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A cutaneous presentation of a common condition
Adam Daunton, Gerald Langman, J M R Goulding
Correspondence to: A Daunton firstname.lastname@example.org
A 25 year old woman was reviewed on the ward by the dermatology team for persistent, painful intertriginous skin erosions beneath both breasts and in both groins. She had been admitted over a month ago with cellulitis around the erosions, which initially responded to intravenous antibiotics but needed several repeat courses owing to recurrence.
Crohn’s disease had necessitated a total colectomy at age 18 years. She also had severe learning difficulties and behavioural problems.
She could not tolerate topical treatments. The only regular drugs at admission were mesalazine 1500 mg daily and clozapine. High dose oral corticosteroids before colectomy caused a marked transient psychosis and she had not received further systemic corticosteroids since.
She was obese with deep sloughy linear erosions demonstrating the “knife-cut” sign beneath the abdominal apron, in the inguinal creases, and under both breasts. She also had an eroded ulcer in her right labium majus.
Fig 1 Knife-cut ulcer in the right submammary fold
Multiple skin swabs were negative for bacterial, candidal, and herpes simplex virus (HSV) infections. Initial skin biopsy showed only non-specific scarring in the dermis, but a deeper one showed non-caseating granulomatous inflammation.
1. Which differential diagnoses must be excluded in this patient?
Linear erosive HSV infection, cutaneous Crohn’s disease, bacterial and fungal infections.
The term “knife-cut” sign was originally used to refer to the linear fissures commonly seen on colonoscopy in patients with Crohn’s disease. It was later used to describe the cutaneous linear erosive lesions seen in some patients with Crohn’s disease affecting the skin (termed metastatic Crohn’s disease).
2. Which investigations are needed to reach a diagnosis?
Histopathological analysis of skin biopsies shows non-caseating granulomatous inflammation, similar to that seen in gastrointestinal Crohn’s disease.
Fig 2 Numerous granulomas, some of which are coalescing, line the ulcer base in the skin biopsy. Haematoxylin and eosin staining; original magnification ×20
Fig 3 Higher power view of the granulomas. Haematoxylin and eosin staining; original magnification ×100
3. How should this condition be managed?
A multidisciplinary approach involving the dermatology and gastroenterology teams. Topical and systemic steroids, metronidazole, and anti-tumour necrosis factor drugs have shown some success.
4. What other cutaneous manifestations can occur in this condition?
Cutaneous features such as fistulas, fissures, or oral aphthous ulcers; erythema nodosum and pyoderma gangrenosum; and cutaneous disorders such as vitiligo, psoriasis, and hidradenitis suppurativa.
Our patient was started on treatment with adalimumab for her cutaneous Crohn’s disease. She received an initial dose of 80 mg, followed by 40 mg every other week. Her skin improved greatly after six weeks, and in the six months since starting treatment she has not had any recurrent episodes of cellulitis that required hospital admission. Methotrexate and ciclosporin were also considered, but because compliance with her existing oral drugs was poor and she sometimes refused oral drugs even while an inpatient, a comparatively infrequent dosing schedule and parenteral route of administration was thought to be most appropriate.
Fig 4 Healing knife-cut ulcer in the submammary fold after treatment with adalimumab for six weeks
Patient consent obtained.
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