[EFFECTIVE TREATMENT FOR BULLOUS PEMPHIGOID WITH OMALIZUMAB].

INTRODUCTION: Bullous pemphigoid is a common autoimmune blistering disorder, characterized by sub-epidermal bullae formation, that tends to affect older patients. We report on a 78 -year-old male patient suffering from bullous pemphigoid, whose disease persisted despite treatment with potent topical corticosteroids, systemic tetracyclines, prednisone and azathioprine. Recently, omalizumab was reported to be effective in several patients with resistant bullous pemphigoid. Omalizumab is a monoclonal antibody against IgE, approved for the treatment of asthma and chronic urticaria and known for its excellent safety profile. The patient was treated accordingly with omalizumab for his bullous pemphigoid with dramatic and rapid regression of his disease.

Cutaneous Chronic Graft Versus Host Disease Following Allogeneic Haematopoietic Stem Cell Transplantation in Children: A Retrospective Study.

Chronic graft versus host disease (cGVHD) is a complication of allogeneic haematopoietic stem cell transplantation (HSCT). The aim of this study was to clinically characterize childhood cutaneous cGVHD. A retrospective study of children treated with HSCT at 2 tertiary medical centres in Israel between 2011 and 2014 was performed. A total of 112 children were included. Cutaneous cGVHD developed in 18% of subjects. Risk factors were older age, HSCT from peripheral blood and acute lymphoblastic leukaemia. The eruption was lichenoid in 90% of subjects, of whom one-third progressed to sclerosis. Topical treatments were usually sufficient in localized disease. Widespread eruption necessitated phototherapy, extracorporeal photopheresis and/or systemic immunosuppressants. Patients presenting with palmoplantar keratoderma, developed sclerosis. To the best of our knowledge, this is the first study describing childhood cutaneous cGVHD. Lichenoid eruption is the most common cutaneous pattern of cGVHD in children. Sclerotic changes may be associated with prior keratoderma. cGVHD poses a therapeutic challenge and better treatments should be sought.

Sclerotic-Type Cutaneous Chronic Graft-Versus-Host Disease Exhibits Activation of T Helper 1 and OX40 Cytokines.

Sclerotic-type cutaneous chronic graft-versus-host disease is a severe complication of allogeneic hematopoietic stem cell transplantation, with profound morbidity. A dearth of effective, targeted treatment options necessitates further investigation into the molecular mechanisms underlying this T-cell-mediated disease. In this study, we compared the transcriptome in skin biopsies from pediatric and young adult (aged <25 years) patients with sclerotic-type cutaneous chronic graft-versus-host disease (n = 7) with that in demographically matched healthy controls (n = 8) and patients with atopic dermatitis (n = 10) using RNA sequencing with RT-PCR and immunohistochemistry validation. Differential expression was defined as fold change > 1.5 and false discovery rate < 0.05. Sclerotic-type cutaneous chronic graft-versus-host disease exhibited strong and significant T helper (Th)1 skewing through key related cytokines and chemokines (CXCL9/10/11, IFNG/IFN-γ, STAT1/signal transducer and activator of transcription 1). Several markers related to the TSLP-OX40 axis were significantly upregulated relative to those in both controls and lesional atopic dermatitis, including TNFSF4/OX40L, TSLP, and IL33, as well as fibroinflammatory signatures characterized in a prior study in systemic sclerosis. Gene set variation analysis reflected marker-level findings, showing the greatest enrichment of the Th1 and fibroinflammatory pathways, with no global activation identified in Th2 or Th17/Th22. Cell-type deconvolution revealed a significant representation of macrophages and vascular endothelial cells. Sclerotic-type cutaneous chronic graft-versus-host disease in young patients may therefore be characterized by strong Th1-related upregulation with a unique TSLP-OX40 signature, suggesting new therapeutic avenues for this devastating disease.

Pityriasis rosea in a woman and her husband - case report and review of the literature.

Pityriasis rosea is an acute, self-limited papulosquamous dermatosis of the trunk and extremities. Many atypical forms of the disease have been reported in the literature [Ahmed et al.: Clin Exp Dermatol 2000;25:624-626; Imamura et al.: Dermatologica 1985;171:474-477]. It is rare to find pityriasis rosea in multiple family members (within a household) at the same time. There have been only 4 reported cases where a couple has contracted pityriasis rosea simultaneously [Miller et al.: Arch Derm Syphilol 1941;44:66-68; Niles et al.: Arch Derm Syphilol 1940;41:264].