Calum Lyon

Severe ulcerative panniculitis caused by alpha 1-antitrypsin deficiency: remission induced and maintained with intravenous alpha 1-antitrypsin.

osteogenesis imperfecta, pseudoxanthoma elasticum, systemic sclerosis, morphea, and acrogeria. In EDS type IV, there is a defect in the synthesis of collagen type III. As a result, patients can develop arterial, intestinal, or uterine fragility and/or rupture. In addition, there can be rupture of the chordae tendinae related to defective collagen; the chordae tendinae structurally support the cardiac valves, and rupture can lead to severe valvular regurgitation, as in this patient. While it is suggested that the laboratory work-up of EPS is usually adequate with a complete history and physical examination in otherwise healthy patients, a genetic evaluation should be pursued in any patient who presents with acute systemic findings, especially vascular events, given that many EDS type IV patients are not diagnosed until an acute life-threatening vascular event occurs. The cause of EPS is unknown, but it may represent a final common pathway in which altered elastic fibers induced by a variety of unrelated connective tissue disorders are eliminated through the epidermis.

Correction of Skin Contour Defects in Leaking Stomas by Filler Injection: A Novel Approach for a Difficult Clinical Problem

BackgroundStomal 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.CasesWe 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.ConclusionsThis 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.

Management of Cicatricial Ectropion Secondary to Pyoderma Gangrenosum

Abstract 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.

Severe Back Pain in a Young Patient with Pyoderma Gangrenosum and Crohn's Disease Controlled with Anti-tumor Necrosis Factor Therapy: Sterile Osteomyelitis.

IntroductionInflammatory 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 ReportWe 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.ConclusionInflammatory 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.

Acute Kidney Injury Due to Interaction of Methyl-1- testosterone with Ciclosporin Metabolism in a Patient with Severe Atopic Dermatitis

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.

Vulval Crohn’s Disease in Childhood

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.

Mesalazine-induced photosensitivity

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 [...]

Crohn's disease of the vulva: A prospective study: A study of vulval Crohn's disease

Vulval Crohns disease (VCD) is a rare extra‐intestinal cutaneous manifestation of Crohns 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.

The secret sensitizer gets out of the bag

Peristomal cutaneous findings can be challenging, owing to the unique environment and local applications of medicaments and dressings. We report an interesting case of allergic contact dermatitis caused by fragrance applied by the patient into the stoma bag that penetrated through it and onto the skin. Stoma appliances comprise a hydrocolloid adhesive ring for attachment to the skin and a polythene collecting bag.

Multiple Squamous Cell Carcinomas Arising in Hyperpigmented Patches: A Newly Recognized Feature of Incontinentia Pigmenti?

A 54-year-old white woman with IP developed multiple painful subungual tumors over a period of 34 years. At birth, she had an erythematous vesicobullous rash, mainly on the extremities and pressure areas, with eosinophilia. At 3 months, warty plaques appeared on the limbs. She then developed whorls and streaks of pigmentation, followed by linear atrophy on the limbs (Figure 1). Over the next few years, she was found to have hypodontia with pegged teeth. Eye abnormalities, includingmacular telangiectasia, retinal gliosis, and chorioretinitis, were noted. In view of the excruciating pain associated with the subungual tumors, surgery was indicated. The first tumor, on the nail fold of the left thumb, was removed using curettage and cautery. Between 2009 and 2011, subungual keratoses on the right middle finger and left little finger were managed with fingertip amputations, and another tumor on the left ring finger was treated with nailbed ablation. The tumors were associated with varying degrees of onychodystrophy. Magnetic resonance imaging of the distal fingers showed evidence of local destruction with bone marrow edema.