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Biomedical subjects

E Grosshans

Publications and source records attributed to E Grosshans.

At least 73 records · Page 4Linked to original sources
PubMed2000

Schnitzler syndrome: heterogeneous immunopathological findings involving IgM-skin interactions.

The Schnitzler syndrome is the association of chronic urticaria, intermittent fever, osteosclerotic bone lesions and a monoclonal IgM gammopathy. It is not yet firmly established whether the monoclonal immunoglobulin component plays a part in the pathophysiology of the urticarial lesions. Immunoblotting on epidermal and dermal human skin extracts as well as immunoelectron microscopic (IEM) studies on Lowicryl K4M-embedded skin sections were performed in three patients with the Schnitzler syndrome. Western blotting on epidermal extracts showed the presence of IgM-kappa antiskin autoantibodies in two patients. These antibodies displayed the same isotype as the monoclonal components and recognized a 280-290-kDa antigen in one patient and a 200-kDa antigen in the other patient. IEM studies showed sparse IgM deposits in the epidermis, around the keratinocytes, near the desmosomes in one patient and dense deposits below the lamina densa, in the region of the anchoring fibrils, in another patient. Antiskin IgM autoantibodies of the same isotype as their monoclonal gammopathies can be present in the serum of some patients with the Schnitzler syndrome. These IgM antibodies seem to deposit in vivo in the epidermis and at the dermal-epidermal junction, in the region of the anchoring fibrils. These findings suggest that the monoclonal gammopathy plays a part in the pathophysiology of the skin rash. They also suggest patient heterogeneity both in the skin antigens that are recognized as well as in their localization.

Autoantibodies↗
PubMed2000

Epithelial sheath neuroma: a new entity.

The authors describe four examples of a peculiar cutaneous lesion characterized histopathologically by a proliferation of enlarged nerve fibers ensheathed by squamous epithelium involving the superficial dermis. The perineural epithelial sheaths were composed of uniform squamous epithelium with evidence of cornification in the form of dyskeratotic cells or resulting in orthokeratotic basket-weave corneocytes. Immunohistochemical studies confirmed the epithelial and neural nature of the two components of the lesions, with the nerve fibers expressing immunoreactivity for S-100 protein, neurofilaments, CD57, and nerve growth factor receptor, whereas the perineural epithelial sheaths showed immunoreactivity for cytokeratins. The authors propose the term "epithelial sheath neuroma" for this lesion and believe that it is a distinct and a previously undescribed benign neoplasm of both cutaneous nerves and epithelial elements.

Aged↗
PubMed2000

[Cutaneous actinomycosis in the perianal area and buttocks].

INTRODUCTION: Actinomycosis is a rare spreading suppurative and granulomatous disease. In 50 to 60 p. 100 of cases, cervico-facial area is involved. Involvement of other cutaneous areas is very uncommon. CASE-REPORTS: Two men (51 and 58 year-old, respectively) were admitted to the hospital because of erythematous and violaceous nodules with fistulization to the skin, localized on the perianal area and buttocks for several months. Bacterial cultures of the pus and of skin biopsy specimens yielded Actinomyces meyeri in case 1 and Actinomyces israelii in case 2. Patients did not have other infectious lesions. Treatment included systemic antibiotics and surgical iterative excisions in case 1. DISCUSSION: The main interest of those two cases is their similar clinical presentation and in one case the rarity of the micro-organism involved (Actinomyces meyeri). Since the lesions involved only the skin, we postulate that the organism may have extended from the oral mucosa through the gastrointestinal tract.

Actinomycosis↗
PubMed2000

[Hereditary progressive mucinous histiocytosis].

BACKGROUND: Hereditary progressive mucinous histiocytosis is an uncommon condition which to date has only been observed in women. The disease is a non-Langerhans histiocytosis with a stereotypic clinical presentation limited to the skin. The clinical, histological and genetic features of this disease differ considerably from other types of histiocytoses and overload diseases. We report the first case observed in France. CASE REPORT: A 49-year-old woman consulted for asymptomatic papules on the dorsal aspect of the hands known since childhood. The lesions had progressively spread to other regions of the body. The woman's mother and two sisters had the same condition but her two sons and her brother and nephews did not. Standard semi-thin slices and electron microscope histology demonstrated dense cellular infiltration of the reticular dermis with interstitial mucinous overload, voluminous histiocytes with characteristic toluidine blue positive granulations, and a cytoplasm rich in myelin and Zebra bodies respectively. The observed clinical, histological and genetic features were characteristic of progressive hereditary progressive mucinous histiocytosis. DISCUSSION: The pedigree in our case and in those reported in the literature suggest a dominant hereditary condition. The fact that only female cases have been reported to date would suggest X-linked transmission with early death of male fetuses. An autosomal dominant transmission limited to woman as a result of mitochondrial heredity or hormone-related phenomena cannot be ruled out. The progressive extension with age without a tendency for spontaneous regression and the electron microscopy aspect suggest a phospholipid deposit disorder similar to that observed in lysosomial diseases. However, primary macrophage proliferation in response to an unknown stimulus cannot be excluded.

Disease Progression↗
PubMed1999

[Benign tumors of the skin and its appendages].

At least, about a hundred of benign specific tumours originating in the epithelial and connective structures of the skin are nosologically individualized. In this count are not included all the clinicopathological variants of these entities and the numerous lesions which only look like tumours such viral warts or cheloidal scars. The most frequent benign skin tumours are seborrhoic keratoses, nevocytic nevi, dermatofibromas and hemangiomas. In this practical review we describe the most common tumours which have some clinical hallmarks and can be easily recognized without a histological checking.

Hemangioma↗
PubMed1999

Chronic urticaria is not significantly associated with hepatitis C or hepatitis G infection: a case-control study.

OBJECTIVE: To study the prevalence of hepatitis C virus (HCV) and hepatitis G virus (HGV) infection in patients with chronic urticaria. DESIGN: Prospective case-control study and literature review. SETTING: Dermatology department of an academic medical center in Strasbourg, France. PATIENTS: One hundred ten consecutive patients with typical urticaria lasting longer than 2 months were seen between March 1, 1997, and August 31, 1998. None had a history of viral hepatitis. Age- and sex-matched patients (n = 110) seen in the same department and during the same period were included for controls. None of the controls had a history of urticaria, pruritic dermatosis, or hepatitis. MAIN OUTCOME MEASURES: The detection of HCV antibodies through a third-generation enzyme-linked immunosorbent assay. To detect early HCV infection without plasmatic antibodies, genomic amplification of HCV RNA was carried out in all patients using 2 different methods. Hepatitis G virus RNA was detected only by genomic amplification. All measures were planned before data collection. RESULTS: Antibodies to HCV were found in 1 patient with urticaria and in 1 of the control group (0.9% of each group). None had circulating HCV RNA, and liver function test results were within the reference range. Genomic amplification without HCV antibodies was not observed. Two patients with urticaria and 2 of the control group (1.8% of each group) had circulating HGV RNA, but they had neither coinfection with HCV nor changes in their liver function test results. CONCLUSIONS: Systematic HCV screening in patients with chronic urticaria is not cost-effective, at least in Europe, because hepatitis C rates were similar to those of the general population. We could not confirm the hypothesis that urticaria occurs in an early phase of HCV infection-ie, before evidence of HCV can be detected by serologic testing. Hepatitis C virus is unlikely to be the cause of urticaria in the infected patient detected in this study because of the absence of HCV RNA and changes on liver function tests. Hepatitis G virus is also unlikely to be a cause of urticaria, as the rate of HGV positivity in this study was even lower than that in the general French population.

Adult↗
PubMed1999

Expression of human hair keratin basic 1 in pilomatrixoma. A study of 128 cases.

Hard keratins are expressed in normal hair and nails, and are characterized by a higher cysteine content than cytokeratins. Previous studies have suggested a coexpression of hard keratins and cytokeratins in pilomatrixoma, a benign follicular tumour which could originate from the hair matrix. Human hair keratin basic 1 (hHb1) is a newly characterized hair keratin which is expressed specifically by cortical cells of the normal hair shaft. A preliminary study has suggested that hHb1 could be expressed in pilomatrixoma. In order to confirm this hypothesis, we have studied a series of 128 pilomatrixomas by in situ hybridization, using a 35S-labelled hHb1-specific probe. The anti-sense probe was used as a negative control. Among these pilomatrixomas, six were early cases, 60 were classified into the intermediate stage (either fully developed or early regressive cases) and 62 were late regressive tumours made of shadow cells only. Forty-seven tumours showed hHb1 expression (37%), all being intermediate stage pilomatrixomas. The areas positively stained by the probe were band-like structures made of transitional cells only, which were very close to cells showing tricholemmal keratinization features. Neither the basophilic matrix cells nor the shadow cells expressed hHb1. Our results suggest that pilomatrixomas can differentiate towards cortical cells during their maturation process, as this keratin is specifically expressed in the cortex of the normal hair shaft. These data are consistent with previous studies which showed the expression of a hard keratin group in transitional cells by immunohistochemistry. The histogenesis of basophilic cells of pilomatrixoma is controversial, but it is likely that transitional cells represent an equivalent of the hair cortex.

Adolescent↗
PubMed1999

Lack of clinical-pathological correlation in the diagnosis of congenital naevi.

Congenital naevocellular naevi (CNN) have classical histological criteria that are thought to allow distinction from acquired naevi. These criteria are mainly the horizontal distribution of melanocytes, forming 'Indian files', and a prominent adnexotropism. In order to check whether the previously described criteria were reliable, we analysed 1349 unselected consecutive cases of naevocellular naevi excised in children under 16 years, during a 54-month period. The slides were analysed in order to determine by histological analysis only if they could be classified as CNN. These results were then compared with the clinical files, in which only the most reliable data from parental and/or medical observations were included. Of the 1349 naevi, 659 had the typical histological criteria of CNN, 32 of them being deep CNN, characterized by massive involvement of the lower dermis and hypodermis. The comparison with clinical data showed that 32 naevi with the histological criteria of congenital naevi were actually acquired, and that 179 naevi present at birth did not fulfil these criteria. This study shows that the classic histological criteria are not absolutely specific and are poorly sensitive as 36% of naevi present at birth lacked the classic criteria. The most specific criteria of true CNN were the involvement of eccrine glands and presence of melanocytes in the septae. In the case of deep CNN which corresponded to large or very large naevi, the clinicopathological correlation was 100%. Deep CNN could easily be distinguished from superficial CNN and often exhibited many histological changes consistent with a complex hamartoma, such as presence of brown fat tissue, abnormal vessels and numerous large terminal follicles. In conclusion, our study suggests that it is not possible to predict, by histological analysis alone, that a naevus was present at birth, except in deep CNN which are likely to be a separate entity among all congenital naevi. Studies dealing with congenital naevus-associated melanoma should take into account the lack of sensitivity of these criteria.

Adolescent↗
PubMed1999

Expression of stromelysin 3 in keratoarcanthoma and squamous cell carcinoma.

Stromelysin 3 (ST3) is a member of the metalloproteinase family, which is expressed in the skin during wound healing and in the stroma of basal cell carcinoma. A high level of expression of ST3 has been observed in carcinomas of poor prognosis. In benign tumors, though, ST3 is not expressed or is at a low level. We have immunohistochemically studied the expression of ST3 in 89 randomly selected cases of squamous cell carcinomas (SCCs), 104 of keratoacanthomas (KA), and 23 cases of metastatic SCC. Stromelysin 3 was expressed only by fibroblasts surrounding the tumors and not by epithelial cells. The proportion of tumors positively stained was 22% of KA, 47% of randomly selected SCC, and 70% of metastatic SCC. In areas of poorly demarcated neoplastic cells, a reinforcement of the staining was observed in the stroma. The intensity and dispersion of staining were used to determine the level of expression. There were significantly more SCC in the groups of high expression levels, and both parameters were significantly higher in SCC than in KA. Expression of ST3 in benign tumors is unusual. Its expression in the the stroma of keratoacanthomas can be related to the high tissue remodeling activity observed in these tumors. It also could be interpreted as in favor of the neoplastic nature of KA. Nevertheless, the level of expression was higher in SCC than in KA and seemed to be related to the prognosis of these tumors. These results correlate well with those obtained in breast cancers and in noncutaneous SCC.

Carcinoma, Squamous Cell↗
PubMed1999

The severity of histopathological changes of leukocytoclastic vasculitis is not predictive of extracutaneous involvement.

Leukocytoclastic vasculitis is defined by histologic features and can be observed in a wide range of entities. Independent of the causative disease, extracutaneous complications are frequent, mainly in the kidneys and gastrointestinal tract. It has been suggested that the severity of histological changes could correlate with the clinical course of the disease. We have therefore compared the severity of histological changes of leukocytoclastic vasculitis to clinical and laboratory findings indicative of extracutaneous involvement in a large group of patients. Among 289 patients followed for cutaneous vasculitis, we included 184 patients with purpuric papules and proven leukocytoclastic vasculitis who all had standardized investigations. A cutaneous biopsy was performed early and standardized laboratory investigations were carried out. The slides were retrospectively randomized and the depth of vasculitis and severity of vascular necrosis were determined according to a semiquantitative scale. These data were compared to the renal, gastrointestinal and articular symptoms using Fischer's exact test, Chi-square test and variance analysis. The intensity of vascular necrosis and the depth of vasculitis were no more severe in patients having renal changes, gastrointestinal involvement or articular symptoms. Both variance analysis and Chi-square tests failed to show a significant increase in the severity score in patients having extracutaneous complications. In this study, the severity of histopathological changes was not predictive of extracutaneous involvement. Thus it appears that the degree of involvement of the cutaneous vessels probably does not correlate with that of vessels in visceral organs.

Adolescent↗
PubMed1999

Differentiating squamous cell carcinoma from keratoacanthoma using histopathological criteria. Is it possible? A study of 296 cases.

BACKGROUND: Squamous cell carcinoma (SCC) and keratoacanthoma (KA) are sometimes difficult to distinguish by histopathological examination, since cytological features are similar in both tumors. Distinctive criteria - mainly architectural - have therefore been proposed as an aid in diagnosis. OBJECTIVE: The purpose of this study was to evaluate the reliability of some of the criteria used to make a distinction between SCC and KA. METHODS: 296 fully excised tumors previously classified as SCC or KA were randomized and examined independently by two examiners. Fourteen criteria, mainly based on the architecture of the tumors, were determined on the 262 slides for which a consensual diagnosis was made. RESULTS: No single criterion was sufficiently sensitive and specific to allow a clear-cut differential diagnosis. The 5 most relevant criteria were epithelial lip, sharp outline between tumor and stroma in favor of KA and ulceration, numerous mitoses and marked pleomorphism/ anaplasia in favor of SCC. Intraepithelial polymorphonuclear abscesses, intraepithelial elastic fibers, parakeratosis and dyskeratosis and extension more lateral than downward were not distinctive criteria, although they are considered as classic distinctive features. CONCLUSION: Many of the criteria commonly used for the differential diagnosis of SCC and KA are not reliable. The combination of the 5 most useful criteria does not significantly increase the specificity or sensitivity of the histological diagnosis in difficult cases. Atypical or difficult cases should therefore be considered and treated as SCC, since a clear-cut distinction is not possible even with the aid of the most relevant criteria.

Carcinoma, Squamous Cell↗