Acute linear IgA dermatosis in a child following varicella.
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Biomedical subjects
Publications and source records attributed to P Thune.
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A 5-year-old girl with the classical clinical features of congenital erythropoietic porphyria (CEP) is described. Different types of CEP are discussed based on varying excretion patterns of porphyrins.
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A case of AIDS manifesting as a necrotic, haemorrhagic anogenital herpes zoster and lymphadenopathy, is described. Investigations of T-lymphocyte subsets showed decreased T4 and slight increase of T8. Electronmicroscopy revealed tubuloreticular virus-like structures in lymphocytes.
Recognizing the growing need for international standardization of photopatch testing techniques, a standard procedure has been used by Scandinavian dermatological clinics since January 1980. The set-up includes a standardized patient examination scheme, including phototest evaluation of the patient's UVA- and UVB-sensitivity, a standard photopatch tray containing 19 substances, and uniform techniques and criteria for the application and evaluation of the tests. Although modifications may be necessary in the future, preliminary experience with the Scandinavian photopatch set-up has been encouraging, and several positive reactions to most of the substances used have been recorded in the first 350 patients tested.
During a period of 2 1/2 years, 7 of 2000 patients routinely tested at our laboratory revealed contact allergy to oak moss in perfumes. All reacted to a mixture of different lichens and to some specific lichen compounds. The sensitivity was probably induced by cosmetics containing lichen substances. The following 3 compounds caused reactions in all patients tested: atranorin, evernic and usnic acids. 3 patients were photosensitive, but stronger reactions were elicited by prolonged contact during occlusion of the patches and complete protection against light, rather than by irradiation alone. The data suggest that the sensitizing capacity of the lichen compounds is primarily of a contact rather than of a photocontact nature.
In a double-blind, cross-over trial with Tigason and placebo, the parameters of palmoplantar pustulosis underwent significant changes during the retinoid therapy. A hand-over effect was clearly indicated during the placebo period and several patients experienced long-lasting remissions.
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In 18 ambulant patients with palmoplantar pustulosis low plasma zinc concentrations were observed. During treatment with an all-trans aromatic retinoid (RO-10-9359) the values increased while at the same time the skin condition improved in particular with regard to the pustule formation. The observed low plasma zinc values may in some way be related to pustulosis but not as causal factor.
A woman aged 54 years developed toxic necrosis of liver cells during treatment with aromatic retinoid (Ro 10-9359) for erythrodermia ichthyosiformis non-bullosa. Her skin was almost normalized when liver function tests and liver biopsy disclosed severe damage. The treatment was stopped, but 4 months later ALAT and ASAT were still elevated.
Eighty patients with untreated dermatitis herpetiformis (DH) were studied. The final diagnosis was made after completion of immunofluorescence (IF) studies of the skin, iodine provocation tests and gastrointestinal investigations. IgA deposits in the skin and the HLA-DRw3 B-cell alloantigen can be demonstrated in nearly all cases of DH. Positive iodine provocation test and gastrointestinal abnormalities are present in about 78% of the cases. For diagnostic purposes tissue typing and intestinal biopsy is of value in addition to IF studies of the skin.
In the present study the participation of lysosomal enzymes in the primary photosensitizing process in the skin of porphyric patients were investigated. Cutaneous photosensitivity reactions were produced in patients with porphyria cutanea tarda by irradiation with near UV light and also to a lesser degree with visible light. The reactions occurred almost exclusively in patients with urinary uroporphyrin excretion exceeding 900 nmol/24 h and preceeded any noticeable increase of lysosomal enzyme activity by several hours. These results indicate that lysosomal enzymes are not primarily involved in the photosensitizing reaction in porphyric patients but that they participate in the cutaneous inflammatory process in porphyric skin at a later stage.
Five psoriasis patients treated with 8-methoxypsoralen and UVA (PUVA) were studied by lymphocyte cultures at the 1st, 5th, 10th and 20th treatment and at a maintenance treatment 6 months later. Abnormal amounts of chromosome aberrations were not found, and the frequency of sister chromatid exchange (examined at the last treatment) was not increased. In vitro experiments with nanogram doses of psoralen (similar to plasma levels in patients) showed no increase in chromosome aberration or SCE freqency. The results indicate that therapeutic doses of PUVA have no clastogenic effect.
A new tablet formulation of 8-methoxypsoralen was used in PUVA treatment for psoriasis. The 8-Mop plasma level showed a maximum at about 1 hour after ingestion (mean values: 1/2 hr, 284 microgram/1; 1 hr, 275 microgram/1; 1 1/2 hr, 198 microgram/1; 2 hrs, 129 microgram/1). Phototoxicity tests showed the optimal time for exposure to be about 1 hour after peroral intake of 8-MOP.
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