Conjunctival involvement in familial chronic benign pemphigus (Hailey-Hailey disease)

Authors

  • Oya Oguz MD,

    Corresponding author
    1. From the Departments of Dermatology, Ophthalmology, and Pathology of Cerrahpşa Medical Faculty of the University of Istanbul, Istanbul, Turkey
      Oya Og̀uz, MD, Bag̀dat Cad. No:400/8, Şaşkinbakkal, Suadiye, Istanbul, Turkey
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  • Gökhan Gokler MD,

    1. From the Departments of Dermatology, Ophthalmology, and Pathology of Cerrahpşa Medical Faculty of the University of Istanbul, Istanbul, Turkey
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  • Özcan Ocakoglu MD,

    1. From the Departments of Dermatology, Ophthalmology, and Pathology of Cerrahpşa Medical Faculty of the University of Istanbul, Istanbul, Turkey
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  • Velittin Og̀uz MD,

    1. From the Departments of Dermatology, Ophthalmology, and Pathology of Cerrahpşa Medical Faculty of the University of Istanbul, Istanbul, Turkey
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  • Cuyan Demirkesen MD,

    1. From the Departments of Dermatology, Ophthalmology, and Pathology of Cerrahpşa Medical Faculty of the University of Istanbul, Istanbul, Turkey
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  • Ertug̀rul H. Aydemir MD

    1. From the Departments of Dermatology, Ophthalmology, and Pathology of Cerrahpşa Medical Faculty of the University of Istanbul, Istanbul, Turkey
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Oya Og̀uz, MD, Bag̀dat Cad. No:400/8, Şaşkinbakkal, Suadiye, Istanbul, Turkey

Abstract

A 25-year-old woman with a 1-year history of malodorous, red, and eroded lesions in frictional sites was seen in the dermatology department. She did not give a family history of a similar condition. She had an 18-month-old baby and her initial complaint was of genital pruritus with profuse vaginal discharge which appeared within 2 months of delivery. She was treated for candidal vaginitis but pruritus persisted and skin lesions occurred on both sides of the groin. The lesions subsided as long as the local treatment for candidal vaginitis was continued, but recurred more than three times in a year, each time with additional and more severe lesions involving the axillae, retroauricular sites, and scalp. She received no specific treatment during this time and her complaint about the eyes was the reason she was referred to a dermatologist.

On dermatologic examination, impetigo-like, serpiginous plaques with severe erythema, erosions, and an active border were observed behind the ears, in the axillae, and in the pubis; these tended to expand peripherally and were partly covered with greasy yellowish scales particularly on the scalp. Satellite pustules and vesicles were observed at the adjacent sites (Fig. 1). Conjunctivae were bilaterally hyperemic and fine yellowish scales were also apparent on the palpebral rim (Figs 2 and 3). Ophthalmologic examination revealed severe conjunctival oedema and ulcerating blepharitis. Cornea appeared normal and vision was unimpaired. Results of Schirmer's test (15 and 17 mm) were within the normal range of tear secretion without an anaesthetic. Swab cultures from the peripheral pustules and erosions showed S. aureus infection while Sabourraud culture remained negative. Skin and conjunctiva biopsies for direct microscopy and immunofluorescence were performed. Histopathologic features were identical in either specimen disclosing suprabasal blister formation, acantholysis, and villus-like protrusions lined by a single layer of basal cells (Fig. 4). IgG, IgA, IgM, and complement C3 immunofluorescence was not detected at the epidermis of the skin adjacent to serpiginous lesions. A direct immunofluorescent test of conjunctival biopsy also remained negative.

The patient was given clarithromycine 500 mg/day for 10 days and topical treatment was also applied. A rapid response was obtained within the first week of treatment.

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