[Bullous mycosis fungoides-clinical indicator for an aggressive course of disease]. / Bullöse Mycosis fungoides ­ ein klinischer Indikator für einen aggressiven Krankheitsverlauf.

Mycosis fungoides (MF) is defined as the most common cutaneous T­cell lymphoma (CTCL). The bullous form is considered one of its numerous variants. Only a few cases of this rare entity have been described. We report the case of a man with an aggressive course of bullous MF, which led to lethal outcome within a few weeks due to a fulminant sepsis.

S1-Guideline Sebaceous Carcinoma.

Sebaceous gland carcinomas are rare malignant cutaneous adnexal tumors with sebocytic differentiation. The typical predilection area is the head and neck region, where sebaceous gland carcinomas are the most common malignant adnexal tumors of the skin. According to their localization a distinction is made between periocular and extraocular sebaceous gland carcinomas. Muir-Torre syndrome (MTS) should always be ruled out if it is suspected. In terms of prognosis, sebaceous gland carcinomas are potentially aggressive tumors with a clear tendency to recur and metastasize. Only small extraocular sebaceous gland carcinomas that have been completely resected have a very good prognosis. Sebaceous gland carcinomas most frequently metastasize lymphogenously to regional or distant lymph nodes; organ metastasis occurs less frequently. Periocular sebaceous gland carcinomas have a higher metastasis rate (up to 15%) than extraocular sebaceous gland carcinomas (up to 2%). Complete micrographically controlled surgery (MCS) of the primary tumor is the therapy of first choice, regardless of periocular or extraocular localization. Adjuvant or therapeutic radiotherapy may be considered. There is currently no established standard therapy for advanced, inoperable, or metastatic sebaceous gland carcinomas. Local procedures and systemic therapies such as chemotherapy or immunotherapy can be considered. The procedure should be determined individually by an interdisciplinary tumor board. Close follow-up care is recommended for these potentially aggressive carcinomas.

Poxvirus infections in dermatology - the neglected, the notable, and the notorious.

The family Poxviridae currently comprises 22 genera that infect vertebrates. Of these, members of the Ortho-, Para-, Mollusci- and Yatapoxvirus genera have been associated with human diseases of high clinical relevance in dermatology. Historically, smallpox had been a notorious health threat until it was declared eradicated by the World Health Organization in 1979. Today, dermatologists are confronted with a variety of poxviral infections, such as farmyard pox, which occurs as a zoonotic infection after contact with animals. In the tropics, tanapox or vaccinia may be in the differential diagnosis as neglected tropical dermatoses. Molluscum contagiosum virus infection accounts for significant disease burden worldwide and is classified as a sexually transmitted infection in certain scenarios. Recently, mpox (monkeypox) has emerged as a public health emergency of international concern, requiring rapid recognition and appropriate management by dermatologists and infectious disease specialists. Advances and new insights into the epidemiology, diagnosis, clinical manifestations and complications, treatment, and prevention of poxviral infections require a high level of expertise and interdisciplinary skills from healthcare professionals linking virology, infectious diseases, and dermatology. This CME article provides a systematic overview and update to assist the practicing dermatologist in the identification, differential diagnosis, and management of poxviral infections.

S3 guideline "actinic keratosis and cutaneous squamous cell carcinoma" - update 2023, part 2: epidemiology and etiology, diagnostics, surgical and systemic treatment of cutaneous squamous cell carcinoma (cSCC), surveillance and prevention.

Actinic keratosis (AK) are common lesions in light-skinned individuals that can potentially progress to cutaneous squamous cell carcinoma (cSCC). Both conditions may be associated with significant morbidity and constitute a major disease burden, especially among the elderly. To establish an evidence-based framework for clinical decision making, the guideline "actinic keratosis and cutaneous squamous cell carcinoma" was updated and expanded by the topics cutaneous squamous cell carcinoma in situ (Bowen's disease) and actinic cheilitis. The guideline is aimed at dermatologists, general practitioners, ear nose and throat specialists, surgeons, oncologists, radiologists and radiation oncologists in hospitals and office-based settings, as well as other medical specialties, policy makers and insurance funds involved in the diagnosis and treatment of patients with AK and cSCC. A separate guideline exists for patients and their relatives. In this part, we will address aspects relating to epidemiology and etiology, diagnostics, surgical and systemic treatment of cutaneous squamous cell carcinoma (cSCC), surveillance and prevention.

Real-world outcomes using PD-1 antibodies and BRAF + MEK inhibitors for adjuvant melanoma treatment from 39 skin cancer centers in Germany, Austria and Switzerland.

BACKGROUND: Programmed death-1 (PD-1) antibodies and BRAF + MEK inhibitors are widely used for adjuvant therapy of fully resected high-risk melanoma. Little is known about treatment efficacy outside of phase III trials. This real-world study reports on clinical outcomes of modern adjuvant melanoma treatment in specialized skin cancer centers in Germany, Austria and Switzerland. METHODS: Multicenter, retrospective study investigating stage III-IV melanoma patients receiving adjuvant nivolumab (NIV), pembrolizumab (PEM) or dabrafenib + trametinib (D + T) between 1/2017 and 10/2021. The primary endpoint was 12-month recurrence-free survival (RFS). Further analyses included descriptive and correlative statistics, and a multivariate linear-regression machine learning model to assess the risk of early melanoma recurrence. RESULTS: In total, 1198 patients from 39 skin cancer centers from Germany, Austria and Switzerland were analysed. The vast majority received anti PD-1 therapies (n = 1003). Twelve-month RFS for anti PD-1 and BRAF + MEK inhibitor-treated patients were 78.1% and 86.5%, respectively (hazard ratio [HR] 1.998 [95% CI 1.335-2.991]; p = 0.001). There was no statistically significant difference in overall survival (OS) in anti PD-1 (95.8%) and BRAF + MEK inhibitor (96.9%) treated patients (p > 0.05) during the median follow-up of 17 months. Data indicates that anti PD-1 treated patients who develop immune-related adverse events (irAEs) have lower recurrence rates compared to patients with no irAEs (HR 0.578 [95% CI 0.443-0.754], p = 0.001). BRAF mutation status did not affect overall efficacy of anti PD-1 treatment (p > 0.05). In both, anti PD-1 and BRAF + MEK inhibitor treated cohorts, data did not show any difference in 12-month RFS and 12-month OS comparing patients receiving total lymph node dissection (TLND) versus sentinel lymph node biopsy only (p > 0.05). The recurrence prediction model reached high specificity but only low sensitivity with an AUC = 0.65. No new safety signals were detected. Overall, recorded numbers and severity of adverse events were lower than reported in pivotal phase III trials. CONCLUSIONS: Despite recent advances in adjuvant melanoma treatment, early recurrence remains a significant clinical challenge. This study shows that TLND does not reduce the risk of early melanoma recurrence and should only be considered in selected patients. Data further highlight that variables collected during clinical routine are unlikely to allow for a clinically relevant prediction of individual recurrence risk.

Effects of the COVID-19 pandemic on care of melanoma patients in Berlin, Germany: the Mela-COVID survey

Background: The COVID-19 pandemic imposes major challenges for care of cancer patients. Objectives: Our aim was to assess the effects of the pandemic on treatment and appointments of patients with malignant melanoma based on a large skin cancer centre in Berlin, Germany, and identify reasons for, and impact factors associated with these changes. Materials & Methods: Patients with melanoma treated from January 1st 2019 received a postal survey with questions on impairment due to the pandemic, fear of COVID-19, fear of melanoma, changes in therapy and/or appointments, including reasons for the changes. Impact factors on postponed/missed appointments were examined using descriptive analyses and multivariate logistic regression. Results: The response rate was 41.3% (n = 324; 57.4% males; mean age: 67.9 years). Among 104 participants currently receiving therapy, four (3.8%) reported treatment changes due to the pandemic. Postponements or cancellations of appointments occurred in 48 participants (14.8%), most frequently, at their own request (81.3%) due to fear of SARS-CoV-2 infection (68.8%). Current treatment was associated with a reduced chance of post-poning/missing appointments (OR = 0.208, p = 0.003), whereas a high or very high level of concern for COVID-19 (OR = 6.806, p = 0.034; OR= 10.097, p = 0.038), SARS-CoV-2 infection among close acquaintances (OR = 4.251, p = 0.026), anxiety disorder (OR = 5.465, p = 0.016) and AJCC stage IV (OR = 3.108, p = 0.048) were associated with a higher likelihood of postponing/missing appointments. Conclusion: Among our participants, treatment changes were rare and the proportion of missed/delayed appointments was rather small. The main reasons for delays/cancellations of appointments were anxiety and concern for COVID-19.

Melanomversorgung während eines Jahres Pandemie in Berlin: abnehmende Terminstornierungen trotz zunehmender Besorgnis über COVID-19.

HINTERGRUND UND ZIELE: Die COVID-19-Pandemie stellt für Krebspatienten eine große Herausforderung dar. Unser Ziel war es, ihren Einfluss auf die Behandlung und auf Arzttermine von Melanompatienten nach einem Jahr Pandemie zu untersuchen. PATIENTEN UND METHODIK: Melanompatienten, die im Vivantes Hauttumorzentrum in Berlin behandelt wurden, beantworteten eine postalische Umfrage zu Pandemie-bedingten Änderungen ihrer Melanomversorgung. Einflussfaktoren auf Terminänderungen wurden mit deskriptiven Analysen und multivariater logistischer Regression untersucht. Daten nach einem Jahr Pandemie wurden mit Daten nach der ersten Welle verglichen. ERGEBNISSE: Von den 366 Teilnehmern (57,7 % Männer; Durchschnittsalter 69,2 Jahre, Rücklaufquote: 36,1 %) berichteten 38 (10,1 %) über verschobene oder verpasste Arzttermine, meist auf eigenen Wunsch (71,1 %) aus Angst vor COVID-19 (52,6 %). Eine aktuelle Therapie war mit einem geringeren Risiko, Termine zu verpassen, assoziiert (Odds Ratio [OR]: 0,194, p = 0,002), höheres Alter (OR: 1,037, p = 0,039), längere Krankheitsdauer (OR: 1,007, p = 0,028) und ein höherer Schulabschluss (OR: 2,263, p = 0,043) mit höherer Wahrscheinlichkeit. Von den 177 Patienten, die aktuell eine Therapie erhielten, erfuhren nur 1,7 % Pandemie-bedingte Behandlungsänderungen. Die Besorgnis über COVID-19 war nach einem Jahr Pandemie signifikant größer als nach der ersten Welle, die Zahl der verpassten Arzttermine jedoch niedriger. SCHLUSSFOLGERUNGEN: Pandemie-bedingte Änderungen waren in unserer Kohorte selten und nahmen trotz zunehmender Besorgnis mit der Zeit ab.

Melanoma care during one year pandemic in Berlin: decreasing appointment cancellations despite increasing COVID-19 concern.

BACKGROUND AND OBJECTIVES: The COVID-19 pandemic poses a great challenge for cancer patients. Our aim was to assess its influence on treatment and appointments of melanoma patients after one year of pandemic. METHODS: Melanoma patients treated in the Vivantes Skin Cancer Centre in Berlin, Germany completed a postal survey on pandemic-related alterations in melanoma care. Impact factors on changes of appointments were examined with descriptive analyses and multivariate logistic regression. Data after one year of pandemic were compared to those after its first wave. RESULTS: Among 366 participants (57.7 % males; mean age 69.2 years, response rate: 36.1 %), 38 (10.1 %) reported postponed or missed appointments, mostly on their own demand (71.1 %) due to fear of COVID-19 (52.6 %). Current treatment was associated with a lower risk of changing appointments (Odds Ratio [OR]: 0.194, p = 0.002), higher age (OR: 1.037, p = 0.039), longer disease duration (OR: 1.007, p = 0.028), and higher school degree (OR: 2.263, p = 0.043) with higher probability. Among 177 patients currently receiving therapy, only 1.7 % experienced pandemic-related treatment alterations. Concern about COVID-19 was significantly higher after one year of pandemic than after its first wave, but the number of missed appointments was lower. CONCLUSIONS: Pandemic-related changes were rare in our cohort and decreased over time despite increasing concern.

S1-guideline atypical fibroxanthoma (AFX) and pleomorphic dermal sarcoma (PDS).

Atypical fibroxanthoma (AFX) and pleomorphic dermal sarcoma (PDS) are rare cutaneous neoplasms representing histomorphological, genetic as well as epigenetic variants of a disease spectrum. Both tumors typically manifest as nonspecific, often ulcerated, skin- to flesh-colored nodules in chronically sun-damaged skin of elderly male patients. AFX is a rather well demarcated, often rapidly growing tumor. PDS tumors are poorly circumscribed and are characterized by aggressive infiltrative growth. Fast as well as slow growth behavior has been reported for both tumors. Histologically, both are composed of spindle-shaped and epithelioid tumor cells with pleomorphic nuclei as well as atypical multinucleated giant cells. Atypical mitoses are common. In contrast to AFX, PDS involves relevant parts of the subcutis and shows areas of tumor necrosis and/or perineural infiltration. Due to the poorly differentiated nature of AFX/PDS (Grade 3), histopathologically similar cutaneous sarcomas, undifferentiated carcinomas, melanomas and other diseases have to be excluded by immunohistochemical analysis. The treatment of choice is micrographically controlled surgery. In cases of AFX, a cure can be assumed after complete excision. Local recurrence rates are low as long as PDS tumors are surgically removed with a safety margin of 2 cm. Metastasis is rare and mostly associated with very thick or incompletely excised tumors; it mainly affects the skin and lymph nodes. Distant metastasis is even more rare. No approved and effective systemic therapy has been established.