Roman Saternus

Article published online: 12 March 2025 © 2025. Thieme. All rights reserved. Georg Thieme Verlag KG Oswald-Hesse-Straße 50, 70469 Stuttgart, Germany

Neoplasien der Hautadnexe (benigne oder maligne) können zum einen von den Talgdrüsen oder Haarfollikel ausgehen, zum andern auch eine Differenzierung Richtung ekkriner oder apokriner Schweißdrüsen zeigen. Auch Mischformen können vorkommen. Maligne Adnextumoren entstehen i. d. R. de novo. Dennoch ist auch die maligne Entartung einer vorbestehenden benignen Läsion möglich. Maligne Adnextumoren der Haut sind im Vergleich zu anderen nicht-melanozytären Hauttumoren insgesamt eine eher seltene Entität. Jedoch zeigt sich, dass die Inzidenz von malignen Adnextumoren in den letzten Jahren zugenommen hat. Ähnlich wie bei anderen Hautkrebsarten sind auch bei malignen Adnextumoren das Alter, UV-Strahlung und Immunsuppression wichtige Risikofaktoren der Tumorgenese. Da sich die Klinik sowohl von malignen als auch benignen Adnextumoren mit Ausnahme des Morbus Paget oftmals ähneln kann und wenig spezifisch ist, kommt der Histopathologie für die korrekte Diagnosestellung eine entscheidende Bedeutung zu. Gemäß der Literatur sind die häufigsten malignen Adnextumore das Talgdrüsenkarzinom (23 %), der extramammäre Morbus Paget (19 %), das Hidradenokarzinom (15 %), das Porokarzinom (7 %) sowie das mikrozystische Adnexkarzinom (MAC) (5 %). Während manche Vertreter prognostisch mit einem Basalzellkarzinom vergleichbar sind (MAC), sind andere durchaus metastasierungsfähig vergleichbar den Plattenepithelkarzinomen (Talgdrüsenkarzinom, Porokarzinom). Andere Vertreter heben sich hier prognostisch besonders negativ ab mit hoher Lokalrezidivquote und Metastasierung, z. B. das Hidradenokarzinom. Dieser Übersichtsartikel fokussiert sich auf diese 5 relativ häufigen Adnex-Malignome, die prognostisch auch die gegebene Streubreite wiedergeben und die Erfordernis einer den jeweiligen Besonderheiten geschuldeten klinischen Versorgung und Nachbetreuung unterstreichen. Adnextumors of the skin (benign or malignant) may originate from the sebaceous glands or hair follicles on the one hand, and may also show a differentiation towards the eccrine or apocrine sweat glands on the other. Mixed forms can also occur. Malignant adnextumors usually develop de novo. However, malignant transformation of a pre-existing benign lesion is also possible. Malignant adnextumours of the skin are a rare entity compared to other non-melanocytic skin tumours. However, the incidence of malignant adnextumors has increased in recent years. As with other skin cancers, age, UV radiation and immunosuppression are important risk factors. Since the clinical presentation of both malignant and benign adnextumors with the exception of extramammary Paget’s disease can often be similar and is not very specific, histopathology is of crucial importance for the correct diagnosis. According to the literature, the most common malignant adnextumors are sebaceous gland carcinoma (23 %), extramammary Paget’s disease (19 %), hidradenocarcinoma (15 %), porocarcinoma (7 %) and microcystic adnexal carcinoma (5 %). While some entities are prognostically comparable to a basal cell carcinoma (MAC), others are quite capable of metastasization comparable to squamous cell carcinomas (sebaceous gland carcinoma, porocarcinoma). Other entities stand out prognostically particularly negatively with a high local relapse rate and metastasis, e. g. hidradenocarcinoma. This review article focuses on these five relatively common adnexal malignancies, which prognostically reflect the given spread and underline the need for clinical care and follow-up due to the particularities. Publication Date: 14 April 2022 (online) © 2022. Thieme. All rights reserved. Georg Thieme Verlag KG Rüdigerstraße 14, 70469 Stuttgart, Germany

We present a 54-year-old male patient with a posttraumatic and postinflammatory elephantiasis nostras verrucosa (ENV) of the left leg due to a minor accident at work (minor injury of the left patella), 5 years ago. After major complications (including development of necrotizing fasciitis), it was necessary to cover the developed skin defects with autologous split skin transplantations. The skin graft showed an initial healing tendency, but during wound dressing changes the patient developed a considerable loss of skin grafts along with an infected wound healing disorder. Additionally, in the following years the patient developed a massive increase in the circumference of the left leg in combination with massive malodorous hyperkeratosis with a sharp circular demarcation line approx. 10cm proximal to the knee. We started an oral therapy with acitretin 40mg/d, an intensified external therapy (stage-adapted antiseptic, aggressive keratolytic) as well as mechanical debridement (scalpel, curette) for mass ablation. In the further course, we could reduce acitretin dose to 10mg every 2 days, showing a long-term stable result. Pathogenetically, an ENV can occur in case of insufficient restoration of lymphatic drainage (after surgery or trauma for example) with a limited clearance of relevant cytokines and growth factors. Due to the rarity of ENV with only a few case reports in literature, there is no established standard therapy. In this case we present a successful treatment of ENV with acitretin.

Although great progress has been achieved during the last decades, the clinical management of organ transplant recipients (OTRs) remains a challenge. OTRs need in general lifelong immunosuppressive therapy that is associated with an increased risk to develop skin cancer and with an unfavorable clinical outcome of these malignancies. Skin cancer prevention measures, including regular full-body examinations, are therefore necessary in OTRs to detect and treat suspicious lesions at an early stage. The frequency of aftercare depends on the individual risk factors of the patient. Patients should apply consistent sun protection with sunscreens and clothing, as well as a monthly self-examination. On the other hand, the need of UVR avoidance increases the risk of vitamin D deficiency, which itself is associated with an increased risk for many diseases, including malignancies. OTRs should therefore be monitored for 25(OH)D status and/or should take vitamin D supplements. It has to be emphasized that an interdisciplinary approach, coordinated by the transplant center, that includes regular skin examinations by a dermatologist, is needed to ensure the best care for the OTRs.

Zusammenfassung: Wir berichten über eine 78-jährige dialysepflichtige Patientin, welche sich mit stark schmerzhaften Nekrosen distal eines Dialyseshunts vorstellte und bei der abschließend ein Steal-Phänomen diagnostiziert wurde. Beim Steal-Syndrom handelt es sich um eine seltene Komplikation eines Gefäßshunts durch Minderperfusion der Extremität distal der AV-Anastomose. Diabetiker sind aufgrund des höheren peripheren Gefäßwiderstandes und der Mikroangiopathie eher gefährdet, ein Steal-Syndrom nach Shuntanlage zu entwickeln. Der vorgestellte Fall soll verdeutlichen, dass das Steal-Syndrom dringlich in die differenzialdiagnostischen Überlegungen bei kutanen Nekrosen am Shuntarm einzuschließen ist und die Patienten schnellstmöglich gefäßchirurgisch vorzustellen sind, um einen Zeitverlust bis zur therapeutischen Intervention mit der Gefahr des irreversiblen Gewebsverlust und damit verbunden ggf. konsekutiv erforderliche Amputationen zu vermeiden.

During the last decade, our scientific knowledge of the pleiotropic biological effects of vitamin D metabolites and their relevance to human health has expanded widely. Beyond the well-known key role of vitamin D in calcium homeostasis and bone health, it has been shown that vitamin D deficiency is associated with a broad variety of independent diseases, including several types of cancer, and with increased overall mortality. Moreover, recent findings have demonstrated biological effects of the vitamin D endocrine system that are not mediated via activation of the classical nuclear vitamin D receptor (VDR) by binding with high affinity to its corresponding ligand, the biologically active vitamin D metabolite 1,25-dihydroxyvitamin D (1,25(OH)₂D). In contrast, many of these new biological effects of vitamin D compounds, including regulation of the circadian clock and many metabolic functions, are mediated by other vitamin D metabolites, including 20-hydroxyvitamin D and 20,23-dihydroxyvitamin D, and involve their binding to the aryl hydrocarbon receptor (AhR) and retinoid-orphan receptor (ROR). In most populations, including the German population, UVB-induced cutaneous vitamin D production is the main source for fulfilling the human body’s requirements of vitamin D. However, this causes a dilemma because solar or artificial UVR exposure is associated with skin cancer risk. In addition to UVB-induced vitamin D production in skin, in humans, there are two other possible sources of vitamin D: from diet and supplements. However, only a few natural foods contain substantial amounts of vitamin D, and in most populations, the dietary source of vitamin D cannot fulfill the body´s requirements. Because an increasing body of evidence has convincingly demonstrated that vitamin D deficiency is very common worldwide, it is the aim of this paper to (i) give an update of the vitamin D status in a population with a western diet, namely, the German population, and to (ii) develop strategies to optimize the vitamin D supply that consider both the advantages as well as the disadvantages/risks of different approaches, including increasing vitamin D status by dietary intake, by supplements, or by UVB-induced cutaneous synthesis of vitamin D.