[Autoinflammatory syndromes]
Internist (Berl). 2009 Jun; 50(6): 676-84Lamprecht P, Gross WLIn its strict sense, the term "autoinflammatory syndromes" comprises the hereditary periodic fever syndromes (HPF), which are caused by mutations of pattern-recognition receptors (PRR) and perturbations of the cytokine balance. These include the crypyrinopathies, familial Mediterranean fever, TNF-receptor associated periodic fever syndrome (TRAPS), hyper-IgD and periodic syndrome (HIDS), pyogenic sterile arthritis, pyoderma gangrenosum and acne (PAPA) syndrome, NALP12-HPF, and the Blau syndrome. The diseases are characterized by spontaneous activation of cells of the innate immunity in the absence of ligands. Autoantibodies are usually not found. HPF clinically present with recurrent fever episodes and inflammation, especially of serosal and synovial interfaces and the skin. Intriguingly, PRR-mediated autoinflammtory mechanisms also play a role in a number of chronic inflammatory and autoimmune diseases.
[Hidradenitis suppurativa]
Tidsskr Nor Laegeforen. 2009 May 14; 129(10): 992-6Tolaas E, Knudsen CW, Sviland L, Tønseth KABACKGROUND: Hidradenitis suppurativa is a chronic inflammatory skin disease characterized by recurrent tender nodules and boils, usually in the armpits and groins. Draining fistulas and hypertrophic scarring are hallmarks of more severe disease. The objective of this article is to review the clinical presentation, diagnostic considerations and treatment of the disease. MATERIAL AND METHODS: The article is based on a non-systematic literature search in PubMed, review of dermatology textbooks and the author's personal clinical experience. RESULTS: Hidradenitis suppurativa, also known as acne inversa, is a follicular occlusion disease that can severely reduce quality of life. Staphylococci and other pathogenic bacteria frequently colonize the lesions, but the disease is not primarily a bacterial infection. Smoking and obesity can worsen disease activity. Moderate and severe disease is usually treated with excisional surgery. Antibiotics, often tetracyclines, are indicated for mild disease and as an adjunct to surgery in more severe disease. Antibiotics, however, are not curative. New treatment options, such as TNF-alpha inhibitors and zinc gluconate should still be considered experimental. INTERPRETATION: Hidradenitis suppurativa is probably underdiagnosed. The disease is often recalcitrant to treatment. The effect of medical treatment is not supported by high quality evidence.
Treatment of Acne with Oral Isotretinoin in Patients with Cystic Fibrosis.
Arch Dis Child. 2009 May 21; Perera E, Massie J, Phillips RJBACKGROUND: Theoretical concerns about liver disease and vitamin A deficiency have limited the use of oral isotretinoin for troublesome acne in adolescents with cystic fibrosis. METHODS: We administered oral isotretinoin to 9 patients with cystic fibrosis who had troublesome acne unresponsive to antibiotics. All patients were followed for 1-4 years after cessation of treatment. RESULTS: Isotretinoin treatment cleared active acne lesions in all patients. It was well tolerated and no patient had significant side effects. All nine patients were pleased or delighted with the improvement in their skin. CONCLUSIONS: Adolescents with cystic fibrosis and acne can be treated with oral isotretinoin. Oral isotretinoin should be considered for adolescents with cystic fibrosis who have acne associated with scarring, acne not clearing with topical and antibiotic treatment, acne associated with depression, or severe cystic acne.
Phototherapy, photodynamic therapy and lasers in the treatment of acne.
J Dtsch Dermatol Ges. 2009 Apr 21; Degitz KModern acne therapy uses anticomedogenic, antimicrobial, antiinflammatory, and antiandrogenic substances. As an additional approach in recent years, treatments have been developed based on the application of electromagnetic radiation. Visible light or infrared wave lengths are utilized by most techniques, including blue light lamps, intense pulsed light, photodynamic therapy and lasers. This review evaluates the various methods with regard to efficacy and their current role in the management of acne. Although UV radiation has been frequently used to treat acne, it is now regarded as obsolete due to the unfavorable risk-benefit ratio. Visible light, especially of blue wavelengths, appears to be suitable for the treatment of mild to moderate inflammatory acne. Photodynamic therapy is effective, but, due to considerable immediate side effects, it is best reserved for selected situations. Despite promising observations, intense pulsed light and lasers have to be evaluated in further studies, before they can be recommended.