Dermatologie (Heidelberg, Germany)最新文献

Medical guidelines serve as systematic decision-making aids to ensure evidence-based and appropriate medical management of specific diseases. In Germany, dermatological guidelines are developed under the leadership of the German Dermatological Society (DDG) and the Professional Association of German Dermatologists (BVDD) following the methodology of the Working Group of Scientific Medical Societies (AWMF). In the period 2023-2024, various guidelines in the field of dermatology were revised or newly issued. These include guidelines on the diagnosis and treatment of epidermal necrolysis (Stevens-Johnson syndrome and toxic epidermal necrolysis), atopic dermatitis, hidradenitis suppurativa/acne inversa, cutaneous Lyme borreliosis, primary hyperhidrosis, circumscribed scleroderma, screening for anal dysplasia and anal carcinoma in HIV-infected individuals, aphthae and aphthous lesions of the oral and pharyngeal mucosa, and allergological diagnostics of hypersensitivity reactions to drugs. Additionally, updates were published on basal cell carcinoma of the skin, anal pruritus, anal eczema, actinic keratosis and squamous cell carcinoma of the skin, bee and wasp venom allergy, and hand eczema. This review summarizes the key recommendations and recent updates in dermatological guidelines.

Systemic sclerosis (SSc) is a rare autoimmune connective tissue disease within the spectrum of rheumatologic disorders. It is characterized by a combination of vasculopathy and inflammatory fibrosis affecting the skin and various internal organs. The disease presents with heterogeneous manifestations, which can complicate diagnosis and must be carefully considered during both monitoring and treatment. In recent decades, advances in research have expanded the range of therapeutic options available for SSc. Treatment strategies need to be tailored individually, depending on the specific clinical manifestations in each patient. The aim of this article is to provide an overview of the diverse disease manifestations and current treatment approaches in SSc. For optimal patient care, close collaboration between rheumatologists, pneumologists, gastroenterologists, cardiologists, nephrologists and general practitioners is essential.

Skin pigmentation by the endogenous pigment melanin is a highly coordinated process in which hormones play a crucial role. They are synthesized not only in classical endocrine organs but also in the skin itself, which acts as an independent endocrine organ. Among the endocrine target structures of the skin, the melanocortin 1 receptor (MC1R) is of particular importance. Via its high expression and tonic activity in melanocytes, as well as by binding to natural melanocortins such as α‑melanocyte-stimulating hormone (α-MSH), being generated in the skin following ultraviolet (UV) light irradiation, MC1R crucially contributes to the different skin phototypes. Gene mutations of MC1R resulting in defective cyclic adenosine monophosphate (cAMP)-mediated signalling can lead to a shift of the eumelanin/pheomelanin ratio towards the pro-oxidant, yellowish-orange pheomelanin. In patients with Addison's disease and associated syndromes, ectopic proopiomelanocortin syndrome and primary adrenal cortex insufficiency elevated melanocortin levels result in increased melanin content of the skin. Two synthetic melanocortins, afamelanotide (NDP-α-MSH) and setmelanotide, are currently approved in Germany. By targeting MC1R directly (afamelanotide) or as a bystander effect (setmelanotide), both agents increase the skin melanin content. Non-licenced synthetic melanocortins, on the other hand, are used as lifestyle products in an unregulated manner. Additional hormones regulating melanogenesis and skin pigmentation include estrogens, thyroid hormones, insulin, insulin-like growth-factor‑1 and melatonin. They are of physiological and clinical relevance during pregnancy and in patients with melasma and vitiligo. Autoimmune thyroid disorders and diabetes are associated with non-segmental vitiligo. Melatonin appears to have a lightening effect on skin pigmentation by melanin.

Background: In the second half of the 19th century, the Swiss autodidact Arnold Rikli developed his concept of the "atmospheric cure" as part of naturopathic efforts and implemented it in Veldes (today Bled, Slovenia).

Objective: What concepts did Riki develop and what indications did he see. How is this to be assessed from today's perspective?

Materials and methods: Rikli's own writings are used to evaluate his "light air cure" as a form of phototherapy. Secondary literature is used to evaluate Rikli's status in his time as well as contradictions and ambivalences from a modern perspective.

Results: For Rikli, the central linchpin of naturopathy is the "light air cure", which he combined with hydrotherapy, physical exercise, phytotherapy, and vegetable nutrition. He was very active as a writer with 53 publications in books and magazines and thus gained a great deal of fame. His views were not without controversy. He appeared as an outspoken opponent of conventional medicine and was a declared opponent of vaccination. His merit was to bring "light therapy" into the focus of attention. In the years that followed, it was increasingly scientifically processed and used in conventional medicine, for example, in the treatment of rickets and tuberculosis. Since 1989, the Arnold Rikli Prize for outstanding leadership in the field of photobiology has been awarded in memory of the "father of phototherapy".

Conclusion: As a nonphysician, Rikli popularized phototherapy as part of a holistic treatment concept in the second half of the 19th century and at the beginning of the 20th century. He eventually helped it gain recognition in conventional medicine, which initially focused on its use in nonpulmonary tuberculosis. Rikli's partly pseudoscientific justifications of his treatment methods have hindered their acceptance in medicine. Nevertheless, he is also a pioneer of phototherapy from a modern point of view and developed his own hydrotherapeutic approaches. His activities were also aimed at the development of tourism in the region around Bled. The combination of physical medicine with physical exercise, vegetable nutrition and phytotherapy seems almost modern compared to the spa medicine of its time. This is also recognized by the Arnold Rikli Prize for Photobiology.

This case report discusses a 58-year-old patient with linear porokeratosis who subsequently developed cutaneous squamous cell carcinoma. Porokeratosis is a rare keratinisation disorder which carries a risk of malignant transformation. The patient initially presented with recurrent erosions and ulcerations. After initial reluctance of the patient to seek medical help, biopsies confirmed squamous cell carcinoma in several lesions. These were successfully excised and the patient opted for secondary wound healing. Due to its potential for malignancy, porokeratosis often requires diligent monitoring. It typically features hyperkeratotic lesions that can progress over time. Although there are various treatment options for porokeratosis, long-term efficacy is limited. This case emphasises the importance of early biopsy in chronic, nonhealing wounds, as well as the need for patient education and regular follow-up to effectively manage the risk of malignancy.

Brooke-Spiegler syndrome is a rare autosomal dominant disorder leading to the development of multiple benign adnexal tumors. A 65-year-old patient with a positive family history and multiple trichoepitheliomas and cylindromas has been receiving intermittent dermatological care at our department. In February 2025, tumors on the right ear were excised due to near-complete occlusion of the external auditory canal. This case highlights the importance of individualized dermatosurgical management in Brooke-Spiegler syndrome to achieve functional restoration and esthetic improvement.