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Background and aims Vulval Crohn's disease (VCD) is a rare extra-intestinal cutaneous manifestation of Crohn's disease. VCD is often unrecognized and misdiagnosed and can be difficult to treat. The aim of the study was to describe the clinical presentation, associated features, and response to treatment modalities in patients with VCD. Methods A prospective review of patients with VCD who were referred to our specialist dermatology clinic from 2003 to 2017 was performed. Data on age at diagnosis, presenting signs, associated features, and response to treatment were collected. Results Thirty-one patients with a median age of 40 years (range 13-66 years) were identified. VCD manifested most frequently with vulval edema (77%), ulceration (35%), and fissures (39%). Eighty-one percent had active gastrointestinal disease. Oral involvement and pyoderma gangrenosum were present in 13% and 19%, respectively. Topical therapy was effective in mild VCD. Tumor necrosis factor-α inhibitors were the most effective second-line therapy, with 53% achieving complete clinical remission. Additionally, we have found compression garments and shorts to be useful adjuncts in treating vulval edema. Conclusions Vulval Crohn's disease is a diagnostic and therapeutic challenge. We recommend consideration of tumor necrosis factor-α inhibitors at an early stage.

Vulval involvement in Crohn’s disease (CD) is rare, particularly in children. The clinical features include erythema, edema, ulceration, and labial skin tags. The authors present two cases of children with vulval CD. In both cases, marked labial edema was the presenting feature. In one patient the immunomodulator tacrolimus ointment 0.03% was used with success. In the second patient control was achieved with intralesional triamcinolone in combination with systemic metronidazole.

Pyoderma gangrenosum is a rare condition with few cases of eyelid involvement reported in the literature. Pathergy is a well-recognised phenomenon that can trigger this condition. Pyoderma gangrenosum should be considered in cases of progressive cribriform cicatrisation where there is a history of antecedent trauma. Surgical management of a resultant ectropion may be challenging as a result of aggressive scarring and the risk of provoking a recurrence. We report a case of pyoderma gangrenosum causing a cicatricial ectropion, and discuss the underlying aetiology of iatrogenic incitement, and its implications for surgical management.

A 75-year-old man with ulcerative colitis (UC) and diet controlled diabetes mellitus presented with a 3-week history of slightly itchy, red plaques on both lower limbs ascending gradually to cover the trunk and arms. One week later, he developed a flare up of his UC. Routine blood tests showed modest drop in haemoglobin (122 g/L) and C reactive protein (85 mg/L). Serology was remarkable for high antiproteinase 3 (c-ANCA). Serum electrophoresis showed a mildly positive paraprotein band (γ region). Stool culture was negative. Urine analysis showed proteinuria. Skin biopsy showed features of urticarial vasculitis (UV). He underwent a flexible sigmoidoscopy after the flare up showed mildly active UC. The patient was given hydrocortisone for 7 days and then prednisolone. Both rash and UC subsided. Electrophoresis was repeated 4 weeks later showing normal pattern. Prednisolone has been gradually reduced. Although rare, UV can be considered as one of the skin manifestations of UC.

Introduction Inflammatory bowel disease has been associated with a number of cutaneous and systemic neutrophilic disorders, including pyoderma gangrenosum. In 1972, the term chronic multi-focal recurrent osteomyelitis was given to a sterile neutrophilic condition which has been associated with inflammatory bowel disease. Case Report We report a case of a 23-year-old man with long-standing severe Crohn’s disease which necessitated subtotal colectomy. He subsequently developed progressive, intermittent back pain that were limiting his functional movement. Numerous investigations to identify what initially was thought to be an infectious process failed to lead to the diagnosis. Biopsy of the spine identified a sterile neutrophilic infiltrate and the diagnosis of chronic recurrent multi-focal osteomyelitis was made which was successfully treated with immunosuppressive drugs. Conclusion Inflammatory bowel disease can present with cutaneous and systemic neutrophilic disorders and this association is becoming increasingly recognized by gastroenterologists and dermatologists. Chronic recurrent multi-focal osteomyelitis is a sterile neutrophilic disorder which can present with bone pain and responds to immunosuppressive therapy. Electronic supplementary material The online version of this article (doi:10.1007/s13555-014-0044-3) contains supplementary material, which is available to authorized users.

Ciclosporin is widely used in a number of inflammatory disorders and has the potential for drug interactions. We report here a case of acute kidney injury due to the interaction of ciclosporin with methyl-1-testosterone. This has not been previously reported and it is relevant as methyl-1-testosterone can be purchased online. Physicians should be aware of any over the counter or online purchased “supplements” and consider possible drug interactions. Electronic supplementary material The online version of this article (doi:10.1007/s13555-013-0038-6) contains supplementary material, which is available to authorized users.

Bullous pemphigoid (BP) is a blistering disorder of the skin and mucosa that may coexist with inflammatory bowel disease (IBD). The authors' experiences with peristomal and generalized BP in five patients (three with ulcerative colitis [UC] post colostomy surgery and two with Crohn's disease [CD] post ileostomy surgery, time since surgery 5 to 20 years) is described. The patients presented with peristomal blisters and erosions, subsequently confirmed as BP by skin biopsy. Treatments for the skin disease included potent alcohol-based topical corticosteroids, oral tetracyclines, and oral corticosteroids. In three patients (two with UC, one with CD), the initially localized peristomal disease later became generalized across the skin; these patients were more likely to require systemic immunosuppressive therapy. Because an involvement of plectin, a cytoskeletal protein that attaches skin and mucosal cells to their extracellular matrix, in IBD has been shown, it is possible that this protein forms the missing link between IBD and BP via epitope spreading. The inflammation of IBD exposes plectin, stimulating a secondary immune response that may, in susceptible individuals, crossreact with the skin, provoking BP. Further research into this area could enable clinical testing for plectin auto-antibodies in patient sera, possibly preempting the development of BP and expediting the initiation of early effective treatment.

We describe two cases of PHACES syndrome that illustrate the importance of recognizing this rare syndrome. As children with this syndrome can present to general pediatricians, dermatologists, pediatric cardiologists, ophthalmologists, and neurologists, it is important that all are aware of the spectrum of associated abnormalities.

ejd.2011.1303 Auteur(s) : Firas Al-NIAIMI1 firas55@hotmail.com, Calum C LYON2 1 Department of dermatology, Salford Royal Hospital Trust, Stott lane, Manchester, UK 2 Department of dermatology, York Hospital, York, UK Acute respiratory distress syndrome (ARDS) is the most extreme form of acute lung injury which can lead to respiratory failure [1]. We report a case of severe ARDS secondary to erythrodermic psoriasis. A 57-year-old man with a history of chronic plaque psoriasis was admitted with erythroderma. [...]

Auteur(s) : Firas Al-NIAIMI1 firas55@hotmail.com, Calum LYON2 1 Department of dermatology Salford Royal Foundation Trust, Stott lane, M50 3TU Manchester, UK 2 Department of Dermatology, York Hospital, York, UK Drugs are known to cause a variety of cutaneous eruptions, including photosensitivity [1]. We report a case of severe photosensitivity resulting in aggravation of existent eczema, due to mesalazine. A marked improvement was noted after subsequent withdrawal of the drug. A 56-year-old man with [...]

Background Stomal leaks can be associated with significant social, psychological and physical morbidity for ostomy patients. Poor fitting of the stoma appliance due to irregularities of skin contours is one cause of stoma leaks which commonly result in secondary irritant dermatitis prompting presentation to a dermatologist. In addition to skin-directed topical therapy and review of stoma appliances, correction of contour defects with intradermal injections of filler materials is one possible treatment to improve adhesion and reduce leaks. Cases We report eight cases of ostomy patients, who presented with stoma leaks and associated dermatitis, who were treated with intradermal injections of the porcine collagen (Permacol™) or subcutaneous injections of polyacrylamide hydrogel (Aquamid Reconstruction™) for correction of skin contour defects. Resolution or improvement of symptoms was achieved for five patients, and no complications were noted as a result of treatment. Conclusions This report represents the largest series of ostomy patients treated for correction of peristomal skin contour defects with injection therapy. Treatment was well tolerated and performed in the outpatient setting under local anesthetic. Attempted correction of peristomal skin contour defects using injection of filler materials represents a potential alternative to surgical intervention and can result in significant benefits for the patient. Electronic supplementary material The online version of this article (doi:10.1007/s13555-014-0058-x) contains supplementary material, which is available to authorized users.

The identification of robust endotypes—disease subgroups of clinical relevance—is fundamental to stratified medicine. We hypothesized that HLA-C∗06:02 status, the major genetic determinant of psoriasis, defines a psoriasis endotype of clinical relevance. Using two United Kingdom–based cross-sectional datasets—an observational severe-psoriasis study (Biomarkers of Systemic Treatment Outcomes in Psoriasis; n = 3,767) and a large population-based bioresource (UK Biobank, including n = 5,519 individuals with psoriasis)—we compared demographic, environmental, and clinical variables of interest in HLA-C∗06:02–positive (one or two copies of the HLA-C∗06:02 allele) with those in HLA-C∗06:02‒negative (no copies) individuals of European ancestry. We used multivariable regression analyses to account for mediation effects established a priori. We confirm previous observations that HLA-C∗06:02–positive status is associated with earlier age of psoriasis onset and extend findings to reveal an association with disease expressivity in females (Biomarkers of Systemic Treatment Outcomes in Psoriasis: P = 2.7 × 10 –14, UK Biobank: P = 1.0 × 10 –8). We also show HLA-C∗06:02–negative status to be associated with characteristic clinical features (large plaque disease, OR for HLA-C∗06:02 = 0.73, P = 7.4 × 10 –4; nail involvement, OR = 0.70, P = 2.4 × 10 –6); higher central adiposity (Biomarkers of Systemic Treatment Outcomes in Psoriasis: waist circumference difference of 2.0 cm, P = 8.4 × 10 –4; UK Biobank: waist circumference difference of 1.4 cm, P = 1.5 × 10 –4), especially in women; and a higher prevalence of other cardiometabolic comorbidities. These findings extend the clinical phenotype delineated by HLA-C∗06:02 and highlight its potential as an important biomarker to consider in future multimarker stratified medicine approaches.