[Acute complications of vascular anomalies in childhood]. / Akute Komplikationen vaskulärer Anomalien im Kindesalter.

Congenital and acquired vascular anomalies are common in childhood. In addition to predominantly harmless vascular skin alterations there are others which must be immediately treated due to the potentially threatening complications. As examples three anomalies and the typical complications are presented. Hemangiomas in infancy can make urgent treatment necessary because they can be complicated by obstruction or painful ulceration. Kaposiform hemangioendothelioma can lead to a life-threatening consumption coagulopathy. Klippel-Trenaunay syndrome can be complicated by thrombosis, localized coagulopathy, and superinfections.

[Psoriasis vulgaris in children and adolescents. Pathogenesis, clinical picture and therapy]. / Psoriasis vulgaris bei Kindern und Jugendlichen. Pathogenese, Klinik und Therapie.

BACKGROUND: The prevalence of psoriasis vulgaris in childhood ranges from 0.12 % in infants to 1.2 % in adolescents. Psoriasis is a polygenic disease triggered by external factors and is influenced by comorbidities such as obesity, metabolic syndrome, chronic inflammatory bowel diseases and rheumatic diseases. CLINICAL FEATURES: Its clinical presentation is variable. Typical complications include erythroderma, disseminated pustulosis and arthropathy. THERAPY: Amongst a wide range of topical or systemic therapeutical options, individualized treatment is based on severity, site and extent of cutaneous involvement, age, potential side-effects and comorbidities.

[Demarcation of infection with Salmonella typhi and S. paratyphi against Salmonella enteritidis in children]. / Abgrenzung von Infektionen mit Salmonella typhi und S. paratyphi gegenüber Infektionen mit S. enteritidis bei Kindern.

BACKGROUND: Typhoid and paratyphoid infections (STP) are an important differential diagnosis for Salmonella enteritidis infections (NTS). The aim of this study is to carve out clinical differences between both infections. PATIENTS/METHODS: We retrospectively reviewed all patients admitted to our hospital between 2007 and 2011 with a diagnosis of STP infection. History, clinical course and laboratory findings were compared with age-matched patients suffering from NTS. RESULTS/DISCUSSION: All patients with an STP-infection showed had a positive travel history (as compared to 11.1% in the NTS group, p<0.01). Salmonella were mostly isolated from blood cultures (n=7/9) in the STP group as compared to 1/16 in the NTS group, where S. enteridis was mainly isolated from faecal cultures (p<0,01). The duration and height of fever as well as the time of hospitalization were significantly longer with STP infections than with NTS infections. Eosinopenia was observed both in STP-(100%) and NTS-infections (77.8%). CONCLUSION: A positive travel history is crucial to the identification of a STP infection, as well as an eosinopenia.

[Skin diseases associated with obesity in children]. / Dermatologische Probleme bei Kindern mit Adipositas.

While the impact of obesity on diabetes, cardiovascular disease and carcinoma development has been studied extensively, only little attention has been paid to its influence on the skin. Obesity alters the skin barrier, can induce skin manifestations, and worsens existing skin diseases like psoriasis. Cutaneous manifestations of obesity may be pseudoacanthosis nigricans, fibroma pendulans (skin tags, fibroepithelial polyps) and striae distensae. Obesity is also associated with hyperandrogenism in women and girls, promoting acne vulgaris, hirsutism, and androgenetic alopecia. In addition, there is a pathogenic association between obesity and psoriasis: the release of pro-inflammatory factors from fat tissue results in the worsening of psoriasis; an association between the severity of psoriasis and the body mass index has been shown. Obesity promotes skin infections like erysipelas and intertrigo.

[Hemangioma. New aspects of pathogenesis, differential diagnosis and therapy]. / Hämangiome. Neue Aspekte zu Pathogenese, Differenzialdiagnosen und Therapie.

With a prevalence of 10­12% infantile hemangiomas (IH) represent the most common skin tumor in infancy. They are characterized by a sequence of growth and, in 90%, spontaneous regression. Special manifestations of IH include hemangiomatosis, congenital hemangioma (RICH/NICH), reticular and segmental hemangiomas (PHACE, PELVIS/SACRAL syndrome). The latter represent a transition between vascular tumor and vascular malformation. Important differential diagnoses of IH include pyogenic granuloma (PG) and hemangioendothelioma (HAE) of which PG is a common, benign vascular tumor with a predilection for the facial region and a proneness to bleed whereas HAE is a slowly growing tumor exhibiting infiltrative growth; unlike IH it is associated with the Kasabach-Merritt syndrome of consumptive coagulopathy. The majority of IHs do not require therapy, however, therapy is absolutely indicated for ulcerating or obstructive IH where propranolol is considered the first-line therapy. Non-obstructive facial IH represents a relative indication for therapy. Cryotherapy is most suitable for IH with diameters below 1 cm and a depth < 4 mm.

Soluble immune receptor serum levels are associated with age, but not with clinical phenotype or disease severity in childhood atopic dermatitis.

BACKGROUND: Soluble immune receptors (SIRs) have been proposed as biomarkers in patients with atopic dermatitis (AD). However, their clinical applicability in affected children has rarely been studied. OBJECTIVE: To assess the diagnostic usefulness of serum SIRs in childhood AD by correlating the obtained receptor profiles with serological parameters and clinical features such as age, AD phenotype and disease severity. METHODS: We investigated 100 children with AD. The sCD14, sCD23, sCD25, sCD30, total IgE (tIgE) and eosinophilic cationic protein (ECP) were determined using sera of all children. The clinical phenotype was classified as extrinsic AD (ADe) or intrinsic AD (ADi) by the presence of allergen-specific IgE antibodies. RESULTS: A total of 55 male and 45 female children were recruited. The sCD23, sCD25 and sCD30 serum levels revealed significant age-dependency. At a mean SCORAD of 40 (range 8-98), none of the evaluated SIRs was correlated to disease severity. In all, 73% of patients suffered from ADe while 27% showed the ADi phenotype. None of the analysed SIRs differed significantly between ADe and ADi patients, while tIgE and ECP levels were elevated in the ADe subgroup. CONCLUSION: The current study provides evidence that sCD23, sCD25 and sCD30 serum levels are highly age-dependent. Serum concentrations of all investigated SIRs did not significantly correlate with disease severity in children with AD and were not differentially expressed in patients of different AD phenotypes. Therefore, we believe that the studied SIRs cannot be regarded as clinically useful biomarkers for the assessment of childhood AD.

[Dermatologic manifestations of infections in pediatric cancer patients]. / Kutane Infektionen in der pädiatrischen Onkologie.

Cutaneous infections in children and adolescents with cancer are frequently associated with a marked morbidity and mortality. Particularly neutropenic patients undergoing intensive cytostatic therapy or after allogenic stem cell transplantation are affected. In this group of patients, atypical manifestations of infectious skin disease are frequently observed which represent a diagnostic challenge. It is therefore essential for clinicians to be familiar with the clinical presentation, diagnostic methods and management of skin infections encountered in pediatric oncology.

[Special features of the skin in newborns and young infants]. / Besonderheiten der Haut des Neugeborenen und jungen Säuglings.

Detailed knowledge of the special features of neonatal and infant skin is a prerequisite for adequate skin care in this age group. Immediately postpartum, the newborn baby's skin assumes vital functions regarding water and electrolyte homeostasis and thermoregulation, as well as innate and adaptive host defense. Due to its functional and structural immaturity, premature skin requires special care. Mature neonates demonstrate benign, transient skin lesions that do not require specific therapy but have to be distinguished from serious and potentially life-threatening illnesses.

[Leprosy - An overview from a pediatric perspective]. / Lepra - Ein Uberblick aus pädiatrischer Sicht.

Worldwide tourism is an increasing industry. One result of this phenomenon is the occurrence of imported infectious diseases, as recently observed even in Germany. Leprosy ranks high among dreaded infectious diseases from tropical and subtropical countries. It remains a major health threat despite marked improvements in diagnosis and therapy. This was achieved by a better understanding of bacteriological and immunological mechanisms over the past decades, resulting in a decline of Leprosy's incidence.

[Naproxen-induced pseudoporphyria]. / Naproxeninduzierte Pseudoporphyrie.

A 12 year old boy developed scars at light-exposed areas following long-term therapy with naproxen for rheumatoid arthritis. Erythrocyte and urine porphyrin levels were not increased, and there was no evidence of increased photosensitivity. Pseudoporphyria is reported in 10-20% of those treated with naproxen for > 4 weeks. As compared to other nonsteroidal anti-inflammatory agents, the specific risk for naproxen is increased about 6fold. While the underlying abnormality has not been elucidated, formation of phototoxic metabolites in a subgroup of genetically predisposed individuals has been suggested as the most likely mechanism. Both dermatologists and rheumatologists should be aware of the risk of naproxen-induced pseudoporphyria and discontinue therapy early in order to avoid scar formation in light-exposed areas.

[Unilateral laterothoracic exanthema. Case report and review of the literature]. / Das unilaterale laterothorakale Exanthem. Kasuistik und Literaturübersicht.

Unilateral laterothoracic exanthem (ULE) developed in a 4-year-old-boy on maintenance therapy for acute lymphatic leukemia (ALL). This is the second reported case of an association between ULE and leukemia. The etiology of ULE is unknown. Endemic cases, frequently associated signs of upper respiratory infections, and the occurrence of ULE in immunocompromised hosts are suggestive of an infectious process.

[Skeletal anomalies in nail-patella syndrome. Case report and overview]. / Skelettanomalien beim Nagel-Patella-Syndrom. Fallbericht und Ubersicht.

The nail-patella syndrome (NPS), also known as hereditary onychoosteodysplasia (HOOD), is a hereditary disorder with an autosomal dominant mode of inheritance involving nails, bones and other tissues. It is characterized by onchodysplasia of the finger nails (most prominent on the thumb and index finger) and V-shaped lunulae. Extraosseous manifestations include ocular (glaucoma, microcornea) and renal involvement (proteinuria, nephrotic syndrome). A variety of skeletal anomalies can be observed. We report a 59 year old male with NPS. In addition to dysplastic patellae and elbow joints and the pathognomonic posterior iliacal horns, he had involvement of humerus, radius, ulna. and finger bones, leading to early and painful degenerative changes. Furthermore, microproteinuria was noted. Early diagnosis of NPS is important to prevent early secondary arthrosis and severe renal damage.

[Treatment of secondary infections in symptomatic HIV-infections in childhood]. / Behandlung von Sekundärinfektionen bei symptomatischer HIV-Infektion im Kindesalter.

Treatment of secondary infections in HIV infected children represents a problem of increasing importance in several children's hospitals. As it is unlikely that the AIDS-problem will be solved by itself within the next years it seems reasonable to summarize our current knowledge about opportunistic infections in order to establish standards for therapy. We will mainly focus on microorganisms endemic in Germany.