Hereditary angioedema in Austria: prevalence and regional peculiarities.

Background: Data on the prevalence and clinical features of Austrian patients with hereditary angioedema (HAE) with C1-inhibitor (C1-INH) deficiency (HAE-1) or dysfunction (HAE-2) are lacking.

Methods: Current baseline data were collected in a national survey. The records of HAE patients at the Medical University of Graz were analyzed with regard to clinical characteristics.

Necrotizing cutaneous lesions caused by interferon beta injections in a patient with multiple sclerosis.

We report a patient with multiple sclerosis who was receiving subcutaneous injections of recombinant interferon beta. During the third month of treatment, painful necrotizing cutaneous lesions appeared at the injection sites. The possible pathogenesis of interferon-induced skin necrosis is discussed.

Cutaneous malakoplakia on the forehead.

A case of cutaneous malakoplakia in an 81-year-old woman in reported. A reddish slowly growing plaque had appeared on her forehead 13 years previously. Histopathology revealed a dense dermal granulomatous infiltrate consisting of lymphocytes and numerous histiocytes containing Michaelis-Gutmann bodies.

Relapsing linear acantholytic dermatosis.

The case of an 81-year-old man with relapsing linear acantholytic dermatosis is described. This is the second description of this disease entity, which is characterized by the histopathologic and ultrastructural features of Hailey-Hailey disease. Clinically, it is characterized by skin lesions that wax and wane in a systematic pattern following the lines of Blaschko.

[Specific IgM tests in syphilis diagnosis].

A total of 359 sera of untreated patients with syphilis were examined by three methods for the detection of Treponema pallidum specific IgM antibodies, the 19S-IgM-FTA-ABS test, the IgM solid phase haemadsorption assay (IgM-SPHA), and the IgM Captia assay. The results were compared and evaluated. In primary syphilis, the 19S-IgM-FTA-ABS and IgM-captia yielded reactive results in all patients, whereas only 40% were positive in the IgM-SPHA; the corresponding values for early latent syphilis wee 96.

[Dyshidrosiform bullous pemphigoid].

Dyshydrosiform bullous pemphigoid is a clinical variant of bullous pemphigoid in which the lesions appear primarily on palms and soles. Knowledge of this unusual manifestation of bullous pemphigoid may be of practical relevance, since it has to be included in the differential diagnosis of blistering palmoplantar dermatoses. The correct diagnosis is confirmed by histological examination and immunofluorescence, which reveal all the characteristic hallmarks of bullous pemphigoid.

[Recurrent venous thromboses in hypoplasia of the vena cava inferior and factor XII deficiency].

The cause of recurrent pelvic and leg venous thromboses in a 24-year-old man was found to be a combination of two rare anomalies, hypoplasia of the hepatic, prerenal segment of the inferior vena cava and factor XII deficiency (factor XII activity 38%, its antigen 39% of normal), the latter considered a risk factor for thromboembolism. Subsequent fibrinolysis was not successful. No thromboembolic phenomena occurred during the following 16 months of oral anticoagulation with phenprocoumon.

[Detection of lupus anticoagulants in the routine blood coagulation laboratory exemplified by 36 patients with systemic lupus erythematosus].

36 patients suffering from systemic lupus erythematosus (SLE) were subjected to various screening and confirmation tests for the presence of lupus anticoagulants (LA) which are a risk for thrombosis. In five out of the 36 patients (14%) lupus anticoagulants could be found. Five out of the 36 patients (14%) showed increased antiphospholipid antibody (APA) levels whereby only two of these patients were at the same time LA-positive.

Ischemic venous thrombosis caused by a distinct disturbance of the extrinsic clotting system.

Ischemic venous thrombosis that led to necrosis of four toes developed in an 81-year-old man. Despite the extensive thrombosis, results of blood clotting tests showed an extremely low prothrombin time (20%). Plasma mixing studies demonstrated an inhibitor that may have features in common with the lupus anticoagulant.

[Successful use of isotretinoin in type Zumbusch generalized pustular psoriasis following recovered etretinate-induced hepatitis].

Administration of etretinate in a 29-year-old female patient suffering from severe pustular psoriasis caused a dramatic increase in liver enzymes. Liver biopsy revealed changes characteristic for drug-induced hepatitis. After normalization of liver parameters following withdrawal of etretinate, isotretinoin was administered during a severe pustular relapse.

Ulcerating elastofibroma dorsi.

Elastofibroma dorsi is a rare, benign tumor in elderly persons that usually occurs in the subscapular region. It represents a degenerative pseudoneoplastic process that resembles a malignant neoplasm. We report a patient with an elastofibroma dorsi of unusual size that ulcerated.

[Plasmapheresis in light urticaria. A rational therapy concept in cases with a proven serum factor].

Plasmapheresis represents a new and powerful treatment for solar urticaria and has been performed in a couple of patients so far. Since therapeutic responses have been observed exclusively in cases exhibiting a pathogenetically important serum factor, plasmapheresis seems to work specifically by elimination of this photoallergen. It is easy to demonstrate a serum factor by in vitro irradiation of the patient's serum and subsequent autologous reinjection into the skin, inducing the generation of wheals.

[Apocrine chromhidrosis].

Apocrine chromhidrosis is an extremely rare and impressive skin disorder. Primarily after emotional stress patients complain of coloured sweat secretion (black, green, blue or yellow). The deposition of lipofuscin in the apocrine glands may be the cause of this disease, the different colours of sweat being due to various oxidation stages of lipofuscin.

[Systemic mastocytosis].

Systemic mastocytosis associated with urticaria pigmentosa seems to be strikingly more common than previously assumed. The diagnosis can be established by the investigation of bone marrow sections, whereas bone marrow smears are less reliable. Some mediators are produced by the enhanced number of mast cells; telemethyl imidazole acetic acid is the most suitable mediator to calculate the size of the mast cell pool.

Treatment of herpes zoster. Recombinant alpha interferon versus acyclovir.

Sixty-four patients received systemic alpha-interferon (10 million units subcutaneously daily) and 63 received systemic acyclovir (5 mg/kg body weight intravenously thrice daily) in a randomized study of acute herpes zoster. Start of healing, complete healing, development of new skin lesions in the primarily affected and in other dermatomes, and degree and duration of pain were evaluated. Both drugs proved equally clinically efficient without statistically different findings between the two groups; herpes zoster neuralgia was not prevented by either interferon or acyclovir therapy.

[Hypereosinophilic dermatitis (Nir-Westfried). A variant in the spectrum of the hypereosinophilia syndrome].

Hypereosinophilic dermatitis represents a clinically distinct disorder in the spectrum of eosinophilic dermatoses. Its major clinical symptoms include pruriginous papular skin eruptions associated with blood eosinophilia. Histological examination reveals a diffuse, dense infiltration with eosinophils.

Bullous pemphigoid after radiation therapy.

Electron beam therapy applied to a lymph node metastasis from a squamous cell carcinoma was followed by the development of histologically and immunologically typical bullous pemphigoid, the lesions being initially strictly confined to the irradiation area. This observation suggests that the bullous pemphigoid antigen may be altered or unmasked by electron beam radiotherapy, leading subsequently to the production of autoantibodies. The disease in this case effectively responded to the administration of tetracycline and niacinamide, a therapeutic regimen described recently.

Persistent light reaction. Successful treatment with cyclosporin A.

A 53-year-old male patient who had suffered for several years from severe persistent light reaction possibly due to tribromsalan photosensitivity was treated with cyclosporin A after long-term low-dose administration of corticosteroids which had to be discontinued. PUVA therapy was impracticable due to the extraordinarily high UVA sensitivity. When cyclosporin A blood concentrations between 100 and 200 ng/ml were reached, the patient was nearly free from symptoms; the excellent clinical response was also documented by phototesting performed prior to and during therapy.

[Chronic lichenoid keratosis].

A 34-year-old female patient presented with the classical features of keratosis lichenoides chronica, a very rare dermatosis. Typical hyperkeratotic papules in a linear and reticular pattern were located on the upper extremities and the trunk. The nails and the perionychial areas showed marked vegetating lesions.

[Dowling-Degos disease: unsuccessful therapeutic trial with retinoids].

A patient with the typical features of Dowling-Degos disease (reticulate pigmented anomaly of the flexures) is described; the clinical picture and differential diagnosis are discussed. Systemic retinoids were used for treatment, but no essential improvement could be observed.