[Detection of lupus anticoagulants in the routine blood coagulation laboratory exemplified by 36 patients with systemic lupus erythematosus]. / Nachweis von Lupusantikoagulanzien im Routinegerinnungslabor am Beispiel von 36 Patienten mit systemischem Lupus erythematodes.

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. The specificity, sensitivity and effectiveness of various tests in respect of LA-demonstrability have been assessed and the results taken as the basis for proposal of a largely automated stepwise diagnostic procedure for LA-determination within the routine coagulation laboratory.

Erythromelanoderma with eosinophilic spongiosis and epidermal intercellular antibodies. Differentiation from pemphigus.

A patient with figurated, erythematous skin lesions which histologically showed eosinophilic spongiosis and an epithelial intercellular staining with IgG, but no acantholysis, developed erythromelanoderma. This case and a review of the pertinent literature indicate that the diagnosis of pemphigus does not seem to be justified even in the presence of eosinophilic spongiosis and epidermal intercellular antibodies, when the clinical signs of this disease and the typical histological feature, i.e., acantholysis, are lacking.

[Systemic mastocytosis]. / Systemische Mastozytose.

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. Investigations like this might offer an alternative to the examination of bone marrow sections in future.

[Plasmapheresis in light urticaria. A rational therapy concept in cases with a proven serum factor]. / Plasmapherese bei Lichturtikaria. Ein rationales Therapiekonzept in Fällen mit nachgewiesenem Serumfaktor.

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. In contrast to transfer tests, this method does not bear the risk of HIV infection. In view of the extreme difficulty in administering conventional therapy for solar urticaria, the present encouraging results suggest that plasmapheresis should be considered in patients who show a serum factor.

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.

[Chronic lichenoid keratosis]. / Keratosis lichenoides chronica.

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. After administration of a combined retinoid-PUVA therapy impressive remission was observed. The clinical picture, the differential diagnosis, in particular from lichen ruber planus, and the therapeutic aspects are discussed.

[Urticarial vasculitis]. / Urtikarielle Vasculitis.

Knowledge of urticarial vasculitis is very important, because its therapy and prognosis are different to other clinically similar diseases, eg idiopathic urticaria. We present a 72-year-old female patient suffering from recurrent urticaria associated with slight fever, arthralgia, occasional abdominal pain, as well as highly elevated erythrocyte sedimentation rate. Histologic examination of involved skin revealed leucocytoclastic vasculitis. Corticosteroid treatment led to clinical improvement. Courses of urticarial vasculitis may vary to a high degree; the clinical spectrum of urticarial vasculitis may turn to ranges from chronic idiopathic urticaria to systemic lupus erythematosus. Clinical hallmarks of urticarial vasculitis are slowly (within 72 hours) regressing wheals, arthralgia, as well as highly elevated erythrocyte sedimentation rate.

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. Cyclosporin A may be a valuable therapeutic alternative to systemic corticosteroids for severe cases of persistent light reaction which cannot be controlled by photoprotective measures.

[Hyperkeratosis lenticularis perstans (Flegel's disease)]. / Hyperkeratosis lenticularis perstans (M. Flegel).

Hyperkeratosis lenticularis perstans (Flegel's disease) is a rare form of dermatosis, which can only be diagnosed microscopically. A typical case is reported; histological differential diagnosis and treatment with systemic retinoids are discussed.

[Successful use of isotretinoin in type Zumbusch generalized pustular psoriasis following recovered etretinate-induced hepatitis]. / Erfolgreicher Einsatz von Isotretinoin bei Psoriasis pustulosa generalisata Typ Zumbusch nach abgelaufener Etretinathepatitis.

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. In contrast to etretinate, isotretinoin was well tolerated and resulted in a good therapeutic response. Thus, isotretinoin can be considered as an effective and safe therapeutic alternative for pustular psoriasis even after the occurrence of etretinate-induced hepatitis.

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.

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.

[Lichen ruber planus--lupus erythematosus/overlap syndrome]. / Das Lichen Ruber Planus--Lupus Erythematodes/Overlap-Syndrom.

Lichen planus/lupus erythematosus/overlap syndrome (OS) comprises those dermatoses which show the clinical, histologic, and immunopathologic characteristics of both diseases. On account of this heterogeneity, the diagnosis of OS may be difficult. About 35 cases have been reported on in the literature so far. We are going to discuss the clinical, histologic, and immunofluorescence findings in OS in detail.

[Leg ulcers in congenital dyserythropoietic anemia]. / Ulcera cruris bei kongenitaler dyserythropoetischer Anämie.

We report on an 18-year-old male patient suffering from leg ulcers due to congenital dyserythropoetic anemia and thrombocytosis. Based on this case, we discuss the importance of hematologic disorders for the development of leg ulcers as well as the pathogenetic mechanisms involved.

[Hypereosinophilic dermatitis (Nir-Westfried). A variant in the spectrum of the hypereosinophilia syndrome]. / Die Hypereosinophile Dermatitis (Nir-Westfried). Eine Variante im Spektrum des Hypereosinophiliesyndroms.

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. We discuss the clinical picture, differential diagnosis, and therapy in the light of two cases.

Lymphomatoid papulosis: remission following intravenously administered acyclovir.

In a 54-year-old male patient suffering from Hodgkin's disease, lymphomatoid papulosis occurred. Complete clearing of the skin lesions was observed immediately after intravenously administered acyclovir. The patient had numerous relapses of his skin eruption with complete responses after each course of intravenously applied acyclovir. This striking therapeutic effect parallels reports of regression of mycosis fungoides and chronic generalized lymphadenopathy after acyclovir application. The mode of action of acyclovir in these disorders is not known. They all are characterized by involvement of the T cell system, and in all these diseases, a virus etiology has been proved or is suggested. Thus, a specific effect of acyclovir on T lymphocytes or selectively on helper T cells is discussed. Alternatively, the virustatic effect of acyclovir could be responsible for the therapeutic success.

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. Ciprofloxacin treatment resulted in partial involution of the lesion.