{"title":"Kongresskalender 2020","authors":"","doi":"10.1111/ddg.14015","DOIUrl":"https://doi.org/10.1111/ddg.14015","url":null,"abstract":"","PeriodicalId":14702,"journal":{"name":"JDDG: Journal der Deutschen Dermatologischen Gesellschaft","volume":"14 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2020-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"90920110","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
A. Weins, M. Theiler, Bettina Bogatu, K. Kerl, M. Pleimes, Jana Pachlopnik-Schmid, L. Weibel
Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a rare and fulminant variant of pityriasis lichenoides et varioliformis acuta (PLEVA) characterized by fever, severe and rapidly progressive ulcerative and necrotic skin lesions with systemic involvement. Febrile ulceronecrotic Mucha-Habermann disease is considered to be part of the spectrum of pityriasis lichenoides, a continuum of inflammatory skin disorders with unresolved pathogenesis [1–4]. To date, only 70 cases of FUMHD have been reported, with predominance in children and adolescents [1, 4, 5]. Unfamiliarity among physicians due to the rarity of the disease may result in delayed or incorrect diagnosis of FUMHD, with a potentially fatal outcome [4]. Due to cardiovascular, hematologic, gastrointestinal, and neurological complications, the mortality rate of FUMHD increases with age up to 15–20 % [5–7]. We report the youngest case of FUMHD so far, initially presenting as Kawasaki disease (KD) and successfully treated with methylprednisolone and methotrexate (MTX) [8]. A 9-month-old infant was admitted due to persistent fever over five days, with an acute diffuse maculopapular exanthematous rash covering the face, trunk and extremities (Figure 1). The boy also had cervical lymphadenopathy, bilateral conjunctivitis with erythema of the oral mucosa, lips (Figure 1a), tongue and glans penis. On examination, he was febrile (39.1°C) and in poor general condition. Auscultation revealed slight rales. His medical history was unremarkable and he had not received any medication before the onset of fever. Laboratory findings on admission showed an elevated CRP (85 mg/L) and microcytic anemia. The white blood cell count and routine chemistry, coagulation studies as well as ANA, ANCA and immunoglobulins were all within normal limits. No viral or bacterial infection could be detected by swabs, blood culture, serology (HSV, VZV, measles, mycoplasma) or PCR (VZV, adenovirus). The chest x-ray, abdominal ultrasound, ECG, and echocardiography were unremarkable. Since the initial diagnosis was KD, treatment with intravenous immunoglobulins (IVIGs 2 g/kg; 19 g/d) and aspirin (20 mg/kg; 200 mg/d) was initiated. Despite this intervention, the patient remained febrile and the exanthema quickly transitioned to papulovesicular and ulcerative papules and plaques with predominantly acral und mucosal involvement (Figures 2, 3). A skin biopsy from the forearm revealed lichenoid dermatitis with degeneration of the basal layer, multiple apoptotic keratinocytes and a dense, predominantly CD8+ lymphocytic infiltrate, highly specific for pityriasis lichenoides (Figure 4). Febrile ulceronecrotic Mucha-Habermann disease was diagnosed based on the acute clinical presentation. Apart from the laboratory abnormalities, there was no sign of other systemic involvement. Treatment was switched to oral methylprednisolone (1 mg/kg; 10 mg/d), MTX (15 mg/ m2 BSA; 7.5 mg/week) and folic acid (5 mg/week). Methylprednisolone was graduall
{"title":"Febrile ulceronecrotic Mucha‐Habermann disease mimicking Kawasaki disease","authors":"A. Weins, M. Theiler, Bettina Bogatu, K. Kerl, M. Pleimes, Jana Pachlopnik-Schmid, L. Weibel","doi":"10.1111/ddg.13989","DOIUrl":"https://doi.org/10.1111/ddg.13989","url":null,"abstract":"Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a rare and fulminant variant of pityriasis lichenoides et varioliformis acuta (PLEVA) characterized by fever, severe and rapidly progressive ulcerative and necrotic skin lesions with systemic involvement. Febrile ulceronecrotic Mucha-Habermann disease is considered to be part of the spectrum of pityriasis lichenoides, a continuum of inflammatory skin disorders with unresolved pathogenesis [1–4]. To date, only 70 cases of FUMHD have been reported, with predominance in children and adolescents [1, 4, 5]. Unfamiliarity among physicians due to the rarity of the disease may result in delayed or incorrect diagnosis of FUMHD, with a potentially fatal outcome [4]. Due to cardiovascular, hematologic, gastrointestinal, and neurological complications, the mortality rate of FUMHD increases with age up to 15–20 % [5–7]. We report the youngest case of FUMHD so far, initially presenting as Kawasaki disease (KD) and successfully treated with methylprednisolone and methotrexate (MTX) [8]. A 9-month-old infant was admitted due to persistent fever over five days, with an acute diffuse maculopapular exanthematous rash covering the face, trunk and extremities (Figure 1). The boy also had cervical lymphadenopathy, bilateral conjunctivitis with erythema of the oral mucosa, lips (Figure 1a), tongue and glans penis. On examination, he was febrile (39.1°C) and in poor general condition. Auscultation revealed slight rales. His medical history was unremarkable and he had not received any medication before the onset of fever. Laboratory findings on admission showed an elevated CRP (85 mg/L) and microcytic anemia. The white blood cell count and routine chemistry, coagulation studies as well as ANA, ANCA and immunoglobulins were all within normal limits. No viral or bacterial infection could be detected by swabs, blood culture, serology (HSV, VZV, measles, mycoplasma) or PCR (VZV, adenovirus). The chest x-ray, abdominal ultrasound, ECG, and echocardiography were unremarkable. Since the initial diagnosis was KD, treatment with intravenous immunoglobulins (IVIGs 2 g/kg; 19 g/d) and aspirin (20 mg/kg; 200 mg/d) was initiated. Despite this intervention, the patient remained febrile and the exanthema quickly transitioned to papulovesicular and ulcerative papules and plaques with predominantly acral und mucosal involvement (Figures 2, 3). A skin biopsy from the forearm revealed lichenoid dermatitis with degeneration of the basal layer, multiple apoptotic keratinocytes and a dense, predominantly CD8+ lymphocytic infiltrate, highly specific for pityriasis lichenoides (Figure 4). Febrile ulceronecrotic Mucha-Habermann disease was diagnosed based on the acute clinical presentation. Apart from the laboratory abnormalities, there was no sign of other systemic involvement. Treatment was switched to oral methylprednisolone (1 mg/kg; 10 mg/d), MTX (15 mg/ m2 BSA; 7.5 mg/week) and folic acid (5 mg/week). Methylprednisolone was graduall","PeriodicalId":14702,"journal":{"name":"JDDG: Journal der Deutschen Dermatologischen Gesellschaft","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-12-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"83009359","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
F. Jockenhöfer, Christopher Knust, S. Benson, M. Schedlowski, J. Dissemond
Placebo effects are used in the treatment of various medical conditions. To date, there is little scientific data in this regard as it relates to skin diseases in general and hardly any data with respect to wound healing in particular.
{"title":"Influence of placebo effects on quality of life and wound healing in patients with chronic venous leg ulcers","authors":"F. Jockenhöfer, Christopher Knust, S. Benson, M. Schedlowski, J. Dissemond","doi":"10.1111/ddg.13996","DOIUrl":"https://doi.org/10.1111/ddg.13996","url":null,"abstract":"Placebo effects are used in the treatment of various medical conditions. To date, there is little scientific data in this regard as it relates to skin diseases in general and hardly any data with respect to wound healing in particular.","PeriodicalId":14702,"journal":{"name":"JDDG: Journal der Deutschen Dermatologischen Gesellschaft","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-12-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"75560519","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
M. Stefanou, Irina Gepfner-Tuma, C. Brendle, M. Kowarik, A. Meiwes, T. Eigentler, Alisa Müller, C. Garbe, U. Ziemann, G. Tabatabai, A. Forschner
A 28-year-old woman was treated with oral vemurafenib (960 mg twice daily) and oral cobimetinib (60 mg once daily, days 1–21) for advanced melanoma with skeletal, pulmonary, hepatic, and peritoneal metastases. Four months later, brain MRI demonstrated new cerebral metastases and treatment was switched to combined immunotherapy with ipilimumab (3 mg/kg) and nivolumab (1 mg/kg) [1]. After two cycles of immunotherapy, the patient was admitted urgently to hospital with a severe headache, nausea, vomiting, and deteriorating consciousness. Brain MRI revealed fulminant progression of the cerebral metastases with up to forty new supratentorial and two new pontine lesions. We also noted prominent leptomeningeal enhancement without obstruction of cerebrospinal fluid (CSF) flow. Microbiological and virological tests of the CSF were negative. Antiedema treatment was initiated with oral dexamethasone (2 mg three times daily) and treatment was then switched to oral dabrafenib (150 mg twice daily) and oral trametinib (2 mg once daily) as a rechallenge of BRAFand MEK-inhibition therapy [2, 3]. Whole brain radiation was discussed as a treatment option, but the patient refused it. Under corticosteroid treatment, the patient’s neurological status improved markedly and symptoms of increased intracranial pressure resolved completely. Seven weeks after cessation of immunotherapy and five weeks after initiation of dabrafenib and trametinib treatment, the patient was hospitalized urgently after suffering a generalized tonic-clonic seizure. Her symptoms were a debilitating headache and a Bálint’s syndrome, which is characterized by a triad of severe neuropsychological deficits, namely optic ataxia, oculomotor apraxia, and simultanagnosia [4]. Brain MRI showed a partial response with reduced size of the cerebral metastases, but also with intralesional hemorrhage. MRI revealed prominent bilateral cortical and subcortical edema in the parieto-occipital regions, compatible with a posterior reversible encephalopathy syndrome (PRES). PRES also explained the clinical symptoms of Bálint’s syndrome [4]. In addition to marked meningeal enhancement, prominent biparietal and left temporal cortical laminar necrosis was noted (Figure 1). CSF analysis showed an elevated cell count (15 cells/μl) with evidence of malignant cells. The long-term blood pressure measurement excluded hypertension. CT angiography and transcranial Doppler ultrasound excluded cerebral vasospasms. Electroencephalography showed no epileptic activity, but the patient had already been treated with levetiracetam. Due to the prominent meningeal gadolinium enhancement, concomitant immune-mediated meningitis was suspected and high-dose corticosteroid treatment was initiated. Therapy with dabrafenib and trametinib was discontinued. One month later, a brain MRI showed regression of PRES, but dramatic progression of the cerebral metastases was noted. Reintroduction of the targeted therapy with vemurafenib and cobimetinib w
{"title":"Posterior reversible encephalopathy syndrome in a melanoma patient with dabrafenib and trametinib treatment following immunotherapy","authors":"M. Stefanou, Irina Gepfner-Tuma, C. Brendle, M. Kowarik, A. Meiwes, T. Eigentler, Alisa Müller, C. Garbe, U. Ziemann, G. Tabatabai, A. Forschner","doi":"10.1111/ddg.13991","DOIUrl":"https://doi.org/10.1111/ddg.13991","url":null,"abstract":"A 28-year-old woman was treated with oral vemurafenib (960 mg twice daily) and oral cobimetinib (60 mg once daily, days 1–21) for advanced melanoma with skeletal, pulmonary, hepatic, and peritoneal metastases. Four months later, brain MRI demonstrated new cerebral metastases and treatment was switched to combined immunotherapy with ipilimumab (3 mg/kg) and nivolumab (1 mg/kg) [1]. After two cycles of immunotherapy, the patient was admitted urgently to hospital with a severe headache, nausea, vomiting, and deteriorating consciousness. Brain MRI revealed fulminant progression of the cerebral metastases with up to forty new supratentorial and two new pontine lesions. We also noted prominent leptomeningeal enhancement without obstruction of cerebrospinal fluid (CSF) flow. Microbiological and virological tests of the CSF were negative. Antiedema treatment was initiated with oral dexamethasone (2 mg three times daily) and treatment was then switched to oral dabrafenib (150 mg twice daily) and oral trametinib (2 mg once daily) as a rechallenge of BRAFand MEK-inhibition therapy [2, 3]. Whole brain radiation was discussed as a treatment option, but the patient refused it. Under corticosteroid treatment, the patient’s neurological status improved markedly and symptoms of increased intracranial pressure resolved completely. Seven weeks after cessation of immunotherapy and five weeks after initiation of dabrafenib and trametinib treatment, the patient was hospitalized urgently after suffering a generalized tonic-clonic seizure. Her symptoms were a debilitating headache and a Bálint’s syndrome, which is characterized by a triad of severe neuropsychological deficits, namely optic ataxia, oculomotor apraxia, and simultanagnosia [4]. Brain MRI showed a partial response with reduced size of the cerebral metastases, but also with intralesional hemorrhage. MRI revealed prominent bilateral cortical and subcortical edema in the parieto-occipital regions, compatible with a posterior reversible encephalopathy syndrome (PRES). PRES also explained the clinical symptoms of Bálint’s syndrome [4]. In addition to marked meningeal enhancement, prominent biparietal and left temporal cortical laminar necrosis was noted (Figure 1). CSF analysis showed an elevated cell count (15 cells/μl) with evidence of malignant cells. The long-term blood pressure measurement excluded hypertension. CT angiography and transcranial Doppler ultrasound excluded cerebral vasospasms. Electroencephalography showed no epileptic activity, but the patient had already been treated with levetiracetam. Due to the prominent meningeal gadolinium enhancement, concomitant immune-mediated meningitis was suspected and high-dose corticosteroid treatment was initiated. Therapy with dabrafenib and trametinib was discontinued. One month later, a brain MRI showed regression of PRES, but dramatic progression of the cerebral metastases was noted. Reintroduction of the targeted therapy with vemurafenib and cobimetinib w","PeriodicalId":14702,"journal":{"name":"JDDG: Journal der Deutschen Dermatologischen Gesellschaft","volume":"122 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-12-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"75382034","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
F. Ferrara, Rossella Lacava, A. Barisani, S. Messori, A. Patrizi, F. Bardazzi, S. Vaccari
In selected cases, conventional photodynamic therapy (C‐PDT) is a valid alternative to surgery for the treatment of basal cell carcinoma (BCC). However, it is limited to superficial BCCs. Pretreatment of BCCs with ablative lasers may enhance its efficacy. We evaluated the C‐PDT and CO2 laser combination therapy for the treatment of superficial and nodular BCCs.
{"title":"Combined CO2 laser and photodynamic therapy enhances the efficacy of treatment of basal cell carcinomas","authors":"F. Ferrara, Rossella Lacava, A. Barisani, S. Messori, A. Patrizi, F. Bardazzi, S. Vaccari","doi":"10.1111/ddg.14004","DOIUrl":"https://doi.org/10.1111/ddg.14004","url":null,"abstract":"In selected cases, conventional photodynamic therapy (C‐PDT) is a valid alternative to surgery for the treatment of basal cell carcinoma (BCC). However, it is limited to superficial BCCs. Pretreatment of BCCs with ablative lasers may enhance its efficacy. We evaluated the C‐PDT and CO2 laser combination therapy for the treatment of superficial and nodular BCCs.","PeriodicalId":14702,"journal":{"name":"JDDG: Journal der Deutschen Dermatologischen Gesellschaft","volume":"5 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"77348840","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Kongresskalender 2019","authors":"","doi":"10.1111/ddg.13999","DOIUrl":"https://doi.org/10.1111/ddg.13999","url":null,"abstract":"","PeriodicalId":14702,"journal":{"name":"JDDG: Journal der Deutschen Dermatologischen Gesellschaft","volume":"38 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"82865837","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"In Memoriam Erich Landes 1921–2019","authors":"R. Kaufmann","doi":"10.1111/ddg.13986_g","DOIUrl":"https://doi.org/10.1111/ddg.13986_g","url":null,"abstract":"","PeriodicalId":14702,"journal":{"name":"JDDG: Journal der Deutschen Dermatologischen Gesellschaft","volume":"11 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"87233801","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
A 52-year-old female patient presented with a history of increasing erythematous and centrally bullous plaques over six days. The patient reported a general feeling of malaise and light-headedness and denied taking any medication during the past eight weeks. She described the lesions as slightly itchy and tender, and did not report a previous infection. A few years ago, the patient experienced a transitory ischemic attack but was otherwise healthy.
{"title":"Disseminated bullae and malaise","authors":"A. Bohne, T. Schwarz, U. Wehkamp","doi":"10.1111/ddg.13938","DOIUrl":"https://doi.org/10.1111/ddg.13938","url":null,"abstract":"A 52-year-old female patient presented with a history of increasing erythematous and centrally bullous plaques over six days. The patient reported a general feeling of malaise and light-headedness and denied taking any medication during the past eight weeks. She described the lesions as slightly itchy and tender, and did not report a previous infection. A few years ago, the patient experienced a transitory ischemic attack but was otherwise healthy.","PeriodicalId":14702,"journal":{"name":"JDDG: Journal der Deutschen Dermatologischen Gesellschaft","volume":"36 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"86793049","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Journal‐Club","authors":"Sigrid Karrer","doi":"10.1111/ddg.13970_g","DOIUrl":"https://doi.org/10.1111/ddg.13970_g","url":null,"abstract":"","PeriodicalId":14702,"journal":{"name":"JDDG: Journal der Deutschen Dermatologischen Gesellschaft","volume":"33 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"79385149","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
K. Gebauer, J. Schirren, Bastian Jaeschke, R. Kaufmann, M. Meissner
We report on a 54-year-old female patient who presented at our oncological outpatient department in 2012. The patient initially complained about ptosis of the right eye and severe right thoracic pain. A Horner's syndrome was suspected, although additional typical symptoms such as miosis and anhidrosis were missing. For further diagnostic imaging the patient underwent a chest MRI. MRI showed a tumor at the apical segment of the upper lobe of the right lung (Pancoast tumor) as well as enlarged right hilar and mediastinal lymph nodes (Figure 1). PET-CT and MRI of the brain revealed no evidence of metastases. A biopsy was performed to determine the characteristics of the tumor. The pathology result showed a metastasis of a malignant melanoma. No primary site was detected by clinical examination of the skin. The patient was diagnosed with stage IV M1b melanoma of unknown primary (CUP). Tissue-based analysis identified a V600E mutation. As the patient was initially inoperable, systemic treatment with the BRAF inhibitor vemurafenib was initiated. Combination with an MEK inhibitor was not yet approved in 2012. After twelve weeks of therapy, a partial response was detected and the decision for surgery was made (Figure 2). Due to remission with neoadjuvant treatment, the entire tumor mass could be resected (Figure 3). The postoperative course was uncomplicated. Adjuvant treatment was not given. No relapse occurred during six years of follow-up. Five Percent of newly diagnosed cases of malignant melanoma are classified as melanomas of unknown primary (CUP). In rare cases the primary lesion is located in the ear, eye, urethra or intestinal mucosa [1]. However, the most favored assumption is immune-mediated degradation of the primary site, while metastases are not affected [2]. DOI: 10.1111/ddg.13983 Neoadjuvant therapy with vemurafenib in Horner's syndrome as a very rare first diagnosis of a malignant melanoma of unknown primary Clinical Letter
{"title":"Neoadjuvant therapy with vemurafenib in Horner's syndrome as a very rare first diagnosis of a malignant melanoma of unknown primary","authors":"K. Gebauer, J. Schirren, Bastian Jaeschke, R. Kaufmann, M. Meissner","doi":"10.1111/ddg.13983","DOIUrl":"https://doi.org/10.1111/ddg.13983","url":null,"abstract":"We report on a 54-year-old female patient who presented at our oncological outpatient department in 2012. The patient initially complained about ptosis of the right eye and severe right thoracic pain. A Horner's syndrome was suspected, although additional typical symptoms such as miosis and anhidrosis were missing. For further diagnostic imaging the patient underwent a chest MRI. MRI showed a tumor at the apical segment of the upper lobe of the right lung (Pancoast tumor) as well as enlarged right hilar and mediastinal lymph nodes (Figure 1). PET-CT and MRI of the brain revealed no evidence of metastases. A biopsy was performed to determine the characteristics of the tumor. The pathology result showed a metastasis of a malignant melanoma. No primary site was detected by clinical examination of the skin. The patient was diagnosed with stage IV M1b melanoma of unknown primary (CUP). Tissue-based analysis identified a V600E mutation. As the patient was initially inoperable, systemic treatment with the BRAF inhibitor vemurafenib was initiated. Combination with an MEK inhibitor was not yet approved in 2012. After twelve weeks of therapy, a partial response was detected and the decision for surgery was made (Figure 2). Due to remission with neoadjuvant treatment, the entire tumor mass could be resected (Figure 3). The postoperative course was uncomplicated. Adjuvant treatment was not given. No relapse occurred during six years of follow-up. Five Percent of newly diagnosed cases of malignant melanoma are classified as melanomas of unknown primary (CUP). In rare cases the primary lesion is located in the ear, eye, urethra or intestinal mucosa [1]. However, the most favored assumption is immune-mediated degradation of the primary site, while metastases are not affected [2]. DOI: 10.1111/ddg.13983 Neoadjuvant therapy with vemurafenib in Horner's syndrome as a very rare first diagnosis of a malignant melanoma of unknown primary Clinical Letter","PeriodicalId":14702,"journal":{"name":"JDDG: Journal der Deutschen Dermatologischen Gesellschaft","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-11-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"76140919","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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