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Mycosis fungoides is the most common type of cutaneous T-cell lymphoma, which is usually observed in mid to late adulthood. We report 5 cases of mycosis fungoides in children, all presenting as patch- and plaque-stage disease most commonly involving the buttocks. Histologic examination showed in every case the typical features of mycosis fungoides. In 4 of the 5 cases, the infiltrating lymphocytes were characterized by the T-cell phenotype CD3(+), CD4(+), CD8(+); and in 3 cases, a monoclonal rearrangement of the T-cell receptor gamma (TCR-gamma) gene was found. Three children received topical PUVA treatment, and the other two were treated with mid-potency topical corticosteroids, resulting in complete clinical remission. A management approach to mycosis fungoides with topical PUVA may be appropriate for children.

POEMS syndrome is a rare condition with cutaneous manifestations commonly including angiomas, hypertrichosis, hyperpigmentation, and thickening of the skin. We describe a male patient with a 2-year history of cervical lymphadenopathy, erythematous thickening of the skin on the neck, and progressive walking difficulties. The patient had an occipital erythema with scarring alopecia and sparse follicular pustules at the edge of the lesion. Further investigation revealed symmetric polyneuropathy, hepatosplenomegaly, monoclonal gammopathy, subclinical thyreopathy, and an osteolytic bone lesion of the skull. Histologically, a plasmacytoma with lambda cell restriction was found. The overlying skin showed marked fibrosis, with loss of hair follicles, and a plasma cell infiltrate of polyclonal origin. The cervical lymph nodes showed histologic characteristics of multicentric Castleman's disease, and the skin of the neck showed thickening and vasoproliferation. There was no evidence of further plamacytomas. After excision of the plasmacytoma and postoperative irradiation, the symptoms gradually resolved within a few months. A cicatricial lesion remained on the occiput without further folliculitis or hair loss on the rest of the scalp. This case illustrates the reactive character of POEMS syndrome as a paraneoplastic syndrome in myeloma patients.

The unusual case of an oesophagitis caused by Candida kefyr in a patient with squamous cell carcinoma of the oropharynx is reported. The further implementation of C. kefyr in the production of milk products is discussed.

More than 400 cases of acanthosis nigricans maligna (ANM) and 23 cases of "florid cutaneous papillomatosis" (FCP) were published. ANM is an obligatory paraneoplastic symptom. A 28-year old female patient with an adenocarcinoma of the stomach presented with ANM. We review the number and types of associated cancer with ANM. In most cases and adenocarcinoma of the gastrointestinal tract has been present. We feel that FCP is not an entity of its own but a variant of ANM; the literature supports this view.

Introduced in 1979, isotretinoin remains a major advance in acne therapy. Both hospital- and community-based dermatologists have experience with this valuable drug, which, owing to its causal principle of action, offers a large proportion of patients the prospect of a cure. Isotretinoin is now in use in more than 80 countries and has been prescribed for some six million patients. Based on accumulated clinical findings with the drug, more comprehensive recommendations can now be made with respect to indications, dosing and duration of treatment than was possible when the drug was launched. Experience with the drug also provides a basis for reevaluating its safety profile. Finally, at a time when healthcare resources are limited, it is important to examine the cost-benefit ratio of this acne treatment versus other options. This paper is accordingly intended to provide a critical status report as well as a detailed and balanced set of recommendations for clinical practice.

Background: Kaposi's sarcoma (KS) is the most frequent neoplasm in patients with AIDS, responsible for death in about 20-30% of the affected patients., Objective: To determine the frequency of opportunistic infections (OI) and change of CD4+ cell counts in patients with KS treated with chemotherapy compared to a group of matched-pair patients without chemotherapy., Methods: In a prospective study, the clinical courses of 35 HIV-infected patients with KS treated with chemotherapy were compared with 35 matched-pair patients without chemotherapy., Results: During the observation period of 6 months, 11 OI occurred in 10 patients of the chemotherapy group and 5 OI in 5 patients of the control group. With respect to the changes of CD4+ cell counts, no significant differences could be observed., Conclusion: The risk for OI in HIV-infected patients with KS is increased while receiving chemotherapy. This should be reflected upon when chemotherapy is taken into consideration.

A livid, sharply defined enanthema of the oral mucosa with ulcerations on the soft palate in a patient presenting with de novo acute myeloid leukaemia with prolonged, therapy-induced granulocytopenia (< 0.5 nl-1 for 113 days!) was diagnosed as geotrichosis. Geotrichum capitatum was identified both in vivo and in vitro. Pneumonic infiltrates in the upper lobes of both lungs were treated with amphotericin B infusions. Healing of the aforementioned enanthema was only achieved after addition of 5-fluorocytosine to therapy. Susceptibility determinations with several Geotrichum capitatum isolates led to the conclusion that amphotericin B was unsuitable as a therapeutic agent in this case. 5-Fluorocytosine and itraconazole exhibited superior antifungal and antimycotic activity.

Bacillary angiomatosis (BA) is a rare infectious disease usually associated with HIV infection. Recent molecular biologic investigations confirm that both Rochalimaea henselae and Rochalimaea quintana can cause BA. The bacteria can be identified by Warthin-Starry staining and electron microscopy. The typical clinical signs are solitary or multiple dermal or subcutaneous nodules. Bone, liver, spleen and other organs may also be involved. We describe the clinical and histological features of a 39-year-old HIV-infected patient with cutaneous and bony lesions of BA. All manifestations of BA disappeared during therapy with erythromycin.

Candidosis, cryptococcosis, and histoplasmosis often occur as HIV-associated mycoses. However, aspergillosis can be observed quite recently. The morphology of the pathogen of HIV-associated mycoses in vivo and in vitro is demonstrated and discussed.

Background: We report on a retrospective study in 544 HIV-positive patients, (42 women, 502 men, mean age 35 years) showing CD4 lymphocyte counts below 200 c/mcl or after their cure of Pneumocystis carinii pneumonia, who received 300 mg pentamidine aerosol as prophylaxis against Pneumocystis carinii pneumonia every four week., Patients and Methods: 277 patients were asymptomatic, 120 in the AIDS related complex stage (ARC) and 147 in the full stage of AIDS. The mean follow-up was 14.4 months., Results: A total of 25 cases of Pneumocystis carinii pneumonia was observed (3.83/year): in the primary prophylaxis group 18 (3.25%/year), in the secondary prophylaxis group seven (6.8%/year). By introducing the loading dose (one inhalation per day for five consecutive days for patients with CD4 cell counts below 150 c/mcl) we reduced the percentage of early manifestations within the first three months from 61% to 14%. No extrapulmonary Pneumocystis carinii manifestation was observed., Conclusion: This study supports the efficacy of pentamidine aerosol prophylaxis of primary and secondary Pneumocystis carinii pneumonia.

In an open phase-III study 103 HIV-positive patients with oral candidiasis were treated with oral fluconazole 100 mg/day for 7-21 days (mean 12.2 +/- 6.1 days). Ninety per cent of the patients presented with the full clinical picture of AIDS, in 83% CD4-lymphocytes were < 100/mm3. Clinical and mycological (smear and mouth rinsing) examinations were performed at the start of therapy, after weeks 1, 2, and 3, and at the end of therapy. The clinical findings showed fluconazole therapy to have achieved cure in 71% of the patients and improvement in 16%. Therapy failed in 13%. Mycological tests revealed elimination in 57% and reduction in colony counts in 23% of patients. Therapy failure according to mycological criteria was observed in 20% of all subjects. Adverse events were recorded for 26% of all patients. A causal connection with study therapy was considered as "unlikely" in 20 cases, "questionable" in 17 cases, and "likely" in three cases. Premature discontinuation of fluconazole therapy was required in seven patients, in three of them because of adverse events due to fluconazole. Even in patients with advanced HIV infection and consequently severe immunodeficiency, fluconazole is an important improvement of the therapeutic spectrum.

Bituminous shale, developed from marine sediments during the Jura era, is the raw substance for the production of shale oil. The shale oil undergoes heating, distillation, fractionated refining and sulfonation (according to a patented method) resulting in water-soluble sulfonated shale oils (Ichthyol) for medical purposes. The antifungal effect of sulfonated shale oils have been described earlier. In this study an in vitro method is applied which is based upon the CO2 detection as a measure for the sensitivity of fungi to sulfonated shale oils. In addition to the minimal inhibitory concentration values (MIC values), sub-inhibitory concentrations are also determined. The actual efficacy of these antifungal agents is demonstrated via dose-effect curves. Our results show that the fractions of sulfonated shale oils refined at 150 to 210 degrees C (Ichthyol, dark) are fungicidal in concentrations between 0.2 and 16.8% for yeasts, dermatophytes and other hyphomycetes. The fractions of sulfonated shale oils refined at 85 to 150 degrees C (Ichthyol, light), on the other hand, showed a clearly higher antifungal activity (concentrations between 0.1 and 5.9%) for all fungi tested. An extended exposition (24 to 168 h) of Candida albicans to these fractions resulted in a further increase of fungicidal activity. Due to the complex nature of sulfonated shale oils the chemically defined antifungal substance(s) have not yet been identified.

In a prospective, open, uncontrolled study 25 patients infected with the human immunodeficiency virus with chronic refractory diarrhea and either confirmed cryptosporidiosis (n = 7) or absence of demonstrable pathogenic organisms (n = 18) were treated with a daily oral dose of 10 g of an immunoglobulin preparation from bovine colostrum over a period of 10 days. Among the 7 patients with cryptosporidiosis, this treatment led to complete remission in 3 and partial remission in 2. Among the 18 patients with diarrhea and negative stool culture, complete remission of diarrhea was obtained in 7 and partial remission in 4. In the remaining 2 patients with cryptosporidiosis and the 7 patients with diarrhea but no demonstrable pathogens treatment produced no significant improvement of the diarrhea. Subsequent doubling of the Lactobin dose (2 x 10 g daily) in 8 of the nonresponders led to complete remission in one case and at least partial remission in a further 4 patients. Treatment of refractory diarrhea with 10 g immunoglobulins from bovine colostrum per day constitutes an important therapeutic approach and led to complete (40%) or partial (24%) remission of diarrhea in 64% of the patients described here.

We report the case of a 20-year-old homosexual man with HIV-1 infection presenting with AIDS. An erythemato-squamous, papulo-crustous, non-itching dermatosis of 4 months duration was finally diagnosed as Norwegian scabies in the immunosuppressed. For clinical and epidemiological reasons the high contagiosity of this rare entity requires an appropriate therapy without delay.

A number of in vivo and in vitro results suggest that interferons have an antiretroviral effect on HIV. To check this, 15 HIV-positive patients who had no full-blown AIDS, were treated with recombinant interferon alpha 2b (5 mill. IU s. c. three times a week) over a period of six months. Twelve to 16 weeks after the initiation of treatment, an increase in CD 4 lymphocytes (+16%), NK cells (+16%), lymphocytes stimulation by con A (+ 176%), neopterin (+66%), and beta-2-microglobulin (+19%) was observed. By the end of the study, all these parameters had slightly decreased again. In all patients with CD4 lymphocytes greater than 0.2 c/nl, we observed a decrease in p24 antigen levels, but in patients with CD4 lymphocytes less than 0.2 c/nl, an increase. It would thus seem that any antiretroviral effect of IFN (as shown by the p24 antigen parameter) is more pronounced in patients with superior immune parameters.

To identify the characteristic features of lues in patients infected with HIV, 402 HIV-positive patients were examined for serological and clinical signs of lues. 141 patients (133 male, 8 female, mean age 36 [18-69] years) had a positive lues serology. Treatment for lues was required in 20 of the 141 cases (14%). In ten patients (one case of lues I, nine cases of lues II) dermatological signs were predominant, macular exanthemas (n = 4) and palmo-plantar syphilides (n = 3) being most frequent. Three patients had seropositive latent lues. Eight patients presented with signs of an active neurolues (lues II: n = 1; lues III: n = 6; lues IV: n = 1). In three of the eight cases the serum FTA-ABS-IgM findings were negative. In these three patients the need for a specific treatment was realized only on the basis of cerebrospinal fluid (CSF) examination in conjunction with the clinical findings and the anamnesis. This result makes it very clear that indication for CSF puncture should be more liberal in some HIV infected patients. The markedly high proportion of cases of neurolues (40% of the luetic patients requiring treatment) is possibly due to reactivation of old lues infections.

In 23 patients with HIV-associated Kaposi's sarcoma 53 tumor lesions were treated with fractioned radiotherapy. Indication for the radiotherapy were mostly cosmetic reasons in stigmatizing tumors, but also in several cases pain, oedema or functional deficits as a result of the tumor lesions. 21 patients received orthovoltage irradiation, the remaining four patients were treated with telecobalt therapy. A complete response was observed in 17%, a partial response in 76% and unchanged lesions in 4%. In two cases (4%), both were treated with telecobalt-therapy by large tumor masses, there occurred a further tumor progression inspite of the radiotherapy. In ten lesions, all with partial remission, we later observed a repeated tumor progression. Important side effects were signs of inflammation as mucositis and edema or hyperpigmentation. The occurrence of acute side effects can be reduced by fractionating of the radiotherapy.

47 HIV-positive male patients with cutaneous Kaposi's sarcoma (KS) were treated with recombinant interferon alpha-2A (IFN) in an open, prospective study. 45 patients received 18 million I.U. IFN s.c. daily for the first three months. From the fourth month, the patients received 18 million I.U. IFN s.c. three times weekly. Four of the 45 patients withdrew from therapy because of side effects during the first weeks (weeks 2 to 6) of treatment. The remaining 41 patients were treated for a minimum period of two years or until their death. Two further patients received 36 million I.U. IFN daily for three months. After three months of IFN therapy we observed complete remission (CR) in five of the 41 patients (12%), partial remission (PR) in seven patients (17%), stable disease in seven patients (17%) and progressive disease (PD) in 22 patients (54%). The mean survival time from the beginning of IFN therapy to date is 26 months for the 19 responders (patients with CR, PR or SD; six are still alive) and nine months for the 22 non-responders (patients with PD; all died). Recurrence of tumor progression occurred in 15 of the 19 responders after an average of ten months of continuous IFN therapy. The longest time of survival in this group is 54 months up to now. Of the four remaining patients, all in the CR group, two died after 24 and 26 months of IFN therapy, without any recurrence of KS growth (causes of death: Burkitt's lymphoma and unknown). The two other patients, both still alive, have not suffered any recurrence of tumor progression after 40 and 41 months, respectively.(ABSTRACT TRUNCATED AT 250 WORDS)

In a prospective study, 29 patients were observed over a period of 42 weeks for signs of oral candidosis (OC), immunological parameters and other typical AIDS-related events. Before the study started, no OC was observed in any of the patients. During the observation period, OC was diagnosed in 12 of the 29 patients (41%). 5 of these 12 patients (42%) developed full-blown AIDS during the 42 weeks. In contrast, a progression to AIDS was observed in only 1 of the 17 patients (5.9%) without OC. The laboratory findings for patients with and without OC showed statistically significant differences for neopterin (23.6 against 14.4 nmol l-1), CD4 counts (417 against 763/mm3) and CD4/CD8 ratios (0.45 against 0.85). Based on these results, it seems justifiable to consider prophylactic measures such as pentamidine inhalation and/or treatment with zidovudine in HIV-infected patients with immunodeficiency and occurrence of OC.

The pathogenesis of the HIV associated Kaposi's sarcoma is still unclear, but it appears to be a multifactorial process. Different investigations have shown, that cytokines and growth factors influence the development of KS. Several cases with tumor manifestation after irritation of the skin are demonstrated. Therefore a mechanism of development comparable with the Koebner Phenomenon can be postulated.

A 37-year-old homosexual man suffering from AIDS (= Acquired Immune Deficiency Syndrome) is described. This man died from disseminated Kaposi-sarcomas about one year after establishing the diagnosis.

The case of a 29 year old man is presented, who showed 'sunburn like' reactions following short exposures to light since early childhood. Demonstration of fluorocytes and analysis of porphyrins confirmed the diagnosis of erythropoietic protoporphyria (EPP). It is noteworthy that the patient failed to show clinical signs, characteristic for EPP at the nasal area or the extensor aspects of the hands, whereas indurated plaques with firm skin coloured papules were observed in the shoulder regions. Biopsies taken from these areas revealed PAS-positive material especially around the capillaries of the dermal papillae, a finding typically for EPP. Electronmicroscopic investigation revealed reduplication of the vascular basal lamina and perivascular deposition of a fine fibrillar material. Therefore the cutaneous changes, yet clinically atypical, seem to be correlated with EPP.

We present a patient with neuro-trophic ulceration of the forehead and right nostril following the section of the trigeminal nerve because of tic douloureux. The condition is differentiated from basal cell carcinoma. The literature is briefly reviewed.

Coronavirus-like particles were identified by electronmicroscopy in the feces of homosexual men. The particles banded at a density of 1.21 g/ml after cesium chloride density gradient centrifugation. To determine whether the presence of this virus might be related to clinical symptoms, several patient groups were studied prospectively. In 8 of 16 (50%) homosexual males with acquired immunodeficiency syndrome (AIDS) or unexplained lymphadenopathy syndrome (LAS), coronavirus particles were found. In contrast, such particles were found in none of 18 heterosexual controls and in only 3 of 20 homosexual males without AIDS or LAS. Thus, coronavirus excretion correlated significantly (2 alpha less than 0.01) with the clinical diagnosis of AIDS or with syndromes belonging to the AIDS-related complex. In addition, such particles identified in the serum of one patient with LAS and diarrhea suggest invasion and systemic spread of the agent and underline that this virus behaves differently from "common cold" human coronaviruses.

Results of the isotretinoin (13-cis-retinoic acid, Ro 4-3780) German Cooperative Study Group, with 198 acne conglobata patients being treated in 19 departments are reported. For the first 12 weeks (phase I) there was an open assignment to 0.2, 0.5 or 1.0 mg/kilogram bodyweight (kg bw). This was followed by further 12 weeks (phase II). If there was at least a two-third improvement of lesions, the 0.2 mg/kg bw was continued, and the 0.5 mg/kg bw dose lowered to 0.2 mg/kg bw. If there was no such improvement, the dose was elevated to 0.5 and 1.0 mg/kg bw respectively. The initial high dose group of 1.0 mg/kg bw was divided after twelve weeks into 0.2 mg/kg bw maintenance therapy, or no therapy at all. Non-inflammatory and inflammatory acne lesions from the entire body were counted. Seborrhea was graded on a four scale (0 to 3+). Subjective side effects were registered. Laboratory data included hematological profile with differential counts, creatinin, SGOT, SGPT, alkaline phosphatase, total bilirubin, serum cholesterol and serum triglycerides, and urine analysis. For statistical analysis 171 patients were available, 27 dropped out of the study, mostly for reasons unrelated to the drug. At least 75 per cent improvement was seen, in the 0.2 mg/kg bw group in 73.7 and 59.5 per cent respectively; in the 0.5 mg/kg bw group in 72.5 and 61.2 per cent respectively; and in the 1.0 mg/kg bw group in 85.4 and 92 per cent respectively (phase I t12 and phase II t24 values, respectively). Sebum suppression was dose-related. Subjective side effects were fairly well dose-related, particularly those of skin and mucous membranes. Myalgia was rare. There was a dose-related elevation of triglycerides and cholesterol, but not significant for the means of each group. Single patients did show significant elevation of blood lipids. All other laboratory parameters did not change significantly. Isotretinoin is presently the most effective drug to control severe forms of acne, leading to long lasting remissions.

The effects of unsaturated fatty acids were examined in a double-blind study on 34 patients suffering from atopic dermatitis. In comparison to the placebo group a clinical improvement (24%) was seen after a three month period during which patients had received linoleic acid and gamma-linolenic acid.

We report on a 73-year-old woman who, 12 years after liquid silicone injection for cosmetic purposes, developed siliconoma of her forehead within 9 months. We discuss the clinical and histopathological aspects of this foreign body granuloma.

Operations on the cutaneous surfaces are mainly performed under local anesthesia. Local infiltrative anesthesia is the most frequently used form in cutaneous surgery. Nerve or field block anesthesia can be used in areas like the external ear, nose, digits or penis. Local anesthesia is easy to administer, rapidly effective and relatively free of side effects. In contrast to general anesthesia it can be used in risk patients e.g. with ischemic heart disease or respiratory illnesses. Adverse reactions must be taken into account, especially toxic effects, allergy, drug interactions or traumatic lesions of nerves or blood vessels.

A case of fasciitis with eosinophilia and hypergammaglobulinaemia (Shulman's syndrome) in a 33-year-old patient is described. Both the lower arms and the lower legs were affected. The diagnosis was established by biopsy which revealed cellular infiltration of the deep fascia with thickening. In addition to typical symptoms there was hepatosplenomegaly. Gastrointestinal or haematological disease was excluded. Under systemic corticosteroid administration all symptoms of fasciitis quickly disappeared.

A seventeen year old female with pemphigus vulgaris was treated by exchange plasmapheresis. Plasmapheresis was performed five times over three weeks. Each procedure significantly reduced the serum level of intercellular antibodies. Without immunosuppressive therapy and with only moderate doses of corticosteroids the clinical symptoms had improved after the three weeks period. Plasmapheresis represents an alternative therapeutical possibility in the treatment of pemphigus vulgaris in order to achieve a rapid reduction in circulating levels of the intercellular antibodies.

Twenty-three patients with biopsy-proven Kaposi's sarcoma and HIV infection were treated with recombinant interferon-alpha-2a (Roferon-A). Two dosage regimens were used: 21 patients received 18 X 10(6) units intramuscularly per day for 3 months, followed by injections of 18 X 10(6) units three times a week. The remaining 2 patients were treated with 2 X 18 X 10(6) units i.m. per day for 3 months. Three patients either refused further treatment or were lost to follow-up within the first few weeks. Thus, a median observation period of 7.5 months (range 1.5-17) was available for 20 patients. Within the first 3 months, 6 patients (30%) responded to treatment, 6 patients (30%) showed no progression, whereas in 8 cases (40%) progressive disease was noted. A similar rate of responders versus nonresponders was found after 6 months of observation. In progressive disease, interferon could be effectively combined with cytostatic drugs. Dose-dependent neurological and hematological side effects were observed in a few patients only. Opportunistic infections were diagnosed in 12 patients with a median onset of 6 months (range 1-10) after start of interferon treatment. The total number of lymphocytes expressing the CD4 antigen or the ratio of CD4 to CD8 positive cells were of prognostic value. These data suggest that interferon alpha is an active agent in the treatment of Kaposi's sarcoma, that it shows tolerable side effects and can be combined effectively with cytostatic drugs in case of progression.

A patient with an inborn cellular immunodeficiency syndrome developed a graft-versus-host reaction after a transfusion with packed red cells. This diagnosis was confirmed by skin biopsy and finally proved by tissue typing.

In a multicenter study, 788 patients suffering from severe acne resistant to therapy were treated with isotretinoin by 758 dermatologists in private practices. The study was conducted using standardized test forms and questionnaires. The goal of the study was to establish the risk/benefit ratio of the drug, if it was applied to patients of a dermatological practice. The initial daily dose amounted to 0.5 mg/kg body weight, the time of treatment was 12 to 16 weeks. The clinical efficacy was judged very good or good by 90% of the doctors and patients. The kind, frequency, and intensity of the side effects observed corresponded with those found in previous studies conducted at hospitals. Our critical analysis of the results left no doubt about the therapeutic value and the safety of isotretinoin, if it is used by dermatologists in private practices.

Retinoids possess regulatory influences on growth and differentiation of epithelial tissues. They induce a population of keratinozytes with normal pattern of differentiation, they have antiproliferative properties, and they show antineoplastic effects by inhibition of malignant transformation of cells in vitro. Also the dermis undergoes distinct alterations under oral administration of retinoids. By stimulating T-lymphocytes and by inhibition of neutrophil migration retinoids seem to develop immunmodulating and antiinflammatory effects. The aromatic retinoid Etretinate is therapeutically used in severe forms of psoriasis and in various genodermatoses with disorders of keratinization as for example ichthyosis, dyskeratosis follicularis Darier, and pityriasis rubra pilaris.