Grotesque bilateral eyelid swelling as a symptom of Munchausen's syndrome.
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Adult↗
Biomedical subjects
S C van der Putte
Publications and source records attributed to S C van der Putte.
The small-cell variant of mycosis fungoides. A clinicopathological and quantitative electron microscopic study on 14 patients.
Small-cell variants of Sézary syndrome and mycosis fungoides (MF) have been described. However, in these studies the nuclear area of the small-cell variant of MF (SC-MF) as compared to histological classical MF (CL-MF) was not characterized objectively by quantitative electron microscopy. In a 14-year follow-up period, of a total of 76 patch/plaque stage MF patients seen in the Department of Dermatology of the University Hospital Utrecht, 14 (18%) had an infiltrate composed of atypical lymphocytes characterized by a distinctly smaller cell diameter and smaller, hyperchromatic, deeply indented nuclei as compared to the usual cell type of MF. The aim of the investigation was to confirm this observation objectively using quantitative electron microscopy (morphometry) and to define SC-MF as compared to CL-MF. The study was performed on the 14 patients with SC-MF, and 10 patients with clinical and histological CL-MF and 4 patients with chronic eczema. Electron micrographs of sections obtained from each biopsy were analysed by computer to produce the following data: a nuclear contour index (NCI), the mean nuclear area (MNA), the mean nuclear area of the cells above the 75th percentile (P75NA) and the percentage of cells larger than 30 microm2. The values of MNA differed significantly between patients with SC-MF and those with CL-MF (17.6 vs 23.2 microm2; P = 0.02), as did the values of P75NA (20.7 vs 27.9 microm2; P = 0.01). The NCI of the SC-MF and CL-MF patients were similar. These results are consistent with our observations that SC-MF does indeed exist.
Adult↗
Papillary "apocrine" fibroadenoma of the vulva.
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Apocrine Glands↗
p53 and bcl-2 expression do not correlate with prognosis in primary cutaneous large T-cell lymphomas.
Overexpression of p53 protein in cutaneous T-cell lymphoma (CTCL) has been reported in primary cutaneous large T-cell lymphomas (PCLTCL) and has been associated with tumor progression and transformation in mycosis fungoides. However, the prognostic significance of p53 expression has not been studied thus far. In the present study we investigated the expression of p53 as well as bcl-2 protein in 27 PCLTCL, including 19 CD30-positive and 8 CD30-negative lymphomas, retrieved from the registry of the Dutch Cutaneous Lymphoma Working Group. The results were correlated with follow-up data and proliferative activity, as assessed by the percentage of MIB-1 positive tumor cells. Overexpression of p53 protein, defined as nuclear staining of more than 5% of the tumor cells, was found in 10 of 27 cases (37%), including 6 of 19 (32%) CD30+lymphomas and 4 of 8 (50%) CD30-PCLTCL. bcl-2 protein was expressed in 6 of 19 (32%) CD30+lymphomas and in only 1 of 8 (12%) CD30-PCLTCL. However, no significant correlation between p53 or bcl-2 expression and prognosis was found, neither in the whole group, nor within the CD30+ or CD30- group. In addition, no relationship between p53 expression and proliferative activity was found. The results confirm that p53 expression is more common in PCLTCL than in mycosis fungoides and Sézary syndrome. However, neither p53 nor bcl-2 expression correlated with survival or proliferative activity.
Adult↗
Immunophenotyping and gene rearrangement analysis provide additional criteria to differentiate between cutaneous T-cell lymphomas and pseudo-T-cell lymphomas.
Differentiation between cutaneous pseudo-T-cell lymphomas and cutaneous T-cell lymphomas (CTCLs) may be extremely difficult. In this study, it was investigated whether demonstration of an aberrant phenotype and detection of clonal T-cell receptor gamma (TCR gamma) gene rearrangements can be used as additional differential diagnostic criteria. Immunohistochemical studies and TCR gamma gene rearrangement analysis using a polymerase chain reaction with primers specific for V gamma 1-8 and V gamma 9 gene segments in combination with denaturing gradient gel electrophoresis (PCR/ DGGE) were performed on frozen material of 11 pseudo-T-cell lymphomas and 17 CTCLs, including 9 cases of mycosis fungoides (MF) and 8 pleomorphic CTCLs. Clonal TCR gamma gene rearrangements were found in 66% of patch/plaque-stage MF and 100% of tumor-stage MF and pleomorphic CTCL, but not in any of 10 pseudo-T-cell lymphomas studied. Aberrant expression of CD2, CD3, and/or CD5 antigens was noted in 3 of 6 (50%) cases of patch/plaque-stage MF, all three cases of tumor-stage MF, and 5 of 8 (62%) pleomorphic CTCLs, but not in any of the 11 pseudo-T-cell lymphomas. Moreover, in pseudo-T-cell lymphomas exhibiting a nodular or diffuse growth pattern, a considerable admixture with reactive CD8+ T cells (15 to 60%), B cells (up to 20%), and macrophages was a characteristic finding. In conclusion, the results of this study suggest that demonstration of clonal TCR gene rearrangements and an aberrant phenotype, as well as demonstration of many admixed CD8+ T cells and B cells can be considered as useful additional criteria in the differentiation between pseudo-T-cell lymphomas and CTCLs.
Antigens, CD↗
Papuloerythroderma of Ofuji in a HIV-infected patient.
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Aged↗
The pathogenesis of familial multiple cylindromas, trichoepitheliomas, milia, and spiradenomas.
Early stages in the development of hereditary cylindromas associated with trichoepitheliomas, milia, and spiradenomas revealed that (a) trichoepitheliomas were derived from the bulges of hair follicles and probably represented an abnormal histogenesis from which rarely poorly differentiated trichoepitheliomas developed; (b) milia were the result of cystic alteration of the trichoepitheliomatous bulge proliferations; and (c) cylindromas and spiradenomas were different appearances of the same tumor and developed from two separate sources, follicular bulge proliferations and eccrine glands. From the bulge proliferations multiple cylindromatous buds developed that, by increasing in number, formed the classical cylindroma and, by increasing in size rather than in number, gave the spiradenomatous variant. Eccrine glands transformed into cylindromas by cylindromatous growth from the basal cell layer. Connections between the original structures and the tumors were mostly lost. Tumors from both sources revealed the same morphology, which was most indicative of eccrine differentiation including secretory and excretory elements. Apocrine differentiation was a rare and possibly secondary event. The multiplicity in derivation and differentiation suggest an adnexal progenitor cell as the most likely source.
Adenoma↗
Mammary Paget's disease confined to the areola and associated with multifocal Toker cell hyperplasia.
A hitherto unreported variant of mammary Paget's disease (MPD) limited to the areola, leaving the nipple unaffected, has been analyzed by serial sectioning of the whole areola and nipple. This otherwise characteristic MPD proved to be confined to the epidermis. There was no underlying carcinoma. This MPD was associated with a multifocal presence of monomorphic but otherwise similar cells in small collections surrounding the ostia of areolar mammary glands in the clinically unaffected area. This condition was interpreted as hyperplasia of mammary gland-related cells also found in normal nipples (so-called Toker cells). The observations hint at a possible derivation of some cases of mammary and extramammary Paget's disease from such Toker cells.
Antigens, Neoplasm↗
Cysts of mammarylike glands in the vulva.
A hidrocystoma which originated in a mammarylike gland (MLG) of the vulva is described. It involved the main collecting duct and was characterized by a peripheral basal layer of myoepithelium and a luminal layer of cuboidal to columnar cells with discrete cytoplasmic snouts and by lobules opening into the cavity. Adhering to it were cutaneous glands, including MLGs, and dartos muscle. These features distinguish it from the other cysts of the vulva, including vestibular, urogenital sinus, and keratinous cysts. Another cystic lesion of the MLGs was evidenced only microscopically and involved multiple whole glands. It resembled similar alterations in eccrine, apocrine, and mammary glands, and most likely represented involution cysts of MLGs.
Adult↗
The diagnostic value of morphometry on blood lymphocytes in erythrodermic actinic reticuloid.
BACKGROUND: As the differential diagnosis of erythrodermic actinic reticuloid vs Sézary syndrome (SS) can be very difficult, we examined the value of the nuclear contour index (NCI) on blood lymphocytes as the criterion for differential diagnoses. The NCI is defined as the nuclear parameter divided by the square root of the nuclear area. Three different parameters were studied: mean NCI, percentage of cells with an NCI of 6.5 or greater, and the highest NCI. These indexes were studied on blood lymphocyte samples obtained from 10 patients with erythrodermic actinic reticuloid and were compared with the findings in 10 patients with other benign forms of erythroderma and in seven patients suffering from SS. RESULTS: The patients with erythrodermic actinic reticuloid differed significantly from the group with SS regarding the percentage of cells with an NCI of 6.5 or greater and the highest NCI, but not when the mean NCI was considered. All three parameters revealed nonsignificant results for erythrodermic actinic reticuloid compared with other benign forms of erythroderma. The group with SS differed significantly from the patients with other benign forms of erythroderma regarding all three parameters. By combining three morphometric criteria (mean NCI, > or = 5.5; > 30% lymphoid cells with an NCI of > or = 6.5; and highest NCI, > or = 11.5), all patients with erythrodermic actinic reticuloid or other benign forms of erythroderma and six of the seven patients with SS were correctly classified. CONCLUSION: Our data indicate that assessment of the NCI on peripheral blood lymphocytes is of value in the differential diagnosis of erythrodermic actinic reticuloid vs SS.
Aged↗
Apoeccrine glands in nevus sebaceus.
Apoeccrine glands were found in 13 of 57 specimens of nevus sebaceus. The glands showed the basic structure of eccrine glands, but differed in that a part of the secretory duct was lined by characteristic apocrine epithelium. The apocrine alteration varied from slight apocrine changes in a few secretory cells to an almost complete alteration of the secretory epithelium in other glands. The association with changes in the excretory ducts of rare glands suggested a transformation of eccrine into apocrine glands. It was nevertheless considered as most likely that a majority of the apocrine glands in nevus sebaceus had developed in the "classic" way in association with pilosebaceus structures. In nevus sebaceus, apoeccrine glands appeared to represent eccrine glands with a variable degree of apocrine metaplasia that could result in complete transformation rather than the formation of a separate category of specialized sweat glands. The capacity of eccrine epithelium to transform into apocrine epithelium is why difficulties are experienced in the precise categorization of sweat gland neoplasms into eccrine and apocrine tumors.
Adipose Tissue↗
Mammary-like glands of the vulva and their disorders.
Supernumerary mammary glands derived from rudiments of the embryonic milk lines or mammary ridges in the vulva are considered a source of a series of unusual tumors resembling lesions of the breasts. This phenomenon was reevaluated in light of recent observations of mammary-like anogenital glands (MLG), which are a normal constituent of the vulva and appear to be closely related to eccrine glands. An analysis of the literature reveals that the concept of "milk lines" originated in a mixture of phylogenetic and ontogenetic theories at the beginning of this century and was not supported by observations in human embryos, which show that primordia of the mammary glands do not extend beyond the axillary-pectoral area. The breasts and the vulva are so widely separated by time and space that the vulvar MLG can not be derived from the mammary ridges or milk lines. The profile of the MLG, which can also reveal some eccrine or apocrine features, makes these glands the most likely source of a series of lesions occurring in the anogenital region and comprising lactating glands, lactating adenoma, fibroadenoma, hidrocystoma, hidradenoma papilliferum, and most cases of extramammary Paget's disease and invasive adenocarcinoma.
Adenoma, Sweat Gland↗
Adenocarcinoma of the mammary-like glands of the vulva: a concept unifying sweat gland carcinoma of the vulva, carcinoma of supernumerary mammary glands and extramammary Paget's disease.
An unusual case of primary adenocarcinoma of the vulva is described. It combined features of the three different types of adenocarcinoma of the skin of the vulva which are currently recognized, i.e. sweat gland carcinoma, adenocarcinoma derived from supernumerary mammary glands, and extramammary Paget's disease (EMPD). Central in this tumor was a recently recognized type of cutaneous gland which appeared special for the anogenital region and was distinguished because it combined morphological features of eccrine, apocrine and mammary glands. As it most resembles mammary glands, it is named "mammary-like gland". On the basis of the case presented and of a critical review of the literature, it was concluded that, with the exception of a few sweat gland carcinomas similar to those elsewhere in the skin, adenocarcinomas of the skin of the vulva form a single category of neoplasms with a variable expression of features reminiscent of eccrine, apocrine and mammary gland carcinomas. The data strongly suggested a common derivation from the mammary-like gland or, in cases of EMPD, its related germinative cells in the epidermis.
Adenocarcinoma↗
Cutaneous cylindroma with malignant transformation.
BACKGROUND: Malignant cutaneous cylindroma is a rare tumor. It has been described in 26 cases, both in the solitary form and in the autosomal dominant inherited multiple tumor form. The authors present two new cases that occurred in one family with a history of multiple cylindromas. METHODS: Clinical and histopathologic data of both tumors were compared with those of 26 other cases in the literature. Immunohistochemical examinations were performed. RESULTS: The malignant tumors were distinguished from the benign lesions by rapid growth, long-standing ulceration, or bleeding. Histopathologic examination showed a well-differentiated carcinoma in one patient and a poorly differentiated tumor in the other. In the latter, lymph node metastasis developed, and the patient died 2.5 years later. Histopathologic criteria of malignancy included cell pleomorphism, frequent mitoses and loss of jigsaw pattern, peripheral palisading, hyaline sheaths, and dual cell population. CONCLUSIONS: These observations are in accord with those in the literature. Malignant cutaneous cylindroma developed more often in the multiple tumor form than in the single tumor form. Malignant cylindroma is an aggressive carcinoma with a tendency to local destructive growth and metastases.
Aged↗
Immunophenotyping on simultaneously occurring plaques and tumours in mycosis fungoides and Sézary syndrome.
Skin biopsy specimens from 15 patients with mycosis fungoides and Sézary syndrome, with simultaneously occurring plaques and tumours, were examined to assess phenotypic deviation. We focused on immunophenotypic differences between the two types of lesions with respect to the T-cell markers CD2, CD3, CD4, CD5 and CD8. In six patients (40%) loss of one or more T-cell markers occurred in at least one of the lesions. Three of the patients studied (20%) showed a difference in immunophenotype between plaques and tumours, with an additional loss of one of the T-cell markers in the tumours (respectively, CD5, CD2 and CD4). All three of these patients showed a larger number of blast cells in the tumour compared with the plaque. No correlation between this loss of antigenicity and the prognosis was observed. The results of this study show that different immunophenotypes can occur simultaneously in an individual patient. Furthermore, we were able to confirm a relationship between the number of intraepidermal CD1+ cells in plaque lesions and the prognosis.
Antigens, CD↗
Familial Jessner's lymphocytic infiltration of the skin, occurring in a father and daughter.
Familial occurrence of Jessner's lymphocytic infiltration of the skin is reported in a father and daughter. A definitive diagnosis of Jessner's disease was greatly facilitated by the presence of large numbers of plasmacytoid monocytes within the dermal infiltrates in both patients. A role for skin-directed lymphocyte migration is suggested to account for the presence of this peculiar cell type.
Adult↗
Cutaneous immunocytomas: a clinicopathologic study of 26 cases.
The clinical and histological features of 16 patients with a primary cutaneous immunocytoma and 10 patients with a secondary cutaneous immunocytoma are reported. In all cases the diagnosis was based on the presence of monotypic plasma cells or lymphoplasmacytoid cells. Our data show that primary cutaneous immunocytomas are a distinct type of cutaneous lymphoma, characterized by (a) the presence of solitary or localized skin lesions (13 of 16 cases); (b) preferential localization on arms and legs (15 of 16 cases); (c) excellent response to local treatment (15 of 16 cases) and (d) a favourable prognosis. Histologically, these primary cutaneous immunocytomas are characterized by the presence of nodular or diffuse infiltrates with monotypic lymphoplasmacytoid/plasma cells located at the periphery of the infiltrates. Important clinical and histological differences were noted between primary and secondary immunocytomas. In the latter group more widespread skin disease was seen, often in the presence of paraproteins and/or autoimmune diseases. In contrast with the peripheral localization of the monotypic cells in primary cutaneous immunocytomas the monotypic lymphoplasmacytoid/plasma cells in secondary immunocytomas formed diffuse infiltrates or these cells were found dispersed throughout the infiltrate. There were no differences in clinical presentation or course between the different subtypes of cutaneous immunocytomas (lymphoplasmacytic, lymphoplasmacytoid and polymorphic immunocytomas). The differential diagnosis between primary cutaneous immunocytomas and cutaneous plasmacytomas, primary follicular centre cell lymphomas and cutaneous 'pseudolymphomas' is discussed.
Adult↗
Epitopes of human immunodeficiency virus regulatory proteins tat, nef and rev are expressed in skin in atopic dermatitis.
We extend our previous documentation that epitopes of HIV regulatory proteins tat, rev, and nef are expressed in tissue from uninfected individuals by the immunohistochemical analysis of normal skin (n = 10) and skin in some selected inflammatory dermatoses including urticaria (n = 6), systemic lupus erythematosus (n = 6), and atopic dermatitis (affected skin, n = 10, and after epicutaneous patch test for allergens, n = 8). A rabbit antibody to HIV-2 tat did not show immunolabeling of skin. Blood vessel endothelium was immunolabeled by one of two antibodies applied to HIV-1 tat, by an antibody to HIV-1 rev, and by two antibodies to HIV-1 nef. In addition one of the anti-nef antibodies labeled Langerhans cells. The anti-rev antibody labeled Langerhans cells and melanocytes in the epidermis, and dendritic cells in the dermis. The labeling of these skin components did not differ in prevalence between controls and groups of dermatosis. For other components, diseased skin conditions especially atopic dermatitis showed additional labeling. In affected skin, keratinocytes were labeled by antibodies to rev and one of two antibodies to nef. Skin after epicutaneous allergen patch testing also showed a statistically significantly increased prevalence of immunolabeling of dendritic cells and Langerhans cells by one of the anti-tat antibodies. We conclude that skin components show expression of epitopes recognized by antibodies to HIV-1 tat, rev, and nef; this expression is more extensive in atopic dermatitis than in normal skin, and can be further increased after epicutaneous allergen patch testing.(ABSTRACT TRUNCATED AT 250 WORDS)
Adult↗