Ocular Manifestations of Hidradenitis Suppurativa
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Background
Hidradenitis suppurativa (HS) is a chronic, inflammatory skin condition characterized by painful nodules, abscesses, and sinus tracts, primarily affecting areas of the body with apocrine sweat glands such as the armpits, groin, buttocks, and under the breasts1. The estimated global prevalence of Hidradenitis suppurativa is found to be 0.00033–4.1%, with a higher prevalence of 0.7–1.2% in European and United States populations2.
The exact cause of HS is not fully understood, but it is believed to involve a combination of genetic, hormonal, and environmental factors1. The disease often starts after puberty and can have a significant impact on quality of life due to pain, scarring, and the psychological burden associated with its symptoms1. Diagnosis is typically clinical, based on the characteristic lesions and their recurrent nature. Management of HS involves a multifaceted approach including lifestyle modifications, medical treatments like antibiotics and biologics, and, in severe cases, surgical intervention.
The eye is a commonly affected organ in several systemic inflammatory diseases including spondylarthritis-related immune diseases, such as psoriasis, psoriatic arthritis, Crohn’s disease, and ulcerative colitis; and vasculitis, such as Bechet’s syndrome, microscopic polyangiitis, and systemic lupus erythematosus3, 4. Understanding the range and patterns of ocular manifestations of systemic inflammatory disease is useful in the diagnosis and management of these patients. Thus, it is advised to review ocular history and symptoms to identify HS patients who should receive an ophthalmologic examination.
Pathophysiology
Hidradenitis suppurativa is an inflammatory skin condition that can cause profound morbidity and affects women more than men5. Patients can present with recurrent nodules, sinus tract formation, abscesses, and/or scarring, affecting the intertriginous areas4. The existing literature has revealed that patients with HS do have increased burden of ocular comorbidities3. One study examining ocular comorbidities in patients with HS demonstrated that uveitis was the most common, followed by scleritis and keratitis; however, they included patients with other autoimmune diseases4.
Pathophysiology
The primary defect in HS pathophysiology involves follicular occlusion of the folliculopilosebaceous unit, followed by follicular rupture, and immune responses (perifollicular lympho-histiocytic inflammation), finally leading to the development of clinical HS lesions6. Histologically, early detectable events include infundibular acanthosis, hyperkeratosis and perifollicular immune cell infiltration7. The pathogenic mechanisms are unclear, but genetic factors and alterations in the skin microbiome play a role. The existing literature points to a combination of genetic factors with alterations in the skin microbiome6. The importance of the genetic background was first noticed when Fitzsimmons and Gilbert observed disease clustering in 14 of 23 patient families, with 30% of patients report a strong family history of HS8.
Furthermore, at tissue (i.e., skin) as well as at serum level, several inflammatory cytokines are upregulated. The most important of the latter are tumor necrosis factor (TNF), interleukin (IL)-1, IL-17, and IL-235. Upregulated inflammatory cytokines cause inflammation that begins around the hair follicles and gradually forms abscesses and nodules, which may progress to pus-draining tunnels and fistulas7. Additionally, hair follicle stem cells in HS lesions show an increased proliferation rate. This is associated with elevated numbers of micronuclei and presence of cytoplasmic single‐stranded DNA in proliferating cells9. Patients may experience irreversible tissue damage, decreased quality of life, decreased mobility due to severe pain and drainage, and systemic inflammation of internal organs10.
Clinical Presentation
Patients with HS may present with ocular comorbidities such as uveitis, most commonly, followed by scleritis and keratitis4; however, the study examining these comorbidities included patients with other autoimmune diseases; therefore these symptoms may not be specific to HS. In another study, episcleritis had the strongest association, followed by keratitis and uveitis3. The pathogenesis of HS is poorly understood but the role of immune dysregulation is believed to be like that of Crohn’s disease. Eye involvement is common in many inflammatory diseases, including Crohn’s. Given the case reports demonstrating association between HS and IED, and the hypothesis that HS shares systemic inflammation dysregulation similar to Crohn’s11. In a few case reports of patients with bilateral blepharokeratoconjunctivitis and HS, shared slit lamp findings included bilateral corneal neovascularization and inferior corneal thinning. Systemic immunosuppression was needed in the first case, which resulted in improvement in the patient’s ophthalmic and dermatological findings12.
Patients with HS are at higher risk for retinal vascular complications in the absence of clinical ocular disease. Recently, a study with Optical coherence tomography angiography (OCTA revealed reduced foveal vascular density (VD) and vessel length density (VLD) in the small capillary density and reduced VD in the deep capillary plexus of the retina13. Similar to findings in diabetic retinopathy, this strengthens the existing association between type II diabetes mellitus as a comorbidity for worsening HS. Additionally, although not typical, HS lesions can appear on the face and periorbital region14. Therefore, it is crucial to conduct a thorough examination to check for any lesions on the eyelids, along the lid line affecting the meibomian glands, or encroaching on the periorbital area11. Furthermore, the patient should be evaluated for symptoms of dry eye caused by glandular dysfunction. Taking a detailed history of any past lesions involving the face or eyes is also important, as these lesions tend to recur and can result in scarring if not treated properly4.
Diagnosis
Hidradenitis suppurativa is diagnosed clinically based on the characteristic appearance and location of the lesions. Patients often present with painful nodules, abscesses, and sinus tracts located in areas of skin friction, such as the armpits, groin, and under the breasts. Biopsies may be performed to rule out other conditions with similar presentations8. Although hidradenitis suppurativa is primarily present in apocrine gland bearing areas, ocular findings have been reported to occur in approximately 13.89% of patients due to systemic inflammation. 3.
Ocular conditions associated with HS can include anterior uveitis, posterior uveitis, scleritis, episcleritis, and interstitial keratitis3, presenting with symptoms like eye redness, pain, light sensitivity, blurred vision, dry eyes, itching, and eye discharge3, 12, 14. The intensity of symptoms may fluctuate based on flares from environmental factors, stress, and comorbidity medical conditions15. In both symptomatic and asymptomatic HS patients, an eye examination should be performed, to check redness of the conjunctiva, inflammation of the eyelids (blepharitis), signs of uveitis or episcleritis/scleritis, and any changes in the cornea or iris4. The presence of cutaneous manifestations on the skin may support the diagnosis4, 9, 11.
Ocular symptoms of hidradenitis suppurativa can resemble those of other eye conditions. Therefore, it is essential to exclude other plausible causes: such as bacterial, viral, or fungal infections; autoimmune diseases like rheumatoid arthritis, Sjögren's syndrome, or lupus; allergies; and dry eye syndrome16. Treatment response can serve as a diagnostic criterion. If ocular symptoms improve following systemic therapies targeted for cytokines or inflammatory markers particularly upregulated in HS it supports the diagnosis17. Early diagnosis and intervention are vital to prevent further complications and preserve eye health.
Differential diagnosis
- Conjunctivitis
- Uveitis
- Corneal Dystrophy
- Ocular Rosacea
- Herpetic Eye Infections
- Keratitis
- Scleritis
- Dry Eye Syndrome (Keratoconjunctivitis Sicca)
- Autoimmune Diseases (e.g., Sjögren's disease, Rheumatoid Arthritis, Sarcoidosis)
- Infection (bacterial, fungal, viral)
Management
Management of HS depends on disease progression. Prompt recognition and initiation of treatment can reduce the risk of HS advancing to end-stage disease. Conservative hygiene practices for ocular manifestations (i.e. uveitis, scleritis, and keratitis) are recommended, such as warm compresses and gentle cleansing of the eyelids and eyelashes. Lubricating eye drops are symptomatic treatment for dry eyes, discomfort, and pain. Pharmacologic options depend on the presentation and severity of the disease. These therapies include topical or oral antibiotics the addition of oral or injection corticosteroids for worsening symptoms and finally biologics such as Humira (adalimumab, AbbVie) for severe symptoms. Procedural interventions are considered for lesions refractory to pharmacologic therapy18. If the HS progresses uncontrolled on those two medications, often biologic therapies are added to the treatment regimen, then surgery is next line on the treatment ladder19.
Saygin et al. found that patients with HS and ocular comorbidities rarely respond to dermatological topical treatment and respond better to anti-TNF-α therapy. Currently, adalimumab (Humira) and infliximab (IFX) are two FDA-approved biologic treatments for HS. th in20. Multidisciplinary management involving dermatologists and ophthalmologists is essential. Regular eye screenings are recommended even if symptoms are not reported4, 12, 16. Treatment should be tailored based on the severity of the symptoms, focusing on preserving vision and minimizing the impact on patients’ lives. Identifying the underlying causes, whether cellular or autoimmune, is crucial for targeted treatment10.
Uveitis Management
If the uveitis is caused by an underlying condition such as hidradenitis suppurativa, management usually involves treating that specific condition which is the treatment ladder as described above. However, the treatment for uveitis remains consistent regardless of its cause, provided it is not infectious21. The primary objective is to reduce inflammation in the eye and any other affected areas of the body. In some instances, treatment may be required for an extended period, ranging from months to years. There are several treatment options available. Traditionally, patients can start with topical corticosteroids such as prednisone then escalate to periocular injection of triamcinolone or oral steroids22. Tumor necrosis factor-α (TNF-α) is implicated in the early pathogenesis of uveitis and can induce the expression of chemokines and adhesion molecules to prolong inflammation. The use of TNF-α antagonists has represented a significant advance in the treatment of refractory uveitis. The systemic use of adalimumab, a fully humanized monoclonal antibody, has shown promising results in controlling intraocular inflammation23, 24. Since TNF-α is also a common treatment modality in HS patients, this would be an ideal choice to treat both conditions simultaneously.
Keratitis Management
Treating keratitis involves addressing the underlying cause of the inflammation and infection. Proper diagnosis of the causative organism is critical, and while culture remains the prevailing diagnostic tool, newer techniques such as in vivo confocal microscopy are helpful for diagnosing fungal keratitis28. For bacterial keratitis, antibiotic eye drops such as moxifloxacin or ciprofloxacin are commonly prescribed to eliminate the infection29. In cases of viral keratitis, antiviral medications like acyclovir or ganciclovir may be used29. Fungal keratitis requires antifungal agents such as natamycin or voriconazole30. Corticosteroid eye drops may be prescribed to reduce inflammation, but only after the infection is controlled to avoid worsening the condition29. When keratitis is associated with inflammatory systemic conditions like rheumatoid arthritis or lupus, managing the underlying disease with immunosuppressive medications such as methotrexate or prednisone is essential to control the eye inflammation4. Regular follow-up appointments are necessary to monitor the response to treatment and adjust medications as needed to ensure complete resolution and prevent complications.
References
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