In about one third of patients, psoriasis starts in the first or second decade of life. In the beginning, involvement is often atypical or mild, and a confident diagnosis may be difficult to establish. Plaque psoriasis is the most frequent type, also in children, but lesions are often smaller, thinner, and less scaly than in adults. Treatment can be a challenge because many therapeutic options have drawbacks or are not approved in childhood. Because psoriasis is a life-long disease, affected children and their parents need special support and guidance. We herein focus on the peculiarities of clinical presentations and of the management of psoriatic children and adolescents.

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Our data demonstrate that hyperproliferation and abnormal differentiation of psoriatic skin is associated with a massive upregulation and secretion of S100A8 and S100A9, suggesting not only a prominent role of these molecules during intracellular calcium-dependent signalling but also implying distinct extracellular functions.

Tumour necrosis factor (TNF)-alpha is thought to play a major role in the pathophysiology of psoriasis. Good clinical responses of psoriasis to anti-TNF-alpha-based therapies have recently been demonstrated. We studied the effect of infliximab, a monoclonal antibody against TNF-alpha, on chemokine expression in pustular psoriasis. A 61-year-old man with a 2-year history of severe pustular psoriasis of von Zumbusch type who did not respond to conventional therapies responded rapidly to treatment with infliximab. The clinical response was reflected by an immediate and effective reduction of the neutrophil-attractant chemokines interleukin (IL)-8 and growth-related oncogene (Gro)-alpha as well as of monocyte chemoattractant protein (MCP)-1, as determined by mRNA in situ hybridization of lesional skin. No expression before or after treatment was seen for monokine induced by interferon (IFN)-gamma (MIG) and IFN-inducible protein (IP)-10. Thus, in pustular psoriasis the chemokine expression pattern is dominated by neutrophil-attractant chemokines and MCP-1 while, in contrast to plaque psoriasis, IFN-gamma-inducible lymphocyte-attractant chemokines such as IP-10 and MIG are not abundant. We conclude that anti-TNF-alpha treatment with infliximab is an effective therapy in severe pustular psoriasis which is reflected by downregulation of disease-promoting chemokines such as IL-8, Gro-alpha and MCP-1.

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Autoantibodies against the 3 desmocollin (Dsc; Dsc1-Dsc3) isoforms have been described in different pemphigus variants. Here, we developed state-of-the-art detection systems for serum anti-Dsc1, Dsc2and Dsc1 IgG and IgA. These assays were applied in 5 different cohorts including pemphigus vulgaris (PV) patients with compatible direct immunofluorescence (IF) microscopy but no reactivity against desmogleins 1 and 3 (n = 24) and sera from patients with autoimmune blistering diseases with positive direct IF microscopy taken at the time of diagnosis (n = 749). We found that detection of anti-Dsc serum reactivity is not helpful in the routine diagnosis of PV, pemphigus foliaceus and paraneoplastic pemphigus but may be valuable in pemphigus vegetans. | BACKGROUNDDesmocollins (Dsc) and desmogleins (Dsg) belong to the Ca 2+ -dependent cadherin adhesion protein family. They are part of the desmosomes and provide proper cohesion between neighbouring cells of various tissues including skin and surface-close epithelia.[1] The 4 Dsg (Dsg1-Dsg4) and 3 Dsc (Dsc1-Dsc3) isoforms share a similar architecture with approximately 30% sequence identity ( Figure 1A). By both homo-and heterophilic transinteraction of the outmost extracellular N-termini and binding to proteins of the desmosomal plaque, Dsc and Dsg mediate connection between the intermediate filaments of neighbouring cells.[1]In pemphigus vulgaris (PV), the major target antigen is Dsg3. [2,3] In patients with the mucocutaneous phenotype of PV, autoantibodies against Dsg1 in addition to Dsg3 reactivity are found. [2,3] In contrast, autoantibody reactivity in paraneoplastic pemphigus (PNP) is more diverse. In addition to antibodies against Dsg3, reactivity against proteins of the plakin family comprising desmoplakins I and II, periplakin, envoplakin, plectin, and BP230 as well as Dsg1, and, more recently, α2-macroglobulin-like 1 and epiplakin has been described. [4,5] Autoantibodies against all 3 Dsc isoforms have been reported in various pemphigus subtypes including PV, pemphigus foliaceus, pemphigus vegetans, pemphigus herpetiformis, IgA pemphigus and PNP. [6][7][8][9] A pathogenic relevance of anti-Dsc antibodies has been suggested by different in vitro models based on the incubation of cultured human keratinocytes and human skin with anti-Dsc3 IgG. These data are supported by the finding of intra-epidermal splitting resulting in erosions and hair loss in mice with Dsc3-null skin. | QUESTIONS ADDRESSEDWhile Dsc1 has been identified as major target antigen in the subcorneal pustular dermatosis type of IgA pemphigus, [18] the diagnostic value of detecting serum anti-Dsc antibodies in PV is still unclear. | EXPERIMENTAL DESIGNTo address this open question, we cloned and expressed the ectodo- This strategy has recently led to highly sensitive and specific test systems for serum autoantibodies against Dsg1, Dsg3, BP230 and the NC1 domain of type VII collagen.To evaluate the diagnostic relevance of detecting anti-Dsc antibodies in pemphigus sera, 5 distinct cohorts we...

Pemphigus vegetans (PVeg) is a rare variant of pemphigus vulgaris characterized by pustules and/or papillomatous vegetations, preferentially affecting intertriginous and periorificial areas. Exceptional manifestations may be misdiagnosed resulting in delayed diagnosis and treatment. Diagnosis is confirmed by immunofluorescence and detection of anti-desmoglein (Dsg) 3 and/or anti-Dsg1 antibodies. We herein report an unusual manifestation of PVeg. At the time of first presentation, lesions were restricted to the right ring finger’s tip. Although mucous membranes were initially not affected, high levels of anti-Dsg3 antibodies were detected while anti-Dsg1 and anti-desmocollin (Dsc) 1, 2, and 3 antibodies were absent. To compare our immunological findings with previous reports, all accessible Anglophone literature published since December 1988 was evaluated. We identified 52 patients suffering from PVeg, 7 of these showed anti-Dsg3 antibodies without any mucous membrane involvement. Notably, the detection of anti-Dsg1 and anti-Dsg3 antibodies does not necessarily correlate with the involvement of skin and/or mucous membranes. This might be due to more specific and complex antibody constellations in nonclassical or atypical pemphigus.

Analysis of the autoantibody profile is not only of diagnostic relevance but may also be helpful in predicting the course of the disease.