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

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.

A novel use for botulinum toxin A in the management of ileostomy and urostomy leaks.

PURPOSE: To assess the efficacy of Botulinum toxin A (BoNT-A) in the treatment of patients with hypercontractile stomas resulting in repeated pouching system failures and leaks. DESIGN: Prospective case series. SUBJECTS AND SETTING: Ten consecutive patients who presented to the outpatient stoma clinic with actively contractile stomas that shortened spasmodically resulting in leaks were offered treatment with BoNT-A if treatment with other measures had been unsuccessful. METHODS: Following an observed reduction in the peristalsic shortening of a stoma after intradermal injection of BoNT-A for hyperhidrosis, we conducted a prospective case series of 10 patients with pouch adhesive failures attributed to spasmodic shortening of the stoma. Ten patients, 3 with urostomies and 7 with ileostomies, were offered BoNT-A injection. The first was treated cautiously with 15 units of BoNT-A injected into the muscularis layer, followed by an additional 25 units injected 1 month later. Subsequent patients received doses varying from 50 to 100 units. Ongoing treatments ranged 50 to 100 units every 3 to 6 months. RESULTS: Seventy percent (n = 7) of patients reported a useful reduction in leakage and pouching system seal failures. In these 7 patients, the frequency of pouch changes changed from an average of 2.18 to 0.44 per day (over all 10 patients this was a change from an average of 2.35 per day to 1.16 per day). No adverse side effects were reported. CONCLUSION: Findings from this clinical case series suggest that BoNT-A may be a promising treatment in the management of patients with leaks caused by actively contracting stomas.

Management of cicatricial ectropion secondary to pyoderma gangrenosum.

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.

Folliculitis-perifolliculitis related to erlotinib therapy spares previously irradiated skin.

Erlotinib, a specific epidermal growth factor receptor tyrosine kinase inhibitor, is used to treat various metastatic cancers. It is known to cause an acneiform rash. Herein, we report a case where the rash spared the previous radiotherapy field. A limitation of this study is that it is an anecdotal case report. Further research into the pathologic process of the rash is warranted to understand the reason for its absence in the irradiated skin.

PHACES syndrome--report of two cases and their evolution over time.

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.

Basal cell carcinoma with a sarcomatous component (carcinosarcoma): a series of 5 cases and a review of the literature.

Cutaneous carcinosarcomas are rare biphasic malignant tumors with a malignant epithelial component together with malignant stroma. Five cases treated in the Yorkshire region of England between 2003 and 2006 are presented. The patients were male with an age range from 58 to 84 years. Four lesions occurred on the face and one on the trunk. Each tumor had an epithelial component resembling a typical nodular basal cell carcinoma. The stromal components demonstrated atypical spindle cells and tumor giant cells, undifferentiated stroma, or osteoid formation. All underwent surgical excision and none showed evidence of recurrence (follow-up 3-17 months, one death from unrelated causes). The recent literature concerning the pathogenesis and prognosis of these unusual tumors is reviewed.

Multidisciplinary care of skin problems in stoma patients.

Skin integrity is essential for the normal usage of a stoma appliance. There is little published on the prevalence, prevention or management of stoma skin problems. Allergic contact dermatitis is often cited as the cause, usually without evidence from formal investigations. The authors approached, by postal questionnaire, 525 patients who had had a stoma formation in the last 10 years. A total of 325 responded. All those who described a skin problem were invited to attend a multidisciplinary clinic for further investigations and appropriate treatment of their peristomal skin. This may be severe and debilitating as well as socially restricting. However, with a multidisciplinary approach a number of conditions can be recognized and easily treated, thus improving the quality of life for stoma patients.

Florid urticarial vasculitis heralding a flare up of ulcerative colitis.

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.

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

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.

Correction of skin contour defects in leaking stomas by filler injection: a novel approach for a difficult clinical problem.

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.

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.

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.