Treatment of haemodialysis-associated pseudoporphyria with N-acetylcysteine: report of two cases.
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Biomedical subjects
Publications and source records attributed to G de Dobbeleer.
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Plexiform fibrohistiocytic tumour has been recently described by Enzinger and Zhang. This is a rare tumour with low grade malignancy which requires large excision. We report two cases, one occurring in a 58-year-old man, presenting a voluminous painless mass of 5 cm on his hand, the other occurring in a 9-year-old boy consulting for a nodule on the chest. On histological examination this dermal hypodermal tumour is characterized by a proliferation of histiocytic-like cells grouped in nodules, where they are associated with multinucleated osteoclastic-like cells and a proliferation of fibroblastic-like cells grouped in fascicles, anastomosing in a plexiform pattern. Immunohistochemistry and ultrastructural aspects are described. Differential diagnosis and histogenesis are discussed.
Sebaceous carcinoma is a skin tumour which frequently metastases to the visceral organs. Needle biopsy is needed for rapid diagnosis. We observed a case in a 65-year-old patient who had a tumour formation below the right maxillary angle and homolateral justamandibular lymph node enlargement. Cytology of the needle biopsy showed a double cell population: small anaplastic cells and an agglutinated cell mass with peripheral maturation and sebaceous differenciation. Treatment was surgical with homolateral node dissection and secondary plasty. The tumour was large, non-encapsulated with local infiltration. On light microscopy, two cell populations were seen. Evident sebaceous differentiation was confirmed by histochemical staining. Immunolabelling confirmed the epithelial nature of the tumour. The clinical diagnosis of sebaceous carcinoma is difficult. Early cytology is needed to identify the two cell components when other cytological signs do not allow a positive diagnosis.
One case of penile verrucous carcinoma (Buschke-Löwenstein tumor) undergoing anaplastic transformation and containing human papillomavirus type 6 is presented. The viral genome is detected by in situ hybridization using biotin-labeled cDNA probes. The clinical, histological and virologic criteria of verrucous carcinoma are discussed in comparison to giant condyloma and highly differentiated squamous cell carcinoma. The importance of viral typing determination for further diagnostic and therapeutic procedures is emphasized.
The authors report a new case of induced pemphigus: a 64-year-old man has had rheumatoid arthritis for 10 years. When he developed his pemphigus, he had been taking D-penicillamine and piroxicam for about 8 months. He needed high doses of prednisolone associated with azathioprine to clear his eruption. The case had a fatal outcome.
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An occlusive dressing was applied to the unaffected skin of the back of a patient with familial benign chronic pemphigus (FBCP). Small lesions of FBCP appeared after 48 h, and the ultrastructure of successive biopsies was studied. It was concluded that: (a) the initial phenomenon in FBCP is the result of an insufficiency of cellular adhesion. This confirms several previous reports. (b) desmosomes are separated in two halves, invaginated in vacuoles and later deeply included in the cell. The particular fate of these disrupted desmosomes has already been observed after the action of several enzymes on the epidermis and in Darier's disease.
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.
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.
Dystrophic calcinosis is a more common form of calcinosis cutis; calcium salts are deposited secondary to local inflammation, tissue damage and degeneration. Various conditions can cause dystrophic calcinosis, including connective tissue disease, infection, inflammatory processes, chronic venous stasis, cutaneous neoplasm and trauma. We report a case of ulcerated cutaneous calcinosis associated with a localised linear scleroderma or morphea. Healing of the ulcerations took place after four months of treatment with colchicine 1 mg per day.
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