[Unclear hip pain in systemic sclerosis. Osteonecrosis]. / Unklare Hüftschmerzen bei systemischer Sklerodermie. Osteonekrose.

Systemic sclerosis (SSc) is an immune-mediated inflammatory disease of connective tissue that leads to widespread small vessel damage, progressive fibrosis of the skin and numerous extracutaneous organs. An often forgotten manifestation of SSc is in bone. We report on a case of SSc where the patient suffered from unexplainable coxalgia that had started 4 years after the diagnosis of SSc was made. Nontraumatic osteonecrosis of the femoral head was identified by magnetic resonance imaging (MRI) as the underlying cause of the hip pain.

[Pansclerotic porphyria cutanea tarda after chronic exposure to organic solvents]. / Pansklerotische Skleroporphyrie nach langjähriger Exposition gegenüber organischen Lösungsmitteln.

A 63 year old man developed generalized scleroderma with massive sclerotic areas, particularly in the abdominal region, four years after being diagnosed with porphyria cutanea tarda (PCT). He had almost daily exposure to organic solvents (benzene, trichlorethylene) for many years. The cutaneous fibrosis progressed dramatically leading to a pansclerosis, even though the uroporphyrin levels were borderline and the liver enzyme values were normal. Organic solvents are among those substances which can cause a cutaneous fibrosis. The unusually complicated clinical development in our patient was a combination of the two initial factors, the PCT and the long term exposure to organic solvents. The pansclerotic PCT was differentiated from a systemic sclerosis, a disabling pansclerotic morphea and a generalized morphea by means of histological examinations, the absence of a Raynaud phenomenon and the non-involvement of additional organs. Auto-antibodies typical for systemic sclerosis were negative. Using a medium dosage of UVA1 phototherapy and intensive physiotherapy, the progression of the skin disease was stopped and the sclerosis improved.

[Dermatologic rehabilitation--chances in crisis]. / Dermatologische Rehabilitation--Chancen in der Krise.

The political activities concerning the health care system in recent months have made the chronic crisis involving dermatological rehabilitation clinics in Germany more apparent. While dermatology has continual to evolve in acute care clinical facilities, no critical analysis of dermatological rehabilitation clinics has occurred. A commonly accepted, standardized document defining precisely the goals of dermatological rehabilitation as well as the appropriate ways to measure the quality of the treatment and its results is not available. An expert panel appointed by the responsible working group of the Bavarian public health care system succeeded in defining mandatory structural requirements which will soon be obligatory for all Bavarian dermatological rehabilitation clinics. In the future, a general concept for the medical care of patients with chronic dermatological diseases on the appropriate level--ambulatory or hospital--has to be established. A close cooperation between clinicians working in acute care facilities and dermatologists in ambulatory practices could, at least in the long run, result in establishing local and national centers of competence for diagnosis, therapy, prevention, and rehabilitation of chronic dermatological diseases.

Keratinocyte growth inhibition by streptococcal proteins.

M proteins are receptor proteins and one of the virulence factors of streptococci. M proteins seem to play a role in inflammatory skin disorders such as psoriasis. It is however unknown whether M proteins have a direct influence on proliferative activity of human keratinocytes. In the present study human HaCaT keratinocytes were exposed to M proteins (M1, M3, M5, M12) and the proliferative and proinflammatory response was analyzed. We found a dose-dependent inhibition of keratinocyte proliferation with crude extract of strain M3 4/55. Following affinity chromatography we found inhibitory activity for keratinocyte proliferation with a maximum of 80% at 10-8 M in the M protein. Additionally tested M1 protein preparation showed an inhibitory activity of 55% whereas other M preparations (5 and 12) did not show any effect. In supernatants from HaCaT cultures IL-1alpha, IL-1beta, IL-6, IL-8, TNFalpha and ICAM-1 were measured by ELISA. The levels of IL-8 were high and TNFalpha was upregulated, whereas ICAM-1 was decreased from around 20 ng/ml to almost zero. In contrast to the streptococcal-derived M3 protein preparation the recombinant M3 did not interfere with the proliferation of HaCaT cells. Because neither recombinant M3 protein nor M3 protein purified by ion exchange chromatography on a Q-resource column had any antiproliferative activity on keratinocytes we suggest, that a component different from M3 protein was responsible.

[Gram-positive septic-toxic shock with bullae. Intraepidermal splitting as an indication of toxin effect]. / Grampositiver septisch-toxischer Schock mit Blasenbildung. Die intraepidermale Spaltbildung als Hinweis für die Toxineinwirkung.

A fourteen-year-old girl with acute otitis media died from gram positive sepsis and toxic shock despite intensive treatment. The definitive bacteriological results showed positive cultures for both S. aureus and S. pyogenes serotype A. In vitro the bacteria produced the bacterial superantigens TSST-1, enterotoxin A, enterotoxin C (S. aureus) and erythrogenic toxin C (S. pyogenes). The patient presented with large flaccid sterile bullae on her chest and arms as well as necrotizing fasciitis. Tzanck test showed keratinozytes without necrosis and no inflammatory cells. Frozen-section and conventional skin biopsy specimens revealed subcorneal intraepidermal cleavage. These cytological and histological findings are those of staphylococcal scalded skin syndrome (SSSS) and differ from bullous erysipelas or toxic epidermal necrolysis (TEN). Therefore bacterial exotoxins are most likely responsible for the intraepidermal blistering in our case just as in SSSS. Bullae are an unfavorable prognostic sign in gram positive toxic shock syndrome. Both Tzanck test and frozen-section biopsy are easy to perform and useful in the early and rapid recognition of gram positive bullous toxic shock syndrome.

Chemiluminescence in semen of infertile men.

Luminol-dependent chemiluminescence (CL) can be used to determine the production of reactive oxygen species (ROS) by cells. Enhanced formation of ROS in human semen was reported to be of pathological significance for a disturbed sperm function. To investigate incidence of elevated CL-signals in semen samples and their correlation to conventional semen parameters, CL-signals in the semen of both 49 consecutive infertile men and 20 controls were measured. Semen was analysed according to WHO-criteria including bovine mucus-penetration- and water-test. A CL-signal of 1.5 x 10(5) counts min-1/2 x 10(6) spermatozoa was considered to be the upper normal limit. The CL in infertile men's semen was elevated with statistically significant differences in oligozoospermia patients/controls (P < 0.0001) and normozoospermia patients/controls (P < 0.05). In the group with elevated CL-signals, a higher percentage of spermatozoa with a pathologic morphology was detected (P = 0.05). In the groups with pathologic results of eosin- and water-tests, the CL-counts were elevated (P < 0.006; P < 0.03). The spermatozoa motility in the group with elevated CL-counts was significantly reduced after 4 h (P < 0.05). The CL-signals correlated inversely with the results of the bovine mucus-penetration-test (r = -0.67), P < 0.0001). In conclusion, semen samples of 28% of our patients showed elevated CL-signals; these were associated with pathological results of membrane integrity-tests. The negative correlation of CL with the results of Penetrak-test reflects its importance to depict the functional capacity of spermatozoa.

T cells involved in psoriasis vulgaris belong to the Th1 subset.

Although the pathogenesis of psoriasis vulgaris is still unknown, several characteristics point to an immunologically mediated process. Epidermal psoriatic lesions are characterized by a hyperproliferation of keratinocytes and an infiltration of T lymphocytes and granulocytes. Because the former may be mediated in part by lymphokines secreted by T cells, we have focused our interest on the in vivo and in vitro cytokine secretion patterns of T lymphocytes from psoriatic lesions. In five patients T lymphocytes were obtained from epidermal specimens. The cells were propagated with lectin and irradiated feeder cells and subsequently cloned by limiting dilution. The resulting T-cell clones were phenotypically and functionally characterized. Our data show that the majority of T-cell clones were CD4+ (74%), whereas only 25% were CD8+ and 1% were CD4-/CD8-. Also, we have further investigated the cytokine secretion pattern of T-cell lines or CD4+ T-cell clones, respectively. All cells tested produced interferon-gamma whereas only a minority secreted interleukin (IL)-4. Moreover, these cells produced high amounts of IL-2 but only little or no IL-10 or tumor necrosis factor-alpha. To correlate these data with the in vivo situation, biopsies from psoriatic lesions of five patients were investigated for the presence of the mRNA of IL-4, IL-10, and interferon-gamma using the polymerase chain reaction. In these biopsies only the mRNA for the Th1 cytokine interferon-gamma but not for the Th2 cytokines IL-4 and IL-10 could be detected. Identical experiments were performed to test the in vivo cytokine production of synovial fluid mononuclear cells of two patients with arthropathia psoriatica. Again, only the mRNA for interferon-gamma but not IL-4 could be detected. This indicates that T cells involved in psoriasis exhibit a Th1-like cytokine secretion profile.

Streptococcal and staphylococcal superantigens (ETA, SEB): presentation by human epidermal cells and induction of autologous T cell proliferation in vitro.

Streptococcal and staphylococcal toxins are responsible for skin-related clinical conditions, e.g. scarlet fever and toxic shock syndrome. Skin involvement may result from a hypersensitivity reaction to these toxins; however, their precise mode of action has still to be elucidated. The aim of the present study was to investigate the capacity of human epidermal cells to present streptococcal erythrogenic toxin A (ETA) or staphylococcal enterotoxin B (SEB) to autologous T-lymphocytes in vitro. We found a significant T-lymphocyte proliferation response to minute amounts of ETA (p < 0.01) and SEB (p < 0.02) when co-cultured with freshly isolated autologous human epidermal cells. We suggest that human skin may serve not only as an entry for microbial toxin-producing strains but also as an important target for streptococcal and staphylococcal toxin-binding and subsequent T cell proliferation.

Quantification of keratinolytic activity from Dermatophilus congolensis.

The bacterium Dermatophilus congolensis is the causative agent of pitted keratolysis, a skin disease. Infection occurs mainly in keratinized tissues and it is necessary for the organism to produce and excrete exoenzymes which are able to degrade keratin. We investigated the amount of keratinase liberated using Keratinazure as substrate and the fungal protease XI as standard. When compared with uninoculated samples, D. congolensis liberated significant amounts of keratinase during a 12-day incubation period with this substrate. An equivalent of 15 units of protease (keratinase) was produced by 10(7) colony-forming units of D. congolensis during a 12-day period at 37 degrees C. We consider the extracellular proteolytic activity of this bacterium to be responsible for keratinized tissues being the main sites of infection.

Fungal flora of human faeces in psoriasis and atopic dermatitis.

Faeces samples taken from 343 patients with psoriasis and 581 patients with atopic dermatitis were subjected to mycological examination. Yeasts were detected in 68% of the psoriatics and in 70% of the patients with atopic dermatitis but in only 54% of the controls (n = 50). Qualitative analysis revealed a predominance of Candida albicans. Non-pathogenic yeasts constituted only 1% in each of these groups. Of the hyphomycetes, Geotrichum candidum occurred in 22% of the psoriatics, in 10% of the atopic dermatitis patients and in 3% of the controls. Aspergillus species were found in 1% of the patients but not in the controls. Stool samples collected on three consecutive days from 141 patients were examined for yeasts. Qualitative correlation between all three samples was shown in 95% of the patients and quantitative correlation in 38%. Deviations were mainly of exponential magnitude. Germ cell concentration of 10(4) cells per ml and above were measured in 38% of the psoriatics and in 28% of the atopic dermatitis patients but in only 22% of the test subjects with healthy skin. There was no correlation between the concentration levels of yeasts in the faeces and the extent of psoriasis or atopic dermatitis.

Dermatological signs of nasopharyngeal linguatulosis (halzoun, Marrara syndrome)--the possible role of major basic protein.

Two hours after ingestion of improperly cooked meat a German tourist in Tunisia showed coughing, hoarseness, dysphagia, anosmia, frontal headache and epistaxis. At the same time a papular non-itching exanthema developed. The nasal discharge contained nymphs of Linguatula serrata. Histological examination of the papules revealed tissue eosinophilia and 'flame figures'. Nasopharyngeal and skin signs subsided spontaneously within 10 days. The possible role of major basic protein in the pathogenesis of nasopharyngeal linguatulosis is discussed.

Papular eruption in helminth infestation--a hypersensitivity phenomenon? Report of four cases.

This communication reports on generalized papular eruption in Man, coinciding with infestation due to dwarf tapeworm (Hymenolepis nana), pinworm (Enterobius vermicularis) and whipworm (Trichuris trichiura). The assumed allergic-hyperergic reaction was evident from itching, blood and tissue eosinophilia, increased serum IgE and clearing up after focus of infestation therapy. Pinworm and whipworm therapy was initially accompanied by Jarisch-Herxheimer phenomenon.

[The significance of yeasts in seborrheic eczema]. / Zur Bedeutung der Hefen beim seborrhoischen Ekzem.

The high prevalence of seborrheic dermatitis in HIV-infected subjects has led to intensified the discussion of the etiopathogenesis of this dermatological disease. There is increasing controversy about the significance of Pityrosporum in seborrheic dermatitis. On the other hand, recent clinical and experimental data favor the role of intestinal candidiasis in seborrheic dermatitis: a high quantity of Candida in the feces of the affected patients, elevated phospholipase activity of the Candida sp. with special pathogenic relevance for mucosal adhesion and fast and long-lasting regression of seborrheic dermatitis after vigorous therapy with oral nystatin. Similar findings have been recorded in the seborrheic forms of psoriasis.

[Pemphigus herpetiformis--the value of simple diagnostic measures]. / Pemphigus herpetiformis--Wertigkeit einfacher diagnostischer Massnahmen.

Herpetiform pemphigus is a rare variety of pemphigus vulgaris. Although the final diagnosis requires thorough clinical, histological and immunological investigations, there are simple examinations which may be carried out at once (such as assessment of blister stability, negative Nikolski's sign, predominance of eosinophils, spongiotic and non-acantholytic epidermal cells in the Tzanck smear). These ad hoc findings may help the practising dermatologist to tell herpetiform pemphigus from other blistering diseases. We discuss and evaluate the various diagnostic procedures regarding herpetiform pemphigus.