Atypical pediatric presentation of alopecic and aseptic nodules of the scalp with features of dissecting cellulitis.

Alopecic and aseptic nodules of the scalp (AANS) and dissecting cellulitis of the scalp (DCS) are rare, closely related conditions of young men that exclusively affect the hair-bearing scalp. We describe a 9-year-old boy who presented with a 6-year history of chronically relapsing, sterile, partially scarring nodules of the scalp and facial skin. Histopathology revealed mixed inflammatory infiltrates consisting of neutrophils, macrophages, lymphocytes, and plasma cells in the deep dermis, consistent with the morphological pattern of suppurative, partly granulomatous dermatitis. The present atypical case is characterized by prepubertal onset and facial involvement which, to our knowledge, has not yet been described before, may be included in the spectrum of "typical" AANS and "typical" DCS.

[Flame figures-eosinophils setting the skin on fire]. / Flammenfiguren ­ Eosinophile, die die Haut in Brand setzen.

A 50-year-old female farmer was initially diagnosed with generalized granuloma annulare and treated with local steroids and ultraviolet (UV) light therapy for 10 years, albeit without success. A histopathological examination in our clinic changed the diagnosis to Wells' syndrome, based on the typical findings of eosinophilic cellulitis together with flame figures. A systemic approach with pulse steroid therapy resulted in complete remission of pruritus and skin manifestations. This case demonstrates successful treatment of a patient with eosinophilic cellulitis.

Lupus erythematosus tumidus: clinical perspectives.

Lupus erythematosus tumidus (LET) is an uncommon and photosensitive inflammatory skin disorder which is characterised by erythematous urticarial plaques. In the last 20 years, extensive research on clinical and histological aspects of the disease have led to a better characterization of this nosological entity and to differentiate it from other similar or related diseases. Today, LET is considered as a separate subtype of cutaneous lupus erythematosus (CLE) with a benign, intermittent clinical course (intermittent CLE, ICLE) and only rarely associated with systemic lupus erythematosus (SLE).

Upregulation of glutathione peroxidase offsets stretch-induced proatherogenic gene expression in human endothelial cells.

OBJECTIVE: Localization of atherosclerotic plaques typically correlates with areas of biomechanical strain where shear stress is decreased while stretch, thought to promote atherogenesis through enhanced oxidative stress, is increased. METHODS AND RESULTS: In human cultured endothelial cells, nitric oxide synthase expression was exclusively shear stress-dependent whereas expression of glutathione peroxidase-1 (GPx-1), but not that of Cu(2+)/Zn(2+)-superoxide dismutase or Mn(2+)-superoxide dismutase, was upregulated solely in response to cyclic stretch. GPx-1 expression was also enhanced in isolated mouse arteries perfused at high pressure. Combined pharmacological and decoy oligodeoxynucleotide blockade revealed that activation of p38 MAP kinase followed by nuclear translocation of CCAAT/enhancer binding protein plays a pivotal role in stretch-induced GPx-1 expression in human endothelial cells. Antisense oligodeoxynucleotide knockdown of GPx-1 reinforced both their capacity to generate hydrogen peroxide and the transient stretch-induced expression of CD40, monocyte chemoatractant protein-1, and vascular cell adhesion molecule-1. Consequently, THP-1 monocyte adhesion to the GPx-1-depleted cells was augmented. CONCLUSIONS: Stretch-induced proatherosclerotic gene expression in human endothelial cells seems to be hydrogen peroxide-mediated. The concomitant rise in GPx-1 expression, but not that of other antioxidant enzymes, may comprise an adaptive mechanism through which the cells maintain their antiatherosclerotic properties in spite of a decreased bioavailability of nitric oxide.

Results of a survey of German dermatologists on the therapeutic approaches to pemphigus and bullous pemphigoid.

BACKGROUND: The lack of controlled trials on the treatment of pemphigus and pemphigoid diseases limits an evidence-based therapy of these disorders.The aim of this survey was to assess the current treatment standards at German dermatological hospitals and the need for future therapeutic trials. METHODS: A two-page questionnaire was sent to 42 German academic and non-academic dermatological hospitals and evaluated at the Department of Dermatology, University Medical Center, Freiburg. RESULTS: The response rate was 76%. Topical clobetasol propionate treatment was regarded as first line therapy of bullous pemphigoid in 27% of the hospitals. More than 50% used systemic corticosteroids at an initial dose of < or = 1 mg/kg prednisolone-equivalent in pemphigus and pemphigoid. Azathioprine is used as first line adjuvant in pemphigoid and pemphigus treatment in 69% and 81% of the hospitals, respectively. Dapsone and mycophenolate mofetil represent alternative options, as well as cyclophosphamide-dexamethasone-pulse therapy in pemphigus. Immunosuppressive treatment of pemphigoid and pemphigus is terminated by 58% and 46% of the hospitals 1-3 months after clinical remission, respectively, but the given time points appeared to be variable. CONCLUSIONS: The high response rate to this survey demonstrates the interest in this topic. The variability of the answers regarding initial doses of corticosteroids and time points for termination of therapy indicates the need for the establishment of guidelines and for controlled therapeutic trials.

Trigeminal trophic syndrome with extensive ulceration following herpes zoster.

The trigeminal trophic syndrome is a rare complication following central or peripheral injury of the trigeminal nerve typically characterized by unilateral distribution of anaesthesia, paraesthesia and ulceration. In one third of cases it is preceded by an iatrogenic damage of the trigeminal nerve, in another third by a history of stroke. Other causes include head trauma, intracranial tumours, herpes virus infection, degenerative diseases of the CNS and idiopathic. Little is known about the pathogenesis. Contribution of neurotrophic factors and an altered sympathetic activity is assumed but a pivotal role of self-mutilation is generally accepted. We report a case of a patient who developed a strictly unilateral crescent ulcer of the ala nasi in addition to an extensive ulceration of the forehead and scalp following herpes zoster ophthalmicus.

Recommendations for the use of rituximab (anti-CD20 antibody) in the treatment of autoimmune bullous skin diseases.

Autoimmune bullous skin disorders are induced by autoantibodies against distinct adhesion complexes of the epidermal and dermal-epidermal junction. Since most of these disorders are characterized by a severe, potentially lethal course,they require long-term immunosuppressive treatment to reduce the de novo synthesis of pathogenic autoantibodies by B lymphocytes. Rituximab, a chimeric monoclonal antibody against CD20 on B lymphocytes, has shown promise in several case reports or cohort studies in the treatment of paraneo-plastic pemphigus,refractory cases of pemphigus vulgaris and foliaceus and in other autoimmune bullous disorders. Treatment with rituximab leads to depletion of pathogenic B-cells which may last up to 12 months resulting in a reduction of plasma cells secreting pathogenic autoantibodies. Rituximab is usually administered in an adjuvant setting at a dose of 375 mg/m(2) i.v.in weekly intervals for four consecutive weeks in addition to the standard immunosuppressive treatment. The present consensus statement of German-speaking dermatologists, rheumatologists and oncologists summarizes and evaluates the current evidence for the use and mode of application of rituximab in autoimmune bullous skin disorders.

Recommendations for the use of immunoapheresis in the treatment of autoimmune bullous diseases.

Despite the use of high-dose systemic corticosteroids in combination with other immunosuppressants, in some patients with autoimmune bullous diseases only insufficient improvement is achieved. In these cases and in acute severe disease, adjuvant immunoapheresis has been increasingly used. A consensus meeting was held in mid-2005 in Hamburg, aiming at developing guidelines for the use of immunoapheresis in the treatment of autoimmune bullous diseases. This paper summarizes the experts' recommendations.