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Biomedical subjects

H Hamm

Publications and source records attributed to H Hamm.

At least 145 records · Page 8Linked to original sources
PubMed1988

Congenital atrichia with nail dystrophy, abnormal facies, and retarded psychomotor development in two siblings: a new autosomal recessive syndrome?

We cared for two sisters, ages 3 and 4 years, who suffered from congenital atrichia. Scalp biopsies performed on both children revealed a marked atrophy of hair follicles with rudimentary hair shafts. The absence of peribulbar infiltrates ruled out alopecia areata. Dystrophy of all nails, distinctive facies, retarded psychomotor development, and a delay in speaking were additional symptoms. The unique combination of findings excludes well-established syndromes such as atrichia with papular lesions, GAPO syndrome, and dominant hidrotic ectodermal dysplasia, as well as X-linked hypohidrotic ectodermal dysplasia. We therefore conclude that we may be dealing with a new genetic entity. The occurrence of the disorder in two siblings with unaffected parents suggests an autosomal recessive mode of inheritance.

Alopecia↗
PubMed1988

Epidermolytic palmoplantar keratoderma of Vörner: is it the most frequent type of hereditary palmoplantar keratoderma?

In a retrospective study, we reevaluated the biopsies that had been obtained, during the past 11 years, from 26 patients presenting with hereditary palmoplantar keratoderma (PPK). Twelve out of 26 biopsies disclosed the histological features of epidermolytic hyperkeratosis, consistent with the diagnosis of epidermolytic PPK of Vörner. A review of the histologically examined cases of the literature revealed a comparable predominance of this hereditary PPK. We conclude that, in contrast to the current opinion, epidermolytic PPK of Vörner represents the most frequent type of hereditary PPK.

Child, Preschool↗
PubMed1988

[Skeletal changes following long-term treatment with retinoids].

The synthetic retinoids, the vitamin-D-derivatives etretinate and isotretinoin, have substantially enlarged the therapeutic arsenal in dermatology. They are primarily used in severe cases of acne and cornification disorders. In the majority of cases, long-term treatment is necessary. Certain side effects in the skeletal system can occur, e.g., osteoporosis, premature epiphyseal closure, and changes similar to DISH (diffuse idiopathic skeletal hyperostosis). We discuss the reports in the literature and our own observations in 31 patients treated at the Westphalian Wilhelms University in Muenster, as well as at the Technical University in Munich. In 3 out of 31 patients treated by retinoids on a long-term basis, skeletal changes were found radiologically as a result of the retinoid medication.

Adolescent↗
PubMed1987

Abnormal expression of class I and class II major histocompatibility antigens in alopecia areata: modulation by topical immunotherapy.

Fifty-eight scalp biopsies were immunohistologically investigated with monoclonal antibodies against HLA-ABC, HLA-DR, and T6 antigens. The following 3 groups were compared: control biopsies obtained from healthy volunteers (n = 5) or patients with unrelated scalp diseases (n = 6); biopsies from untreated alopecia areata (AA), obtained either from untreated patients (n = 19) or from the untreated side in patients receiving unilateral treatment with the contact allergen diphencyprone (DCP) (n = 13); biopsies obtained from the treated side in patients receiving unilateral treatment with DCP (n = 13). While HLA-ABC antigens were strongly expressed by epidermal keratinocytes and the infundibular epithelium of hair follicles in all biopsies, these antigens were either not detectable or only faintly expressed on the subinfundibular epithelium and the hair matrix in the control series. By contrast, 30 out of 32 biopsies from untreated AA showed expression of HLA-ABC antigens on hair matrix epithelium, and the subinfundibular epithelium was HLA-ABC-positive in 15 out of 32 cases. In the biopsies from treated AA, HLA-ABC antigens were expressed on hair matrix epithelium in 9 out of 13 cases, and on the subinfundibular epithelium in 1 case. In the controls and untreated AA, HLA-DR expression was confined to dendritic cells in the epidermis and the follicular infundibulum. Its expression on hair matrix epithelium was found in 15 out of 32 biopsies from untreated AA and in 4 out of 13 biopsies from treated AA. In the control series, intrabulbar T6+ dendritic cells were either absent or present in low numbers. High numbers of intrabulbar T6+ cells were present in 7 out of 32 biopsies from untreated AA and in 0 out of 13 biopsies from treated AA. The data show that abnormal expression of class I major histocompatibility (MHC) antigens on hair matrix epithelium is a constant feature in AA, whereas class II MHC antigens are less frequently expressed. Topical immunotherapy with DCP, which induced expression of HLA-DR in epidermal keratinocytes in 6 out of 13 cases, reduced the abnormal expression of both HLA-ABC and -DR antigens in the epithelium of lower hair follicles in AA.

Alopecia Areata↗
PubMed1987

Multiple agminate Spitz naevi: review of the literature and report of a case with distinctive immunohistological features.

We describe a 13-year-old girl with multiple pigmented nodules and plaques arranged in a cluster in the right lumbar region, which had developed since infancy. Eleven of 15 lesions which were examined histologically were found to be Spitz naevi. The remaining four lesions were compound naevocellular naevi, and two of them showed focal dysplasia. Eight Spitz naevi were investigated immunohistologically with monoclonal antibodies against HLA-antigens and malignancy-associated melanocytic antigens which are rarely present in common naevi. Naevus cells in all lesions expressed HLA-ABC antigens, but lacked HLA-DR antigens in seven of the eight lesions. All naevi were positive for 'constitutive' (KG-6-56) and 'early' (K-1-2) markers of naevomelanocytic cells. In five of the eight Spitz naevi, at least one of the three malignancy-associated melanocytic antigens PAL-M1, A-1-43 and A-10-33 was found. The expression of malignancy-associated antigens in multiple agminate Spitz naevi is at variance with their benign clinical course.

Adolescent↗
PubMed1987

Cholesterol in pleural effusions. A diagnostic aid.

In this prospective study of 70 patients with pleural effusion, the underlying disease could be identified in 62 cases. By predefined criteria, 31 of these effusions were classified as transudates and 31 as exudates. Pleural fluid protein content, LDH activity and cholesterol level were measured to investigate their utility in differentiating the exudates from the transudates. Protein and LDH levels, and their pleural fluid-to-serum ratios, resulted in erroneous classification of 11 to 15 percent of the effusions. Mean cholesterol level in malignant effusions was 94 mg/dl, 76 mg/dl in inflammatory effusions and 30 mg/dl in the transudates. Using a dividing line of 60 mg/dl to separate the exudates from the transudates, only 5 percent were incorrectly classified. Elevated cholesterol levels in exudates seem to be independent of the serum levels. Our findings indicate that the pleural fluid cholesterol level is a simple and cost-effective aid in differentiating exudative from transudative pleural effusions.

Cholesterol↗
PubMed1986

Rhodopsin and retinoblastoma. A monoclonal antibody histopathologic study.

Rhodopsin was identified in formaldehyde-fixed, paraffin-embedded human fetal retina, and in five retinoblastomas using monoclonal antibody (MAb) MAb-E. The binding pattern corresponding to rhodopsin immunoreactivity was then compared with S-antigen using another monoclonal antibody, MAbA9-C6. Rhodopsin and S-antigen were first observed in the 18-week-old human fetal eye, at a stage preceding photoreceptor differentiation. In adult eyes containing normal photoreceptor cells, rhodopsin immunoreactivity was restricted to the rod outer segments, whereas S-antigen immunoreactivity was localized to the entire photoreceptor cell. In retinoblastomas both monoclonal antibodies bound to the same area of the tumor; however, different and distinct staining patterns associated with each monoclonal antibody were recognized. In four cases, an intense well-circumscribed "halo" pattern, characteristic of cell-surface binding, was associated with rhodopsin, whereas the binding pattern associated with S-antigen was intense, well localized, and cytoplasmic in all cases. Our results show that some well-differentiated retinoblastomas express both rhodopsin and S-antigen, and as such express proteins that participate in the initial events in the phototransduction of vision.

Adult↗
PubMed1986

Inflammatory linear verrucous epidermal nevus (ILVEN) in a mother and her daughter.

Inflammatory linear verrucous epidermal nevus (ILVEN) is a rare skin disease with characteristic clinical and histological changes. The approximately 100 cases so far reported include only one familial occurrence involving a woman and her nephew. We report the occurrence of typical ILVEN in a 47-year-old mother and her 17-year-old daughter. So far, no clear-cut genetic interpretation of this observation can be given. Apart from coincidence, various genetic explanations are considered, including X-linked inheritance with extreme lyonization.

Adolescent↗
PubMed1986

Ichthyosis bullosa of Siemens: a unique type of epidermolytic hyperkeratosis.

We report the second family of ichthyosis bullosa, an entity that was first described by Siemens in 1937 and since then has fallen into oblivion. Clinically, ichthyosis bullosa is characterized by blistering resembling epidermolysis bullosa simplex and by generalized, yet circumscribed dark gray hyperkeratoses covering mainly the arms and the legs. Lichenification and superficially denuded areas (mauserung) are further prominent features. Histology disclosed intracorneal blister formation corresponding to the mauserung phenomenon and epidermolytic hyperkeratosis that was confined to the granular layer and to the uppermost layers of the prickle cells. On electron microscopic examination the keratinocytes of these layers displayed structural alterations of tonofilaments as usually observed in epidermolytic hyperkeratosis. Thus ichthyosis bullosa shares with bullous ichthyosiform erythroderma blistering and epidermolytic hyperkeratosis, but can be distinguished from this wellknown disease by the lack of erythroderma, by the mauserung phenomenon, by the confinement of acanthokeratolysis to the superficial layers of the epidermis, and by intracorneal blistering.

Adult↗
PubMed1986

Extramammary Paget's cells: further evidence of sweat gland derivation.

Tissue from a 66-year-old male patient with extramammary Paget's disease of the right side of the scrotum and the right groin was studied for the presence of several antigens with the immunoperoxidase technic. Adenokeratin (cytokeratin No. 18) and carcinoembryonic antigens were positive in Paget's cells, whereas squamokeratin (cytokeratin No. 10) was expressed only in normal epidermal cells. Langerhans cells were decreased in the region of the tumor. Many transferrin receptors were present on the tumor cells, indicating a high cellular proliferation rate. Enzyme histochemical studies of the extramammary Paget's cells showed positive reactions for several enzymes typical of sweat glands, except for leucine aminopeptidase, which was negative. A comparison with three other cases showed that these enzyme reactions varied greatly from case to case. Both immunohistochemical and enzyme histochemical findings provide further evidence that extramammary Paget's cells are related to sweat gland epithelial cells, with variable expression of cellular characteristics.

Aged↗
PubMed1986

[Mixed vascular nevus. Report of 4 cases].

We describe four patients with a combined vascular nevus, consisting of telangiectatic and angiospastic parts, and give a review of 28 observations previously reported. In all of these cases the two different nevoid skin changes are situated directly adjacent to each. Therefore we consider this combination not to be an incidental finding but to represent a distinct entity. For this anomaly, we propose the designation "nevus vascularis mixtus".

Adult↗
PubMed1984

[Monilethrix: exclusive involvement of body hair].

In a family suffering from monilethrix, at first sight only one generation seemed to be affected, suggesting a recessive mode of inheritance. A thorough examination of the scalp and body hair of all family members, however, revealed three other affected persons in two more generations proving autosomal dominant transmission. We consider this observation an additional argument against the existence of a recessive type of monilethrix which has been postulated in the past.

Adult↗