[Juvenile systemic lupus erythematosus with unusual manifestation of lupus-associated panniculitis]. / Juveniler systemischer Lupus erythematodes mit der ungewöhnlichen Manifestation einer Lupus-assoziierten Pannikulitis.

Juvenile systemic lupus erythematosus (JSLE) is a rare multisystem autoimmune disease with broad heterogeneity of clinical manifestations. Diagnosing JSLE is often very challenging. This life-threatening, unpredictable, and relapsing disease, which may affect various organ systems, requires interdisciplinary, lifelong care. Here, we report the case of a 13-year-old patient with JSLE suffering from recurrent arthralgia, lupus panniculitis, and rashes that were successfully treated with hydroxychloroquine and prednisolone.

[Multiple erythema migrans and facial nerve paralysis: clinical manifestations of early disseminated Lyme borreliosis]. / Multilokuläre Erythemata migrantia und Fazialisparese : Klinische Manifestationsformen der disseminierten Frühinfektion der Lyme-Borreliose.

Lyme borreliosis is a common vector-borne disease in Europe. The infection follows different stages with a broad variability of clinical symptoms and manifestations in different organs. A 49-year-old man presented with flu-like symptoms, facial nerve paralysis and multiple erythematous macular on his trunk and extremities. We diagnosed Lyme disease (stage II) with facial nerve paralysis and multiple erythema migrans. Intravenous ceftriaxone led to complete healing of hissymptoms within 2 weeks.

[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.

[Tinea barbae profunda by Arthroderma benhamiae. A diagnostic challenge]. / Tinea barbae profunda durch Arthroderma benhamiae. Eine diagnostische Herausforderung.

Dermatomycoses due to pets and farm animals are often a clinical and diagnostic challenge for dermatologists. A 24-year-old man presented with inflammatory skin changes on his cheeks and chin. Because of negative fungal culture and the clinical appearance of a highly inflammatory process, our first diagnosis was a bacterial pyoderma. Polymerase chain reaction (PCR) identified Arthroderma benhamiae in both the patient and his guinea pig. A. benhamiae is a zoophilic dermatophyte which belongs to the Trichophyton mentagrophytes-complex. The fungus is acquired from guinea pigs and causes highly inflammatory forms of tinea. PCR-based diagnostics are quick and simple tools to identify this pathogen, so that suitable antimycotic therapy can be initiated quickly.

[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.

[Fiddler's neck]. / Geigerknoten.

The fiddler's neck is an uncommon variant of acne mechanica in violinists and violists. It is a single firm red-brown dermal nodule usually on the left side of neck. This special form of acne mechanica represents a therapeutic challenge since the triggering mechanical factors persist, unless they can be corrected by changes in positioning or modifications of the chin pad. A 72-year-old woman who had played the violin since childhood presented with a red-brown nodule on her neck for 18 months. Cushioning provided no relief. Excision of the affected area with primary closure represented one therapeutic option. Further supportive measures include improved posture to reduce the pressure between skin and instrument and interposing a neck cloth.

[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.

[Linear IgA bullous dermatosis of children]. / Lineare IgA-Dermatose im Kindesalter.

Linear IgA bullous dermatosis is an acquired autoimmune subepidermal blistering disease, characterized by linear IgA deposits at the basement membrane zone. Described in both children and adults, it occurs as tense pruritic vesicles and bullae in a "cluster of jewels" configuration with central crusting on an inflammatory elevated base. It is typically located on the face, anogenital region and trunk. Whilst the adult manifestations can be chronic, in children a spontaneous remission has often been reported. Our patient showed a spontaneous remission after 8 weeks of symptomatic topic treatment with methylprednisolone and oral cetirizine dihydrochloride.

[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.

[Incontinentia pigmenti (Bloch-Sulzberger syndrome)]. / Incontinentia pigmenti (Bloch-Sulzberger-Syndrom).

A female infant, aged two weeks, presented with linear erythematous crusted papules, plaques and blisters on the right leg which had occurred two days after birth. Histological examination revealed typical features of incontinentia pigmenti in the inflammatory stage. Incontinentia pigmenti is a rare X-linked dominant genodermatosis caused by mutations in the NEMO gene located at Xq28 affecting the skin, different organ systems, the central nervous system, eyes, teeth and skeleton with variable expression. We summarize important clinical and diagnostic aspects of incontinentia pigmenti as well as its genetic and molecular basis.

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.

[Classical and atypical presentations of Galli-Galli disease]. / Klassische und atypische Präsentation des Morbus Galli-Galli.

Galli-Galli disease is a rare genodermatosis which classically presents with reticulate hyperpigmentation of the flexures and intertriginous areas. Recently, an atypical clinical presentation of Galli-Galli disease with monomorphic lentigo-like maculae and papules has been reported. We describe two cases of Galli-Galli disease, one patient presenting with a classical form and another with atypical skin lesions. In spite of the clinical differences, the histopathological examination - with filiform elongated rete ridges and acantholysis - confirmed the diagnosis of Galli-Galli disease in both cases.

[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.