favorable clinical prognosis. Spindle cell liposarcoma is a rare variant of atypical lipomatous tumour (well-differentiated liposarcoma) and must be distinguished from dedifferentiated liposarcoma with metastatic potential and benign spindle cell lipoma. The advent of cytogenetic and molecular investigations of lipomatous neoplasms has contributed to a better understanding of the biology of these neoplasms and led to a modification of conventional classification schemes.

Diagnosis, Differential↗

Association between sleep apnea and reactive astrocytes in brainstems of victims of SIDS and in control infants.

Among 27,000 infants studied prospectively to characterize their sleep-wake behavior, 38 infants died under 6 months of age; 27 infants died from sudden infant death syndrome (SIDS), 5 from congenital cardiac abnormalities, 2 from infected pulmonary dysplasia, 2 from septic shock with multi-organ failure, 1 with a prolonged seizure, and another with prolonged neonatal hypoxemia. The frequency and duration of sleep apneas recorded some 3-12 weeks prior to the infants' death were analyzed. Brainstem material was retrospectively collected from these 33 infants and studied in an attempt to elucidate the relationship between sleep apnea and hypoxic gliosis. The findings were compared between the SIDS victims and the control infants. Brainstem materials were immunohistochemically studied for quantitization of reactive astrocytes using an anti-glial fibrillary acidic protein (GFAP) antibody. The pathological materials were collected within 24h of death. This study focuses on the association between respiratory characteristics and pathology. Physiological and pathological data in the arousal pathway of the brainstem were linked for each infant and variant-covariant analyses were carried out using physiological data as dependent variables and pathological data and categorical data to evaluate the association with SIDS or non-SIDS as independent variables. The study failed to statistically support an association between hypoxic loads, reflected by the GFAP-positive reactive astrocytes in brainstems, the classification of being SIDS or non-SIDS infants, and the characteristics of sleep apnea.

Analysis of Variance↗

Primary lymphoma of the liver: clinical-pathological features and relationship with HCV infection in French patients.

Primary lymphoma of the liver (PLL) is rare. In some cases, the hepatic lymphoma has been diagnosed in patients who were infected by the hepatitis C virus (HCV). It has been suggested that HCV plays a role in the pathogenesis of lymphoma. The aim of our multicentric retrospective study was to assess the characteristics of PLL and to determine the prevalence of HCV infection in PLL. Thirty-one immunocompetent patients (anti-human immunodeficiency virus, anti-human T-cell leukemia/lymphoma virus negative, no history of allograft) with PLL fulfilled the entire selection criteria. The liver biopsy specimens were reassessed by the same pathologist. The non-Hodgkin's lymphomas were classified according to the World Health Organization classification. Blood samples were tested in 28 patients for antibodies to HCV, and HCV RNA was detected by reverse transcription polymerase chain reaction. In the majority of cases, the clinical, biologic, and radiologic data were nonspecific. Twenty-seven of 31 patients presented a B-cell lymphoma corresponding to the centroblastic morphologic variant of a diffuse, large B-cell lymphoma (22 cases), a Burkitt's lymphoma (1 case), an extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue type (3 cases), and unclassified, small B-cell lymphoma (1 case). The 4 other cases were T-cell lymphomas. The prevalence of HCV infection was 21% (6 of 28 cases). All of these patients were positive for HCV RNA by polymerase chain reaction in blood. Most of the HCV-infected patients presented a high-grade, B-cell type lymphoma. In conclusion, our study confirms the rarity of PLL and demonstrates an increased prevalence of HCV infection.

Adult↗

Winchester syndrome caused by a homozygous mutation affecting the active site of matrix metalloproteinase 2.

The inherited osteolysis syndromes are a heterogeneous group of skeletal disorders whose classification is still uncertain. Three osteolysis syndromes show autosomal recessive inheritance and multicentric involvement: Torg syndrome (OMIM 259600), Winchester syndrome (OMIM 277950) and Nodulosis-Arthropathy-Osteolysis syndrome (NAO; OMIM 605156). The 2001 Nosology of the International Skeletal Dysplasia Society (Hall CM, Am J Med Genet 2002: 113: 65) classifies NAO as a variant of Torg syndrome, while Winchester syndrome is considered as a separate disorder. Recently, mutations in the matrix metalloproteinase 2 (MMP2) gene were identified in affected individuals with a clinical diagnosis of NAO in two Arab families. We report a homozygous missense mutation (E404K) in the active site of MMP2 in a 21-year-old woman with a severe form of osteolysis best compatible with a diagnosis of Winchester syndrome. The clinical and molecular findings suggest that Torg, NAO and Winchester syndromes are allelic disorders that form a clinical spectrum.

Adult↗

Banding studies on chromosomes in diffuse "histiocytic" lymphomas: correlation of 14q+ marker chromosome with cytology.

Chromosomes were studied in cells from tissues primarily involved by diffuse "histiocytic" lymphoma in nine patients. Two of the patients had stage II disease; their tumors were fibrotic and had no mitotic cells. One patient was in stage III, and the remaining six patients had stage IV disease. The modal chromosome number of abnormal cells from these last seven patients was hypodiploid in two, hyperdiploid in four, and near-triploid in one. Complete banding studies of six cases and partial analysis of the seventh indicate that (1) every patient had a distinct cell line with common markers, with a few cells showing minor variants; (2) although certain chromosomes (Nos. 1, 2, 3, 9, 12, and 14) were structurally affected more often than others, no markers with the same banding pattern were noted among them; and (3) the cytologic type of lymphoma could be correlated with the karyotype in all seven patients. When the Lukes and Collins classification was used, three patients whose tumors were composed predominantly of large noncleaved cells showed a 14q translocation leading to the formation of a 14q+ marker chromosome. This marker was not observed in four patients whose tumors had a majority of large cleaved cells. These preliminary results, if confirmed in a larger series of patients, will provide additional evidence that there are consistent chromosome changes associated with specific subtypes of lymphoproliferative disorders analogous to the Ph1 chromosome in chronic myelogenous leukemia.

Chromosome Aberrations↗

Expression of CD44 standard and variant-v6 proteins in transitional cell bladder tumours and their relation to prognosis during a long-term follow-up.

The expression of the standard CD44 (CD44s) and its v6 isoform (CD44v6) was analysed immunohistochemically in 173 cases of transitional cell bladder cancer. The results of immunohistochemical analyses were related to established prognostic factors and clinical follow-up data. The expression intensity of CD44s in non-basal tumour cells was significantly related to TN classification, S-phase fraction (SPF), mitotic index, grade, density of tumour infiltrating lymphocytes. The expression intensity of CD44v6 in non-basal tumour cells was inversely related to DNA ploidy, SPF, and mitotic index. The expression intensity of CD44v6 in basal tumour cells was also inversely related to T-category, grade, papillary status, DNA ploidy, SPF, and mitotic index. Strong expression of CD44s in non-basal tumour cells was related to unfavourable outcome in univariate analysis (p = 0.008), whereas the strong expression of CD44v6 in both non-basal cells (p = 0.005) and basal cells (p = 0.0008) was related to high survival probability. In multivariate survival analysis, the expression intensity of CD44v6 was independently related to favourable outcome in muscle invasive tumours, while in superficial tumours, CD44s was an independent prognostic factor. The results suggest that the expression of CD44s and CD44v6 is associated with malignant features and prognosis in bladder cancer.

Adult↗

Downregulation of p27KIP1 and Ki67/Mib1 labeling index support the classification of thyroid carcinoma into prognostically relevant categories.

The cyclin-dependent kinase inhibitor p27KIP1 has been proposed as a valuable prognostic indicator for a variety of human neoplasms. Immunohistochemical reactivity for p27KIP1 and the proliferation marker Ki67/Mib1 were investigated in 90 thyroid carcinomas of follicular cell origin. The neoplasms were divided into three prognostic groups on the basis of their morphologic features: group 1, well-differentiated papillary or follicular carcinomas with favorable pathologic features (43 papillary carcinomas and 4 minimally invasive follicular carcinomas); group 2, papillary or follicular carcinomas with unfavorable pathologic features (21 poorly differentiated carcinomas and 2 papillary carcinomas, tall cell variant); and group 3, undifferentiated, or anaplastic, carcinomas. p27KIP1 expression (p = 0.007) and Ki67/Mib1 labeling index (p = 0.02) showed a strong correlation with the subdivision of the thyroid carcinomas in the three prognostic groups with a significant linear trend for tumors with low p27KIP1 (p = 0.002) and high Ki67/Mib1 labeling index (p = 0.005) to segregate into the unfavorable categories (groups 2 and 3). Low p27KIP1 expression, but not cellular proliferation, was related to adverse prognostic factors, such as large tumor size (p = 0.03) and extrathyroidal extension (p = 0.01), but the correlation was not independent of the subdivision in the three groups. Low p27KIP1 expression (p = 0.03) and high proliferative rate (p = 0.02) were associated with poor survival, reflecting the close association between patient morbidity and mortality rates and tumor differentiation. No significant association could be seen between p27KIP1 or cellular proliferation and clinicopathologic parameters (e.g., age, sex, tumor size, extrathyroidal extension, vascular invasion, lymph node metastases, distant metastases, tumor stage, and survival rate) within any of the groups, or the histologic diagnosis of papillary versus follicular carcinoma irrespective of their degree of differentiation. Modulation of p27KIP1 and cellular proliferation patterns in thyroid carcinoma correlate with tumor differentiation and support the morphologic classification of thyroid carcinoma into prognostically relevant categories.

Adenocarcinoma, Follicular↗

A comparison of the performance of different methods of disease classification for rheumatoid arthritis. Results of an analysis from a nationwide twin study.

OBJECTIVE: We have assessed the importance of the distinction between classification criteria for rheumatoid arthritis (RA) that recognize the presence of currently active disease from those that, in addition, incorporate evidence of past disease activity in ascertaining disease occurrence. We applied 7 classification schemes to a population of twins with inflammatory arthritis to determine (a) the number of individuals classified as RA positive by each scheme and hence the effect on the estimate of disease concordance in the twins and (b) their performance in correctly assigning a diagnosis compared with a physician's opinion. METHODS: The schemes assessed were the 1958 ARA (Rome) criteria which detect active disease, the 1966 New York (using both the 2/4 and 3/4 published cutoffs) which detect "ever" disease and 4 variants of the 1987 ARA criteria. These were the 4/7 and decision tree approaches applied on the basis of the relevant features (1) being present at the time of the study and (2) being present ever and allowing current joint deformity to substitute for absent joint swelling. RESULTS: In all, 283 individuals with a history of joint swelling were assessed, 255 of whom were considered to have RA by their physician. Criteria used to recognize "current" RA identified only about 70% of those which recognized RA ever. These differences in ascertainment level produced a marked effect on the monozygotic twin RA concordance estimates with percentages ranging from 10 to 18%. The results from receiver operating curves confirmed that criteria used to assess only current RA were too insensitive to be of value. Of the criteria that recognized RA status ever the 1987 ARA performed best overall. CONCLUSION: The use of classification methods that incorporate past as well as current evidence of disease activity is essential to avoid important misclassification in epidemiological and family studies. The 1987 criteria, applied retrospectively and allowing joint deformity to substitute for swelling, are of enhanced value over other existing schemes.

Adult↗

Erythrokeratodermas: a classification in a state of flux?

The term 'erythrokeratodermas' or 'erythrokeratodermias' has been applied to a group of inherited disorders characterized by well-demarcated erythematous lesions and hyperkeratotic plaques. Connexin mutations have been demonstrated to be responsible for most cases of erythrokeratoderma variabilis but there remain some cases without demonstrated connexin mutations, suggesting genetic heterogeneity. The position of progressive symmetric erythrokeratoderma has become rather unclear. Loricin mutations have been found in some cases that clinically resemble variant Vohwinkel syndrome and other cases have features that overlap with those of erythrokeratoderma variablis. Whether progressive symmetric erythrokeratoderma exists as a distinct entity is under question.

Child↗

Eccrine angiomatous hamartoma: a multiple variant.

A case of multiple eccrine angiomatous hamartoma present in a boy since birth is reported. Clinically, this condition must be differentiated from other neonatal angiomatoses. Sometimes the clinical findings are nonspecific, whereas histologic examination may exclude angiomatoses with visceral involvement. In our case the hamartomatous nature of this tumor is documented also by the presence of pilar structures intimately related to the eccrine-angiomatous complex in one of two lesions histologically examined. Therefore, the histologic classification of eccrine angiomatous hamartoma into subgroups seems to be excessive.

Child, Preschool↗

[Common disorders and diseases of the nails. Anatomy, physiology, disorders, clarification and therapy].

Disorders of anatomical variants of the nails are often seen in clinical practice. Their correction identification and interpretation are essential dermatological skills. The increasing emphasis on aesthetic considerations in dermatology means that even the slightest nail changes may assume significance for their patients. In order to skillfully evaluate nails, one must be familiar with the terminology and classification of nail disorders. The nail plate, nail bed and periungual tissue may also be affected. Longitudinal melanonychia is especially important as a possible clue to subungual melanoma.

Diagnosis, Differential↗

Inter- and intra-specific variation within the genus Psoroptes (Acari: Psoroptidae).

In this paper the taxonomy and relative importance of various species within the genus Psoroptes are reviewed with respect to inter- and intra-specific variations in cross infestivity and host specificity, site of infestation and morphological characters. Intra-specific variation within populations of the sheep scab mite, Psoroptes ovis will then be discussed critically with respect to virulence (the relative growth of the scab lesion and mite burdens), epidemiology, susceptibility to the systemic acaricide, ivermectin, and resistance to synthetic pyrethroid and organophosphate acaricides. Finally, it is suggested that the ear mite, P. cuniculi, and the sheep scab mite, P. ovis are variants of the same species. Information was collected from published literature and from the results of unpublished studies carried out at the VLA, Weybridge over the last 10 years.

Animals↗