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Biomedical subjects

H Hintner

Publications and source records attributed to H Hintner.

108 records · Page 6Linked to original sources
PubMed1981

Immunofluorescence mapping of antigenic determinants within the dermal-epidermal junction in the mechanobullous diseases.

The classification of mechanobullous diseases often depends on the electron microscopic distinction of intradermal (dermolytic), junctional and intraepidermal sites of cleavage. Electron microscopy is tedious and time consuming. In this report we describe a different approach to the determination of the cleavage plane by using a method which recognizes subtle differences in the localization of antigenic structures relative to the cleavage plane. Cryostat sections of lesional and extralesional skin of 3 patients with dermolytic epidermolysis bullosa, 3 with epidermolytic epidermolysis bullosa and 8 with junctional epidermolysis bullosa were examined by immunofluorescence, with specific antisera against type IV collagen (localized within the basal lamina); against laminin (noncollagenous protein, localized in the lamina lucida); and with bullous pemphigoid antibodies (directed against the bullous pemphigoid antigen localized in the lamina lucida). All specimens were also examined by electron microscopy. In dermolytic epidermolysis bullosa (where cleft formation occurs intradermally) type IV collagen, laminin and the bullous pemphigoid antigen were consistently found in the roof of the blister, whereas in junctional epidermolysis bullosa (where the cleft occurs in the lamina lucida) type IV collagen and laminin were found on the floor of the blister whereas bullous pemphigoid antigen was present mainly on the roof, but focally also on the floor, of the blister. In epidermolytic epidermolysis bullosa (where the cleft is intraepidermal) all antigens were localized below the cleavage plane. In all cases electron microscopy confirmed the level of cleft formation predicted from the immunofluorescence mapping of the antigenic sites. The described method equals electron microscopy in accuracy but it is more rapid and simpler to perform.

Collagen↗
PubMed1981

Hormone binding globulin levels in patients with hereditary angiooedema during treatment with Danazol.

Five patients with hereditary angiooedema were treated with Danazol for periods up to 10 months. This therapy resulted in normalization of serum levels of the inhibitor of the first complement component and disappearance of clinical disease symptoms in all patients. Although a variety of hormones were measured, only those of the pituitary-gonadal axis altered during treatment: a moderate decrease in leuteinizing hormone levels and a sharp fall of testosterone in adult men were observed. In all patients, however, both sex hormone binding globulin and thyroxine binding globulin concentrations were considerably suppressed during Danazol therapy. However, levels of corticosterone binding globulin and thyroxine binding globulin concentrations were considerably suppressed during Danazol therapy. However, levels of corticosterone binding globulin remained unchanged. The fraction of endogenous testosterone not bound to sex hormone binding globulin invariably increased. The possible relevance of these findings to the understanding of the mode of action of Danazol both in these patients and in general, are discussed.

Adult↗
PubMed1981

[Pyoderma gangraenosum].

Pyoderma gangrenosum is a relatively rare, destructive, inflammatory disease of unknown cause which may present as a purely cutaneous disorder or may be associated with an underlying internal disease (ulcerative colitis, Crohn's disease, rheumatoid arthritis, multiple myeloma, lymphoma and others). Four selected patients are described which reflect the clinical spectrum of this condition; these cases and a review of the literature serve as a background for analysis of pyoderma gangraenosum as an entity and a discussion of its pathogenesis.

Aged↗
PubMed1981

[Fasciitis with eosinophilia - Shulman syndrome].

Fasciitis with eosinophilia (also Shulman's disease or eosinophilic fasciitis) is characterized by an inflammatory thickening of the fascia, eosinophilia and hypergammaglobulinemia. It has clinical and histopathological similarities with scleroderma, though evidence for systemic involvement is rarely found. In this report, we describe the clinical and laboratory features of two patients with this disease and their response to treatment and we discuss its relationship to scleroderma and the pseudosclerodermatous syndromes and its prognosis.

Adult↗
PubMed1981

[The multiple hamartoma syndrome (Cowden syndrome)].

The multiple hamartoma syndrome is a genetic disorder of autosomal dominant inheritance. It is characterized by an impressive diversity and multitude of both mesodermal and epithelial hamartomas and tumors of all organ systems. The dermatological hallmarks of this probably not too rare syndrome are lichenoid centrofacial and akral papular lesions and a marked papillomatosis of the entire oral mucosa of a highly characteristic morphology which usually extends throughout the entire length of the gastrointestinal tract. The importance of this syndrome is its frequent association with malignant tumors, predominantly carcinomas of the mammary and thyroid glands. In this paper, we describe the first three cases of the German literature. No malignancies were detected; in one case, a meningeoma causing severe increase of intracranial pressure was discovered.

Adult↗
PubMed1980

Expression of basement membrane zone antigens at the dermo-epibolic junction in organ cultures of human skin.

Using the epithelial outgrowth in organ cultures of human skin ("epiboly") as a model system for basement membrane zone neogenesis, the emergence of various antigenic determinants of the junction zone (bullous pemphigoid antigen, type IV collagen and laminin) was studied and the time sequence of their appearance assessed. All 3 antigens were found at the newly built dermo-epibolic junction; their synthesis, however, followed a distinct time sequence: bullous pemphigoid antigens emerged synchronously with the advancing tip of the migrating epithelium, whereas type IV collagen and to a greater extent, laminin, appeared with considerable delay. At the ultrastructural level, the formation of basal lamina accompanied the emergence of type IV collagen and laminin.

Antigen-Antibody Reactions↗
PubMed1980

[Netherton syndrome: weakened immunity, generalized verrucosis and carcinogenesis].

Two patients with Netherton's syndrome are presented who, in addition to the classical triad of trichorrhexis invaginata, ichthyosis linearis circumflexa and atopy, exhibit signs of reduced cellular immunity (negative skin tests to a battery of microbial antigens, impaired stimulation of lymphocytes with various mitogens and widespread viral warts resembling epidermodysplasia verruciformis). One patient had a squamous cell carcinoma possibly arising from a viral wart. It is suggested that Netherton's syndrome is part of the group of immunodeficiency syndromes with atopy-like symptoms.

Adult↗
PubMed1979

[Treatment of common warts with topically applied DNCB (author's transl)].

Twenty-one patients suffering from multiple common warts were treated by topically-applied dinitrochlorobenzene (DNCB). After an average treatment period of 8.7 weeks, the warts had completely disappeared in twelve patients. Therapeutic failure must be admitted in five patients, whereby, sensitization to DNCB was not possible in two of these cases. Four patients did not complete the therapeutic regimen. Indications, advantages, disadvantages and side effects of this therapeutic method are discussed and an attempt is made to explain the underlying mechanism of this form of treatment.

Administration, Topical↗
PubMed1979

[Danazol treatment of hereditary angioneurotic oedema (author's transl)].

Danazol, an attenuated androgen, was administered to four patients with hereditary angioneurotic oedema, with rapid and complete response without side-effects. The follow-up period has now been up to 17 months. In all patients there was an indirect indication that their hormonal state influenced the course of the disease.

Adult↗
PubMed1979

Hereditary angio-oedema: treatment with danazol. Report of a case.

An 8-year-old boy with hereditary angio-oedema was treated with danazol under close endocrinological supervision. The boy's C1 esterase inhibitor (C1inh) and C4 levels increased rapidly to near normal values under a daily dose of 400 mg and were maintained at about 50% of the normal by maintenance doses of 200 mg danazol every other day. Throughout the entire treatment period (11 months) the boy has been maintained free from attacks of angio-oedema. No hormonal imbalance was detected in the follow-up period. Our results indicate that danazol should be suitable for the treatment of HAE not only in adults but also in prepubescent children.

Angioedema↗
PubMed1979

[Disseminated perforating annular granuloma].

A case of disseminated perforating granuloma annulare is presented. Concomitant diseases and exogeneous factors previously described as eliciting mechanisms were not found. Treatment with corticosteroids or cloroquin was not successful. All lesions were cleared with photochemotherapy within eight weeks.

Female↗
PubMed1979

Lichen planus and bullous pemphigoid.

A patient is described who had clinical and histopathological features of lichen planus and bullous pemphigoid; deposits of in vivo bound C3 at the basement membrane zone, which, at the electron microscopy level, were deposited in the lamina lucida; and with circulating IgG antibasement membrane zone antibodies which exhibited a pronounced C3 binding capacity. The similarity of our case to others described in the literature suggests that bullous lichen planus in fact represents the coexistence of two distinct diseases, namely lichen planus and bullous pemphigoid.

Adolescent↗
PubMed1979

[Necrotizing vasculitis: its manifestation as urticaria].

A case of urticarial vasculitis, a distinct form of necrotizing vasculitis is described. This entity is characterized by a chronic, persistent urticaria with petechiae and the histological features of necrotizing vasculitis. Serum complement is depressed and multisystem involvement can often be found. There is evidence for immune complexes to play a role in the pathogenesis of this disease.

Female↗
PubMedDate not supplied

Squamous cell carcinoma in junctional and dystrophic epidermolysis bullosa.

We report here on three patients suffering from recessive dystrophic epidermolysis bullosa and one suffering from generalized atrophic benign epidermolysis bullosa, all of whom developed cutaneous squamous cell carcinoma. Our observations and a review of the literature suggest that squamous cell carcinoma in generalized atrophic benign epidermolysis bullosa is very infrequent and has a better outcome compared to skin cancer in recessive dystrophic epidermolysis bullosa. These differences could be explained by the distinct pathophysiology and clinical course of each of these variants of epidermolysis bullosa. In contrast to UV-induced skin cancer, the tumours in epidermolysis bullosa develop on distal extremities at sites of chronic wound healing. The cases reported here underline the exceptional importance of early histopathological assessment of suspicious skin lesions in patients with epidermolysis bullosa.

Adult↗
PubMedDate not supplied

Ocular involvement in anti-epiligrin cicatricial pemphigoid.

PURPOSE: To report an anti-epiligrin cicatricial pemphigoid (AECP) patient with severe ocular involvement and to provide a practical approach to distinguishing AECP patients from those with other subepidermal blistering diseases. METHODS: Techniques included direct and indirect immunofluorescence microscopy, Western blot and immunoprecipitation studies, as well as interdisciplinary examinations of mucous membranes and skin. RESULTS: This study describes a patient with clinical features of cicatricial pemphigoid, circulating anti-basement membrane zone IgG antibodies, and subepidermal blisters. Histopathology and immunofluorescence analysis suggested the diagnosis of a cicatricial pemphigoid-like type of epidermolysis bullosa acquisita. However, Western blot and immunoprecipitation studies demonstrated that the patient's serum contained autoantibodies against laminin 5 alpha3 subunit, leading to the diagnosis of an AECP. CONCLUSION: Since patients with AECP have an increased relative risk for malignant tumors, it is important to distinguish this entity within the spectrum of cicatricial pemphigoid patients by additional studies such as Western blot or immunoprecipitation.

Autoantibodies↗