Unilateral refractory (erosive) conjunctivitis: a peculiar manifestation of pemphigus vulgaris.
A woman aged 57 years had conjunctivitis of the right eye since February 2003. It had started with a pinhead-sized blister at the margin of the upper eyelid and was progressive in nature. Intense redness of the right eye, lacrimation, and severe pain confined to the right forehead were the major complaints. She had been under medical care ever since, without any tangible outcome. Its refractory nature impelled the ophthalmologist to seek dermatologic consultation, for apparently the diagnosis seemed to have eluded the consultant. Accordingly, she reported on March 2, 2004 for the opinion of severe incessant itching, profuse lacrimation, and pain that was confined only to the right eye and forehead. The very fact that she had reported with continuous rubbing of the right eye re-enforced the suspicion of the episode being an exclusive expression of pemphigus vulgaris of the eye that probably was the reason for ineffectiveness of the drugs given thus far to her. Examination of the right eye was marked by intense inflammation of the palpebral and bulbar conjunctiva, apparent in the form of severe redness (Figure 1). The conjunctiva was studded with several scattered minute erosions. Corneal opacity and cataract were its accompaniment, whereas the left eye was normal. Mucous membrane of the oral cavity was thoroughly scanned for blister and/or erosions but was normal. The rest of the skin surface was also unaffected. Nikolsky's sign was elicited by asking the patient to rub the eye. In fact, this was responsible for periodic recurrence of the episode. Tzanck test was performed by preparing, fixing, and Giemsa staining the smear from one of the erosions over the conjunctiva. The stained slides were examined under oil-immersion, which revealed plentiful acantholytic cells characterized by large nucleus containing nucleoli and occupying almost the entire eosinophilic cytoplasm and basophilic cell wall. The two biopsies from the conjunctiva were also taken with the help of a 3-mm punch. One of the biopsies was subjected to serial sections. The sections were stained with hematoxylin and eosin stain and examined by light microscopy. The presence of split/cleavage in the epidermis (intraepidermal) above the basal (suprabasal) was cardinal. The cleavage was filled with acantholytic cells of characteristic morphology (vide supra) (Figure 2). The other biopsy was subjected to direct immunofluorescence and was found complementary to the preceding microscopic pathology. Routine investigations comprising total and differential leukocyte count, liver and kidney function tests, chest skiagram, and electrocardiogram were normal. The preceding findings were fairly conducive to form the diagnosis of erosive conjunctivitis emanating from pemphigus vulgaris. Accordingly, pulse therapy (intermittent high-dose) containing 100 mg dexamethasone in 5% glucose daily by slow IV infusion on 3 consecutive days, along with 500 mg of cyclophosphamide on Day 1 only followed by continuous low-dose 50 mg cyclophosphamide, was administered. The patient has since recovered completely and is now under surveillance.
Sehgal
Virendra N
VN
Dermato-Venereology (Skin-VD) Centre, Sehgal Nursing Home, Panchwati, Delhi, India. drsehgal@ndf.vsnl.net.in
Sharma
Sonal
S
Sardana
Kabir
K
D003231:Conjunctivitis / Q000209:etiology* / Q000473:pathology; D005260:Female; D006801:Humans; D008875:Middle Aged; D010392:Pemphigus / Q000150:complications*