[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.

[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.

[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.

[Hyperhidrosis of childhood and adolescence: clinical aspects and therapeutic options]. / Hyperhidrose im Kindes- und Jugendalter : Klinik und aktuelle Therapieoptionen.

Hyperhidrosis is characterized by localized or general excessive sweating which is severe enough to be perceived as pathological. Since excessive sweating often starts in childhood and adolescence, usually in children between 6 and 16 years of age, hyperhidrosis is an important disorder for children and juveniles. This condition causes considerable disruption of both social life and educational career, leading to severe deterioration in the patient's quality of life. In addition, therapy is often challenging since many treatment modalities are not approved for children. Nonetheless, there are still effective therapeutic options for children with hyperhidrosis.

[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.

[Familial benign chronic pemphigus (Hailey-Hailey disease): successful treatment with carbon dioxide laser]. / Pemphigus chronicus benignus familiaris (Morbus Hailey-Hailey) : Erfolgreiche Behandlung mittels CO2-Laser.

Hailey-Hailey disease is a rare, autosomal dominantly inherited genodermatosis, which manifests with recurrent intraepidermal blistering and erythematous hyperkeratotic patches in intertriginous areas. Conventional therapeutic approaches include topical and systemic steroids, antibiotic agents, as well as oral retinoids. Alternative treatments include surgical interventions such as excision, dermabrasion and laser ablation. A 56-year-old woman presented with 15-year history of severe therapy-resistant Hailey-Hailey disease. We performed laser ablation with carbon dioxide laser under general anesthesia. Six weeks after treatment complete remission within ablated areas was achieved. Six-month follow-up showed no relapse. In our opinion carbon dioxide laser ablation represents an effective and safe therapeutic option for Hailey-Hailey disease.

[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.

A proinflammatory activity of interleukin 8 in human skin: expression of the inducible nitric oxide synthase in psoriatic lesions and cultured keratinocytes.

Psoriasis is a common chronic skin disease mediated by cellular immune mechanisms and characterized by an intense neutrophil cell infiltrate and proliferative activation of epidermal keratinocytes. We have previously described the expression of the inducible nitric oxide synthase (iNOS) in epidermal keratinocytes of psoriatic skin lesions. In this study, the role of iNOS in psoriatic inflammation was explored ex vivo in psoriatic skin biopsies and in vitro in primary cultures of human keratinocytes. Messenger RNA for the iNOS enzyme (iNOS mRNA) was detected by reverse transcriptase polymerase chain reaction in skin biopsies from patients with psoriasis, but not in skin specimens from patients with atopic eczema or from healthy volunteers. As demonstrated by in situ hybridization and immunohistochemistry, expression of iNOS mRNA and its gene product was localized to the epidermal keratinocytes of psoriatic skin lesions. In situ hybridization further revealed a complete colocalization of mRNA expression for iNOS with interleukin (IL) 8 receptor-specific mRNA either in the basal germinative cell layer or at focal sites of ongoing neutrophil inflammation in suprabasal cell layers. Because psoriatic keratinocytes have previously been shown to express mRNA transcripts for IL-8, it seemed reasonable to hypothesize that iNOS expression could be induced in an autocrine loop by IL-8. This hypothesis was substantiated by our in vitro experiments showing that a combination of IL-8 and interferon gamma induces the expression of iNOS-specific mRNA and of the functional enzyme in cultured human keratinocytes. These results suggest an important role for iNOS in concert with IL-8 and its receptor early during the formation of psoriatic lesions.

[Infantile hemangioma. Successful treatment with propranolol]. / Infantiles Hämangiom. Erfolgreiche Behandlung mit Propranolol.

We report on an infant, aged four months, suffering from a severe hemangioma of the left labium majus. We induced systemic treatment with propranolol in off-label-use over a period of 5 1/2 months. A few weeks after onset of the treatment, the size and color of hemangioma was obviously reduced; finally there was an almost complete regression. This result underlines the role of propranolol in treatment of problematic hemangioma.

[Langerhans cell histiocytosis. Solitary facial lesion]. / Langerhans-Zell-Histiozytose. Isolierte kutane Manifestation im Bereich des Gesichtes.

Langerhans cell histiocytosis is the general term for all clinical entities characterized by a proliferation of dendritic cells that are phenotypically identical to the Langerhans cells of the skin. As dendritic cells are present in nearly every tissue of the body, Langerhans cell histiocytosis shows a broad spectrum of clinical manifestations, mostly in the bone (approximately 80%) and skin (approximately 60%). Langerhans cell histiocytosis is basically a disease of the childhood and early youth, but can rarely occur in the elderly. Here, we report on a 70-year-old man presenting with a single facial lesion of Langerhans cell histiocytosis and summarize the most important clinical aspects as well as current therapeutic concepts.

[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 role of hematopoietic stem-cell transplantation in cutaneous T-cell lymphoma.

Cutaneous T-cell lymphoma represents a broad spectrum of disorders with various manifestations, clinical courses, and therapeutic considerations. Mycosis fungoides is the most common form of cutaneous T-cell lymphoma, the skin being affected by erythematous patches, plaques, or tumors. The related Sézary syndrome is a more aggressive form of cutaneous T-cell lymphoma with cutaneous and leukemic manifestations. Recent advances in the understanding of the molecular and biologic behavior of T cells in these disorders have facilitated the development of novel treatments, using biologic agents, phototherapeutic methods, and irradiation techniques. Nevertheless, there remains a continued need to develop new therapeutic options, particularly for patients with advanced-stage and treatment-refractory disease. At present, hematopoietic stem-cell transplantation has the best chance for expanding the therapeutic armamentarium of cutaneous T-cell lymphoma. In advanced-stage mycosis fungoides and the Sézary syndrome, stem cell transplantation may result in sustained remissions and improvement of progression-free survival. However, physicians have to be aware of severe clinical problems like graft-versus-host disease as well as increased risk of infection and secondary cancers. In this review, we focus on the role of hematopoietic stem cell transplantation for the therapy of mycosis fungoides and the Sézary syndrome.

[Dermatomycoses of childhood]. / Dermatomykosen im Kindesalter.

The clinical relevance of mycotic infections of the skin and mucous membranes is often underestimated, although their number and duration are far greater than for all other microbial infections, and a rising incidence can be observed in childhood. An overview of important mycotic infections and their dermatologic manifestations is given here, with special emphasis on dermatophytoses and candidoses because mould infections only rarely lead to cutaneous lesions, even in immunocompromised children.

[Subungual squamous cell carcinoma. An important differential diagnosis of subungual tumors]. / Subunguales Plattenepithelkarzinom. Eine wichtige Differenzialdiagnose subungualer Tumore.

A 63-year-old patient presented with a 12-month history of a subungual tumor on the right middle finger. The patient had had a similar lesion involving his left index finger 5 years ago, which was identified as a subungual squamous cell carcinoma. Ablation of the nail plate, the nailbed and the matrix was performed. Histopathology confirmed another subungual squamous cell carcinoma. The patient received a full thickness skin graft. Subungual squamous cell carcinomas are among the most frequently reported types of subungual malignancies. However, subungual squamous cell carcinomas arising in more than one digit have been reported only rarely. The cause of subungual squamous cell carcinoma has not been clearly identified, they has been associated with radiation, chronic infection, arsenic ingestion, HPV infection and trauma.

[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.

[Tuberculosis cutis colliquativa. Even today an important differential diagnosis]. / Tuberculosis cutis colliquativa. Auch heute eine wichtige Differenzialdiagnose.

An 82-year-old woman presented with perforating abscesses in the cervical and axillary region over 7 months. Histopathological and extensive microbiological examinations lead to the diagnosis of tuberculosis cutis colliquativa, a form of postprimary cutaneous tuberculosis. Tuberculosis cutis colliquativa can be caused by either contiguous spread from an underlying structure, direct inoculation or hematogenous dissemination. Morphologically it is characterized by a subcutaneous node forming an abscess with secondary perforation of overlying skin. The parotid, submandibular, and supraclavicular regions are the sites most likely to be affected. Today it still remains an important differential diagnosis for an abscess with fistulas. Correlation with histopathologic findings and microbiological examinations including polymerase chain reaction and mycobacterial culture being the most reliable method is required. Standard therapy regimens consist of 2 months of quadruple therapy followed by another 4 months of a continuation phase using a two-drug regimen.

[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.

[Trombiculiasis in humans]. / Trombidiose des Menschen.

Trombiculiasis is an epizoonosis that occurs worldwide and is caused by various types of chiggers. Clinical findings after accidental contact with the parasitic mite larvae include erythematous papules and urticarial plaques, to some extent with vesicular transformation, which can lead to excoriations due to the intense pruritus. Prophylaxis against exposure is recommended when visiting areas known to be colonized by mites. Reactions to bites can be treated by local application of alcohol compresses or zinc lotions and external application of agents containing polidocanol or glucocorticosteroid. Systemic antihistamines are indicated to control itching.

[Successful use of allogeneic stem cell transplantation for treatment-refractory mycosis fungoides]. / Erfolgreicher Einsatz einer allogenen Stammzelltransplantation bei therapierefraktärer Mycosis fungoides.

Mycosis fungoides is the most common type of cutaneous T-cell lymphoma and characterized by a chronic progressive course spanning decades. The choice of treatment options should be tailored to the stage depending on the extent and aggressiveness of the disease and taking the individual situation of the patient into consideration. Long-term complete remissions can only be achieved in the early phase of the disease, while there is no therapy that results in a cure or long-term remission in advanced stages. In young patients with a treatment-refractory course of mycosis fungoides, allogeneic stem cell transplantation represents an important alternative option to manage the disease since complete clinical remission can be obtained even in advanced stages.