[Congenital poikilodermas]. / Kongenitale Poikilodermien.

We discuss the diagnoses and differential diagnoses of the various forms of congenital poikiloderma. In addition, we differentiate this disease from dysplastic syndromes, progeria, and connotal metabolic disorders.

[Multicenter skin tumor of the interdigitating dendritic cell]. / Multizentrischer Hauttumor der interdigitierenden dendritischen Zelle.

This report concerns a multicentric skin tumor of dendritic cells. Skin-colored to reddish-brown papules and nodules erupted on the right arm of a 68-year-old man and, during the following years, spread to the upper extremities, shoulders, legs and face. Immunohistochemically the large, histiocyte-like tumor cells are Leu-6 (CD1), Leu-3a (CD4), HLA-DR/DQ and S-100 protein positive, but on thorough ultrastructural investigation were found to lack Birbeck granules. Thus, this disease represents a skin tumor of the interdigitating dendritic cell, an accessory cell which, in this case, was associated with a high content of T-lymphocytes within the cutaneous cellular infiltrate. The clinical course is characterized by periodic progression with intermittent, spontaneous regression. During a 7-year follow-up it was not possible to ascertain involvement of either lymph nodes or internal organs.

Skin tumor of T accessory cells (interdigitating reticulum cells) with high content of T lymphocytes.

A case of T-accessory cell tumor of the skin in a 67-year-old man is reported. The limbs, shoulders, and face were affected, but no visceral involvement is evident 6 years after onset. Tumor cells are nonphagocytic mononuclear cells with folded irregular nuclei. Immunologically, cells were positive for S100 protein, HLA-DR, Ki-M1, Leu 3a (CD4), Leu 6 (CD1); that is, they are identical to the phenotype of Langerhans or interdigitating reticulum cells (IDCs). Birbeck granules were absent. The clinical course appears to be less aggressive than that of the reported IDC sarcomas in other anatomical sites. The similarity of our case to some cases of so-called "non-X histiocytosis" of the skin is discussed. It is suspected that the "non-X histiocytosis of the skin" reported in the literature might have included T-accessory cell tumors, especially those of IDC origin. More immunological studies on the histiocytic disorders of the skin are necessary to clarify their cytogenesis.

[Chronic systemic toxicity of oral photochemotherapy using 8-methoxypsoralen and UVA]. / Untersuchungen zur chronisch-systemischen Toxizität einer oralen Photochemotherapie mit 8-Methoxypsoralen und UVA.

The purpose of the study conducted was to clarify the question of chronic, systemic toxicity from PUVA therapy. Five groups of patients, classified according to length of treatment were examined with respect to 43 laboratory parameters. The results indicated that PUVA had no chronic toxic influence on the liver, kidneys, bone marrow, metabolism, or immune response.

[Skin and bone findings in mastocytosis]. / Haut- und Knochenbefunde bei der Mastozytose.

The syndrome of mastocytosis extends from cutaneous urticaria pigmentosa through systemic mastocytosis to the rarely occurring mast cell leukaemia. Our investigations with a large patient collective have shown that systemic forms of the disease occur more often than is generally supposed. In particular the incidence of bone marrow manifestations deserve more attention. The inflammatory-granulomatous findings in the bone marrow suggest an immunoactive component in the pathogenesis of this disease. The bone lesions that occur in about 50% of patients are probably the result of the common endosteal site of the mastocytosis granuloma. These lesions can be generalized (osteoporosis-osteosclerosis) or localized (osteolytic-osteosclerotic foci). In clinical practice bone biopsy and skeletal radiology complement one another; in addition to skin biopsy bone biopsy supplies the initial diagnosis of mastocytosis and documents systemic manifestation; the X-ray picture informs the clinician about the type and extent of the bone pathology.

[Bullous angiolymphoid hyperplasia with eosinophilia]. / Bullöse angiolymphoide Hyperplasie mit Eosinophilie.

Two patients are described having a bullous variant of angiolymphoid hyperplasia with eosinophilia on the head. This disease is characterized by inflammatory, infiltrated, plaque-like lesions with recurring bullous eruptions. At the same time there may be prurigo-like papules on the trunk and arms. The course is a characteristic histological pattern revealing cutaneous lymphocytic infiltrate, lymphoid follicle formation, and atypical vascular proliferation associated with tissue eosinophilia. Blister formation takes place subepidermally.

[Mastocytosis and the gastrointestinal tract]. / Mastozytose und Gastrointestinaltrakt.

Gastrointestinal symptoms frequently occur in patients with systemic mastocytosis. The gastrointestinal tract, liver, and spleen may be involved in this disease. In addition to investigations of our own in patients with mastocytosis, gastroenterologica manifestations from the literature are reviewed.

[Improvement of psoriasis by haemodialysis (author's transl)]. / Besserung der psoriasis durch Hämodialyse. Beziehung zu einer bestimmten HLA-Antigen-Konstellation?

Haemodialysis treatment of 5 patients with therapy-resistant psoriasis showed that certain clinical forms seem to respond to such treatment. Each patient had at least one of the frequently occurring antigens of psoriatic patients in his HLA match (B 13, B17, B 27, B 37, Cw 6). Of these antigens B 37, B 13 and B 27 were found only in such patients where treatment was successful. The HLA-B 37 antigen was associated with generalised, HLA-B 13 with chronic stationary and HLA-B 27 with arthropathic manifestation of psoriasis.

[Internal findings in mastocytosis]. / Interne Befunde bei Mastozytose.

45 patients with urticaria pigmentosa were thoroughly investigated and a systemic involvement concerning the bone marrow, the skeletal system, liver, spleen, and coagulation was often encountered. This systemization is no sign of malignancy. Benign systemic mastocytosis should be differentiated from malignant mastcell-reticulosis.

[Allergic eczematous contact dermatitis due to fatty acid polypeptide condensates (author's transl)]. / Allergisches Kontaktekzem auf Eiweiss-Fettsäure-Kondensate.

After an electrocardiogram an eczematous contact dermatitis was found on the skin of a 34-year-old soldier, where the contact of the leads had been fixed to the chest and extremities. 5 months later, he caught an eczematous contact dermatitis which spread over the face after irrigation of the external auditory meatus. In both cases, the contact allergen was a fatty acid polypeptide condensate. This article describes the production, properties and application of the fatty acid polypeptide condensates as well as effects and side-effects in comparison to other detergents.

Comparison of three methods of susceptibility testing of bacteroidaceae, peptococcaceae and other anaerobes to doxycycline.

The in vitro activity of doxycycline against 147 strains of gram-positive and gram-negative anaerobes was determined by broth dilution, agar dilution and agar diffusion tests. The strains were isolated from clinical specimens in 1977. Saccharolytic Bacteroides strains (39 B. fragilis, 6 B. thetaiotaomicron, 4 B. vulgatus) showed broth dilution MICs of less than or equal to 0.0625-4.0 microgram/ml after 5 h incubation and MICs of less than or equal to 16 microgram/ml after 15 h incubation at 37 degrees C. With strains of B. oralis, B. melaninogenicus ss. intermedius, B. corrodens, Veillonella sp. and Megasphaera elsdenii the broth dilution MICs were less than or equal to 0.0625-32 microgram/ml after 15 h incubation. Peptostreptococcus spp., Propionibacterium acnes, Eubacterium sp., Bifidobacterium sp. and Clostridium sp. had broth dilution MICs of less than or equal to 2 microgram/ml, whereas Peptococcus spp. were inhibited by less than or equal to 0.0625 to greater than 32 microgram/ml. With the great majority of strains tested, MICs were 2 to 256 times higher in agar than in broth dilution tests. Clinical and experimental studies seem to be needed to determine which of the in vitro data are correlated to the outcome of doxycycline therapy. Several groups and species were separately considered for statistical analysis of the relationship between zone size and MIC. With gram-positive anaerobes, correlation was poor between broth dilution MIC and zone size (correlation coefficients r = - 0.168 for Peptococcaceae, and r = - 0.108 for P. acnes). When calculating the regression lines for agar dilution MICs and zone diameters, a higher correlation was found (r = - 0.9 for Peptococcaceae; r = - 0.397 for P. acnes). With B. fragilis and other Bacteroides species correlation coefficients were r = - 0.807 to r = - 0.891 for broth and agar dilution MICs and zone size but stochastic linearity was lacking.

[The Zinsser-Cole-Engman syndrome. A contribution to congenital poikilodermias as well as a contribution to familial pancytopenias]. / Das Zinsser-Cole-Engman-Syndrom. Ein Beitrag zu den kongenitalen Poikilodermien, zugleich ein Beitrag zu den familiären Panzytopenien.

The Zinser-Cole-Engman-syndrome (Dyskeratosis congenita), which is characterized by the cardinal symptoms pigmentatio reticularis, onychodystrophia and leukoplakia oris, is discussed as a clinical entity, which is different from Fanconi's anemia and Braun-Falco-Marghescu-syndrome. A review of the literature is given.

[Bloom syndrome: review and definition]. / Das Bloom-Syndrom: Ubersicht und Abgrenzung.

The cases of Bloom-syndrome as described in the literature (till...) are reviewed and this syndrome is discussed as a defined entity which can be separated from congenital poikilodermias. The prognosis of this autosomal recessive disease is dubious.

[Scintigraphic changes on the liver and spleen in urticaria pigmentosa]. / Szintigraphische Veränderungen an Leber und Milz bei Urticaria pigmentosa.

In 36 patients with mastocytosis pathological changes in liver and spleen were scintigraphically demonstrated. These changes are due to the histologically proven involvement of the reticuloendothelial system. The infiltration of the liver by mast cells was not evident in liver biopsy.