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Classical variants of specific cutaneous LE lesions are chronic discoid LE (CDLE) and subacute cutaneous LE (SCLE). CDLE and SCLE may appear at any age; however, the most common age of onset is between 20 and 40 years, with a female predominance of 3:1 in CDLE and 3-6:1 in SCLE. Nonspecific LE skin lesions such as generalized or acrolocalized vasculitis (4-30%), livedo reticularis (22-35%), and alopecia (38-78%) are frequently seen in patients with cutaneous LE. Other typical cutaneous LE subsets such as LE profundus/panniculitis, LE tumidus, urticaria vasculitis, hypertrophic LE, and bullous LE are rather rare variants. Butterfly rash and/or macular exanthema are characteristic skin lesions of systemic lupus erythematosus (SLE) rarely found in patients with cutaneous LE.

Shower PUVA is a new variant of photochemotherapy suitable for therapy of various skin disorders. Psoralen, e.g. trioxsalen-containing water recirculates in a closed shower system and wets the skin continuously. After showering, whole-body UVA irradiation (320–400 nm) is performed. In order to prove the equal distribution of photosensitivity in vivo minimal phototoxic dose (MPD) was determined in different skin areas of healthy individuals. Additionally, we investigated the accumulation of trioxsalen in psoriasis lesions under the conditions described by quantifying psoralen in scales collected after showering. In a randomized study 20 healthy volunteers (skin type I–III) took showers for 5 and 10 min in trioxsalen (0.27 mg/l)-containing water at 37°C. Immediately afterwards, MPD was tested on the inside of the upper arms and on the buttocks by using a polychromator light source (315–400 nm). The applied UVA doses were 0.06–0.75 J/cm2 with steps of 0.125 J/cm2. MPD was evaluated after 72 h. Equal distribution of photosensitivity was defined as equal MPD on the insides of the upper arm and the buttocks (±0.125 J/cm2). Skin scales of 21 patients with psoriasis were collected by scratching after showering with trioxsalen-containing water (0.27 mg/l) for 5 min. For quantification of trioxsalen in the scales HPLC was performed. An equal distribution of photosensitivity was achieved in 70% (14/20) cases after 10-min showering in trioxsalen-containing water. Showering for 5 min only revealed a 30% (6/20) rate of equal distributed photosensitivity. After 10-min shower time MPD was 0.325 J/cm2 (median; range: 0.06–0.625 J/cm2). The average amount of trioxsalen found in the scales was 2.03 ng/mg scales (range: 0.38–7.2 ng/mg). For shower PUVA using trioxsalen, 10 min shower time is recommended to achieve sufficient distribution of photosensitivity on the skin. Clinical efficacy of shower PUVA can be explained by skin accumulation of trioxsalen which enters from the aqueous phase into the upper skin layers in detectable amounts. This is the first report demonstrating the efficacy of shower PUVA which in short shower time allows an uptake of psoralen by the skin.

The proapoptotic potential of tumor necrosis factor-alpha (TNF-alpha) has been demonstrated for various cell types, whereas nuclear factor-kappaB (NF-kappaB) is known to support the transcription of prosurvival genes. In the present study, investigation of normal human melanocytes revealed induction of apoptosis after TNF-alpha treatment (100 U/ml) in only 3 out of 11 cultures analyzed, whereas 8 cultures remained largely resistant. In sensitive cultures, NF-kappaB binding activity was found increased after TNF-alpha treatment; apoptosis-resistant cells were characterized by relatively high basic NF-kappaB binding activities and did not show NF-kappaB activation after TNF-alpha treatment. Inhibition of NF-kappaB by a specific inhibitor, Bay-11, either induced apoptosis itself or resistant melanocyte cultures became sensitive to TNF-alpha treatment. No correlation was found between apoptosis sensitivity and the expression of TNF receptor-1 or the expression of Bax, Bcl-2 and Bcl-X(L). A strong correlation, however, was found regarding the pigmentation degree, as high pigmentation correlated with apoptosis resistance and sensitive melanocyte cultures were weakly pigmented. These data may indicate that in cultured melanocytes, high levels of melanogenesis lead to an increase in oxidative stress which itself causes NF-kappaB activation. NF-kappaB mediates the transcription of antiapoptotic factors which may block TNF-alpha-induced apoptosis at early steps of the signal cascade.

It has been described that treatment of cells with high dose psoralen and UVA induce the production of reactive oxygen species (ROS) leading to DNA damage. Transcription factor nuclear factor kappa B (NFkappaB) plays a crucial role in regulating not only cell growth but also cell differentiation, and ROS seem to be partly involved in these mechanisms. The aim of this research was to find out the effect of a combined treatment with trioxsalen (TMP)/UVA on NFkappaB binding activity in HaCaT keratinocytes. HaCaT keratinocytes were treated with 27 microg/l TMP. This concentration did not affect the proliferation rates, nor was it toxic, as shown by cytotoxicity assays. After treatment with TMP with or without UVA (1 J/cm(2)), NFkappaB binding activity in nuclear protein extracts was measured by electrophoretic mobility shift assays. The effect on cytokines and cytokine receptor genes was investigated using cDNA expression arrays. An inhibitory effect on NFkappaB binding activity was found between 30 and 60 min after TMP supplementation of the culture media. UVA irradiation induced a 2-fold increase in NFkappaB binding activity in TMP supplemented HaCaT keratinocytes compared with the non-irradiated control. In addition, NFkappaB binding activity was higher after UVA irradiation with TMP than in UVA irradiated cells in the absence of TMP. TGF-alpha, IL-1R, IL-2Ralpha, IL-12beta and PDGF expression was induced by UVA. However, all of them except PDGF were inhibited by combined TMP/UVA treatment. Using an inhibitor of NFkappaB activation, we found out that under these conditions, these cytokines or cytokine receptor genes are apparently not regulated by NFkappaB. Our results indicate that a combined TMP/UVA treatment of HaCaT keratinocytes induces NFkappaB binding activity, and that this is a synergistic effect. The investigated cytokines, and cytokine receptor genes do not seem to be NFkappaB regulated; however, TMP shows anti-inflammatory capacities in vitro.

Nuclear factor-ĸB (NFĸB) is a pleiotropic transcriptional activator, which is a sensitive transcriptional factor for free radicals and activates multiple target genes. UVA is very efficient in inducing free radicals in human skin cells. L-ascorbic acid is regarded as a scavenger of UVA-induced free radicals in human keratinocytes. In epidermis, melanocytes and keratinocytes play an important protective role against skin photodamage. In the present study, we aimed to investigate the role of NFĸB on photodamage in melanocytes and keratinocytes. Normal human melanocytes (NHM) and HaCaT keratinocytes were treated with UVA (500 mJ/cm², 1,000 mJ/cm2) and/or L-ascorbic acid (100 µM, 250 µM). NFĸB binding activity was analysed by electrophoretic mobility shift assay. NFĸB binding activity was increased by UVA irradiation in HaCaT keratinocytes, but it was not affected in NHM. On the other hand, L-ascorbic acid decreased NFĸB binding activity both in UVA-irradiated and in non-irradiated NHM. In contrast, NFĸB binding activity in HaCaT keratinocytes was increased after treatment with L-ascorbic acid. In addition, L-ascorbic acid synergistically induced NFĸB binding activity with UVA irradiation. The contrary response on NFĸB binding activity in NHM and HaCaT keratinocytes indicated that the redox regulation might be different on photoprotective action in melanocytes and keratinocytes.

Classic variants of cutaneous borreliosis are erythema chronicum migrans (ECM), lymphadenosis benigna cutis (LBC) and acrodermatitis chronica atrophicans (ACA). Other dermatoses have been reported in the literature as possibly linked to borreliosis. A 59-year old female patient was seen in the late phases of cutaneous borreliosis with histologically confirmed ACA. In addition, prominent livedo racemosa was seen on the legs, also showing tissue changes similar to those of ACA. Borrelia burgdorferi infection was serologically confirmed by the presence of anti-IgM and anti-IgG antibodies. The clinical spectrum of late cutaneous borreliosis should be enlarged to include livedo racemosa.

Radiation-induced acne is a rare, clinically and pathogenetically ill-defined acneiform dermatosis with special features that may occur in irradiated skin areas especially after high doses of deeply penetrating radiation. We report on a patient with an oropharyngeal carcinoma who developed severe radiation-induced acne including comedones and cysts as well as few inflammatory papules and pustules in a skin area irradiated with up to 63 gray of a 6 MeV photon beam. Acnegenic drugs may precipitate the disease; our patient was on longterm therapy with carbamazepine whose acnegenic potency is less well documented than that of testosterone or glucocorticoids. Treatment of radiation-induced acne is comedolytic; topical retinoids are especially valuable.

Contact allergy to oil of turpentine was reported to have become rare. However, the evaluation of standardized data of 45,005 patients tested 1992-1997 in 30 Dermatological Centers associated with the German-Austrian Information Network of Departments of Dermatology (IVDK) showed an increase in positive patch test reactions to turpentine from 0.5% during the years 1992-1995, up to 1.7% in 1996 and 3.1% in 1997. In particular, 17,347 patients tested in 1996-1997 were evaluated in detail by comparing 431 individuals with positive patch test reactions with the rest of the group found negative to turpentine. Using the so-called MOAHLFA index, the following characteristics were shown. Turpentine allergy (a) was found to be significantly less frequent in men and in patients with occupational dermatitis, (b) showed no difference in its association with atopic dermatitis, (c) patients with turpentine allergy had significantly less symptoms of the hands, more symptoms of the legs or in the face and (d) were significantly more often aged over 60 years. Also, patients sensitized to turpentine had increased rates of additional sensitizations. The definite reason for the increase in turpentine sensitization in the population tested here is not clear. Therefore, a detailed exposure analysis is necessary; the new increase in turpentine allergies may be due to popular topical remedies or household chemicals.

A 76-year-old female patient developed severe manifestations of a kaposi-like acroangiodermatitis (so-called Mali's disease) due to chronic venous insufficiency of the lower extremities. The patient presented with large areas of confluent, violaceous or brown-black papules on both lower legs. Histologically, proliferation of thick-walled capillaries was seen in the upper dermis consisting of fully differentiated endothelial cells, as shown by immunohistochemistry. In contrast to true Kaposi's sarcoma, human-herpes-virus-8 DNA could not be detected by polymerase-chain-reaction in this condition. We review the diagnostic criteria used to distinguish between acroangiodermatitis, also called pseudo-Kaposi's sarcoma, and the true Kaposi's sarcoma.

Die perforierenden Dermatosen bilden eine heterogene Gruppe von Erkrankungen, die durch transepidermale Eliminationsphanomene gekennzeichnet sind. Abzugrenzen sind die primar perforierenden Dermatosen von solchen perforierenden Dermatosen, bei denen Perforation oder Elimination sekundar und als seltenes Teilsymptom bei bestehenden Dermatosen anderer Provenienz auftreten konnen. Zu den primar perforierenden Dermatosen zahlen dabei als Prototypen die Hyperkeratosis follicularis et parafollicularis in cutem penetrans (M. Kyrle), die Elastosis perforans serpiginosa und die perforierende Follikulitis. Die erworbene reaktiv perforierende Dermatose (syn. erworbene reaktiv perforierende Kollagenose) stellt zusammen mit der hereditaren, reaktiv perforierenden Kollagenose eine weitere Variante der primar perforierenden Dermatosen dar. Wir stellen hier eine 84jahrige und eine 96jahrige Patientin mit einer erworbenen reaktiv perforierenden Dermatose vor. Bei beiden Patientinnen lagen eine diabetogene und eine hyperurikamische Stoffwechsellage vor. Eine Therapie mit Allopurinol (100 mg/d) fuhrte bereits nach kurzer Dauer (1 bis 2 Wochen) zu einer weitgehenden Abheilung der disseminierten Lasionen. Uber einen Nachbeobachtungszeitraum von jeweils 6 Monaten befanden sich beide Patientinnen in Vollremission. Diese Beobachtungen belegen erneut die Existenz und Massivitat der Erkrankung und zeigen, das Allopurinol neben der bekannten urikostatischen Wirkung moglicherweise in der Haut immunologische oder differenzierungsmodulierende Effekte vermittelt.

Allergische Reaktionen induziert durch das Antimykotikum Nystatin sind trotz des jahrelangen weitverbreiteten Einsatzes eine Raritat. Wir konnten einen Fall beobachten, bei dem es nach oraler Nystatinanwendung zur Entwicklung einer akuten generalisierten exanthematischen Pustulose mit Allgemeinsymptomatik (Fieber, Arthralgien) kam. Die Kausalitat wurde durch eine positive Intrakutantestung mit Nystatin-haltigen Arzneimitteln gesichert. Immunhistologisch fanden sich in den Testreaktionen positive Ablagerungen von Komplement C3, wohingegen zirkulierende Immunkomplexe nicht nachweisbar waren. Immunologisch ist der beschriebene Fall am ehesten als Typ-II-Reaktion nach Coombs und Gell einzuordnen. Unter systemischer Prednisolongabe kam es innerhalb von 11 Tagen zu einer kompletten Remission. Bei 2 Probanden, die berichteten, zuvor niemals Nystatin eingenommen oder lokal angewandt zu haben, wurde ebenfalls eine Intrakutantestung mit Nystatin-haltigen Arzneimitteln durchgefuhrt, die zunachst unauffallig war. Allerdings entwickelten sich bei beiden Probanden an den Injektionsstellen nach 3 Wochen juckende erythematose Papeln. Offenbar besteht fur Nystatin ein bislang nicht bekanntes Sensibilisierungsrisiko durch die Intrakutantestung.

Insektengiftallergien fuhren in Deutschland zu 10–40 Todesfallen pro Jahr. Die Einleitung und Aufrechterhaltung einer Hyposensibilisierungsbehandlung kann fur die betroffenen Kranken lebensrettend sein. Im Rahmen einer prospektiven Studie mit einem gereinigten Wespengiftpraparat an 20 Wespengiftallergikern (klinischer Schweregrad II–IV) wurden die Wirksamkeit und das Auftreten von Nebenwirkungen wahrend der einleitenden Schnellhyposensibilisierung genau dokumentiert. Die Indikation zur Behandlung wurde individuell auf Grund einer Punkteskala gestellt, mit der a) die Schwere der klinischen Symptomatik, b) die Befunde der In-vivo- und In-vitro-Diagnostik und c) das Expositionsrisiko bewertet wurden. Die Schnellhyposensibilisierung erfolgte unter stationaren Bedingungen nach einem standardisierten 8-Tage-Schema. Nach Abschlus der Behandlung vertrugen alle Patienten die angestrebte Erhaltungsdosis von 100 μg des gereinigten Wespengiftpraparates komplikationslos, auch wenn Dosen von 0,01 bis 10 μg (Median 0,2 μg) zu Lokalreaktionen bei allen Patienten fuhrten. Bei 40% der Kranken traten subjektive Nebenwirkungen (Median der Schwellendosen: 30 μg, Spannbreite 0,2–60 μg) und bei 30% objektive systemische Nebenwirkungen auf (Median der Schwellendosen 55 μg, Spannbreite 20–80 μg), doch die gesamte Nebenwirkungssymptomatik war leichten klinischen Schweregrades. Die kumulativen Tagesdosen beim Auftreten objektiver systemischer Nebenwirkungen betrugen 39 bis 210 μg (Median: 135 μg) und lagen bei 83% der Patienten oberhalb von 100 μg. Unsere prospektive Studie bestatigte, das die Schnellhyposensibilisierung mit dem gewahlten Schema zur Toleranzinduktion gegenuber dem Wespengiftpraparat wirksam ist. Die Verwendung lyophilisierter, gereinigter Praparate brachte allerdings im Hinblick auf die Nebenwirkungen keine Vorteile mit sich.

Summary Scleromyxoedema, a disseminated papular and sclerotic variant of lichen myxoedematosus, is a rare disease with a chronic progressive course, and little tendency towards spontaneous remission. The treatment of scleromyxoedema has been largely ineffective. Aggressive chemotherapeutic agents have been used, often leading to therapy-related morbidity and mortality. We report a 41-year-old woman with scleromyxoedema, associated with a monoclonal gammopathy of IgG-k type, whose condition almost completely cleared with 12 monthly sessions of extracorporeal photopheresis. The patient had previously not responded to isotretinoin, and chlorambucil with prednisolone.

Wir berichten uber einen 32jahrigen Patienten mit fortgeschrittenem, erworbenen Immundefizienz-Syndrom (AIDS), bei dem eine schwere gastrointestinale Kandidose festgestellt wurde. Initial erfolgte eine Behandlung mit Fluconazol 200 mg/d uber5 Monate, die dann mit Itraconazol 200 mg/d uber einen weiteren Monat kombiniert wurde. Die Kandidose zeigte sich klinisch resistent auf beide Azol-Antimykotika. Die antimykotische Aktivitat von Fluconazol, Itraconazol und auch von Ketoconazol gegen Candida albicans wurde in vitro mit dem Mikrodilutionstest durch Bestimmung der 90%-Hemmkonzentration ermittelt. Eine hohe minimale Hemmkonzentration (MHK) wurde fur Fluconazol (50 μg/ml) festgestellt gleichbedeutend mit einer Fluconazol-Resistenz. Die Empfindlichkeit war gegen Itraconazol grenzwertig (MHK 0,125 μg/ml) und gegen Ketoconazol deutlich erniedrigt (MHK 0,25 μg/ml). Im Blutplasma lies sich daruber hinaus ein erniedrigter Itraconazol-Spiegel nachweisen. Bei HIV-Patienten ist die gastrointestinale Absorption von Azol-Derivaten haufig vermindert, so das sich die klinische Resistenz von Itraconazol, neben einer grenzwertig erniedrigten Empfindlichkeit von Candida albicans, auch durch eine reduzierte Absorption erklaren last.

We report the case of drug-induced, acrolocalized Kaposi sarcoma (KS), arising multicentrically in both palms and soles of a male patient who has had widespread psoriasis since 12 years of age. This 59-year-old man, of Mediterranean origin, was HIV antibody-negative and had received oral prednisolone treatment over 5 months for chronic obstructive lung disease (initial dose: 75 mg/d). Eight months after discontinuing oral treatment the KS nodules regressed spontaneously and finally disappeared completely without additional treatment. Light and electron microscopic investigations confirmed the diagnosis of KS, whereas laboratory tests excluded HIV infection and suggested mild immune dysfunction. The existence of HLA loci predisposing to KS and to psoriasis (A1, DR5, DR7, DR11) was characteristic for the simultaneous occurrence of these two diseases. This case report demonstrates the complex interrelationships between genetic predisposition, drugs leading to immune suppression, and the evolution of an unusual neoplasm.

Reactive oxygen species can cause harmful effects in keratinocytes and fibroblasts if antioxidative defence mechanisms are exhausted. Therefore, it seems to be reasonable to prove if oral supplementation with various nutrient antioxidants is useful in prevention or treatment of skin disorders especially in those mediated by UV irradiation. Betacarotene, ascorbic acid and tocopherol have been tested alone or in combination for prevention of sunburn, photodermatoses and photocarcinogenesis with divergent results. Other candidates for oral antioxidative supplementation in humans are selenium and polyphenols. However, clinical data are limited or missing up to date.

2-Chloracetophenone (CN) is widely used as tear gas by police and civilians for self-defence. It may affect the eyes, respiratory system and skin, sometimes causing serious injuries. Both irritative and allergic contact dermatitis have been described. We report three police officers who experienced accidental escape of CN from their professional tear gas canisters. All of them showed localized dermatitis at the site of contact to CN, while widespread lesions appeared after 4 days in one case. Patch tests with the original involved tear gas dissolved in acetone (at 0.1-0.0001%) indicated an allergic reaction in two patients and an irritative reaction in the third. Occupational contact dermatitis due to CN seems to occur among police officers more often than is generally known. Infrequently, extensive health problems may be caused by CN when lesions spread over the integument. Therefore, an improvement of safety measures in occupational CN gas use is needed, especially aiming at avoidance of accidental leakage of canisters.

An HIV-1 infected immunosuppressed patient (CD4+ cell counts: 382 cells/microL; viral load 94,000 copies/mL) with recurrent perianal herpes simplex virus type 2 (HSV-2) infections is described, showing an unusual exophytic tumour resembling a squamous cell carcinoma in the lateral part of the tongue. He also had persistent facial herpes infection, oral candidosis, oral hairy leukoplakia and lymphadenopathy. The presence of HSV-2 was detected by polymerase chain reaction both in smears and in a tissue biopsy taken from the involved tongue area. Treatment with brivudin, a new oral virustatic drug, led to rapid regression of the tumour.

A cutaneous metastatic angiosarcoma was diagnosed in a 79-year-old woman 19 years after radiotherapy for a carcinoma of the uterine cervix. The diagnosis was confirmed by immunohistochemical staining (factor VIII-related antigen and BMA 120) and electron microscopic examination. Surgical treatment of the large tumour, which was situated in the gluteal region, was not feasible, but electron beam therapy resulted in complete remission. However, a further metastasis occurred in the inguinal region, and management by total excision, radiotherapy, and interferon-alpha treatment was unsuccessful. The patient died 28 months after the initial diagnosis of the neoplasm.