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Year : 2022  |  Volume : 67  |  Issue : 6  |  Page : 835
Localized linear IgA bullous dermatosis and a recurrent cicatricial ectropion of the eyelid: A case report of a sixteen-year follow-up

Eye Hospital, University Medical Centre Ljubljana, Slovenia

Date of Web Publication23-Feb-2023

Correspondence Address:
Katja Matović
Grablovičeva 46, 1000 Ljubljana
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijd.ijd_909_20

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Cicatrizing ectropion of the eyelid is an uncommon condition, possibly resulting in significant ocular morbidity. A potential cause can be a systemic disease, including autoimmune blistering disease (ABD). We herein report a case with a sixteen-year follow-up of a patient with a chronic cicatrizing unilateral ectropion caused by linear IgA bullous dermatosis (LABD). LABD is an ABD, characterized by the accumulation of IgA anti-basement membrane autoantibodies. It has a varied presentation; however, localized or ophthalmic manifestations have been infrequently described. The case demonstrates the benefits of immunohistochemistry in obtaining the correct diagnosis, and difficulties in the medical and surgical management of a recurrent cicatricial ectropion caused by chronic systemic disease.

Keywords: Autoimmune blistering disease, cicatrizing ectropion, linear IgA bullous dermatosis

How to cite this article:
Drnovsek-Olup B, Matović K. Localized linear IgA bullous dermatosis and a recurrent cicatricial ectropion of the eyelid: A case report of a sixteen-year follow-up. Indian J Dermatol 2022;67:835

How to cite this URL:
Drnovsek-Olup B, Matović K. Localized linear IgA bullous dermatosis and a recurrent cicatricial ectropion of the eyelid: A case report of a sixteen-year follow-up. Indian J Dermatol [serial online] 2022 [cited 2023 Mar 23];67:835. Available from:

   Introduction Top

Ectropion is an abnormal eversion of the eyelid, primarily involving the lower eyelid. Per pathophysiology, acquired ectropion is classified as involutional, paralytic, cicatricial, or mechanical. Cicatricial ectropion is a relatively uncommon condition, resulting from the shortening of the anterior lamella of the eyelid, caused by scarring. It may follow an eyelid trauma or surgery; a potential cause can as well be a systemic disease.[1] We herein report a case with a 16-year follow-up of a patient with recurrent cicatricial ectropion associated with linear IgA bullous dermatosis (LABD). LABD is a rare entity of subepidermal autoimmune blistering disease (ABD).[2]

   Case History Top

In 2003, a 63-year-old male was referred to the oculoplastic unit for treatment of a presumed squamous cell carcinoma (SCC) of the left lower eyelid. He had a history of smoking, arterial hypertension, chronic obstructive pulmonary disease, and peripheral arterial occlusive disease. He had been noticing skin changes for over one year. On examination, ectropion of the left lower eyelid was present, and the skin of the eyelid was dry and scaling.

Excision of the left lower eyelid and reconstruction with a local skin flap was performed. Histological examination showed changes consistent with squamous cell carcinoma in-situ and clear surgical margins. Shortly after the surgery, recurrent bullous changes appeared on the reconstructed eyelid. Microbiological examination excluded herpetic disease.

Three months later, a surgical revision of the retracted cicatricial left lower eyelid was performed and a histopathological sample was obtained. The eyelid was reconstructed with a retroauricular skin graft. The histological examination reported a local, atypical bullous pemphigoid [Figure 1]. Additional immunofluorescent investigation showed linear deposits of IgA and complement C3 along the basement membrane, by which the diagnosis of LABD was made. As the disease was limited, no systemic therapy was prescribed.
Figure 1: A neutrophil and eosinophil-rich sub-epidermal vesicle and a mixed cellular inflammatory infiltrate in the dermis beneath the vesicle. Haematoxylin and eosin, 200x original magnification

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At a follow-up in 2012, the patient reported recurrent bullous changes of the eyelid. He was found to have a pronounced cicatricial ectropion of the left lower eyelid and keratinization of the everted, lower tarsal conjunctiva [Figure 2]. The ocular surface was otherwise well preserved.
Figure 2: Cicatricial ectropion of the left lower eyelid

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Due to a highly unusual course, a revision of the first biopsy (2003) was carried out and showed findings, consistent with subepidermal bullous dermatosis.

In 2015, the patient experienced a pruritus efflorescence on the buttocks, abdominal skin and hands. A biopsy was performed and the histopathological examination showed prebullous phase of an ABD. Systemic corticosteroid treatment was prescribed but was refused by the patient. The skin changes disappeared after local corticosteroid therapy.

In the same year, surgical correction of the ectropion with a transpositional, zygomatic flap was performed [Figure 3]. In the absence of an active LABD, preventive systemic therapy was not prescribed. Bullous changes reappeared and led to a recurrence of the cicatricial ectropion.
Figure 3: Surgical correction of the cicatricial ectropion

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The patient remained under the care of the oculoplastic unit and dermatological department, being treated with local dexamethasone to the eyelid. Another surgical correction of the ectropion along with systemic therapy with dapsone was to be performed. However, in 2019, priorly to carrying out the planned treatment the patient died of an unrelated condition.

   Discussion Top

Cicatricial ectropion is a relatively uncommon condition and may be caused by a systemic disease. In a case as such, surgically challenging management should be carried out along with systemic treatment.

A case of a patient with recurrent cicatricial ectropion of an eyelid caused by LABD was herein presented. LABD is a rare disorder with a reported incidence of 0.23–2.3 cases per million per year.[3] It has a bimodal age predilection, occurring in children and in adults, which are most commonly affected in their 60s.[4] The adult-onset form usually presents with lesions on the trunk, head and limbs.[3] In both forms, mucous membranes may be affected.[2],[3] LABD was rarely described in the ophthalmic literature, and only a few cases described a localized disease.[5],[6]

What is unique in the case presented is that recurrent signs of systemic disease were present on the left lower eyelid. The lower conjunctiva was affected indirectly due to exposure, otherwise, mucosal membranes were unaffected.

LABD is usually idiopathic, however, there as well is a subset of drug-induced LABD, vancomycin being the most commonly reported.[2],[4] There have as well been documented cases of LABD in association with malignancies.[4],[7] The pathogenetic connection between ABD and malignancies remains unclear, however, proinflammatory molecules may lead to tissue damage, chronic inflammation and increased risk of carcinogenesis.[8] The time-onset relation between ABD and malignancy is as well uncertain.[8]

In the herein-reported case, the diagnosis was delayed. LABD was first thought to have been preceded by SCC excision; however, the review of the specimen nine years later showed that reactive changes in the sample were misinterpreted as SCC.

Biopsy with immunofluorescence analysis is important in confirming the diagnosis and planning treatment for LABD. Direct immunofluorescence shows linear deposition of IgA along the dermo-epidermal junction.[2] In mild LABD topical corticosteroid as a sole therapy can be effective.[4] In a more severe course, the treatment of choice is dapsone, and sulphonamides are a second line-therapy.[4],[9] In more resistant cases corticosteroids or other immunosuppressive agents are required.[4] After clinical remission systemic therapy is gradually tapered.[10]

As in our patient, the disease was limited to a small area, he was treated with local corticosteroids only. However, the disease caused recurrent ectropion of the eyelid, potentially causing ocular morbidity. The decision to initiate systemic treatment with dapsone before further surgical management was made, but was not carried out due to the decease of the patient.

The report aims to emphasize the need to consider the differential diagnosis of a systemic cutaneous disease even in localized recurrent lesions. Along with medical treatment, cicatricial changes in a case as such necessitate multiple surgical procedures, thus requiring a multidisciplinary approach.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Bedran EG, Pereira MV, Bernardes TF. Ectropion. Semin Ophthalmol 2010;25:59-65.  Back to cited text no. 1
Lammer J, Hein R, Roenneberg S, Biedermann T, Volz T. Drug-induced linear IgA bullous dermatosis: A case report and review of the literature. Acta Derm Venereol 2019;99:508-15.  Back to cited text no. 2
Lings K, Bygum A. Linear IgA bullous dermatosis: A retrospective study of 23 patients in Denmark. Acta Derm Venereol 2015;95:466-71.  Back to cited text no. 3
Fortuna G, Marinkovich MP. Linear immunoglobulin A bullous dermatosis. Clin Dermatol 2012;30:38-50.  Back to cited text no. 4
Letko E, Bhol K, Foster CS, Ahmed AR. Linear IgA bullous disease limited to the eye: A diagnostic dilemma: Response to intravenous immunoglobulin therapy. Ophthalmology 2000;107:1524-8.  Back to cited text no. 5
Carruth BP, Meyer DR. Linear IgA bullous dermatosis: An unusual cause of upper eyelid cicatricial entropion. Ophthalmic Plast Reconstr Surg 2013;29:e151-4.  Back to cited text no. 6
van der Waal RI, van de Scheur MR, Pas HH, Jonkman MF, Van Groeningen CJ, Nieboer C, et al. Linear IgA bullous dermatosis in a patient with renal cell carcinoma. Br J Dermatol 2001;144:870-3.  Back to cited text no. 7
Pietkiewicz P, Gornowicz-Porowska J, Dmochowska MB, Dmochowski M. Malignancy in relation to autoimmune blistering dermatoses: Molecular and clinical aspects. In: Vereecken P, editor. Highlights in Skin Cancer. London: IntechOpen; 2013.  Back to cited text no. 8
Souza BC, Fregonesi NC, Tebcherani AJ, Sanchez AP, Aoki V, Fernandes JC. Linear IgA bullous dermatosis: Report of an exuberant case. An Bras Dermatol 2013;88(Suppl 1):67-70.  Back to cited text no. 9
Chen S, Mattei P, Fischer M, Gay JD, Milner SM, Price LA. Linear IgA bullous dermatosis. Eplasty 2013;13:ic49.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3]


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