CONJUNCTIVAL INVOLVEMENT IN FAMILIAL CHRONIC BENIGN PEMPHIGUS (HAILEY-HAILEY-DISEASE)

A 25-year-old woman with a 1-year history of malodorous, red, and erod ed 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 wi th profuse vaginal discharge which appeared within 2 months of deliver y. She was treated for candidal vaginitis but pruritus persisted and s kin lesions occurred on both sides of the groin. The lesions subsided as long as the local treatment for candidal vaginitis was continued, b ut 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 dermato logist. On dermatologic examination, impetigo-like, serpiginous plaque s with severe erythema, erosions, and an active border were observed b ehind the ears, in the axillae, and in the pubis; these tended to expa nd peripherally and were partly covered with greasy yellowish scales p articularly on the scalp. Satellite pustules and vesicles were observe d at the adjacent sites (Fig. 1). Conjunctivae were bilaterally hypere mic and fine yellowish scales were also apparent on the palpebral rim (Figs 2 and 3). Ophthalmologic examination revealed severe conjunctiva l 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 cultu res from the peripheral pustules and erosions showed S. aureus infecti on while Sabourraud culture remained negative. Skin and conjunctiva bi opsies for direct microscopy and immunofluorescence were performed. Hi stopathologic features were identical in either specimen disclosing su prabasal blister formation, acantholysis, and villus-like protrusions lined by a single layer of basal cells (Fig. 4). IgG, IgA, IgM, and co mplement C-3 immunofluorescence was not detected at the epidermis of t he skin adjacent to serpiginous lesions. A direct immunofluorescent te st of conjunctival biopsy also remained negative. The patient was give n clarithromycine 500 mg/day for 10 days and topical treatment was als o applied. A rapid response was obtained within the first week of trea tment.