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Biomedical subjects

H Hamm

Publications and source records attributed to H Hamm.

At least 109 records · Page 6Linked to original sources
PubMed1994

Hailey-Hailey disease. Eradication by dermabrasion.

BACKGROUND: Chronic benign familial pemphigus (Hailey-Hailey disease) is a rare dominant genodermatosis that bothers patients by chronic recalcitrant plaques occurring mainly in the intertriginous areas. We looked for a surgical treatment that is not impaired by the complications and disadvantages of radical excision and grafting of involved areas. In 10 patients with debilitating Hailey-Hailey disease (five men, five women; age, 36 to 57 years), nine different involved areas (neck, axilla, cubital area, inframammary area, periumbilical area, groin and adjacent inner thigh, scrotum, vulva, and the perineal/perianal area) were treated by dermabrasion for a total of 46 treated regions. The plane of abrasion was located in the upper to middle dermis as verified by random sampling before and after surgery, thus documenting removal of the entire epidermis. RESULTS: In most instances, reepithelialization was completed after 7 to 10 days followed by excellent functional and cosmetic long-term results. When examined 3 to 79 months (median, 42 months) later, all treated areas remained completely disease free since surgery, except for major recurrences in four sites and minor recurrences in another four sites. Four of these areas were dermabraded again without subsequent relapse. CONCLUSIONS: Our results show that dermabrasion may be considered an excellent treatment of Hailey-Hailey disease that is unresponsive to conservative therapy. As a probable explanation, the intrinsic defect of cell adhesion in Hailey-Hailey disease seems to be limited to epidermal keratinocytes but spares adnexal structures from which rapid reepithelialization occurs.

Adult↗
PubMed1993

Actinic comedonal plaque--a rare ectopic form of the Favre-Racouchot syndrome.

A 59-year-old woman with a single cuff-like plaque, consisting of multiple skin-coloured to bluish cysts and comedones on her right forearm is reported. The solitary lesion resembled, morphologically and histologically, the Favre-Racouchot syndrome. The above condition is another case of the previously described actinic comedonal plaque, which seems to be a rare entity within the spectrum of disease caused by actinic damage to the skin.

Dermatitis, Photoallergic↗
PubMed1993

[Incontinentia pigmenti in a male infant].

Bloch-Sulzberger incontinentia pigmenti (IP) is a rare X-linked neuroectodermal syndrome. Over 97% of the patients are female. We report on a male baby who developed blisters in linear groups or bands shortly after birth. When the child was 3 months old the blisters were followed by verrucous papules, which cleared after 1 year leaving areas of brownish grey hyperpigmentation. In addition to the skin involvement, our patient showed central motor dysfunction on the right side of the body and also dental and ocular anomalies. Both parents were in good health. Chromosome analysis yielded a normal karyotype (46, XY). The genes for coagulation factor VIII and biglycan in the Xq28 region were not deleted. The presence of the disease in this male infant may be due to an early somatic mutation or a half-chromatid mutation. A further possibility is mosaic expression of an unstable premutation. This model offers a good explanation for the reports in the literature of transmission of the disease from mother to son.

Diagnosis, Differential↗
PubMed1993

[The Köbner phenomenon in Duhring dermatitis herpetiformis].

We report on four female patients with confirmed dermatitis herpetiformis DH and Köbner phenomenon in identical localizations. All four had typical bilateral lesions in a band-like arrangement along the straps of their brassieres, and two patients also had additional lesions in other sites prone to pressure. Obviously, the Köbner phenomenon is not as rare in DH as would be expected in view of a single previous case report.

Adult↗
PubMed1992

[Anti-basement membrane antibody disease of the lungs without clinical kidney involvement].

Anaemia (haemoglobin 10.7 g/dl) and small spotty infiltrates in both lungs were found in an 18-year-old man who had increasing haemoptysis over the preceding 3 weeks. Bronchoscopy revealed diffuse bilateral pulmonary haemorrhage. Further diagnostic measures provided no evidence of involvement of other organs, in particular the kidneys. The demonstration of anti-basement membrane antibodies confirmed the diagnosis of a disease within the group of immune-induced alveolar haemorrhage. The radiological signs in the lungs regressed over 3 weeks of administering prednisone, initially 100 mg daily, then 60 mg daily, and the patient was discharged. While being treated as an out-patient, with reduction of prednisone to 10 mg daily, the haemoptysis recurred so that a single dose of cyclophosphamide, 1.5 g, was added to the immunosuppressive treatment. There was no further haemoptysis and the prednisone was discontinued after having been given for 15 months. The patient has now been in complete clinical remission for 3 years and anti-basement membrane antibodies are no longer demonstrable. At no time was there any evidence of renal involvement in the sense of the classical Goodpasture's syndrome.

Adolescent↗
PubMed1992

The surfactant system of the adult lung: physiology and clinical perspectives.

Pulmonary surfactant is synthesized and secreted by alveolar type II cells and constitutes an important component of the alveolar lining fluid. It comprises a unique mixture of phospholipids and surfactant-specific proteins. More than 30 years after its first biochemical characterization, knowledge of the composition and functions of the surfactant complex has grown considerably. Its classically known role is to decrease surface tension in alveolar air spaces to a degree that facilitates adequate ventilation of the peripheral lung. More recently, other important surfactant functions have come into view. Probably most notable among these, surfactant has been demonstrated to enhance local pulmonary defense mechanisms and to modulate immune responses in the alveolar milieu. These findings have prompted interest in the role and the possible alterations of the surfactant system in a variety of lung diseases and in environmental impacts on the lung. However, only a limited number of studies investigating surfactant changes in human lung disease have hitherto been published. Preliminary results suggest that surfactant analyses, e.g., from bronchoalveolar lavage fluids, may reveal quantitative and qualitative abnormalities of the surfactant system in human lung disorders. It is hypothesized that in the future, surfactant studies may become one of our clinical tools to evaluate the activity and severity of peripheral lung diseases. In certain disorders they may also gain diagnostic significance. Further clinical studies will be necessary to investigate the potential therapeutic benefits of surfactant substitution and the usefulness of pharmacologic manipulation of the secretory activity of alveolar type II cells in pulmonary medicine.

Adult↗
PubMed1992

Macrophages in melanocytic naevi.

Whereas the inflammatory infiltrates of malignant melanoma have been widely investigated, little is known about the infiltrates accompanying benign melanocytic naevi. Using monoclonal antibodies directed against HLA-DR antigens, the CD1 antigen, the transferrin receptor and functionally divergent macrophage subpopulations, frozen fresh material of 87 melanocytic naevi (MN), ten primary cutaneous melanomas (PCM) and ten samples of normal skin were studied. Compared with normal skin, abundant HLA-DR+ cells were found in the stroma of MN equivalent to the quantity present in PCM. In MN we found higher numbers of dermal CD1+ dendritic cells compared with PCM and normal skin. There were more macrophages that expressed the transferrin receptor or the antigens 27E10, RM3/1 and 25F9 in MN than in normal skin but fewer than in PCM. No significant differences were found between congenital MN (n = 40), common acquired MN (n = 27) and dysplastic MN (n = 20) macrophage subpopulations. Also, no correlations were evident between macrophage infiltrates and naevus location or patients' age. Our data show that potential melanoma precursors among melanocytic naevi cannot be identified by the pattern of macrophage infiltrates.

Antigens, CD↗
PubMed1992

[Cervix cancer in HPV16-associated Bowenoid papulosis].

In a 38-year-old female patient with bowenoid papulosis of the anogenital region and an extensive carcinoma in situ of the cervix uteri, HPV16 DNA was found in the biopsy specimens and HPV16/18 DNA was detected in the cervical swab. The known coincidence of both these diseases indicates the necessity for careful regular gynaecological check-ups of patients with bowenoid papulosis.

Adult↗
PubMed1992

The genetic risk for alopecia areata in first degree relatives of severely affected patients. An estimate.

Substantial evidence indicates that genetic factors may have a role in the etiology of alopecia areata (AA). Most studies, however, provide only general information on the familial incidence but fail to specify family relationships. We therefore obtained information on the incidence of AA in first degree relatives of 348 severely affected patients. In 7% one of the parents was affected. Among the siblings of the patients 3% had developed AA, while AA was present in 2% of the children. Taking into account the age of the children, their lifetime risk was calculated to approach 6%. However, a severe type of AA is to be expected only in about 2% of the children. The degree of involvement observed in the patients did not influence the frequency and type of AA present in their first degree relatives.

Adolescent↗
PubMed1992

[Elastosis perforans serpiginosa. Considerations on the pathogenesis based on a typical case].

Elastosis perforans serpiginosa (EPS) is a rare entity belonging to the group of primary perforating dermatoses. A 13-year-old male patient with Down's syndrome developed reddish hyperkeratotic papules in a serpiginous and ellipsoid configuration on the face. Histological examination revealed transepidermal elimination of thick coarse elastic fibres from the papillary dermis. The dermal infiltrate showed an immunohistological pattern consistent with an acute cell-mediated immune response. It consisted mainly of activated T-lymphocytes, with a predominance of CD4-positive cells. Considerable numbers of CD1-positive cells were also present. Inflammatory macrophages of the 27E10 phenotype were found in considerable numbers, whereas 25F9-positive resident macrophages were almost completely absent. The role of a cell-mediated immune response in the mechanism of transepithelial elimination is discussed.

Adolescent↗
PubMed1992

[Rhabdomyosarcoma: differential diagnosis of cutaneous tumors in childhood].

Primary rhabdomyosarcoma can arise in the skin, but there are few reports on this common childhood malignancy in the dermatological literature. We report on a male infant with a cutaneous tumour growing on the right nasal bridge since his 10th week of life. Clinically the tumour mimicked pilomatrixoma. Histological and immunohistological examination of the skin tumour and of subsequent lymph node metastases revealed rhabdomyosarcoma of the alveolar growth pattern. Our patient died at the age of 4 years of disseminated organ metastases.

Biomarkers, Tumor↗
PubMed1992

[Unilateral lentiginosis--a segmental neurofibromatosis without neurofibromas].

Segmental neurofibromatosis (NF) is a usually non-inherited form of NF that is characterized by unilateral neurofibromas and/or café-au-lait spots. In partial unilateral lentiginosis (PUL), apart from unilateral lentigines, café-au-lait spots of different dimensions have been described in some patients without fulfilling the diagnostic criteria for NF. We report on three patients representing the 4th to 6th cases of segmental NF without neurofibromas. The diagnosis resulted from unilateral café-au-lait spots of different sizes in all patients and axillary "freckling" and associated skeletal alterations in two patients each. As these findings are clinically and histologically comparable to reported cases of PUL and moreover fulfil the diagnostic criteria for segmental NF, our cases support the hypothesis that PUL is a segmental NF lacking neurofibromas.

Child↗
PubMed1992

Lipoproteins and apolipoproteins in human pleural effusions.

We investigated the lipoproteins and apoproteins in human serum and pleural effusions of different origin: transudates, inflammatory exudates, and malignant exudates. Transudates had a low cholesterol content of 35 +/- 12 mg/dl (mean +/- SD) because of low levels of low-density lipoprotein (LDL) cholesterol--representing 16% of serum levels--whereas inflammatory exudates (cholesterol 92 +/- 26 mg/dl) and malignant exudates (cholesterol 86 +/- 6 mg/dl) exhibited high levels of LDL, with 67% and 69% of serum levels. Apolipoprotein (apo) B level corresponded with LDL and presented with multiple split-products in sodium dodecyl sulfate-polyacrylamide gel electrophoresis in exudative effusions. LDL levels in effusions correlated with serum levels in exudates but did not correlate with those in transudates. In contrast, lipoprotein(a) appeared in all effusions from patients with detectable serum levels. The isoforms were similar as demonstrated by immunoblotting. Differences were found in the composition of the high-density lipoprotein (HDL) fraction: transudates had cholesterol-rich HDL when compared with serum. HDL particles of malignant exudates were poor in cholesterol, and isoelectric focusing demonstrated more sialized apolipoprotein E. A strongly abnormal HDL level with accumulation of cholesterol was found in a long-standing tuberculous effusion. In conclusion, cholesterol in acute effusions is bound to lipoproteins and derived from the blood. The difference in total cholesterol levels between transudates and exudates is based on the lack of LDL in transudates. Transudates show the lipoprotein characteristics of interstitial fluid. Alterations of lipoproteins occur in chronic inflammation and in malignancy with possible de novo synthesis of apolipoprotein E by tumor cells. Lipoprotein(a) accumulates independently from LDL in the pleural space, a finding that supports the view that the physiologic function of lipoprotein(a) is located in the interstitial space.

Aged↗