Objective: This paper describes two different clinical presentations of diffuse cutaneous mastocytosis (DCM), based on the largest series published to date. As far as we are aware, these two variants of clinical presentations have not yet been reported. Design: We undertook a case controlled analysis of 8 children with DCM. Results of laboratory testing including mast cell mediator levels, and clinical symptoms on presentation and during follow-up were analyzed. Results: The levels of relevant mast cell mediators were initially high in all cases but declined sharply later on. There was a reduction of 20% in 2 of the 7 cases, whereas there was a reduction of 80% in the remaining 5. No reduction occurred in 1 case. Clinical improvement followed the same pattern. Conclusions: DCM is a rare variant of cutaneous childhood onset mastocytosis. Various forms show the same or overlapping features at various times. It appears to follow a course similar to that in other types of childhood onset mastocytosis, taking into account the decreased symptoms and the levels of mast cell mediators during follow-up. Obtaining a bone marrow biopsy should be considered only in those cases where there is no improvement or even worsening of signs or symptoms and persistent elevated levels of mast cell mediators.

Accessible online at: www.karger.com/drm James Ferguson, Dundee, United Kingdom Gerardo Ferrara, Benevento, Italy Joachim W. Fluhr, Jena, Germany Ola Forslund, Malmö, Sweden Thilo Gambichler, Bochum, Germany Marie-Jeanne P. Gerritsen, Nijmegen, The Netherlands Paolo Gisondi, Verona, Italy Aditya K. Gupta, London, Canada Antoine Hadengue, Genève, Switzerland M. Haedersdal, Copenhagen, Denmark Henning Hamm, Würzburg, Germany C.W. Hanke, Carmel, USA Rudolf Happle, Marburg, Germany Michael Hertl, Marburg, Germany Daniel Hohl, Lausanne, Switzerland Thomas Hunziker, Bern, Switzerland Gregor B.E. Jemec, Roskilde, Denmark Pascal Joly, Rouen, France Dennis Jullien, Lyon, France Jean Kanitakis, Lyon, France Behrooz Kasraee, Geneve, Switzerland Harald Kitller, Vienna, Austria C. Kowalewski, Warsaw, Poland Roger Kuffer, Paris, France Christine Labrèze, Bordeaux, France Jean-Marie Lachapelle, Brussels, Belgium Emmanuel Laffitte, Geneva, Switzerland Michael Landthaler, Regensburg, Germany Fréderique-Anne Le Gal, Genève, Switzerland Christine Léauté-Labrèze, Bordeaux, France Celeste Lebbe, Paris, France Dan Lipsker, Strasbourg, France Tommaso Lombardi, Geneva, Switzerland A.W. Lucky, Cincinnati, USA Joseph Malvehy, Barcelona, Spain Isabelle Masouyé, Genève, Switzerland Bruno Matard, Croissy Sur Seine, France Laurent Misery, Brest, France Silvia Moretti, Firenze, Italy Sei-ichiro Motegi, Maebashi, Japan U. Mrowietz, Kiel, Germany Luigi Naldi, Bergamo, Italy Bernard Noel, Lausanne, Switzerland Alberto Orfao, Salamanca, Spain M.C. Pasch, Nijmegen, The Netherlands Carle Paul, Toulouse, France Gérald E. Piérard, Liège, Belgium Paula Aguilera, Barcelona, Spain Giuseppe Argenziano, Naples, Italy B.S. Atiyeh, Beirut, Lebanon Martine Bagot, Créteil, France P. Bahadoran, Nice, France Sebastien Barbarot, Nantes, France Jens Malte Baron, Aachen, Germany Sylvie Bastuji-Garin, Créteil, France Christiane C. Bayerl, Wiesbaden, Germany J.C. Beani, Grenoble, France Carola Berking, Munich, Germany Philippe Bernard, Reims, France Maria Grazia Bernengo, Torino, Italy Paul L. Bigliardi, Lausanne, Switzerland Andreas J. Bircher, Basel, Switzerland Luca Borradori, Bern, Switzerland J.N. Bouwes Bavink, Leiden, The Netherlands Ralph-Peter Braun, Zurich, Switzerland Irwin M. Braverman, New Haven, USA Helmut Breuninger, Tübingen, Germany Magnus Bruze, Malmö, Sweden L. Bugatti, Jesi, Italy J.C. Bystryn, New York, USA Eric Caumes, Paris, France Lorenzo Cerroni, Graz, Austria Carlo Chizzolini, Genève, Switzerland Olivier Chosidow, Paris, France Arnon Cohen, Omer, Iceland Arnon D. Cohen, Hod Hasharon, Israel O. Cottencin, Lille, France Vicente Crespo, Malaga, Spain Bernard J. Cribier, Strasbourg, France Ophelia Dadzie, London, United Kingdom Esteban Dauden, Madrid, Spain Vincenzo de Giorgi, Firenze, Italy Pierre A. de Viragh, Zürich, Switzerland Pascal Del Giudice, Fréjus, France Olivier Dereure, Montpellier, France Magdalena A. Dohil, San Diego, USA Nicolas Dupin, Paris, France Alain Dupuy, Paris, France Madeleine Duvic, Houston, USA James T. Elder, Ann Arbor, USA Rüdiger Eming, Marburg, Germany Sabine Eming, Köln, Germany Odile Enjolras, Paris, France Ervin H. Epstein, Oakland, USA Gabriella Fabbrocini, Naples, Italy M.C.W. Feltkamp, Leiden, The Netherlands The Editor extends his gratitude and appreciation to the following reviewers whose comments and criticisms ensure the quality of the papers published in this journal.

Mast cells have a prominent, although not completely understood, participation in both immunity and disease. Well-known disorders in which mast cells play a prominent and undisputed role are mastocytosis and urticaria. Mastocytosis has been classified more clearly, based on international consensus meetings and reports of the European Network on Mastocytosis (Consensus Mastocytosis 2007) in the last 5 years. Urticaria is elicited by a great diversity of factors and entities. Treatment points at avoidance, elimination or treatment of the eliciting stimulus or cause, inhibition of mast cell mediator release or therapy of target tissues of mast cell mediators (Consensus Urticaria 2006). Chronic urticaria is believed to have an underlying autoimmune pathogenesis in almost 50% of cases. In a disease such as atopic dermatitis, the role of mast cells is probably underexposed, but is beyond the main scope of this review. In this review; we stress the important differences between children and adults with these disorders. Recent advances in mast cell-mediated skin diseases, such as mastocytosis and urticaria, and differences based on age are subject of discussion, with focus on the literature published in the last 5 years.

Mastocytosis is characterized by an increased number of mast cells with an abnormal growth and accumulation in one or more organs. In most children mastocytosis is limited to the skin (cutaneous mastocytosis) and often transient as compared with that in adults in whom mastocytosis is usually progressive and systemic. Generally, we recognize three more common forms of cutaneous mastocytosis: maculopapulous mastocytosis (formerly urticaria pigmentosa), mastocytoma of skin, and diffuse cutaneous mastocytosis. Childhood mastocytosis can further be divided into cutaneous mastocytosis (nonpersisting and persisting) and systemic mastocytosis (extremely rare). An approach to management using a set protocol is described in table form. In most cases of mastocytosis, only yearly checkups are necessary and no treatment is required; preventive recommendations are warranted in those individuals with systemic disease and constitutional symptoms. Symptomatic therapy is advised in only a minority of cases. This article is meant as a guideline for physicians involved in the care of children with mastocytosis and their parents.

Background: Mastocytosis is a disorder that can be subdivided into two forms: cutaneous and systemic. Patients with cutaneous mastocytosis only may suffer from cosmetic problems. Topical steroid application has been shown to be effective in cases of limited skin lesions. Methods: A case-controlled pilot study was conducted during a 6-weeks treatment using diluted 25% fluticasone propionate 0.05% cream under wet-wrap occlusion in 5 adults and 6 children. Improvement was measured up to the 24th week after treatment using the SCORMA Index. Results: The results of this pilot study showed a partial but clear cosmetic improvement in 9 of the 11 patients. The mean SCORMA Index decreased after treatment from 38 to 26. Conclusion: 25% dilution of fluticasone propionate 0.05% cream under wet-wrap occlusion is an alternative treatment modality for alleviating the symptoms of cutaneous mastocytosis, but the improvement may be moderate and fall short of the patient’s expectations.