摘要:

Type VII collagen, the major structural component of the anchoring fibrils, was assayed in normal unaffected skin of patients with different forms of hereditary epidermolysis bullosa. Immunofluorescence staining with affinity-purified polyclonal antibodies to type VII collagen revealed a complete absence of staining in the skin of patients with severe dystrophic recessive epidermolysis bullosa. In all other forms, localized recessive dystrophic, dominant dystrophic, junctional and simplex forms there was an intense continuous linear staining of type VII collagen at the dermoepidermal junction. Also, obligate heterozygote carriers of the gene for severe dystrophic recessive form showed a normal pattern of staining. As internal controls and to define the clinical diagnosis, staining with antibodies to type IV collagen, laminin and bullous pemphigoid antigen was also performed. All these antibodies showed a normal staining pattern indicating an intact general morphology of the dermoepidermal junction zone. These results suggest that there is a defect of type VII collagen in patients with severe recessive dystrophic epidermolysis bullosa. The data also suggest that the group of recessive dystrophic epidermolysis bullosa may be heterogeneous not only clinically, but also at the molecular level.

ISSN:1018-8665    

DOI:10.1159/000248673

出版商:S. Karger AG    

年代:1988

数据来源: Karger

摘要:

By using a microhandling system, we have recorded the force necessary to elongate isolated corneocytes. According to certain hypothesis, it appears that the elastic modulus of corneocyte is far higher than that of stratum corneum itself. Aggregates of corneocytes are disassembled by forces with a wide range of intensity. In the light of these experiments, stratum corneum appears as a very supple material where the deformations occur more by a plastification of the intercellular medium and an unfolding of the microrelief lines than an elongation of the corneocytes themselves. Moreover, desmosomes remnants could play a certain role in the corneocytes cohesiveness.

ISSN:1018-8665    

DOI:10.1159/000248674

出版商:S. Karger AG    

年代:1988

数据来源: Karger

摘要:

This study deals with 13 patients who volunteered for serial biopsies during proquazone treatment which proved to be effective in the majority of the cases. The immunocompetent cells in the inflammatory cell infiltrates were characterized by a panel of monoclonal antibodies: OKT11, OKT4, OKT8, OKT6, NK-9 and Leu-14. Before proquazone treatment there was only a slight predominance of OKT4-positive cells as compared to OKT8-positive cells but a rather high number of NK-9-positive natural killer or activated cytotoxic T cells. The pattern of inflammatory cell subclasses changed during the treatment and the most striking difference was the reduction in the number of the NK-9-positive cells. It is proposed that Jessner’s lymphocytic infiltration of the skin (LIS) should be viewed as an entity and that proquazone possibly acts by modulating the pattern and activation stage of lymphocyte subclasses in LI

ISSN:1018-8665    

DOI:10.1159/000248675

出版商:S. Karger AG    

年代:1988

数据来源: Karger

摘要:

A male newborn presented at birth with marked skin fragility and widespread erosions of the face, scalp, trunk and extremities. Abnormal associated findings included: cleft palate, hypoplastic low-set ears, bilateral synechiae of the first and second toes, incomplete fusion of both eyelids, and dystrophic nails. There was no evidence of consanguineous parentage. The karyotype was normal. The baby died at 3 days of age because of pulmonary distress. As demonstrated by light and electron microscopy, there were both epidermolytic and dermolytic features. Desmosomes and tonofilaments were decreased in most basal cells. Hemidesmosomes were normal. Anchoring fibrils were rare. This type of epidermolysis bullosa does not fit any known categories of disease.

ISSN:1018-8665    

DOI:10.1159/000248676

出版商:S. Karger AG    

年代:1988

数据来源: Karger

摘要:

There are at least six variants of junctional epidermolysis bullosa (JEB). About 20 cases of the generalized atrophic benign variant of JEB (GABEB) have been previously reported. We present an additional case of GABEB, occurring in a 14-year-old girl. Generalized cutaneous blisters occurred since birth and healed without severe scarring or milia, but with slight atrophy. In addition, mucous membrane involvement and hair, nail and tooth abnormalities were found. Electron microscopic examination showed a cleavage within the lamina lucida and the presence of numerically and structurally abnormal hemidesmosomes.

ISSN:1018-8665    

DOI:10.1159/000248677

出版商:S. Karger AG    

年代:1988

数据来源: Karger

摘要:

Dominant dystrophic epidermolysis bullosa of Cockayne-Touraine occurred in a father and his son. Clinically, the child presented evident blistering, while the father, despite of his work, where minimal skin traumata frequently occur, was rarely affected. Absent or rudimentary anchoring fibrils were seen in the normal-appearing skin of the father; this suggests that other factors may play a role in the dermolytic separation.

ISSN:1018-8665    

DOI:10.1159/000248678

出版商:S. Karger AG    

年代:1988

数据来源: Karger

摘要:

A 43-year-old male with a 23-year history of small-plaque-type parapsoriasis developed a chronic recurrent self-healing papulonodular and tumoral cutaneous eruption. The microscopic findings were consistent with eosinophilic histiocytosis. Immunohistochemical and ultrastructural studies revealed a population of T lymphocytes, eosinophils and monocyte-macrophage cells in the dermal infiltrate. These findings confirm the similarities between eosinophilic histiocytosis and lymphomatoid papulosis suggesting that the former may be a clinicopathologic variant of the latter.

ISSN:1018-8665    

DOI:10.1159/000248679

出版商:S. Karger AG    

年代:1988

数据来源: Karger

摘要:

25 patients with chronic urticaria suspected to be of ‘alimentary origin’, were studied for a quantitative or qualitative deficiency of pancreatic enzyme secretion. All showed a normal fecal chymotrypsin excretion and 23/25 a normal bentiromide (PABA) and pancreolauryl test. In 2 females the urinary PABA and pancreolauryl tests were borderline pathological. This does not support the hypothesis that a pancreatic deficiency (of the kind which could be identified with the methods used) is associated with chronic urticaria in patients in whom improvement of urticaria occurs under a hydric or low antigenic d

ISSN:1018-8665    

DOI:10.1159/000248680

出版商:S. Karger AG    

年代:1988

数据来源: Karger

ISSN:1018-8665    

DOI:10.1159/000248681

出版商:S. Karger AG    

年代:1988

数据来源: Karger

ISSN:1018-8665    

DOI:10.1159/000248682

出版商:S. Karger AG    

年代:1988

数据来源: Karger