Management and complications of cicatricial eyelid retraction secondary to Pyoderma Gangrenosum in a patient with Takayasu Arteritis
Author: Maria Mas Castells
Base Hospital / Institution: Sahlgrenska University Hospital, Sweden
ePoster presentation
Abstract ID: 25-332
Purpose
1 To describe the management of cicatricial eyelid retraction secondary to facial Pyoderma Gangrenosum (PG) in a patient with Takayasu Arteritis (TA).2 To present the surgical complications encountered.3 To review the occurrence of PG in association with TA.4 To differentiate PG from the similarly presenting necrotising fasciitis (NF).
Methods
A 35-year-old woman of Somalian origin presented with a rapidly progressive and extremely painful erythemato-ulcerative lesion on her right upper face. No documented history of trauma or surgery. Previous medical history included a clinically stable Takayasu arteritis medically treated with Azathioprine and low-dose oral steroids, epilepsy, thrombocytopenia and chronic generalised ache of unclear nature.
Results
Clinical presentation, negative swab cultures, as well as skin biopsy showing a neutrophilic infiltration of the dermis suggested the diagnosis of pyoderma gangrenosum. Aggressive oral steroids and Cyclosporine rapidly controled the inflammatory episode. An extensive scar developed involving the right upper eyelid and causing eyelid retraction, severe lagophthalmos and symptomatic exposure keratopathy. Unsuccessfull temporary tarsorraphies preceeded a surgical eyelid scar release and full-thickness skin graft from the arm. Preoperative high-dose steroids failed to prevent postoperative PG on the arm, a phenomenon known as pathergy. The skin graft suffered mild contraction and currently doesn’t completely correct the lagophthalmos.
Conclusion
This case describes the occurrence of facial PG compromising eyelid closure. PG is an ulcerative cutaneous condition characterised by non-infectious painful necrotic ulcers. Despite being a rare manifestation of TA, PG should be suspected in patients with these characteristic skin lesions. It is of paramount importance to differentiate PG from NF, an aggressive polimicrobial cutaneous infection which shares a similar presentation, because the treatment is completely opposite: aggressive debridement and systemic antibiotics used to treat NF would worsen PG.
Additional Authors
| First name | Last name | Base Hospital / Institution |
|---|---|---|
| John | Paoli | Sahlgrenska University Hospital |
| Karin | Svedberg | Sahlgrenska University Hospital |