SCLEROSING KERATITIS AND EPIDERMOLYSIS BULLOSA ACQUISITA: UNCOMMON MANIFESTATIONS IN A SLE PATIENT WTH A GOOD RESPONSE TO RITUXIMAB

SCLEROSING KERATITIS AND EPIDERMOLYSIS BULLOSA ACQUISITA: UNCOMMON MANIFESTATIONS IN A SLE PATIENT WTH A GOOD RESPONSE TO RITUXIMAB

SANTOS, FABIANA MIRANDA MOURA DOS; ANDRADE, JULIANA D'AVILA; LOBÊ, SAMARA DE QUADROS; XAVIER, CLARICE GARCIA VALADARES; RIOCHA, ANNA CHRISTINA HIGINO; VALE, EVERTON SIVIERO; LANNA, CRISTINA COSTA DUARTE; TELLES, ROSA WEISS

Pôster:

Patients with systemic lupus erythematosus (SLE) may present several ocular and cutaneous manifestations which could be related to the disease itself, to its treatment, to infections, and to superimposed different autoimmune diseases. We report a patient with SLE and epidermolysis bullosa acquisita (EBA), a very rare diseases association, who developed sclerosing keratitis.

Case report

CJO, female, 32 years-old, with SLE since 2004. She presented multisystemic manifestations with severe complications associated with numerous infectious related to usual and opportunistic agents (candida and cytomegalovirus) that caused many hospital admissions on her follow up. During the course of the disease, the patient showed articular manifestations, cutaneous discoid lesions, convulsion, serositis (pericardium and pleura), hemolytic anemia and thrombocytopenia, nephritis (three relapses), high titers of autoantibodies (FAN 1/640 speckled, Anti-Ro 1/800, Anti-RNP 1/3200, Anti-Sm 1/600, Anti-DNAds 1/160) and low complement. She also presented diffuse blisters and erosions on mucocutaneous areas, initially diagnosed as bullous systemic lupus erythematosus (BSLE), besides loss of the nails, with an unsatisfactory treatment response. These lesions were classified as EBA, the inflammatory type, after skin biopsy and clinical evaluation by an experienced dermatologist. The patient was treated with dapsone, colchicine, several immunosuppressants (azathioprine, cyclophosphamide, cyclosporine, mycophenolate mofetil) and belimumab, combined with high doses of corticosteroid, with no or only partial response. In 2018, she developed an inflammation in the right eye. The patient referred pain, tearing and a mucopurulent discharge and the eye examination revealed decrease of the transparency, stromal vascularization and thickening of the lower third of the cornea with fluorescein staining area at the upper margin of the lesion, besides symblepharon points. Infectious causes were excluded, the final diagnosis was sclerosing keratitis, and she was treated with topical corticosteroids eye drops. There was good response of the ocular and renal manifestations, as well as of the EBA to corticosteroids (topical and systemic) and rituximab/cyclosporine.

Conclusion

This case highlights a rare and difficult diagnosis of cutaneous and ocular involvement in a SLE patient. The association of EBA and SLE is rarely described. Differentiating EBA from BSLE remains a challenging task due to their identical histological and immunological findings. The evolution of the bullous lesions which left scars, and the extension and severity of mucous and nails involvement supported the EBA diagnosis. The multi-professional team involved considered the keratitis as an immunological manifestation in a patient who fulfilled the criteria for the diagnosis of both SLE and EBA.

Patients with systemic lupus erythematosus (SLE) may present several ocular and cutaneous manifestations which could be related to the disease itself, to its treatment, to infections, and to superimposed different autoimmune diseases. We report a patient with SLE and epidermolysis bullosa acquisita (EBA), a very rare diseases association, who developed sclerosing keratitis.

Case report

CJO, female, 32 years-old, with SLE since 2004. She presented multisystemic manifestations with severe complications associated with numerous infectious related to usual and opportunistic agents (candida and cytomegalovirus) that caused many hospital admissions on her follow up. During the course of the disease, the patient showed articular manifestations, cutaneous discoid lesions, convulsion, serositis (pericardium and pleura), hemolytic anemia and thrombocytopenia, nephritis (three relapses), high titers of autoantibodies (FAN 1/640 speckled, Anti-Ro 1/800, Anti-RNP 1/3200, Anti-Sm 1/600, Anti-DNAds 1/160) and low complement. She also presented diffuse blisters and erosions on mucocutaneous areas, initially diagnosed as bullous systemic lupus erythematosus (BSLE), besides loss of the nails, with an unsatisfactory treatment response. These lesions were classified as EBA, the inflammatory type, after skin biopsy and clinical evaluation by an experienced dermatologist. The patient was treated with dapsone, colchicine, several immunosuppressants (azathioprine, cyclophosphamide, cyclosporine, mycophenolate mofetil) and belimumab, combined with high doses of corticosteroid, with no or only partial response. In 2018, she developed an inflammation in the right eye. The patient referred pain, tearing and a mucopurulent discharge and the eye examination revealed decrease of the transparency, stromal vascularization and thickening of the lower third of the cornea with fluorescein staining area at the upper margin of the lesion, besides symblepharon points. Infectious causes were excluded, the final diagnosis was sclerosing keratitis, and she was treated with topical corticosteroids eye drops. There was good response of the ocular and renal manifestations, as well as of the EBA to corticosteroids (topical and systemic) and rituximab/cyclosporine.

Conclusion

This case highlights a rare and difficult diagnosis of cutaneous and ocular involvement in a SLE patient. The association of EBA and SLE is rarely described. Differentiating EBA from BSLE remains a challenging task due to their identical histological and immunological findings. The evolution of the bullous lesions which left scars, and the extension and severity of mucous and nails involvement supported the EBA diagnosis. The multi-professional team involved considered the keratitis as an immunological manifestation in a patient who fulfilled the criteria for the diagnosis of both SLE and EBA.

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DOI: 10.5151/sbr2019-236

Referências bibliográficas

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Como citar:

SANTOS, FABIANA MIRANDA MOURA DOS; ANDRADE, JULIANA D'AVILA; LOBÊ, SAMARA DE QUADROS; XAVIER, CLARICE GARCIA VALADARES; RIOCHA, ANNA CHRISTINA HIGINO; VALE, EVERTON SIVIERO; LANNA, CRISTINA COSTA DUARTE; TELLES, ROSA WEISS; "SCLEROSING KERATITIS AND EPIDERMOLYSIS BULLOSA ACQUISITA: UNCOMMON MANIFESTATIONS IN A SLE PATIENT WTH A GOOD RESPONSE TO RITUXIMAB", p-236-236. In: Anais do 36º Congresso Brasileiro de Reumatologia. [ISBN 978-85-212-1892-0]. São Paulo: Blucher, 2019.
ISSN 23577282, DOI 10.5151/sbr2019-236