Pyoderma Gangrenosum of the Eyelid: Report of Two Cases and Review of the Literature

Abstract

Pyoderma gangrenosum (PG) of the eyelid is extremely rare, and its proper management is essential for the preservation of visual function. Here, we report 2 cases of PG of the eyelid with intraorbital involvement. In both cases, the skin and intraorbital lesions improved after systemic immunosuppressive therapies; however, corneal perforation occurred in 1 case. In order to assess the clinical features of PG of the eyelid and to obtain clues for optimal treatment, we reviewed 15 well-documented cases in the literature, including the present cases. Corneal perforation occurred in 4 cases and defective ocular motility in 1 case. Three patients eventually underwent enucleation of the affected eye. Our cases and the literature review clearly indicate that MRI is a powerful tool for evaluating the extent of extracutaneous PG lesions around the eye and that early diagnosis and immediate immunosuppressive therapy are crucial for the preservation of visual acuity.

Pyoderma gangrenosum of the eyelid

“…Only 15 other cases have been reported, and they have a wide range of clinical presentations, treatments, and outcomes. [4][5][6][7][8][9][10][11][12][13][14] Our case is instructive for its dramatic response to oral steroids and for highlighting clinical features that may be predictive of a good outcome.…”

“…5,6,11,14 In the other 11 cases, the eyelid was part of a wider area of disease, with involvement of the scalp, cheek, temple, lateral canthus, inferior oblique, inferior rectus and lateral rectus muscles, orbital apex, sclera, anterior chamber, and maxillary sinus or nasopharynx. 4,[7][8][9][10][11][12][13] The initial diagnosis in our case delayed immunosuppressive treatment by 4 weeks, during which time there was complete breakdown of the anterior lamella. However, the eyelid was recovered with aggressive immunosuppression, and the corneal health and vision were preserved.…”

“…Diagnostic delays of 7 days to 3 years occurred in three-quarters (9/12 in which diagnosis time is reported) of previous cases, even when the diagnosis of PG was already known, with misdiagnoses of hordoleum, stye, abscess, chalazion, herpetic infection, preseptal cellulitis, tumour, and nodular scleritis. [4][5][6][7][8][10][11][12]14 Useful clues to the diagnosis include high levels of pain inconsistent with cystic swellings and the presence of other systemic diseases; leukopaenia, aplastic anemia, ulcerative colitis, diabetes, and rheumatoid arthritis have all been reported in patients with eyelid PG. 4,7,8,11 Oral or intravenous steroids are the mainstay of treatment for PG and, in combination with recommencement of infliximab infusions, brought about a dramatic and almost complete recovery in our patient.…”

Retinal vasculitis associated with pyoderma gangrenosum: a case report

“…PG-associated nodular scleritis [10], orbital inflammation [10], eyelid involvements [11–13] and peripheral ulcerative keratitis [14] have been reported. In the vast majority of cases, immunosuppressive therapy had been successful.…”

Pyoderma gangraenosum

“…Ebenfalls bekannt ist die Koinzidenz mit F Vaskulitiden wie der Takayasu [16] oder am Nasenrücken [9]. Zusätzlich ist eine extrakutane Manifestation an den Schleimhäuten der oberen Atemwege, des Auges und des Genitalbereichs möglich [7,11,13].…”