Biomedical subjects
Publications and source records attributed to G Achten.
A healthy 26-year-old patient presented a primary herpetic type 2 infection. The infection disseminated to the skin, the mucosa and the liver. The clinical course of the infection had a favorable issue.
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A total of 330 patients with eczematous lesions were tested with 27 topical substances. Among the 88 patients with leg ulcers, 85 (2%) were allergic to one or more substances present in one out of two cases of allergy to wool alcohol. Among the 242 patients suffering from eczematous dermatitis, the percentage of allergic reactions reached 54.6%. A great number of allergic reactions of cetyl alcohol (11.2%), chloramphenicol (10%) and sodium lauryl sulfate (6.4%) was observed. These allergens are not included in the European standard series adopted by the ICDRG. The findings are discussed and compared with data taken from the literature.
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Observations of 3 different great ichthyotic disorders, no longer responding to local keratolytics and to oral vitamin A are reported: a case of erythrokeratoderma variabilis, one of ichthyosiform erythroderma, and one of bullous ichthyosiform hyperkeratosis. After a week of oral treatment by the ethylester retinoid (Ro 10-9359), the hyperkeratotic component of each disease already improved dramatically. A maintenance treatment had to be continued. With equal or superior keratolytic effects, the retinoid has lower toxicity and less important side-effects than retinoic acid itself. The ethylester retinoid is nowadays the choice oral treatment in these great hyperkeratotic disorders.
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Electron microscopic studies have been carried out on three cases of fixed drug eruption, with particular regard to the dyskeratotic cells. The authors have tried to show the sequence of events leading from a normal basal keratinocyte to a dyskeratotic body. Ribosomes were first increased in number; then the tonofibrillar system looked thicker, cytoplasmic organelles degenerated and numerous melanosomes appeared. Many dyskeratotic bodies were later found in epidermal macrophages and in the intercellular space. Several intra-cytoplasmic desmosomes were found in the dyskeratotic cells and their evolution is discussed. The increased number of melanosomes seen in the dyskeratotic bodies could be due to injury to epidermal lysosomal catabolism or it could be due to an apparent increase in melanosome numbers, within cells whose volume has been reduced.
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A 34 year's old female patient died of nodular liquefying panniculitis after a six months evolution. The only systemic lesions found at post mortem histological examination were an involvement of both pleural cavities with an oily fluid. This seemed to have been the cause of death. This symptomatology was not observed in any of the 26 fatal cases of Weber-Christian disease published in the literature among which 11 cases with no other lesions than adipous tissue lesions, 6 cases where death was related to secondary effects of panniculitis and 9 cases where death was related in intercurrent diseases.
Complete alpha-1-antitrypsin deficiency of the type PiOO associated with chronic obstructive lung disease, cutaneous hyperextensibility and hyperlaxity of joints were found in a nineteen-year-old Moroccan boy. On a nosological point of view, this patient could be included as a 8th form, in the Ehlers-Danlos syndrome which now groups seven clinical variants. A causal relationship between the biological deficiency and the clinical alterations (pulmonary, cutaneous and articular) could be assumed according to the biological and ultrastructural findings.
The authors investigated the ultrastructure of the epidermis of two children who suffered from the staphylococcal scalded skin syndrome (or the Ritter's type of toxic epidermal necrolysis). This syndrome is attributed to the action of an exfoliative toxin produced by Staphylococcus aureus phage group II. A characteristic bullous cleavage was selectively observed at the level of the granular layer, without any damage in other epidermal layers. This cleavage was the result of disruption of of desmosomes between granular cells in two halves, each half desmosome conserving the tonofilaments which were attached to its attachment plaque. No remarkable cytoplasmic alteration occurred in the granular layer, with the exception of the development of thickened tonofilaments among dilated endoplasmic reticulum. Odland bodies were particularly numerous in the areas of desmosomal disruption. This syndrome must be considered as an entity clinically, histologically and ultrastructurally separate from the drug form of toxic epidermal necrolysis.
A 19-year-old Moroccan male was found to have total absence of serum alpha1-antitrypsin, a major inhibitor of elastase. This patient had chronic obstructive lung disease, hyperextensibility of the skin over the cheeks and wrists, and hyperlaxity of the hand joints. Microscopic sections of the skin revealed a thickened dermis with shortened and rarefied elastic fibers. Ultrastructural study showed collagen fibers with variable and irregular diameters. Elastic fibers were scarce and their relatively poor matrix was surrounded by numberous microfibrils. The outline of the fibers was irregular with deep recesses filled with microfibrils. The ergastoplasm of the fibroblasts was well developed. The differential diagnosis with other connective dystrophies showed the original characteristic of this case. Clinically and histopathologically, the skin abnormalities are probably related to the deficiency in elastase inhibitor.
The staphylococcal form of toxic epidermal necrolysis or staphylococcal scalded skin syndrome (SSSS) is exceptionally seen in an adult patient. We report a case of SSSS in a 21-year-old male who suffered from a fulminant pneumopathia due to a phage group II Staphylococcus aureus. The onset of that clinical picture in an adult patient is unusual and could be due to a deficient immunity, as previous case reports have emphasized. Unfortunately, the sudden death of our patient did not allow us to investigate his immunological defences.
A double labeling method with two levels of tritiated thymidine was used to study 6 patients with seborrheic keratosis, 1 with a fibroepithelial tumor of Pinkus, and 1 with basal cell nevus syndrome manifesting three pits on the palm of the hand. The two latter types of lesions, known to be able to evolve into basal cell carcinoma, had an increased S-phase duration (18 hr for the germ cells of the palmar pits) as compared with normal epidermis (10 hr). This situation was similar to that observed in basal cell carcinoma, However, the S phase was not lengthened in seborrheic keratosis (9.2 plus or minus 1.6), a benign tumor in which malignant transformation is extremely rare. S phase was also of normal duration in the benign eosinophilic septa of the tumor of Pinkus.
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