Pyoderma gangrenosum of the upper eyelid

Abstract

A 3-year-old woman presented with a large ulcerated lesion involving the left upper eyelid and left earlobe with erythematous, swollen, undermined and advancing borders. Culture of the ulcer showed no growth of organisms. Treatment with empiric intravenous and topical antibiotics showed no improvement. Workup revealed an elevated white blood cell count with elevated neutrophil count, elevated erythrocyte sedimentation rate and C reactive protein and positive antinuclear antibody. A skin biopsy showed a neutrophilic dermatitis with vasculitis. The patient was started on oral prednisone, which resulted in halting of the progression of ulceration and clinically decreased skin inflammation. The skin biopsy and positive response to corticosteroids confirmed the diagnosis of pyoderma gangrenosum. Pyoderma gangrenosum is a rare inflammatory skin condition and rarely presents as lesions of the eyelid. Early initiation of immunosuppressive therapy prevents disfigurement. Eyelid reconstruction in these cases may prove to be difficult.

Background

Pyoderma gangrenosum is a rare skin condition characterised by multiple ulcerations in various parts of the body, most commonly the lower extremities and sites of skin trauma. Its pathophysiology is currently unknown, but it is theorised to be an immune-mediated condition due to neutrophilic infiltration of the dermis with subsequent destruction of tissue.¹ The incidence is estimated to be 3–10 patients per million population per year, with peak incidence occurring in adulthood between the ages of 20–50 years.¹ Pyoderma gangrenosum occurring in the paediatric population is extremely rare, comprising only 4% of cases. The occurrence of lesions in sites other than the lower extremities is rare as well.²

Case presentation

A 3-year-old girl presented with a 2-week history of a rapidly enlarging ulcer on the left upper eyelid. The mother noted that a few days prior the child was playing and had sustained some abrasions on the left side of her face. The patient developed skin ulcers over the left upper eyelid as well as the left earlobe. Over the next few days, there was rapid progression of the ulcers in both sites, resulting in large areas of tissue loss. There were no associated systemic symptoms such as fever, nausea, vomiting, diarrhoea, gastrointestinal bleeding and joint pains. The patient initially consulted with a local hospital who started a course of intravenous and topical antibiotics. Despite empiric antibiotic treatment, there was still progression of the ulcers.

The patient was seen around 2 weeks after her initial symptoms. Examination of the left eye showed a 30×32×30 mm ulcer of the left upper eyelid with tissue loss of the anterior lamellae and partial tissue loss of the tarsus. There was also loss of normal eyelid margin architecture of the lateral third of the eyelid. The borders of the ulcer were erythematous, swollen and had undermined borders (figure 1). The left globe was unaffected. Its visual acuity was 20/20 and was grossly normal on examination using slit lamp biomicroscopy. The right globe and adnexae were likewise also unremarkable. There was an ulcerative lesion of similar characteristics in the left earlobe as well, with tissue loss mainly on the lobule. There were no other lesions in other parts of the body. Systemic examination was unremarkable.

Figure 1.

Gross photograph showing an ulcer in the left upper eyelid with erythematous and undermined borders. The central part of the lesion shows complete tissue loss of the anterior lamellae and partial tissue loss of the tarsus with eschar formation.

The patient was started on empiric antibiotics comprising of a course of intravenous ceftriaxone and topical erythromycin ointment. Workup was done while the patient was undergoing treatment. Bacterial, fungal and tuberculous cultures from the ulcer sites showed no growth of organisms. Complete blood count showed an elevated white blood cell count with elevated neutrophil count. Erythrocyte sedimentation rate and C-reactive protein were elevated. Antinuclear antibody was positive. Pathergy test was negative. A skin biopsy was done with the sample taken from the erythematous ulcer edges. Histopathology showed a neutrophilic infiltration within a loose dermis and leukoclastic vasculitis (figure 2). The results of the skin biopsy were consistent with a diagnosis of pyoderma gangrenosum.

Figure 2.

Skin biopsy of the erythematous margins of the left upper eyelid ulcer showing neutrophilic infiltration of the dermis (black arrowhead) with vasculitis (arrow). There is also reactive acanthosis and keratosis of the overlying epidermis (white arrowhead).

Differential diagnosis

An important differential to consider in cases of skin ulceration, particularly in the eyelid, is bacterial infection. These lesions are usually caused by gram-positive bacteria, most commonly Staphylococcus aureus.³ Progression of skin infection can lead to preseptal or orbital cellulitis. Thus, obtaining cultures from the ulcer site is crucial in the management of these cases since initiation of antibiotic therapy is curative.

Another differential that comes to mind in cases of rapidly progressive skin infections is necrotising fasciitis, which is commonly caused by Streptococcus.³ The condition is characterised by severe pain and its very rapid progression. The management of cases requires early initiation of antibiotic therapy as well as debridement of all infected and necrotic tissue. The prognosis for these cases are often poor.³

Once infection has been ruled out, it is important to consider that a number of inflammatory conditions can present as skin lesions. An example is Wegener’s granulomatosis, which can present with skin lesions with granulomatous inflammation. These cases are often systemic and can be diagnosed with appropriate serum markers and biopsy. Consultation with a rheumatologist and dermatologist who are knowledgeable on skin inflammation may help.

Treatment

The patient was started on a course of oral prednisone with a dose of 1 mg/kg/day. There was rapid improvement in the appearance of the inflamed skin over the next 3 days, exhibited by halting of the progression of the ulceration and transformation of the raw ulcer bed into eschars. The results of the skin biopsy and positive response of the therapeutic trial of corticosteroids confirmed the diagnosis of pyoderma gangrenosum. The patient was planned for initiation of immunosuppressants. Eyelid reconstruction will be planned once the inflammation is controlled.

Outcome and follow-up

The patient was followed up over a course of 2 weeks where there was decreased erythema and swelling of the borders of the ulcerated lesion. The patient was advised regarding the possibility of development of sensory amblyopia and the need for eyelid reconstruction in the future. The patient was unfortunately lost to follow-up.

Discussion

The diagnosis of pyoderma gangrenosum is clinical and supported by findings on histopathology and response to immunosuppressants.² There have been numerous efforts to formulate diagnostic criteria for this condition, with most including the characteristic appearance of skin ulcers with violaceous and undermined borders and exclusion of other causes of skin ulceration. The presence of other associated conditions such as inflammatory bowel disease, arthritis, collagen vascular disease and various haematologic malignancies may contribute to the diagnosis. A skin biopsy is essential to the diagnosis.² Histopathology most commonly reveals massive neutrophilic infiltration and granuloma formation, often with associated vasculitis. The timing of biopsy is critical since it must be done during the period of active inflammation to be able to demonstrate the classic findings of neutrophilic infiltration.³

There are various forms of pyoderma gangrenosum. The case presented is consistent with the vegetative form of pyoderma gangrenosum. It is a localised and less aggressive variant characterised by ulcerative and fungating lesions. Peculiar to this variant is the occurrence of lesions within the head and neck region, no association with systemic diseases and negative pathergy testing⁠—all of which was seen in the case.³ The vegetative form of pyoderma gangrenosum also shows a mixed infiltrate in the dermis on histopathology, comprising of neutrophils, eosinophils and histiocytes.³

Immunosuppressive therapy is the cornerstone of management of pyoderma gangrenosum, usually involving high-dose corticosteroids and eventually immunomodulating drugs.⁴ Both local wound care and systemic drugs have a role in its management. Early diagnosis and subsequent early initiation of treatment are key to reducing tissue destruction and scarring.⁴ In cases where tissue loss has occurred due to progressive ulceration, such as in the case, reconstruction may be challenging.

Learning points.

• Pyoderma gangrenosum is a rare skin condition. The diagnosis can be challenging due to its multiple variants.

• A skin biopsy done during a period of active skin inflammation confirms the diagnosis.

• Early diagnosis and initiation of immunosuppressive therapy are key to preventing tissue loss and disfiguring sequelae.