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Biomedical subjects

K Konrad

Publications and source records attributed to K Konrad.

At least 55 records · Page 3Linked to original sources
PubMed1989

[Hyalinosis cutis et mucosae of the ear-nose-throat].

Hyalinosis cutis et mucosae (HCM) is a rare autosomal recessive disease of unknown aetiology and pathogenesis. In the skin and mucous membranes there is characteristically a deposition of hyaline-like material in the papillary dermis, around the small blood vessels and around skin appendages. Besides the manifold skin lesions, the mucous membranes affected are found in the nasal and oral cavities, pharynx and larynx. The latter causes the most characteristic symptom, namely, hoarseness, from birth. The lesions of the vessel walls are the most significant histopathological alterations. Ultrastructurally, massive deposits of amorphous, hyaline-like material in the dermis, reduction in number and size of the collagen fibrils and, finally, thickening of the basal laminae could be observed. The findings suggest an abnormal production of noncollagenous proteins as well as the alteration of the lysosomal systems of fibroblasts, endothelial cells and pericytes in the pathogenesis of hyalinosis.

Adult↗
PubMed1989

Multiple apocrine hidrocystomas on the eyelids.

A 31-year-old man with multiple cystic tumors symmetrically distributed on his eyelids is presented. Histopathology and immunohistochemistry suggest the diagnosis of apocrine hidrocystomas. Apocrine hidrocystomas occur frequently on the face, but multiple and symmetrical occurrence on the eyelids has not been reported up to now.

Adult↗
PubMed1989

Photosensitivity and hyperpigmentation in amiodarone-treated patients: incidence, time course, and recovery.

Amiodarone (AD) induces photosensitivity in 75% of the patients treated with this drug. Phototoxic reactions can be experimentally elicited with UVA but not with UVB. The UVA-MED is significantly reduced after 12 months of treatment. The development of photosensitivity depends on the total dose of AD; 40 g is the minimal cumulative dose requirement. Under the regimens commonly used, photosensitivity can be expected after 4 months of continuous AD treatment and appears to be unrelated to the skin type. Photosensitivity gradually decreases and returns to normal between 4 and 12 months after the withdrawal of AD. AD-related hyperpigmentation develops after an average of 20 months of continuous AD treatment and a minimal total dose of 160 g AD in about 8% of the patients (mainly of skin type I). Electron microscopic examination of the sun-exposed skin of patients without AD discoloration shows pigment deposits similar to those already described in patients with AD hyperpigmentation in exposed and non-exposed skin. Light and electronmicroscopic examination of sun-exposed skin of both clinically photosensitive and non-photosensitive patients reveals perivascular inflammation even in the absence of a clinical rash. Reduplications of vascular basal laminae occur in sun-exposed skin of both patients with and without UVA photosensitivity but are absent from non-exposed skin. In one patient, followed for 33 months after drug withdrawal, massive AD-induced hyperpigmentation was found to be reversible.

Amiodarone↗
PubMed1989

[Staphylococcal scalded skin syndrome in 2 adults with acute kidney failure].

We report on two cases of staphylococcal scalded skin syndrome (SSSS) in adults. In contrast to the previously reported cases our patients were neither immunosuppressed nor haemodialysed, nor did they have cancer. In both cases, acute renal failure in the presence of a rather banal staphylococcal infection preceded the SSSS. The pathogenic role of acute renal failure in the development of SSSS is discussed.

Acute Kidney Injury↗
PubMed1989

[Cornu cutaneum].

We report on 62 cases of cornu cutaneum. In contrast to the general opinion that the majority of these lesions arise on the basis of an underlying actinic keratosis or a squamous cell carcinoma, on histological examination we found actinic keratoses in only 25% and squamous cell carcinomas in only 3.2% of cases; 58% of all lesions were identified as common warts. We therefore recommend removal of these skin lesions by the shaving technique followed by electrodesiccation of the base. This has the advantage of supplying the histopathologist with a representative specimen for histological examination and avoiding an unnecessarily large surgical excision. In the rare cases of histologically confirmed squamous cell carcinoma, these lesions can be excised in a second session.

Aged↗
PubMed1988

[Histology of cutaneous AIDS manifestations].

An increasing number of skin and mucous membrane biopsies from AIDS patients is submitted for histopathologic examination. The dermatopathologist must be aware about the wide spectrum of AIDS-associated non-specific cutaneous manifestations, of cutaneous infections and of skin neoplasms. The histological criteria essential for the diagnosis of early Kaposi's sarcoma and for oral hairy leukoplakia are presented.

Acquired Immunodeficiency Syndrome↗
PubMed1988

[Initial clinical experiences in a large Austrian patient sample with immunodeficiency syndrome (AIDS): I. Type and course of encountered infections].

This study presents a report on the first clinical experiences gained in 68 hospitalized HIV antibody-positive patients from Austria, covering a period of twelve months. 36 patients (52.9%) belonged to risk group I or Ib (homo- or bisexual), whereas 26 (38.2%) patients were i.v. drug abusers (risk group II). 5 (7.4%) patients fulfilled the criteria of stage II of the CDC classification of HIV-associated clinical symptoms, 10 (14.7%) were classified as stage III and the remaining 53 patients (78%) as stage IV. The most frequent and also the most serious problem was the development of opportunistic infections. Multiple infections were found in 45.7% of all cases. Kaposi's sarcoma was found in 9 patients who all belonged to risk group I. During the entire observation period 10 patients died as a consequence of HIV-1-induced immunodeficiency and the resulting opportunistic infections and/or neoplasms.

Acquired Immunodeficiency Syndrome↗
PubMed1988

Wells' syndrome is a distinctive disease entity and not a histologic diagnosis.

Wells' syndrome is a distinctive dermatosis clinically resembling acute cellulitis with solid edema; it resolves spontaneously after weeks or months without residues. Recurrences over many years are common. Light microscopy is characteristic for the disease, with diffuse tissue eosinophilia and marked edema, fibrinoid "flame figures," and palisading microgranuloma. Vasculitis is never found. Eosinophilia of the peripheral blood is a frequent feature. Etiology and pathogenesis are unknown, but the disease has been found to be associated with hematologic disorders in several cases, and recurrences can often be related to infections, arthropod bites, drug administration, or surgery. The diagnosis of Wells' syndrome should be based on the typical clinical picture and the course of the disease with its recurrences and histopathology. Flame figures in histologic sections are an important diagnostic feature but not diagnostic per se for the disease because they represent a reaction pattern that can occur in other conditions. A dilution of Wells' syndrome by making flame figures the central criterion of diagnosis and by lumping all flame figure-positive skin reactions together is therefore unjustified.

Adrenal Cortex Hormones↗
PubMed1988

Epidermolysis bullosa herpetiformis Dowling-Meara in a large family.

A large Arab family, originating from Jerusalem, including 38 affected members (19 male and 19 female) with epidermolysis bullosa herpetiformis Dowling-Meara over four consecutive generations is described. Fourteen of 38 affected members of the family were examined clinically; their ages ranged from 2 to 35. The main clinical features were bullae, generalized, solitary, and in groups, with predilection to the skin of the palms and soles. Mild to moderate patchy hyperkeratosis of the palms and soles was found in 5 affected members of the family; their ages ranged from 2 to 7; 9 other affected adults were free. Blisters in oral mucous membranes were noted and found in summer and in periods of fever; hair, teeth, and nails were normal. Age of onset of the disease was from birth to 2 weeks. Expressivity appeared equally variable within and between sibships. Improvement was noted by progression of age from 5 to 23 years, and by some in summer and by others in winter. In contrast to previous reports, aggravation of the disease was noted during fever periods. Ultrastructural studies from a fresh blister disclosed intraepidermal blister via cytolysis of basal cell cytoplasm. The pedigree shows the transmission of an autosomal dominant gene. Affection of both consanguineous parents and their six offspring with epidermolysis bullosa herpetiformis Dowling-Meara is the most striking feature of the family--probably 25% of their offspring were homozygote.

Child, Preschool↗
PubMed1988

The phenotypic spectrum of histiocytosis X cells.

Proliferating cells in histiocytosis X (histiocytosis X cells) share many structural and immunophenotypic features with Langerhans cells, leading to the assumption that histiocytosis X represents a proliferative disorder of Langerhans cells. Because, depending on their state of activation and/or differentiation, Langerhans cells exhibit a varying immunophenotype, we investigated whether histiocytosis X cells display a similar phenotypic heterogeneity and, if so, whether the heterogenous biological behavior of histiocytosis X is reflected by differences in the immunophenotype of the proliferating cells. In 21 patients suffering from different clinical manifestations of histiocytosis X, proliferating cells uniformly expressed class I and II alloantigens, T200, CD1, CD4, and S100 protein. In 12 of 21 cases, histiocytosis X cells additionally exhibited immunocytochemically detectable amounts of C3b and C3bi receptors and certain monocyte/macrophage antigens (CDw14, Ki-M1, Ki-M6). This immunophenotypic heterogeneity of histiocytosis X cells could not be correlated with clinical course, prognosis, and final outcome of the disease in a given patient. The capacity of histiocytosis X cells to immunophenotypically mimic various states of Langerhans cell activation and/or differentiation, however, underscores the concept of histiocytosis X as a proliferative disorder of Langerhans cell origin.

Adolescent↗
PubMed1988

Langerhans' cells are an actual site of HIV-1 replication.

Human epidermal Langerhans' cells (LC) are HLA-DR+/DQ+, CD1+, CD4+ dendritic antigen-presenting leukocytes. Based on the observation that in certain human immunodeficiency virus type 1 (HIV-1) infected individuals, LC are the only epidermal cells to react with monoclonal antibodies against HIV-1 isolate termed human T-lymphotropic virus IIIB/83 core proteins p17 and p24, we have proposed that LC can serve as a target for HIV-1. This contention was strengthened by the ultrastructural finding of HIV-1-like particles in the close proximity of LC and by the demonstration of signs of moderate to severe LC damage. Detailed electron microscopic analysis of skin and mucosal biopsies from an AIDS patient with p17/p24-positive LC now revealed not only mature HIV-1-like virions in the extracellular space surrounding LC and in intracytoplasmic LC vacuoles, but also developmental forms of HIV-1-like particles budding from LC surface membranes. Using peripheral blood derived monocytes/macrophages as targets for HIV-1 isolation, a virus isolate, designated human T-lymphotropic virus III WR-SK/86, was recovered from skin tissue from this patient by cocultivation and identified as unique by nucleic acid hybridization analysis. These findings now conclusively show that HIV-1 replicates in and is released from LC and support the concept that antigen-presenting cells (mononuclear phagocytes, LC) can serve as a reservoir for the acquired immunodeficiency syndrome virus.

AIDS-Related Complex↗
PubMed1988

Verrucous malignant melanoma.

Five cases of verrucous malignant melanoma--a rare variant of melanoma that was described in 1967, but is rarely mentioned today--are presented and the clinical and histopathologic criteria defining this variant are discussed. The importance of this rare entity in the differential diagnosis of verrucous pigmented skin lesions is emphasized.

Adult↗
PubMed1988

[Acquired epidermolysis bullosa. A clinico-pathologic study].

Epidermolysis bullosa acquisita is an immunologically mediated mechano-bullous dermatosis presenting with a variety of clinical appearances. In mild cases the skin lesions, serous blisters, erosions and scars are restricted to the extremities. Scarring alopecia, dystrophy and even loss of nails as well as extensive erosions of mucous membranes may complicate severe cases. The characteristic histopathological feature is a subepidermal blister, which is located within the dermis by electron microscopy. Linear deposits of IgG and C3 along the basement membrane zone have been found on the dermal side of the lamina densa by immunoelectron microscopy. This close anatomical relationship with anchoring fibrils strongly suggests a functional disturbance of these anatomical structures, leading to dermolytic blistering. Treatment with sulphones, in combination with cortico-steroids in the more severe cases, has resulted in long-term improvement in two of our three patients.

Adult↗
PubMed1987

Vegetating cicatricial pemphigoid. A new subset of the cicatricial pemphigoid spectrum.

A case with widespread vegetating-pustular skin lesions, oral erosions, ulcerations and scarring, and conjunctival synechiae is reported. Clinically, histopathologically, and by immunofluorescence and electron microscopy this patient combined the features of pemphigoid vegetans, as described by Winkelmann and Su, and the mucocutaneous type of cicatricial pemphigoid. This observation suggests that a third subset of cicatricial pemphigoid can now be added to the two existing ones, the mucocutaneous and Brunsting-Perry types, and the designation vegetating cicatricial pemphigoid is proposed for this heretofore undescribed condition.

Aged↗
PubMed1987

Epidermal Langerhans cells--a target for HTLV-III/LAV infection.

Langerhans cells (LC) are bone marrow-derived, Ia+, CD1+, CD4+, ATPase+ dendritic antigen-presenting cells within the human epidermis. Since the CD4 molecule has been implicated as a receptor structure for HTLV-III/LAV (human T-cell leukemia virus/lymphadenopathy-associated virus), we asked whether LC from HTLV-III/LAV-seropositive individuals display signs of HTLV-III/LAV infection. In skin biopsies from 7/40 HTLV-III/LAV-infected persons (1 asymptomatic carrier, 2 patients with acquired immunodeficiency syndrome (AIDS)-related complex and 4 patients with AIDS), LC were the only epidermal cells to react with a monoclonal antibody specific for the HTLV-III core protein p17. A varying percentage of p17+ LC were morphologically altered with blunt dendrites and poorly demarcated cellular contours. In one of these biopsies, the presence of LC-associated viral particles characteristic of HTLV-III/LAV as well as cytopathic changes in approximately one-third of the LC population were demonstrated by electron microscopy. These results strongly suggest that LC may harbor HTLV-III/LAV. The infection of LC with this retrovirus may have deleterious consequences for the immunologic functions of this cell system and may thus contribute to both the acquisition of immunodeficiency and the infectious and neoplastic complications of AIDS.

Acquired Immunodeficiency Syndrome↗
PubMed1987

[The glucagonoma syndrome].

We report on our experience with two patients with glucagonoma syndrome and review the recent dermatologic literature. The clinical features are described with special emphasis on necrolytic migratory erythema, the characteristic cutaneous sign of glucagonoma syndrome. Using our histological and ultrastructural findings, we discuss the pathogenesis of necrolytic migratory erythema. Pathognomonic laboratory data and the diagnostic procedures recommended for the evaluation of patients with glucagonoma syndrome are presented. Finally, we discuss the differential diagnosis and describe therapeutic possibilities in the management of this syndrome.

Adenoma, Islet Cell↗
PubMed1987

[South African tick bite fever].

Two male patients developed South African tick bite fever after visits to South Africa. The clinical and laboratory findings in this condition, which is rarely seen in central Europe, are presented and its differential diagnosis to other rickettsial diseases is discussed.

Bites and Stings↗