[CME Dermatology 24/Answers: Porokeratosis]. / CME-Dermatologie 24/ Antworten: Porokeratose.

CME Dermatology 24/Answers: Porokeratosis Abstract. Porokeratoses are a heterogeneous group of cornification disorders with the characteristic histological feature of the cornoid lamellae in the area of the marginal ridge. It is a rare but characteristic disease that occurs primarily in adulthood. Men are slightly more likely to be affected. The etiopathogenesis that leads to the transformation of the keratinocytes remains unclear; associations with genetic mutations and trigger factors such as UV rays and immunosuppression were observed. Due to the risk of malignant degeneration, consistent sun protection and regular clinical controls should take place. Cryotherapy, ablative laser therapy, curettage, photodynamic therapy or topical application of fluorouracil (5-FU), imiquimod and retinoids can be used to treat itchy, painful or cosmetically disturbing porokeratoses, depending on the location and severity.

[CME Dermatology 24: Porokeratosis]. / CME-Dermatologie 24: Porokeratose CME-Fragen.

CME Dermatology 24: Porokeratosis Abstract. Porokeratoses are a heterogeneous group of cornification disorders with the characteristic histological feature of the cornoid lamellae in the area of the marginal ridge. It is a rare but characteristic disease that occurs primarily in adulthood. Men are slightly more likely to be affected. The etiopathogenesis that leads to the transformation of the keratinocytes remains unclear; associations with genetic mutations and trigger factors such as UV rays and immunosuppression were observed. Due to the risk of malignant degeneration, consistent sun protection and regular clinical controls should take place. Cryotherapy, ablative laser therapy, curettage, photodynamic therapy or topical application of fluorouracil (5-FU), imiquimod and retinoids can be used to treat itchy, painful or cosmetically disturbing porokeratoses, depending on the location and severity.

[CME Dermatology 23/Answers: Hidradenitis suppurativa]. / CME-Dermatologie 23/Antworten: Hidradenitis suppurativa.

CME Dermatology 23/Answers: Hidradenitis suppurativa Abstract. Hidradenitis suppurativa (Acne inversa) is a chronic skin disease characterized by recurrent inflammatory nodules, abscesses, and scarring mainly in the intertriginous areas. The prevalence is estimated to be 0.05 to 4.1 % worldwide, with women being more commonly affected. The exact pathogenesis remains unknown, but hair follicle occlusion and inflammation seem to be the initiating events. Several epidemiological correlations with smoking and obesity as well as mutations in gamma secretase genes were described. The therapy depends on the extension of the disorder and should respect the current guidelines. Topical clindamycin is suitable for mild forms of the disease whereas moderate to severe disease requires systemic anti-inflammatory treatment. Surgical intervention is currently the only definitive treatment for hidradenitis suppurativa.

[CME Dermatology 23: Hidradenitis suppurativa]. / CME-Dermatologie 23: Hidradenitis suppurativa.

CME Dermatology 23: Hidradenitis suppurativa Abstract. Hidradenitis suppurativa (Acne inversa) is a chronic skin disease characterized by recurrent inflammatory nodules, abscesses, and scarring mainly in the intertriginous areas. The prevalence is estimated to be 0.05 to 4.1 % worldwide, with women being more commonly affected. The exact pathogenesis remains unknown, but hair follicle occlusion and inflammation seem to be the initiating events. Several epidemiological correlations with smoking and obesity as well as mutations in gamma secretase genes were described. The therapy depends on the extension of the disorder and should respect the current guidelines. Topical clindamycin is suitable for mild forms of the disease whereas moderate to severe disease requires systemic anti-inflammatory treatment. Surgical intervention is currently the only definitive treatment for hidradenitis suppurativa.

Quarantine as a public health measure against an emerging infectious disease: syphilis in Zurich at the dawn of the modern era (1496-1585).

Syphilis is considered as one of the most devastating sexually transmitted diseases in human history. Based on historical records, the "Böse Blattern" (German for "Evil Pocks") spread through Europe after 1495 and shared symptoms with what we know today as syphilis. Many cities took measures to protect their population. Here, transliterations of archival documents from the 15(th) and 16(th) century (provided in the appendix) are used to trace the steps taken by the governing authorities in Zurich to deal with this emerging infectious disease. One of the central measures taken by the city was to establish a quarantine facility referred to as the "Blatternhaus". The city doctors, including the well-known physician and naturalist Conrad Gessner, oversaw the obligatory quarantine and treatment of patients with symptoms. Treatment could range from better nutrition, herbal remedies and skin ointments to aggressive heat therapy and "smoking". Furthermore, the affliction was suspected as a sexually acquired disease, hence prostitutes and infected foreigners were extradited from the city. Meanwhile, the church used its social influence to promote a more "Christian" behavior. In several respects, the public health measures taken in the 16(th) century against a new and menacing epidemic do not diverge in their basic rationale from approaches used during the 20(th) century and today.

Swiss clinical practice guidelines on field cancerization of the skin.

Actinic keratosis (AK) affects millions of people worldwide, and its prevalence continues to increase. AK lesions are caused by chronic ultraviolet radiation exposure, and the presence of two or more AK lesions along with photodamage should raise the consideration of a diagnosis of field cancerization. Effective treatment of individual lesions as well as field cancerization is essential for good long-term outcomes. The Swiss Registry of Actinic Keratosis Treatment (REAKT) Working Group has developed clinical practice guidelines for the treatment of field cancerization in patients who present with AK. These guidelines are intended to serve as a resource for physicians as to the most appropriate treatment and management of AK and field cancerization based on current evidence and the combined practical experience of the authors. Treatment of AK and field cancerization should be driven by consideration of relevant patient, disease, and treatment factors, and appropriate treatment decisions will differ from patient to patient. Prevention measures and screening recommendations are discussed, and special considerations related to management of immunocompromised patients are provided.

Primary cutaneous follicle center lymphoma with diffuse CD30 expression: a report of 4 cases of a rare variant.

BACKGROUND: CD30 is expressed in aggressive and Epstein-Barr virus-associated forms of B-cell non-Hodgkin lymphomas, but is rarely expressed by the majority of tumor cells in primary cutaneous B-cell lymphomas (CBCLs). The expression of CD30 in CBCLs may be at risk for misinterpretation as an unequivocal indicator of a highly aggressive form of the disease. OBJECTIVE: We report 4 cases of low malignant primary cutaneous follicle center lymphoma (PCFCL) with diffuse and strong expression of CD30 by the majority of neoplastic cells. RESULTS: The patients included 3 men and 1 woman with tumors on the scalp (3 patients) and chest wall (1 patient). The histologic examinations revealed a mixed, diffuse, and follicular growth pattern with CD20(+), bcl-6(+), and bcl-2(-) tumor cells. Seventy percent to 90% of the tumor cells expressed CD30. Clonal rearrangement of immunoglobulin heavy chain genes was found in 1 of 4 cases. None of the 3 cases yielded positivity for Epstein-Barr virus RNA. LIMITATIONS: The study is limited by the small number of patients. CONCLUSIONS: This rare variant of CD30(+) PCFCL needs be distinguished from CD30(+) aggressive B-cell lymphomas. CD30 in this variant of CBCLs may serve as a therapeutic target for anti-CD30 antibody-based strategies.

Gamma-linolenic acid levels correlate with clinical efficacy of evening primrose oil in patients with atopic dermatitis.

INTRODUCTION: Atopic dermatitis (AD) has been related to a deficiency of delta-6-desaturase, an enzyme responsible for the conversion of linoleic acid to gamma-linolenic acid (GLA). Evening primrose oil (EPO) contains high amounts of GLA. Therefore, this study investigated whether EPO supplementation results in an increase in plasma GLA and its metabolite dihomo-gamma-linolenic acid (DGLA) correlating with clinical improvement of AD, assessed by the SCORing Atopic Dermatitis (SCORAD) index. METHODS: The open study included 21 patients with AD. EPO (4-6 g) was administered daily for 12 weeks. Before treatment, and 4 and 12 weeks after initiation of EPO supplementation, objective SCORAD was assessed and plasma concentrations of GLA and DGLA were determined by gas chromatography. RESULTS: A significant increase in plasma GLA and DGLA levels and a decrease in the objective SCORAD were observed 4 and 12 weeks after initiation of EPO treatment. In the per-protocol population (n = 14), a significant inverse correlation between the changes in plasma GLA levels and SCORAD was found (P = 0.008). CONCLUSION: The clinical disease activity under EPO treatment correlates with the individual increase in plasma GLA levels. Thus, the results of this pilot study indicate that an increase in plasma GLA might be used as predictive parameter for responsiveness of AD to EPO therapy.

[Anorectal manifestations of sexually transmitted infections]. / Sexuell übertragbare Infektionen - anorektale Manifestationen.

The incidence of sexually transmitted infections is rising in Europe and in Switzerland since the beginning of the third millenium. Many organisms may affect the perianal skin and the anorectum. While some of these infections are a result of contigous spread from genital infection, most result from receptive anal intercourse affecting males who have sex with males but is seen increasingly in females as well since there is evidence of the increasing popularity of anal sex among heterosexuals. The symptoms of specific infections are largely dependent on the route and site of inoculation. Organisms that cause typical genital symptoms - such as syphilis, chancroid, herpes simplex or HPV-infection - result in similar symptoms when the perianal skin, the anoderm or the distal anal canal are the site of infection. Patients with proctitis may have unspecific signs in various degrees including mucous discharge, rectal bleeding, anorectal pain, superficial ulcers and sometimes generalized lymphadenopathy and fever. It is of utmost importance to include STIs (e. g. lymphogranuloma venereum, gonorrhea, non-LGV-chlamydia and herpes simplex) in the differential diagnosis in these patients. Unfortunately rectal infection with chlamydia and gonorrhea is asymptomatic in the majority of cases of men having sex with men and in high-risk females. A careful history and physical examination is essential in establishing a correct diagnosis, usually supported by proctoscopy, culture, PCR, serology and histology. Certain organisms, more commonly thought of as food- or water-borne disease may be sexually transmitted by direct or indirect fecal-oral contact from various sexual practices.

[Sexually transmitted infections: update 2013]. / Sexuell übertragbare Infektionen: Update 2013.

Although there have been significant advances in diagnosis, treatment, and education, the occurence of STI continues to increase worldwide as well as in Switzerland. The most common STI include human papillomavirus (HPV), chlamydia, herpes, simplex virus types 1 and 2, gonorrhea, syphilis, hepatitis B and trichomoniasis. Diagnosis and treatment continue to be major public health challenges because of the asymptomatic nature of many of these infections. Recent studies showed favorable epidemiologic trends for HPV infection in vaccinated females as well as in some males. On the other hand we are confronted with rising incidences e.g. in urethritis with various causative agents and in syphilis cases. Alarmingly during the last 3 years gonococci have become less susceptible to our remaining antibiotics, necessitating strict adherence to updated treatment guidelines. Other trends in STIs and their consequences are discussed.

Mycoplasma pneumoniae and mucositis--part of the Stevens-Johnson syndrome spectrum.

BACKGROUND: Mycoplasma pneumoniae may induce mucosal inflammation, referred to as M. pneumoniae-associated mucositis (MPAM). There is no generally accepted definition of MPAM, since there may be mucosal lesions only, or mucosal and minimal skin lesions. PATIENTS AND METHODS: We conducted a literature review of MPAM, paying particular attention to pathogenesis, clinical manifestations, treatment decisions, and prognosis. RESULTS: We identified 32 cases of MPAM (median age 13.5 years), whereof 23 patients were otherwise healthy children and young adolescents (72%). M. pneumoniae infection was associated with fever and respiratory symptoms in all calls; it was confirmed by serology (n = 30) and/or PCR (n = 9). Oral lesions were present in all cases, followed by ocular (97%) and uro-genital lesions (78%). Despite the syndrome's name postulating the absence of cutaneous involvement, minimal skin lesions occurred in 31%. Treatment regimens included systemic antibiotics (100%) and systemic anti-inflammatory treatment with corticosteroids (31%) or immunoglobulins (9%). Macrolides were given in 81%, with failure, relapse, and/or worsening in one-third of patients. No patient suffered long-term sequelae. CONCLUSION: MPAM is a distinct extra-pulmonary manifestation falling into the continuum of Stevens-Johnson syndrome. This entity may be due to inflammatory mechanisms suggesting that systemic anti-inflammatory treatment is even more important than antimicrobials.

Photoprotection in transplant recipients.

Skin cancers remain the most commonly diagnosed neoplasms among organ transplant recipients (OTRs), comprising nearly 40% of all posttransplant malignancies. The occurrence of skin tumors has a considerable impact on the quality of life and the overall survival of OTRs. Ultraviolet radiation (UVR) is the major environmental cause of nonmelanoma skin cancers, and it is therefore pivotal to protect the skin from UVR in order to prevent de novo and progression of preinvasive and invasive malignancies. The present chapter delivers background information on UVR, photoprotection and accompanying concerns regarding vitamin D, as well as educational and behavioral aspects of photoprotection in OTRs; some common misconceptions and mistakes regarding photoprotection are addressed as well.

Congenital syphilis in Switzerland: gone, forgotten, on the return.

INTRODUCTION: Acquired syphilis has re-emerged in many Western European countries. In contrast to international guidelines, screening for syphilis in pregnancy is not generally recommended in Switzerland. There has been an increase in the incidence of laboratory syphilis notifications in recent years, regardless of gender and age. METHODS: We conducted a retrospective study, evaluating the total numbers of pregnant women with positive syphilis serology (Treponema pallidum Particle Agglutination assay, TPPA) from 2000 to 2009, and evaluated the clinical management and outcome of their offspring. In addition, we sought to determine cases of infectious syphilis (primary, secondary, and early latent syphilis) reported to the Swiss Federal Office of Public Health in women of childbearing age, which has previously been shown to precede changes in the incidence of congenital syphilis within a population. RESULTS: Out of 13,833 women who gave birth at our institution, positive syphilis serology was found in 9 pregnant women during the 10 years studied. A total of 6 pregnant women were diagnosed having syphilis infection during pregnancy. Regarding their offspring, 8 of the 9 newborns were tested serologically. One neonate experienced congenital syphilis because the adequately treated mother developed re-infection after treatment. Within the Swiss population, infectious syphilis cases in women of childbearing age increased substantially from 2006 to 2009. CONCLUSIONS: The epidemiologic data suggest that congenital syphilis could become a medical problem in Switzerland due to the rise of infectious syphilis cases in women of childbearing age that have been shown to be followed by changes in the congenital syphilis incidence. The persistence of congenital syphilis in Switzerland along with this rise of infectious syphilis in women of childbearing age suggests a potential for improvement of prenatal care and syphilis control programmes.