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PURPOSE: Uveal melanoma (UM) is an orphan cancer of high unmet medical need. Current patterns of care and surveillance remain unclear as they are situated in an interdisciplinary setting. METHODS: A questionnaire addressing the patterns of care and surveillance in the management of patients with uveal melanoma was distributed to 70 skin cancer centers in Austria, Germany and Switzerland. Frequency distributions of responses for each item of the questionnaire were calculated. RESULTS: 44 of 70 (62.9%) skin cancer centers completed the questionnaire. Thirty-nine hospitals were located in Germany (88.6%), three in Switzerland (6.8%) and two in Austria (4.5%). The majority (68.2%) represented university hospitals. Most patients with metastatic disease were treated in certified skin cancer centers (70.7%, 29/41). Besides, the majority of patients with UM were referred to the respective skin cancer center by ophthalmologists (87.2%, 34/39). Treatment and organization of follow-up of patients varied across the different centers. 35.1% (14/37) of the centers stated to not perform any screening measures. CONCLUSION: Treatment patterns of patients with uveal melanoma in Germany, Austria and Switzerland remain extremely heterogeneous. A guideline for the treatment and surveillance is urgently needed.
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Cuidados Posteriores , Melanoma/terapia , Monitoreo Fisiológico , Pautas de la Práctica en Medicina/estadística & datos numéricos , Neoplasias de la Úvea/terapia , Cuidados Posteriores/métodos , Cuidados Posteriores/estadística & datos numéricos , Austria/epidemiología , Estudios Transversales , Estudios de Seguimiento , Alemania/epidemiología , Necesidades y Demandas de Servicios de Salud/estadística & datos numéricos , Humanos , Tamizaje Masivo/métodos , Tamizaje Masivo/estadística & datos numéricos , Melanoma/epidemiología , Melanoma/patología , Monitoreo Fisiológico/métodos , Monitoreo Fisiológico/estadística & datos numéricos , Metástasis de la Neoplasia , Recurrencia Local de Neoplasia/epidemiología , Vigilancia de la Población/métodos , Derivación y Consulta/normas , Derivación y Consulta/estadística & datos numéricos , Neoplasias Cutáneas/epidemiología , Neoplasias Cutáneas/patología , Neoplasias Cutáneas/terapia , Encuestas y Cuestionarios , Suiza/epidemiología , Neoplasias de la Úvea/epidemiología , Neoplasias de la Úvea/patologíaRESUMEN
BACKGROUND: Eosinophilic fasciitis is a rare fibrosing disorder of muscle fascia with rapid onset of erythema, induration, oedema and tenderness affecting extremities bilaterally. CASE REPORT: We report three cases of eosinophilic fasciitis in 3 females aged 64, 65 and 73 years, in two of them in association with morphea. They fulfilled the proposed diagnostic criteria. Associated malignancies could be excluded in all of them. They were treated by systemic corticosteroids. In the two females with associated morphea higher prednisolone dosages and a combination with methotrexate was necessary. CONCLUSIONS: Eosinophilic fasciitis is a differential diagnosis of systemic scleroderma. Response to treatment is often delayed. Systemic corticosteroids are the first line therapy. Patients with associated morphea need combined drug therapy, in our patients with methotrexate. There is no close correlation between laboratory signs of inflammation and clinical response to treatment.
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BACKGROUND: Cutaneous angiosarcoma of the head and neck region is a subtype of cutaneous angiosarcoma with an unfavourable prognosis. Diagnosis is often delayed. PATENTS AND METHODS: The setting is an Academic Teaching Hospital Skin Cancer Center. Eight Caucasian patients could be identified, 5 men and 3 women. Delay to diagnosis was between 12 to 4 months (mean 7.8 ± 2.9 months). The diagnosis was confirmed in all cases by histopathology and immunohistochemistry. Hematoxylin-eosin, Giemsa, PAS, iron and reticulin stains were performed. Endothelial markers such as CD31, CD34, and Ki67 for proliferation assessment were used in all tumours. Other markers used included pan-cytokeratin (CK), CK7, CK20, ERG, CD 40 and c-MYC. Tumours were classified as localised versus multifocal or diffuse form. Tumour staging was performed according to the 8th edition of the AJCC. The mean age of patients was 79 years ± 26.4 years. The male to female ratio was 1.7. Tumour classification was diffuse in 2 patients, multilocular in one and localised in 5 patients. In 5 of 8 patients, a multimodal treatment was performed, one had radiotherapy alone, in another patient surgery was performed, and radiotherapy is planned. The mean OS was 26.4 months ± 24.5 months. CONCLUSION: Cutaneous angiosarcoma of the head and neck is an aggressive tumour with a poor prognosis. Although surgery remains a cornerstone of treatment, the tumour size at first presentation may be too large, and the elderly patients maybe not suitable for extensive surgery. Therefore, multimodal treatment with adjuvant radiotherapy and/ or chemotherapy is necessary. Multimodal treatment offers a better outcome than radiotherapy or chemotherapy alone. Stealth liposomal encapsulated doxorubicin is a therapeutic option for elderly patients with improved safety compared to conventional doxorubicin.
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BACKGROUND: Eccrine poroma is a benign tumour of eccrine duct epithelium. The usual clinical presentation is nodular. CASE REPORT: We present a 78-year-old man with a painful pendulating flesh-coloured malodorous plantar tumour. Differential diagnoses included telangiectatic granuloma, acrochordon, basal cell or squamous cell carcinoma, cylindroma, amelanotic melanoma, and verruca. Microbiological investigations identified numerous bacteria including Corynebacterium striatum, Streptococcus dysgalactiae, Staphylococcus aureus, Citrobacter koseri. We performed surgery since the tumour hampered his mobility. Histopathology revealed a well-circumscribed tumour composed of cuboidal cells with eosinophilic cytoplasm. Healing was unremarkable. CONCLUSIONS: Pendulating plantar eccrine poroma is a rare clinical presentation of this benign adnexal tumour. Often asymptomatic, in some cases the tumour may become painful. Because of the bacterial colonisation, it could lead to deep soft tissue infections. Malignant transformation is possible. Surgical removal is the treatment of choice.
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BACKGROUND: Cutaneous B-cell lymphomas represent about 25% of all cutaneous lymphomas. Peripheral diffuse large B-cell lymphoma of the leg type is the most aggressive subtype seen mainly in elderly patients. Treatment is not standardised. CASE REPORT: An 87-year-old female patient was presented in May 2018 because of the development of painless subcutaneous nodules on the legs since late 2017. On examination, we observed up to 5 cm large erythematous nodules on the legs and a smaller plaque in the left submammary fold. The histology of a skin demonstrated tumour infiltrate that was separated from the overlying epidermis by a grenz zone. It consisted of densely packed, blastoid lymphocytic cells with numerous, and some atypical mitoses. The cells were positive for CD20, CD79A and CD5. Almost 100% of the cells were labelled with Ki67. The diagnosis of a diffuse large B-cell lymphoma (PCLBCL-LT) of the leg was confirmed. Histologic analysis of a bone marrow biopsy demonstrated a hypercellular bone marrow without malignant lymphatic infiltrates. Diagnostic ultrasound of cervical nodes and computerised tomography (CT) scans (native and with contrast medium) of head, neck and trunk excluded an extracutaneous manifestation of the PCLBCL-LT. Treatment with rituximab plus bendamustibe was initiated, but tumour progress was noted after the second course. Suggested palliative therapy with radiation and rituximab was refused. The patient died 7 months after diagnosis. CONCLUSIONS: Although some trials suggested a beneficial effect of immuno-chemotherapy, the prognosis of (PCLBCL-LT) remains poor. Standardised treatment is missing due to the relative rarity of this malignancy.
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BACKGROUND: Familial chronic pemphigus or Hailey-Hailey disease (OMIM 169600) is a rare, autosomal dominant blistering skin disorder the genetic background are mutations of the ATP2 C1 gene. The treatment is challenging. CASE REPORT: A 48-year-old Caucasian female patient presented to the department with a relapse of her Pemphigus chronicus familiaris (Hailey-Hailey). No other medical diseases were known. On examination, we observed an otherwise healthy woman with widespread erosive lesions on the neck, axillae, groins, submammary fold and anal fold. She reported burning sensations and an unpleasant odour. The diagnosis had been confirmed earlier by histopathology of a skin biopsy with acantholysis, and the relapsing and remitting course. Family history was positive for father and brother. Since she had not responded well in the past to systemic retinoids and did not tolerate the adverse effects of these drugs, we suggested an ablative erbium-YAG laser treatment in general anaesthesia. Laser treatment was performed with the MCL 29 Dermablate (Asclepion Laser Technologies, Jena, Germany) on two occasions. We used a 5 mm focus, pulse energy of 1200 mJ at 8 Hz. The resulting superficial wounds were treated with an ointment containing fusidic acid 0.2% and betamethasone 0.1%. Wound healing was completed after 12 days. No adverse events were observed. CONCLUSIONS: Ablative erbium-YAG therapy is an option for pemphigus chronicus familiaris, in particular in young women and patients who do not tolerate the adverse effects of retinoid therapy.
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BACKGROUND: Acanthosis nigricans (AN) is acquired hyperpigmentation of the intertriginous body regions. Histologically, AN is characterised by a thickened stratum corneum and a variable amount of acanthosis. Although benign and rarely symptomatic, AN may be a red flag for underlying pathologies. CASE PRESENTATION: We analysed our patients with AN and could differentiate three different patterns, that are illustrated by one case report each. The is the benign AN associated with metabolic syndrome including obesity. The second type is the paraneoplastic AN malignancy which is associated with a wider range of malignancies. This type may occur before, after or with the clinical appearance of the malignancy. The third type is relapsing AN after complete remission. We present a patient who had a malignant AN and was treated successfully for his cancer. Years later, however, AN relapsed. In that case in association with the appearance of skin tags. Cancer restaging excluded a tumour relapse. His BMI was 31.2 kg/m2, and the diagnosis of benign AN was confirmed. CONCLUSIONS: The diagnosis of AN remains incomplete without screening for metabolic syndrome and/ or cancer. The combination of AN and skin tags is more often associated with metabolic syndrome. AN may be considered as a red flag for malignancies and the metabolic syndrome.
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Subungual melanoma (SUM) is a rare subtype in Caucasians. Histologically, most tumors are of the acrolentiginous type (ALM). This is a retrospective analysis of the years 2002-2019 at a certified skin cancer center. We observed 12 SUM patients with a median age of 76 years, seven men and five women (0.6% of all melanomas). The delay of diagnosis reached from 30 years to several months. Hallux and thumb were the most affected localizations. The dominant histologic type was ALM. Clinical symptoms were nail plate destruction (90.9%), bleeding (50.0%), pigmentation of the nail plate (33.3%), and a positive Hutchinson sign (25.0%). All tumors were treated surgically with three-dimensional margin control. In six patients, amputation was performed. Median relapse-free survival and overall survival were (56 ± 73.5) months and (112.5 ± 135.1) months, respectively. SUM is rare among Caucasian patients. Nail plate pigmentation and Hutchinson sign were not frequent. Amelanotic ALM with nail plate destruction was the dominant presentation in our series. Dermoscopy is of limited value for amelanotic subungual tumors. Early biopsy of unusual or treatment resistant nail disorders is recommended.
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Diagnóstico Precoz , Enfermedades de la Uña/diagnóstico , Uñas/patología , Anciano , Anciano de 80 o más Años , Biopsia , Dermoscopía/métodos , Diagnóstico Diferencial , Femenino , Estudios de Seguimiento , Humanos , Masculino , Melanoma/diagnóstico , Persona de Mediana Edad , Estudios RetrospectivosRESUMEN
BACKGROUND: Cutaneous squamous cell carcinoma (SCC) of the hand is the most common soft-tissue malignancy in this particular region. A literature survey suggested a higher rate of metastases in advanced SCC of the hand compared to head-and-neck cutaneous SCC. CASE REPORT: An 84-year-old man presented with an ulcerated firm tumour on the dorsum of his right hand. A diagnostic biopsy confirmed the diagnosis SCC. Imaging suggested an involvement of the tendons of digits 3 and 4. A diagnostic ultrasound suggested a loco-regional axillary lymph node metastasis. After discussion in the interdisciplinary tumour board, amputation of the affected digits followed by lymph node excision was recommended. CONCLUSIONS: Advanced SCC of the hand requires interdisciplinary management. Amputation is part of the surgical spectrum in advanced cases.
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BACKGROUND: Eruptive xanthomas are rare and often asymptomatic. On the other hand, these cutaneous lesions are a red flag for serious underlying metabolic disorders that demand an early diagnosis to prevent morbidity and mortality. CASE REPORT: We report on two male patients, aged 23 and 27 years, who presented with eruptive xanthomas. Clinical, histological and laboratory investigations disclosed a metabolic syndrome in the younger patient and an alcohol-induced chylomicronemia in the elder one. Two types of macromorphology of cutaneous lesions were observed. Treatment was tailored according to underlying pathologies and resulted in significant improvement of the metabolic parameters and improvement of skin lesions. CONCLUSION: Dermatologists should be aware of the diagnostic importance of eruptive xanthomas for serious metabolic disorders.
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BACKGROUND: Leg ulcers are a burden to patients, their families and society. The second most common cause of chronic leg ulcers is the mixed arterio-venous type. An 80-year-old female patient presented to our department due to painful enlarging chronic leg ulcer of mixed arteriovenous origin on her left lower leg. She suffered from peripheral arterial occlusive disease stage I and chronic venous insufficiency Widmer grade IIIa, and a number of comorbidities. AIM: The aim of our ulcer treatment was a complete and stable wound closure that was hampered by arterial occlusion, exposed tendon, and renal insiffuciency. CASE REPORT: To improve the prognosis for ulcer surgery, we performed percutaneous transluminal angioplasty, transcutaneous CO2 and deep ulcer shaving. The wound was closed by sandwich transplantation using elastin-collagen dermal template and meshed split skin graft. She had a 100% graft take with rapid reduction of severe wound pain. CONCLUSION: Complex approaches are necessary, to gain optimum results in leg ulcer therapy in mixed leg ulcers. Therapeutic nihilism should be abandonend.
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Acrocyanosis is an uncommon complaint belonging to the acro-syndromes. It typically presents with coolness and bluish discolourations of hands, feet, ears, nose, lips and nipple. The most frequently affected parts of the body are the hands. This review discusses physical factors, vascular disorders, infectious diseases, haematological disorders, solid tumours genetic disorders, drugs, eating disorders, and spinal disease presenting as or leading to acrocyanosis.
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Langerhans cell histiocytosis (LCH) is a rare disorder that is now recognized as a true malignancy of dendritic Langerhans cells. Various somatic mutations have been discovered for BRAF and MAP kinases. The clinical variability is remarkable. A very rare variant is cutaneous LCH without internal organ manifestations. We present a case series of cutaneous LCH illustrating the spectrum of clinical symptoms, treatment, and disease course. Cutaneous LCH needs a careful investigation of possible internal involvement and a long follow-up since second malignancies may develop over time. Overtreatment should be avoided, since too-aggressive treatment will not improve the outcome.
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Histiocitosis de Células de Langerhans , Neoplasias Cutáneas , Anciano , Biopsia , Femenino , Histiocitosis de Células de Langerhans/patología , Histiocitosis de Células de Langerhans/terapia , Humanos , Inmunohistoquímica , Lactante , Masculino , Persona de Mediana Edad , Piel , Neoplasias Cutáneas/patología , Neoplasias Cutáneas/terapiaRESUMEN
Skin tumours are among the most frequent tumour types of mankind. In the case of large tumours, field cancerization, or satellitosis scalping surgery is a possible option. The procedure can also be used in a palliative setting with tumour debulking. Less common indications are multiple benign tumours of the scalp and chronic inflammatory scalp dermatoses not responding to medical treatment. We present a case series and discuss surgical modalities beyond curative surgery of primary skin cancer.
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Melanoma affecting two interdigital spaces has been recently described by our group for the first time in medical literature. Here we present a second patient with acrolentiginous melanoma of the sole affecting the 1st and 2nd interdigital space. The tumour was removed by delayed Mohs surgery. Due to the extension of the melanoma, digit II and III had to be removed. Staging excluded metastatic spread. The tumour was classified as pT4bN0M0.
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BACKGROUND: Lentigo malignant melanoma is a melanoma subtype of chronic sun-damaged skin in elderly Caucasians. Amelanotic variants of lentigo malignant are extremely rare. CASE PRESENTATION: This is a case report of an 80-year-old male patient who presented with a non-pigmented exophytic tumour of his bald head. After complete surgical excision under the suspicion of squamous cell carcinoma, three-dimensional histologic examination confirmed an amelanotic lentigo malignant melanoma with a tumour thickness of 1.76 mm, resected R0. Five years later he developed the first relapse, the other year a satellite metastasis was surgically removed. One year later, this patient had developed a large relapsing lentigo malignant melanoma with skull roof invasion. There was no evidence of distant metastatic spread. Amelanotic lentigo malignant melanoma is a very rare tumour. CONCLUSIONS: Serial excision or slow Mohs and Mohs micrographic surgery are the treatments of choice especially in the head and neck area. These tumours may be locally very aggressive as it is shown by skull invasion in the present case.
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BACKGROUND: The sudden development of facial plaques and nodules may be an alarming clinical sign for underlying malignancies. Nevertheless, a broad range of inflammatory and infectious diseases must be considered as well in the differential diagnosis. CASE REPORT: We report on a 53-year-old male patient with a left-sided cheek infiltration with oozing but no lymphadenopathy. He had a medical history of head-and-neck cancer. The primary differential diagnosis was herpes zoster with secondary impetiginization or pyoderma facial. About eight weeks later, the patient presented with progressive formation of nodules and plaques on the face and isotretinoin was stopped. Skin biopsy suggested mycosis fungoid and an oral treatment with bexarotene was started. After limited response for another eight weeks, he returned later with massive facial swelling, nodules and impetiginization. Another skin biopsy was performed to exclude diagnostic error or investigate possible disease progression. Microscopic evaluation and multiplex-polymerase chain reaction confirmed the diagnosis of peripheral T-cell lymphoma, not otherwise specified (PTL-NOS), stage Ia (T1 N0 M0). Imaging techniques excluded metastatic spread. By interdisciplinary tumour board, R-CHOP (rituximab, cyclophosphamide, hydroxyl-doxorubicin, vincristine, and prednisolone) was recommended and initiated by hemato-oncologists. CONCLUSIONS: PLT-NOS confirmed in the present patient has a poor prognosis with a 5-year survival rate of less than 20%.
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Herpes zoster is a common disease caused due to varicella zoster virus (VZV) infection with increasing incidence by age. If the patient has a severe, extended, or treatment-recalcitrant course of herpes zoster, this must be a red flag to search for underlying pathologies. Here, we report about a 64-year-old male patient with diabetes, who came to our emergency department because of general malaise, fever, chills, and a pronounced nuchal and facial swelling on the left side. Based on herpetiform-grouped vesicles and yellowish crusts, an impetiginized facial herpes zoster was diagnosed, and combined antiviral and antibiotic treatment was initiated. He was HIV negative. Despite intensified treatment, his situation worsened. We observed blasts in peripheral blood, but bone marrow biopsy was initially denied. Some days later after deterioration of his disease, he accepted further diagnostics. A myelodysplastic syndrome with blast excess (refractory anemia and blast excess II, RAEB II) could be confirmed. The following translocations were detected: t(2;12)(p13; q13) and t(6;9)(p22;q34). REAB II has an unfortunate prognosis. Cytoreductive treatment was initiated by the hematooncologist. Unfortunately, the patient deceased due to septic shock.
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Moulages are individualized wax models that were very popular as teaching aids in medicine during the 19th and until the first half of the 20th century. After a period of decline, moulages have become a subject of interest again in more recent times since they represent unique artifacts of art, craftsmanship, and medical history. Werther's collection at Dresden-Friedrichstadt Hospital was one of the most important in Dresden-the capital of Saxony-during the period before World War II. Some relicts have survived and have been restored.