Eye problems in patients with Acquired Boil Epidermolysis

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Thursday, 18 July 2019

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L'Acquired Boil Epidermolysis (EBA) is a disease rare, chronic and non-hereditary, characterised by the presence of tense blisters on skin and mucous membranes which can affect both adults and children. It is caused by a form of autoimmunity against collagen, and in particular against collagen type VII, and it is estimated that one person in every 5 million suffers from acquired epidermolysis bullosa.

This disease not easy to diagnose because of clinical manifestations similar to other bullous diseases, because of symptoms that may vary from patient to patient, but also because of technical difficulties in correctly identifying the target molecule (antibodies against collagen type VII). The classical form of EBA lawsuit skin fragility and tense blisters which may appear on any part of the skin and mucous membrane, but which are predominant in areas exposed to slight trauma and especially in the superficial and extensible areas of the skin.

Injuries heal leaving scars hypo- or hyper-pigmented, cysts and nail dystrophy. In addition to this classic form, there are other types of non-classical EBA: mucous membrane-associated EBA (MM-EBA), bullous pemphigoid-like EBA (BP-like EBA), immunoglobulin A EBA (IgA-EBA) and Brunsting-Perry EBA.

Although boils appearing on skin and mucous membranes have been well characterised, until recently little was known about the ocular involvement in patients with EBA. This knowledge comes from a limited number of clinical studies in which, unfortunately, the diagnosis of EBA and the ophthalmic description had not been standardised.

Acquired bullous epidermolysis: the study on The British Journal of Ophthalmology

However, a new study published in The British Journal of Ophthalmology describes the characteristics of ocular involvement from a clinical and pathological point of view in patients from a referral centre specialising in bullous diseases. The study included nine patients (5 women and 4 men), with a mean age at diagnosis of 32 years and a follow-up of approximately 11 years.

At the time of treatment enrolment, all patients presented conjunctivitis scar7 patients with damage to both eyes and 2 patients with damage to only one eye. In addition, 60% of patients reported severe conjunctival fibrosis.

The cornea had been damaged severely and extensively in eight patients. Although corneal involvement was most frequently associated with irritation due to trichiasis or scarring conjunctivitis, some of the corneal lesions developed even in the absence of other defects. In addition to involvement of the conjunctiva or cornea, 6 patients reported a eyelid involvementwith the presence of trichiasis.

In addition, at the time of enrolment, the better corrected visual acuity (BCVA) was 20/40 or higher in 60% of the eyes, between 20/50 and 20/200 in 16% and 20/400 or lower in 10% of the eyes. As found in the monitoring of 4 patients, the patient's clinical condition may worsenwith progression of conjunctival fibrosis, diminished vision, corneal ulceration, healing defects following corneal surgery, or even deficiency of limbal stem cells, cells responsible for the differentiation and proliferation of corneal epithelium cells. Moreover, these problems can occur in all subtypes of EBA and have been observed an association between the presence of severe lesions in the head and neck region and severe forms of conjunctival fibrosis. Patients with progressive fibrosis, on the other hand, do not have lower BCVA

In addition to systemic treatmentsIn an attempt to halt the loss of visual acuity, patients were subjected to ocular surgical procedures such as keratoprosthesis, superficial keratectomy for resection of the corneal cloth, corneal keratoplasty with buccal mucosa graft, 'hot' keratoplasty, amniotic membrane grafts, marginoplasty.

The authors of the study showed that Although ocular involvement is relatively rare in patients with EBA, when it does occur, it appears severely causing severe visual loss. However, the corneal surgical procedures and keratoplasties performed in this patient group had a poor outcome, probably due to the lack of sufficient immunosuppressive drugs.