Subungual melanoma: A single center series from Dresden.

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.

Cutaneous Langerhans cell histiocytosis : The spectrum of a rare cutaneous neoplasia.

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.

Werther's collection of medical moulages.

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.

Patterns of care and follow-up care of patients with uveal melanoma in German-speaking countries: a multinational survey of the German Dermatologic Cooperative Oncology Group (DeCOG).

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.

Successful treatment of relapsing disseminated coccidioidomycosis with cutaneous involvement with posaconazole.

A 52-year-old woman with pulmonary sarcoidosis on immunosuppressive therapy developed pulmonary infiltrates and cutaneous granulomatous abscesses after a trip to the USA in April 2005. A hyphomycete was identified, further characterized by a gene probe as Coccidioides spp. and then definitively identified as Coccidioides posadasii by polymerase chain reaction and sequencing. Antibodies towards Coccidioides spp. were detected. The infection was successfully treated with posaconazole (Noxafil), 2 x 400 mg/d.

Myxoid neurothekeoma--a painful case in a less common location.

Myxoid neurothekeoma is a rare benign asymptomatic tumor of the skin. Most tumors are located on the head, shoulders and arms. We present a 19-year-old female patient with a relapsing tumor of this type in a less common localization, i.e., lower trunk, and with painful sensations under mechanical pressure. Wide excision surgery led to complete remission.

Advanced Ulcerated Squamous Cell Carcinoma of the Hand with Locoregional Axillary Lymph Node Metastasis - Case Report and Literature Review.

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.

Successful Treatment of a Widespread Pemphigus Chronicus Familiaris (Hailey-Hailey) By Erbium-YAG-Laser.

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.

Cutaneous Angiosarcoma of Head and Neck - A Single-Centre Analysis.

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.

Large B - Cell Lymphoma of the Leg - Unfavourable Course with Rituximab/Bendamustin.

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.

Acanthosis Nigricans - A Two-Sided Coin: Consider Metabolic Syndrome and Malignancies!

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.

Eosinophilic Fasciitis - Report of Three Cases and Review of the Literature.

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.

A Painful Step - Pendulating Plantar Eccrine Poroma.

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.

Tumor necrosis factor-alpha inhibitor-induced psoriasis or psoriasiform exanthemata: first 120 cases from the literature including a series of six new patients.

Tumor necrosis factor-alpha (TNFalpha) inhibition is effective in the treatment of moderate-to-severe psoriasis. We report on 120 patients from the literature including six new patients (three women and three men) who developed pustular lesions during treatment with TNFalpha inhibitors. We identified 72 women and 36 men (several papers did not specify the gender of patients) with an age range of 13-78 years (mean 42.3 years). The primary diagnoses were rheumatoid arthritis (n = 61), ankylosing spondylitis (n = 21), psoriasis (n = 10), Crohn disease (n = 8), SAPHO (synovitis acne pustulosis hyperostosis osteitis) syndrome (n = 3), psoriatic arthritis (n = 2), and other diagnoses (n = 15). Psoriasis (except palmoplantar pustular type) was the most common adverse effect during anti-TNFalpha treatment (n = 73), followed by palmoplantar pustular psoriasis (n = 37) and psoriasis of the nail (n = 6), sometimes combined in the same patient. Palmoplantar pustulosis and psoriasiform exanthema was the diagnosis in ten patients each. A positive personal history of psoriasis was recorded in 25 patients. A positive family history was noted in eight patients. No data about personal (n = 7) or family history (n = 46) were available in a number of patients. Newly induced psoriasis was diagnosed in 74 patients whereas an exacerbation or aggravation of a pre-existing psoriasis was noted in another 25 patients. All three TNFalpha inhibitors available on the market were involved: infliximab (63 patients), etanercept (37 patients), and adalimumab (26 patients). Several patients were treated with more than a single TFNalpha inhibitor. The timing of cutaneous adverse effects (psoriasis and psoriasiform rash) varied considerably among patients, ranging from after a single application to a delayed response of up to 63 months after initiation of treatment. The mean time to appearance of the cutaneous adverse effect for all TNFalpha inhibitors was 9.5 months. Cessation of the responsible TNFalpha inhibitor was carried out in 47 patients either alone or in association with adjuvant anti-psoriatic therapy (mostly topical). This resulted in complete remission in 21 patients, partial remission in 20 patients, and stable disease in another three patients; in the other three patients, the outcome was not reported. TNFalpha inhibition was continued in 47 patients but anti-psoriatic adjuvant therapy was introduced. The outcome in this group was complete remission in 22 patients, partial remission in 25 patients, and stable disease in 2 patients. The response rate (complete remission plus partial remission) was 93.2% and 95.9%, respectively, in each group. In six patients, switching from one TNFalpha inhibitor to another one immediately after cutaneous adverse effects occurred resulted in an improvement in five patients. In nine patients, a second TNFalpha inhibitor was initiated after a break in TNFalpha inhibition. The response to a second or third drug in these patients was mixed. The underlying pathomechanisms of induction of psoriasis or psoriasiform exanthemata by TNFalpha inhibitors remain elusive but there is reason to assume that induction of such adverse events has more than one pathophysiology.

The hospital as a work of art: one hundred thirty years of dermatology in Dresden-Friedrichstadt.

The department of dermatology at the Hospital Dresden-Friedrichstadt (Dresden, Germany) was founded in 1874 as one of the oldest departments of dermatology in a municipal hospital in Germany. Dresden was the capital of Saxony and, as such, one of the most influential cultural centers of Germany. This particular situation is also reflected by the hospital's history itself.

Deep ulcer shaving combined with split-skin transplantation in distal calciphylaxis.

Calciphylaxis is a cause of painful deep ulcers. There is controversy about best wound management in this disease. A retrospective study of inpatients during the 3 years was made. Seven calciphylaxis patients were identified. All patients suffered from various associated pathologies including diabetes mellitus type II and chronic renal insufficiency. Ulcers were treated by aggressive and deep shaving combined with autologous split-skin grafting in the same session. A 30% to 90% take rate of the grafts eventually with a complete ulcer healing in 6 of 7 patients was achieved. No patient developed a deep cutaneous infection or sepsis. All patients are still alive except one. The single death was related to cardiovascular complications. In distal calciphylaxis, aggressive ulcer surgery with defect closure offers a marked improvement in quality of life and prevents early deep skin infections and sepsis as major causes of mortality.

Eruptive Xanthomas - Two Case Reports With Distinct Features.

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.

The Role of Complex Treatment in Mixed Leg Ulcers - A Case Report of Vascular, Surgical and Physical Therapy.

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.