[Eosinophilic granulomatosis with polyangiitis presenting as livedo racemosa]. / Livedo racemosa als kutane Manifestation bei eosinophiler Granulomatose mit Polyangiitis.

As a rare antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis, eosinophilic granulomatosis with polyangiitis (EGPA, formerly Churg-Strauss syndrome) is characterized by asthma, severe peripheral eosinophilia and the presence of extravascular granulomas. Cutaneous involvement usually includes palpable purpura or cutaneous to subcutaneous nodes. We present the case of a 43-year-old woman with EPGA and the unusual cutaneous manifestation of livedo racemosa.

[Nodular granuloma of the lip. An unusual manifestation of secondary syphilis]. / Epitheloidzellige granulomatöse Entzündungsreaktion an der Lippe. Ungewöhnliche Manifestation im Stadium II bei Frühsyphilis.

Syphilis is a sexually transmitted disease caused by Treponema pallidum which evolves through three overlapping stages. A 50-year-old woman presented with an expanding painless granulomatous nodule on her lower lip in combination with a maculo-papular exanthem. Both serologic studies and microscopic examination indicated an infection with Treponema pallidum. This case shows an unusual granulomatous nodular presentation of syphilis on the lower lip, emphasizing the variable clinical and histological manifestations syphilis, which shows an increasing number of new infections worldwide in recent years.

[Metastatic malignant melanoma. Successfull treatment with ipilimumab]. / Metastasiertes malignes Melanom. Erfolgreiche Behandlung mit Ipilimumab.

A 73-year-old man, in whom 26 years ago a malignant melanoma with cervical lymph node metastases of the right retroauricular region was diagnosed, developed BRAF V600E-negative distant metastases, which progressed during both monochemotherapy and polychemotherapy. Therefore he was started on ipilimumab in a dose of 3 mg/kg body weight four times in intervals of 3 weeks. Subsequently, there was an almost complete regression of distant metastases. In several phase III trials a significant survival benefit has been identified for patients treated with ipilimumab. The human monoclonal antibody has been approved since July 2011 as a second-line treatment in Germany and was incorporated in January 2013 into the new guidelines for the treatment of malignant melanoma. The CTLA-4 antibody is the first drug that can improve significantly survival in patients with metastatic melanoma. In advanced (unresectable or metastatic) melanoma, immunostimulatory treatment with ipilimumab represents a new therapeutic option.

[Radiation-induced Favre-Racouchot disease]. / Strahleninduzierter Morbus Favre-Racouchot.

A 71-year-old man presented with disfiguring skin changes of the nose and cheek. The patient had undergone a surgery of a malignant melanoma of the right paranasal sinus and then 5 months later received radiation therapy with 60 Gy total dose to the endonasal area. Physical examination revealed elastosis, open and closed comedones, and cysts in the field of radiation exposure. Taking in account the exclusive affection of the irradiated skin, we diagnosed a radiation-induced Favre-Racouchot disease. We recommended topical treatment with vitamin A derivatives in combination with physical comedo extraction.

[NK/T-cell lymphoma, nasal type with cutaneous dissemination]. / NK/T-Zell-Lymphom vom nasalen Typ mit kutaner Dissemination.

Extranodal NK/T-cell lymphoma, nasal type, is a lymphoproliferative disorder originating from peripheral T-cells or natural killer (NK) cells. While it is a rare disease in Europe, it is more frequent in Asia and South America. It is associated with Epstein-Barr virus (EBV) infection and characterized by an extremely aggressive course and poor prognosis. We report a 46-year-old Caucasian woman who presented with multiple subcutaneous, painful nodules on the trunk first noticed a few weeks earlier. In addition to dermatological findings, the patient reported a 4-months history of necrotizing nasopharyngeal inflammation of unclear origin. Due to nonspecific histological and clinical findings mimicking a chronic inflammatory condition, a diagnosis of Wegener disease was made and immunosuppressive therapy with azathioprine was initiated. However the disease progressed under therapy. Histopathological reevaluation and immunophenotyping revealed a disseminated NK/T-cell lymphoma, nasal type. In the case of an unspecific chronic inflammatory process in the nasopharyngeal space one should always consider the possibility of this rare lymphoma, even in Europe.

[Epithelioid sarcoma of the right hand]. / Epitheloidzelliges Sarkom der rechten Hand.

A 35-year-old man presented with swelling, indurations and nodules on the thumb, wrist and fingers of the right hand. History revealed that the findings were slowly progressive and had been present for at least eight years. Histopathologic analysis of a nodule showed a diffuse infiltrate with atypical spindle-shaped cells and expression of cytokeratin, epithelial membrane antigen (EMA) and CD34; the diagnosis of epithelioid sarcoma (ES) was made. Because of diffuse extension of the tumor, forearm amputation was performed along with axillary dissection and local radiotherapy because of axillary lymph node metastases. ES is a rare subtype of soft tissue sarcoma with a harmless appearance and indolent course over years. ES represents a diagnostic challenge, with consequent delay in diagnosis and adequate treatment. The most important measure in the treatment of ES is early surgical excision with adjuvant radiotherapy if local metastases are present.

[Hereditary cutaneous leiomyomatosis]. / Hereditäre kutane Leiomyomatose.

The occurrence of multiple cutaneous leiomyomas can be indicative of hereditary cutaneous leiomyomatosis. This autosomal dominant disorder is due to germline mutations in the fumarate hydratase (FH) gene. Associations with uterine myomas and renal cell carcinomas have been described and are referred to as Multiple Cutaneous and Uterine Leiomyomas (MCUL) or Hereditary Leiomyomatosis and Renal Cell Cancer (HLRCC), respectively. A 34-year-old man presented with multiple red-brown papules and nodules. After histopathologic confirmation of piloleiomyomas, we made the diagnosis of hereditary cutaneous leiomyomatosis. Taking into consideration the aforementioned complications, close interdisciplinary management of these patients and regular screening examinations within affected families are mandatory.

[Anetoderma with positive Borrelia serology]. / Anetodermie bei positiver Borrelienserologie.

Anetoderma is an uncommon disease characterized by multiple circumscribed atrophic, herniated skin lesions on trunk, thighs and upper arms caused by loss of elastic fibers. Associations with autoimmune diseases or infections, especially spirochetal infections, have been described. We report a case of anetoderma with an increased serum Borrelia burgdorferi IgM-titers. After treatment with doxycycline 200 mg/day for three weeks, the progression of the disease stopped and no new lesions appeared.

[Malassezia folliculitis in an immunocompromised patient]. / Malassezia-Follikulitis bei einem immunsupprimierten Patienten.

Malassezia folliculitis (synonym: Pityrosporum folliculitis) is a polymorphic dermatomycosis characterized by the development of small papules and pustules. In severe cases, molluscoid comedonal papules are typical. The disease occurs mainly in sebum-rich areas of the face, back and chest, frequently affecting patients under immunosuppressive therapy. We report a case of Malassezia folliculitis observed in a heart transplant recipient who was successfully treated with itraconazole. Moreover, we summarize the taxonomy of the genus Malassezia as well as diagnostic and therapeutic measures of the related folliculitis.

[Erythema elevatum et diutinum]. / Erythema elevatum et diutinum.

Erythema elevatum et diutinum (EED) is a rare, chronic, cutaneous, leukocytoclastic vasculitis. It is characterized by symmetric, plaques, papules and nodules occurring preferentially on the extensor aspects of the arms. An association with chronic infections, neoplasms, especially myeloproliferative diseases and paraproteinemia, as well as autoimmune disorders (rheumatoid arthritis, Crohn disease) has been described. An 83-year-old woman with EED presented with symmetric, skin colored, firm nodules on the extensor surfaces of her fingers for two years. This case report summarizes the central clinical aspects and differential diagnosis of EED as well as its therapeutic options.

The new polyomavirus (MCPyV) does not affect the clinical course in MCCs.

Since 2008, a new polyomavirus (MCPyV) in Merkel cell carcinomas (MCC) has been described, but little is known about its impact on the clinical course. The purpose of this study was to determine the presence of MCPyV in a large sample and to correlate the results with the clinical course of the disease. 59 samples from 44 patients were analysed for the presence of MCPyV using the primers LT3, VP1 and LT1. The clinical records of these patients were evaluated and correlated with the presence of MCPyV. 58% of specimens were positive for MCPyV. Of these, LT3 was positive in 53%, VP1 in 37% and LT1 in 10%. 57% of primary tumours and 53% of metastases were positive for LT3; the numbers for VP1 and LT1 were lower. There was no correlation between the detection of MCPyV in the primary tumour and the appearance of metastases. The survival time was statistically independent from the presence of MCPyV. There is a striking occurrence of MCPyV in MCC, but whether it affects the clinical course remains unclear.

[Metastatic Crohn disease. Atypical manifestation]. / Metastatischer Morbus Crohn. Atypische Manifestation.

Crohn disease may be associated with many different typical and atypical cutaneous manifestations, also known as "metastatic Crohn disease". A 33-year-old man presented with generalized erythematous plaques with bullae and pustules. Histological examination revealed dense epidermal and dermal neutrophilic infiltration, as well as dermal edema with signs of vasculitis. The patient recovered with topical and systemic glucocorticosteroids. The lesions healed with both flat and hypertrophic scars. In summary, we present a generalized atypical case of metastatic Crohn disease.

[Giant condyloma of the penis with malignant transformation associated with lichen sclerosus et atrophicus]. / Riesenkondylom des Penis mit maligner Transformation bei vorbestehendem Lichen sclerosus et atrophicus.

A 72-year old patient presented with a 6 months history of a rapidly growing tumor of the glans and foreskin. He had a long history of phimosis with lichen sclerosus et atrophicus-like lesions on the foreskin which had not been treated. The rest of the personal, family and sexual history was unremarkable. Treatment consists of circumcision and tumor excision. Histopathology confirmed a squamous cell carcinoma within a giant condyloma with a concomitant lichen sclerosus et atrophicus. CT- and ultrasound scans showed no metastases. Giant condylomas are a rare sexually transmitted disease usually caused by human papilloma virus subtypes 6, 11, but also by 16 and 18 among others. They are expansive, cauliflower-like destructive lesions that most frequently affect the anogenital region. In about 30 percent a giant condyloma progresses into a squamous cell carcinoma. Therapy of choice is the histopathologically controlled excision. Recurrences are often seen, so the patients should be monitored frequently after therapy.

[Shulman's syndrome (eosinophilic fasciitis)]. / Shulman-Syndrom (eosinophile Fasziitis).

Eosinophilic fasciitis is a rare disease characterized by edema, painful indurations, and progressive muscle weakness. Mainly the extremities are involved. We report on a 22-year-old woman with eosinophilic fasciitis presenting with progressive muscle weakness of both hands and feet and a reduced general condition. She showed symmetrical and firm swelling of the extremities with painful restriction of joint movement. Systemic treatment with glucocorticosteroids as well as physiotherapy and manual lymphatic drainage led to continuous improvement of her symptoms. The differentiation from other diseases, such as systemic scleroderma, eosinophilia-myalgia syndrome, and pseudoscleroderma, might be difficult at the beginning of the disease. The gold standard for diagnosis is--as was done in our case--a deep skin-to-muscle biopsy. Further imaging, especially magnetic resonance imaging, can support the diagnostic procedure.

[Merkel cell carcinoma. Viral genesis and new therapeutic options?]. / Merkel-Zell-Karzinom. Virale Genese und neue Therapieoptionen?

Merkel cell carcinoma (cutaneous neuroendocrine carcinoma) is a rare, highly malignant, neuroendocrine tumor of the skin with predominance in older patients. The tumor is most often located in the sun-exposed skin of the head, the neck and -as in our patient - the extremities. Notably, the tumor bears a high risk of an early regional lymph node as well as distant metastases. Clinically, only a presumptive diagnosis of Merkel cell carcinoma can be established. The definite diagnosis is made by histological and immunohistological methods. Surgical excision with a safety margin should be combined with sentinel lymph node biopsy. In advanced tumor stages (lymph node or visceral metastasis), a remission can be achieved by different chemotherapy schedules in combination with radiation. Recently, a previously unknown polyomavirus, named Merkel cell polyomavirus (MCV or MCPyV), has been identified in 80% of Merkel cell carcinomas. In the near future, these novel findings could be utilized to distinguish Merkel cell carcinoma from small round cell cancers and could lead to the development of new therapeutic options.

[Tuberous sclerosis: diagnostic criteria and new treatment approaches]. / Tuberöse Sklerose : Diagnosekriterien und neue Therapieansätze.

With a prevalence of 1 in 6,000 births, tuberous sclerosis is a relatively frequent hamartoma and tumor syndrome inherited as an autosomal dominant trait, which manifests primarily on the skin and in the central nervous system. Decisive factors for morbidity and mortality and thus for the prognosis are the changes in the central nervous system in the form of cortical hamartomas. Treatment for many years consisted solely in using nonspecific symptomatic approaches; dermatological therapy comprised mainly laser or electroacoustic ablation of facial angiofibromas. New models of therapy hinder the pathogenesis of tuberous sclerosis. Various studies provided evidence that the macrolide rapamycin decreases growth of brain and kidney tumors by specific inhibition of mTOR kinase. Synergistic effects were observed in combination therapy with the cytokine IFN-gamma.

[Treatment of carcinoma in situ of erythema ab igne with photodynamic therapy]. / Behandlung eines Carcinoma in situ bei Erythema ab igne mittels photodynamischer Therapie.

Erythema ab igne (EAI) occurs at skin locations that have been repeatedly exposed to heat or infrared radiation, a single dose of which results in erythema but the intensity of which does not suffice to cause a burn. The clinical picture of EAI is characterized by reticulate erythema, desquamation, and teleangiectasias. Complications of EAI include increasing cutaneous atrophy and predisposition to malignant tumors. The paramount goal of therapy is to eliminate the cause. Individual case reports of EAI with established epithelial dysplasia in the sense of carcinoma in situ have described good response of the neoplastic alterations to topical application of 5-fluorouracil or imiquimod. In our case we achieved successful results with photodynamic therapy.

Multicentre intraindividual randomized trial of topical methyl aminolaevulinate-photodynamic therapy vs. cryotherapy for multiple actinic keratoses on the extremities.

BACKGROUND: Methyl aminolaevulinate-photodynamic therapy (MAL-PDT) is an effective treatment in facial/scalp actinic keratosis (AK). OBJECTIVES: The aims of this study were to compare efficacy, safety, cosmetic outcome and patient preference of MAL-PDT vs. cryotherapy in patients with AK at other locations. METHODS: A multicentre, controlled, randomized, open, intraindividual, right-left comparison was performed. Patients with nonhyperkeratotic AK were treated once with MAL-PDT and cryotherapy on either side of the body. At week 12, lesions showing noncomplete response were retreated. The primary efficacy variable was the lesion response at week 24. Investigator's assessment of cosmetic outcome, patient's preference in terms of cosmetic outcome and a patient preference questionnaire were also analysed at week 24. RESULTS: In total, of 121 patients with 1343 lesions (98% located on the extremities and the remainder on the trunk and neck) were included. Both treatments provided a high mean percentage reduction in lesion count at week 24 with significantly higher efficacy for cryotherapy: 78% for MAL-PDT and 88% for cryotherapy (P=0.002, per protocol population). Investigator's assessment of cosmetic outcome was significantly better for MAL-PDT than cryotherapy (P<0.001), 79% of lesions having an excellent cosmetic outcome with MAL-PDT vs. 56% with cryotherapy at week 24. The cosmetic outcome achieved by MAL-PDT compared with cryotherapy was also preferred by patients (50% vs. 22%, respectively, P<0.001), and 59% of patients would prefer to have any new lesions treated with MAL-PDT compared with 25% with cryotherapy (P<0.001). Both treatment regimens were safe and well tolerated. CONCLUSIONS: MAL-PDT showed inferior efficacy for treatment of non-face/scalp AK compared with cryotherapy. However, both treatments showed high efficacy, and MAL-PDT conveyed the advantages of better cosmesis and higher patient preference.

Multiple eruptive myxoid dermatofibromas: report of first case and review of literature.

Multiple eruptive dermatofibromas are a rare presentation of dermatofibroma which are frequently associated with underlying diseases such as human immunodeficiency virus infection or lupus erythematosus. Eruptive dermatofibromas generally present a characteristic histology with a poorly circumscribed lesion showing hyperplasia of the epidermis, prominent bundles of collagen and a diffuse proliferation of fibrocytes. We report an unusual case of multiple eruptive dermatofibromas showing massive depositions of mucin within the dermis. A 20-year-old woman presented with nearly 100 red to yellowish papules and nodules distributed symmetrically all over the integument which developed over a period of 9 years. Comprehensive clinical and laboratory diagnostics showed no signs indicating any underlying disease. To our knowledge this is the first report of multiple eruptive myxoid dermatofibromas. We consider this case to be a unique presentation of multiple eruptive dermatofibroma showing massive deposition of mucin.