Dear Editor, Primary cutaneous Tcell lymphoma (CTCL) comprises a heterogeneous spectrum of nonHodgkin's lymphomas, ranging from indolent forms (e.g. earlystage mycosis fungoides) with predominantly favourable prognosis to rare and aggressive CTCL subtypes with poor course of disease. Treatment of advancedstage mycosis fungoides (MF) and aggressive subtypes of CTCL is challenging, and responses are often short lived. Bendamustine is an alkylating agent licensed for the treatment of several lymphoproliferative diseases including chronic lymphocytic leukaemia, multiple myeloma and nonHodgkin's lymphoma. Due to its efficacy and beneficial toxicity profile, bendamustine (alone or in combination) has gained a renaissance over the last years for several haematologic malignancies, including aggressive lymphomas in elderly patients with high comorbidity. We herein report a case series of primary CTCL patients with either aggressive CTCL type or pretreated advancedstage MF treated with bendamustine monochemotherapy. A total of 11 patients (four women, seven men; median age 67 years, range: 55– 83 years) treated with bendamustine between 2016 and 2022 were included (Table 1). Eight patients had advancedstage MF (≥stage IIB) and three patients had aggressive type CTCL (two cases of primary cutaneous peripheral Tcell lymphoma, not otherwise specified and one case of primary cutaneous gammadelta Tcell lymphoma). CTCL classification and treatment response were evaluated according to the international criteria of the International Society for Cutaneous Lymphomas (ISCL), the U.S. Cutaneous Lymphoma Consortium and the EORTC Cutaneous Lymphoma Task Force. Patients received a median of 4 bendamustine cycles (range: 3– 9). Overall response (ORR) was 91% (10/11 patients), with complete remission (CR) in skin observed in 2 (18%) patients, partial response (PR) in 8 (73%) and progressive disease in one patient (9%), respectively (Figure 1). In 4 (36%) patients with CR or PR, longterm control (mean 59 months) was achieved with maintenance treatment using extracorporeal photopheresis (ECP) or bexarotene. Relapses occurred in 6 (54%) patients, and time to next treatment (TNT) on average was 3.5 months (range: 1– 6 months). Overall survival on average was 41.2 months (range: 5– 100 months). Toxicities were observed in 5 of the 11 patients, including 1 severe adverse event (severe acute renal failure with sepsis, but complete recovery). Other adverse events were lymphocytopenia (grade 3) and nausea (grade 2). The advent of brentuximab vedotin (BV) and mogamulizumab has significantly improved the therapeutic armamentarium against aggressive and advancedstage CTCL. Nevertheless, patients with refractory or progressive disease usually require singleor multiagent chemotherapy, with potential high levels of toxic reactions and frequent lack of durable response. Accordingly, there is need for further alternative treatment options, preferably with low toxicity profile. Bendamustine has previously shown an encouraging high response rate (50% ORR, 28% CR) in patients with refractory or relapsed systemic Tcell lymphomas. A French series of nine patients with advancedstage CTCL has evaluated the efficacy of bendamustine combined with BV, and revealed a CR in two patients, PR in five patients, and stable disease in two patients, respectively. So far, only few data exist on bendamustine as monochemotherapy for CTCL. In a study on bendamustine salvage therapy for Tcell neoplasms, PR was observed in 2 of 3 patients with mycosis fungoides. Moreover, our group has reported a case of primary cutaneous gammadelta Tcell lymphoma that completely cleared with bendamustine monochemotherapy (see patient 11). In conclusion, this retrospective singlecentre analysis revealed an encouraging high ORR of bendamustine monochemotherapy in advancedstage MF and aggressive CTCL, including elderly patients with comorbidity. In four patients with PR, subsequent maintenance therapy with ECP or bexarotene enabled longterm disease control. Nevertheless, other six patients had early relapses and required further systemic treatment. This study has several limitations. With the help of patient records and clinical photographs, we performed an unblinded retrospective singlecentre analysis of a relatively small number of patients. More investigations, preferably prospective trials or large retrospective multicentre analyses performed under more rigorous conditions are now warranted to evaluate the role of bendamustine (as a monotherapy or combination therapy) in the management of CTCL. Received: 31 May 2022 | Accepted: 18 October 2022
Clinical and Experimental DermatologyVolume 46, Issue 8 p. 1619-1621 Letter to the Editor • Correspondence HPV-5-associated cutaneous squamous cell carcinoma in situ in poikiloderma with neutropenia A. Kreuter, Corresponding Author A. Kreuter alexander.kreuter@helios-gesundheit.de orcid.org/0000-0003-2275-499X Department of Dermatology, Venereology and Allergology, HELIOS St Elisabeth Hospital Oberhausen, University Witten/Herdecke, Witten, GermanySearch for more papers by this authorB. Koushk-Jalali, B. Koushk-Jalali Department of Dermatology, Venereology and Allergology, HELIOS St Elisabeth Hospital Oberhausen, University Witten/Herdecke, Witten, GermanySearch for more papers by this authorB. Akgül, B. Akgül Institute of Virology, National Reference Center for Papilloma- and Polyomaviruses, University of Cologne, Cologne, GermanySearch for more papers by this authorS. Silling, S. Silling Institute of Virology, National Reference Center for Papilloma- and Polyomaviruses, University of Cologne, Cologne, GermanySearch for more papers by this authorF. Oellig, F. Oellig Institute of Pathology, Mülheim an der Ruhr, GermanySearch for more papers by this authorC. Has, C. Has Department of Dermatology, Medical Center, University of Freiburg, Freiburg, GermanySearch for more papers by this authorU. Wieland, U. Wieland Institute of Virology, National Reference Center for Papilloma- and Polyomaviruses, University of Cologne, Cologne, GermanySearch for more papers by this author A. Kreuter, Corresponding Author A. Kreuter alexander.kreuter@helios-gesundheit.de orcid.org/0000-0003-2275-499X Department of Dermatology, Venereology and Allergology, HELIOS St Elisabeth Hospital Oberhausen, University Witten/Herdecke, Witten, GermanySearch for more papers by this authorB. Koushk-Jalali, B. Koushk-Jalali Department of Dermatology, Venereology and Allergology, HELIOS St Elisabeth Hospital Oberhausen, University Witten/Herdecke, Witten, GermanySearch for more papers by this authorB. Akgül, B. Akgül Institute of Virology, National Reference Center for Papilloma- and Polyomaviruses, University of Cologne, Cologne, GermanySearch for more papers by this authorS. Silling, S. Silling Institute of Virology, National Reference Center for Papilloma- and Polyomaviruses, University of Cologne, Cologne, GermanySearch for more papers by this authorF. Oellig, F. Oellig Institute of Pathology, Mülheim an der Ruhr, GermanySearch for more papers by this authorC. Has, C. Has Department of Dermatology, Medical Center, University of Freiburg, Freiburg, GermanySearch for more papers by this authorU. Wieland, U. Wieland Institute of Virology, National Reference Center for Papilloma- and Polyomaviruses, University of Cologne, Cologne, GermanySearch for more papers by this author First published: 22 June 2021 https://doi.org/10.1111/ced.14811 Conflict of interest: the authors declare that they have no conflicts of interest. Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat No abstract is available for this article. Volume46, Issue8December 2021Pages 1619-1621 RelatedInformation
Evidence is accumulating that COVID-19 vaccines might induce or exacerbate autoimmune rheumatic diseases. The currently available COVID-19 vaccines include mRNA and recombinant adenoviral vector vaccines, both encoding SARS-CoV-2 spike protein production as the primary target for neutralizing antibodies. We report a case of subacute cutaneous lupus erythematosus (SCLE) following mRNA vaccination with the Pfizer mRNA vaccine BNT162b2, and summarize the current literature on CLE occurring after COVID-19 vaccination.
Human papillomaviruses (HPV) induce a broad spectrum of cutaneous and mucosal lesions. Phylogenetically, HPVs are classified into five genera called alpha, beta, gamma, mu, and nu.1 More than 40 alpha-HPV-types infect the anogenital region. Alpha-HPVs are divided into low-risk (LR) and high-risk (HR)-HPV-types, according to their oncogenic potential.1,2 Several alpha-HPV-types rarely (<1%) detected as monoinfections in cervical cancers are classified as probably (HPV68) or possibly (HPV26,30,34,53,66,67,69,70,73,82,85,97) carcinogenic (pHR-HPV-types).
Background In contrast to adults, only limited data are available on the human papillomavirus (HPV)-type spectrum in anogenital warts (AGW) of children. Objective This study aimed to evaluate the HPV-type spectrum in AGW of prepubertal children. Materials & methods In a retrospective German multicentre study, HPV genotyping was performed in AGW biopsies of 55 1- to 12-year-old children using HPV group-specific PCRs followed by hybridization with type-specific probes or sequence analysis. Results Human papillomavirus-DNA was found in 53 of the 55 AGW. In 58.5% (31/53) of the HPV-positive AGW, mucosal HPV types were detected. HPV6 (27/53, 50.9%) was the predominant type. 43.4% (23/53) of the lesions were induced by cutaneous HPV types (HPV2, HPV27, HPV57). Mucosal HPV types were significantly more common in children under 5 years of age than in children 5 years of age and older (22/25, 88.0% [95% CI: 70.0-95.8] vs. 9/28, 32.1% [95% CI: 17.9-50.7], P < 0.001). In contrast, cutaneous HPV types were significantly more prevalent in the 5- to 12-year age group (4/25, 16.0% [95% CI 6.4-34.7] vs. 19/28, 67.9% [95% CI 49.3-82.1], P < 0.001). Conclusion Anogenital warts in 5- to 12-year-old children are frequently associated with cutaneous HPV types, possibly due to horizontal transmission. HPV typing, in addition to comprehensive clinical and psychosocial evaluation, can potentially help in the assessment of these cases.
Die retikuläre erythematöse Muzinose (REM-Syndrom) ist eine seltene Hauterkrankung, die vorwiegend Frauen betrifft und sich durch flächige, zum Teil retikuläre, unregelmäßig konfigurierte blasse Erytheme im Bereich des Dekolletés und des Rückens auszeichnet. Die Hautveränderungen sind oftmals symptomlos oder nur von geringem Juckreiz oder Brennen begleitet. Extrakutaner Befall oder Organbeteiligung kommt beim REM-Syndrom nicht vor. Histopathologisch imponieren in der Dermis lokalisierte, perivaskulär und periadnexiell akzentuierte lymphozytäre Entzündungsinfiltrate sowie eine vermehrte Ablagerung von Muzin. Aufgrund der ähnlichen Histologie zum Lupus erythematodes (LE) tumidus wird diskutiert, ob das REM-Syndrom dem Spektrum des kutanen LE zugeordnet bzw. als LE-artiges Krankheitsbild gewertet werden kann. Beide Krankheitsbilder zeigen zudem ein gutes Ansprechen auf eine Therapie mit Hydroxychloroquin.
ZusammenfassungBei einem Patienten mit diffusen Angiokeratomen im Bereich des unteren Abdomens und Genitalbereichs konnte mithilfe einer genetischen Untersuchung die Diagnose eines Morbus Fabry gestellt werden. Der Morbus Fabry ist eine X‑chromosomal vererbte Speicherkrankheit mit teilweise schwerwiegenden Multiorganbeteiligungen, unter anderem des Herzens und der Niere. Pathogenetisch liegt ein Mangel des lysosomalen Enzyms α‑Galaktosidase A (α-GAL A) vor. Unbehandelt ist die Lebenserwartung der Patienten besonders durch kardiale Komplikationen verkürzt. Aktuell zugelassene Therapieoptionen sind eine lebenslange Enzymersatztherapie und eine Chaperontherapie.
CD30-positive primary cutaneous anaplastic large cell lymphoma (C-ALCL) is an indolent type of cutaneous lymphoma with favourable clinical prognosis. Pseudocarcinomatous hyperplasia (PCH) is a rare benign epithelial condition that can resemble invasive squamous cell carcinoma both clinically and histopathologically. PCH predominantly occurs in CD30-positive lymphoproliferative disorders. We report a 75-year-old woman with PCH in a multifocal C-ALCL located on the scalp and right retroauricular area, which rapidly responded to treatment with psoralen ultraviolet A photochemotherapy. Comprehensive virological analyses for potential oncogenic viruses, including Epstein-Barr virus, human herpesvirus-8, human papillomaviruses, the recently discovered cutavirus and nine different human polyomaviruses, were negative.
severe drug eruption. Methazolamide, a sulfonamide derivative, acts as a carbonic anhydrase inhibitor and reduces intraocular pressure through inhibition of carbonic anhydrase in the ciliary body. We speculate that methazolamide may induce sulfonamide hypersensitivity reactions. TEN developing a reaction to sulfa drugs is not uncommon, but its development following the application of eye drops is a rare occurrence. We cannot ignore the subsequent complications after successful treatment of TEN. Long-term and high-dose glucocorticoids caused complications, including infections and multiple organ failure, which can eventually lead to altered fluid balance, hypoalbuminaemia, cachexia and immunosuppression, which was lethal in our case.
Background It has recently been reported that atonal homolog 1 (ATOH1) gene is down-regulated in Merkel cell carcinoma (MCC) and thus may represent a tumor suppressor gene. Objectives We aimed to test for ATOH1 gene mutations and expression levels in MCC tissues and cell lines. Methods Genomic DNA isolation and amplification via PCR was successfully performed in 33 MCCs on formalin-fixed paraffin-embedded tissue and three MCC cell lines, followed by Sanger sequencing of the whole ATOH1 gene to detect genomic aberrations. ATOH1 mRNA levels were determined by RT-PCR. Immunohistochemistry of ATOH1 was performed to quantify protein expression in tumor samples and cell lines. Results Neither in any of the 33 MCC tissue samples nor in the three cell lines ATOH1 mutations were present. ATOH1 was expressed in all lesions, albeit at different expression levels. Univariate analysis revealed that the total immunohistology score significantly correlated with the occurrence of tumor relapse ( r = 0.57; P = 0.0008). This notion was confirmed in multivariate analysis suggesting that ATOH1 expression is a potential independent predictor for tumor relapse in MCC patients ( P = 0.028). MCC-related death also correlated with ATOH1 expression ( r = 0.4; P = 0.025); however, ATOH1 expression did not retain its predictive value in the regression model. Conclusions In contrast to anecdotal reports ATOH1 expression is not lost by genetic alterations in MCC. However, protein expression of ATOH1 is increased in advanced MCC indicating that ATOH1 is involved in MCC progression.
Lateral distribution of cancer has been observed previously. Most evident is this laterality in ultraviolet (UV)-induced skin cancer, based on an unequally distributed UV exposure.
ANAMNESIS AND CLINICAL FINDINGS:A 83-year old patient with "myelodysplastic / myeloproliferative neoplasia", type chronic myelomonocytic leukemia (CMML), presented at our clinic with an intermittent bleeding, growing tumour located at the glabella. The tumour occurred after a trauma, and thus the suspected diagnosis was pyogenic granuloma. In the context of outpatient care, a severe and persistent bleeding occurred, so that the patient was hospitalized. EXAMINATIONS AND DIAGNOSES: Histopathological analyses of the excized tumour nodule revealed specific cutaneous leukemic infiltrates. Laboratory check-up showed thrombocytopenia with thrombocytopathy and hypofibrinogenaemia.THERAPY AND CLINICAL COURSE:Despite appropriate intraoperative hemostasis, sustained post-treatment bleeding occurred, necessitating the application of several erythrocyte concentrates as well as substitution of prothrombin, fibrinogen, and factor XIII. After stationary dismissal, the patient experienced a transition into acute myelomonocytic leukemia and died a short time thereafter.CONCLUSIONS:Skin lesions are frequent in "myelodysplastic / myeloproliferative neoplasias" and myelodysplastic syndromes. Specific cutaneous infiltrates associated with CMML may be a clinical indicator for a transition into acute leukemia.