|Year : 1977 | Volume
| Issue : 1 | Page : 13-17
Ocular manifestations in bullous dermatoses
NS Venugopal, D Sam Raj
Government Ophthalmic Hospital, Egmore, Madras, India
N S Venugopal
Superintendent, Government Ophthalmic Hospital, Egmore, Madras-600 008
|How to cite this article:|
Venugopal N S, Raj D S. Ocular manifestations in bullous dermatoses. Indian J Ophthalmol 1977;25:13-7
Pemphigus vulgaris is a rare, acute or chronic, usually fatal disease, characterized by a generalised eruption of bullae, containing, serous, haemarrhagic or purulent material on a previously normal skin. Mucus membrane complications may occur simultaneously with the skin eruption in the mouth and throat which often interfere with mastication and swallowing and in late state the mucous-membrane of the eyes may be involved. Death usually ensue in a matter of weeks from sepsis or broncho-pneumonia. The conjunctiva is nearly always involved and the lesions in both acute and chronic types are similar. They consist of bullae which rupture rapidly leaving ulcerated base. The bullae are transient. It is often difficult to find a case with an intact bullae. In acute cases death supervenes before cicatricial changes can develop but in the chronic cases repeated bullae formation leads to conjunctival cicatrization (symblepharon) with progressive contraction of the conjunctival sac, always most marked in the lower fornix.
Chronic Ocular Pemphigus: (Benign mucous membrane pemphigoid) may occur either with or without associated mucous membrane lesions of the nose, and throat and in the absence of skin changes. When there are no mouth changes the diagnosis is difficult but it can be made on the basis of bullous lesions on the conjunctiva with progressive cicatrisation which is most marked in the lower fornix over a period of month. The low grade conjunctival eosinophilia of pemphigus is usual in differentiating it from erythema multiforme bullosum. The prognosis is poor in acute case and death results in a matter of weeks (in some cases steriod give temporary effect). Chronic cases are characterised by long remissions and exacerbation until death finally ensues Death may not occur however until vision has been greatly diminished or lost through cicatrization of the conjunctiva and cornea. Loss of tear function due to cicatrization in the upper fornix results in keratinization of the corneal epithelium and contributes to loss of vision.
Etiology is still uncertain for both pemphigus and ocular pemphigus but the isolation of a virus from Pemphigus Vulgaris has been claimed, current reports suggest an autoimmune aetiology.
Treatment of both conditions is unsuccessful. Steroids produce temporary effect but are of no use for ocular conditions. Antibiotics for secondary infections, artificial tears where there is loss of tear function, are of same help.
Erythema Multiforme bullosum is an acute inflammatory disease with multiform eruptions and large bullae surrounded by red haloes. In contrast to pemphigus the disease is of short duration and the prognosis is good. Symmetrical eruption of bluish red macules, papules and bullae are seen. Unlike the lesions of Pemphigus the bullae do not appear to arise from the normal skin, but are either surrounded by a red halo or develop from the edge of an erythematous patch. Simultaneous lesions of the lips, mouth and conjunctiva are almost the rule. It usually attacks children and young adults whereas pemphigus and ocular pemphigoid usually affect the older age group.
It may mainfest as (1) Simple catarrhal conjunctivitis or (2) Purulent conjunctivitis or (3) as a severe destructive membraneous conjunctivitis which leads to cicatrization and even to destruction of the globe-described as Stevens Johnson syndrome.
In majority of cases it has followed the use of drugs. Virus and bacterial infections may be responsible in some cases. In other cases no definite cause can be detected.
The treatment is symptomatic, antibiotics for secondary infection and steroid are useful.
| Pemphigus|| |
Case 1:-55 years old male was admitted in the Skin Ward, Government General Hospital for multiple skin lesions all over the body of 20 days duration. There was erythema progressing to vesicle and bullae on the face, trunk, back and extremties resulting in scaling [Figure - 1]
Eyes showed conjunctival congestion with purulent discharge without bullae formation.
Investigation:---Total and differential counts-6000/cmm.,- P54 L36 E8 M2 RBC2.5 millionlcms. ESR -½ hours 6 mm. and 1 hour -- 15 mm. Urine-albumin and sugar-Nil- Blood sugar 125 mg.%- Blood Urea - 23 mg. % B.P.-140/90.
Patient was treated with prednisolone and tetracycline internally and methylene blue in zinc cream for external use and terramycine ointment for eye.
Case 2:-A 28 year old male was admitted in Skin ward, Government Ophthalmic Hospital for pain and diminished vision with redness in both eyes of 1½ years duration.
On examination his skin was normal and no past history of any cutaneous eruption.
There was conjunctival congestion with frothy secretion in both eyes, symblepharon in lower fornix with subconjunctival cyst in the left eye and superficial vascularisation around the periphery on both cornea which were hazy. The vision was reduced to 2/60 in right side and 5/60 in left side.
Histopatbologically the conjunctival cyst with a bit of symblepharon showed subepithelial bullae with underlying stromal hyaline changes [Figure - 2]. A diagnosis of benign mucous membrane pemphigoid or ocular pemphigus was entertained the above findings.
Case 3 :-A 15 year old male was admitted in Government Ophthalmic Hospital for defective vision on both eyes of one year duration.
He gave no history of skin infection at any time.
On examination both the conjunctivae were congested and there was symblepharon [Figure - 3] in the lower fornices of both eyes with corneal opacity. No details could Le made out in the anterior chamber. Vision was reduced to perception of light in both eyes.
Case 4:-A male patient aged 48 years was admitted for generalized vesicular bullous lesion all over the body, face and throat of 2 years duration.
On examination the eyelids were normal. Conjunctiva was dry, cornea hazy and there was total symblepharon. The vision was reduced to hand movement.
Right eye:-The right eye was removed three years ago in Tiruchirapalli Private Hospital (He had a similar attack of skin condition with pain and redness on the right eye for which the eye was removed-).
Investigation:-BP-110190, TC-6600/cmm, DC -P61 L32, E7, RBC-3.25 million) cmm-Hb-70%, ESR-}hour 8 mm. and I hour-20mm., Urine albumin and sugar nil.
He was treated with prednisolone and tetracycline orally and gantrisin eye drops locally.
Comment:-The case no. I was examined at an early stage of ocular involvement of the conjunctiva before the bullae formations. In this case the reduction of the vision was due to senile cataract,
Case no. 2:-As per history there was no skin or mucuous membrane manifestations of pemphigus but the symblepharon in the lower fornix and the subconjunctival cyst biopsy proved histopathologically a typical picture of ocular pemphigus.
Case no. 3:-He showed the same picture as case no. 2 i.e. ocular involvement such as symblepharon, hazy cornea (keratinization) without the skin or mucous membrane lesion.
Case no. 4:-showed the ocular pemphigus as the patient lost one eye (right eye) which was removed probably due to secondary infection and perforation and total symblepharon in the left eye with reduction of vision to hand movements as a result of kertinisation of the cornea.
4 cases of ocular pemphigus were diagnosed on clinical grounds and historathological study was made in one case (No.2) which showed typical picture of ocular pemphigus.
| Erythema Multiformae Bullosum|| |
Case I:- A male 40 years old was admitted in Government General Hospital in Skin Department for drug eruption following ingestion of some tablet for head-ache 25 days before the eruption.
On examination he had generalized erythamatous purpuric papules and bullae all over the body, skin and oral cavity including his throat. The eyes were swollen, conjunctiva congested and there was severe epiphora. He was treated with anti-histamine, steroids and liquid paraffin externally.
Patient was found to be allergic to many antibiotics like chloramphenicol and tetracycline.
After 15 days he recovered from his skin lesion but left with pigmented macules which persist even now.
As a sequelae he developed symblepharon both eyes with loss of vision in the right eye (No PL), due to hazy cornea and low intraocular tension and eye passed in to the stage of phthisis bulbi-Steven's Johnson Syndrome  [Figure - 4].
In the left eye the symblepharon formed only in the lateral part and the cornea was clear, vision was 6/9 with fundus normal.
Case 2:-- A female aged 68 years was admitted in Skin Department, Government General Hospital for generalized bullous lesion of 2 months duration involving face, trunk [Figure - 5] and limbs of varying size from 1 cm. to 2 cm., tense with clear fluid and healing with hypo and depigmented macules. The mucus membrane was normal.
Right eye was swollen with conjunctival congestion and chemosis with purulent discharge.
There was mild conjunctival congestion with purulent discharge in left eye.
The cornea and iris were normal, the pupils were dilated and reacted well to light, tension was notmal in both eyes. The dilatation of the pupils were due to mature cataract on both sides-(senile cataract) [Figure - 6] and the vision was reduced to hand movement on right side and to 1 meter on the left side. Fundus--no view could be seen due to lens changes.
Investigations:- Total and differential Count 6000/ cm.,-P55 L35E 10-RBC 3.25 million per cmm. Hb. 65%-ESR ½ hour 3 mm- 1 hour 6 mm- Blood sugar 93 mg.%. Patient was put on cortisone and antibiotics and for the skin lesion Zinc cream, and for eye Soframycin Eye Ointment. The patient recovered well and was discharged on 1-12-1973.
Case 3:- A man 50 years was admitted in Eye Hospital for defective vision of three months duration.
Three months before he had redness on : both eyes with fever followed by the bullae formation all over the body which started after taking some medicines for fever and later on he was treated outside hospital.
On examination he had dark pigmented areas all over the body [Figure - 6] with the following eye changes.
Right Eye:- There was Symblepharon in the lower fornix; conjunctiva and cornea were dry and hazy, Pupil was dilated and reacting sluggishly to light, vision was reduced was to 6/60, lens showed early cataract changes and anterior chamber was normal.
Left Eye:- There was adherent leucoma at 6'O clock position with symblepharon in the lower fornix [Figure - 7], conjunctiva and cornea were dry and cornea was hazy, the vision was reduced to 2/60, lens showed early cataract changes. Anterior chamber was normal except that at 6 `O Clock position where it was shallow.
Comments :-3 cases of Erythema Multiforme were recorded. In one there was destruction of the globe on the right side due severity of the conditions and there was adherent leucoma as a result of corneal perforation (No. 2). In all the cases there were cicatrization in the upper fornix resulting in keratinization of the corneal epithelium and thus contributed to the loss of vision. In two cases (i.e. I & 3) the bullous lesion developed after ingestion of some drugs which was aetiologifactor for the condition. Case 1 and 2 were admitted in an acute stage responded well to anti-histamines and steroids. The eye conditions were treated with Gantrisin drop as a substitute for artificial tears which contains methyl cellulose.
| Acknowledgement|| |
We sincerely thank Prof. A.S. Thambiah, Professor of Dermatology, Madras Medical College, Madras for permission to publish the cases seen in Skin Department, Government General Hospital, Madras.
| References|| |
|1.||Sorsby Arnold. Modern Ophthalmology. 2. 56566, Buttermenth London. |
|2.||Crossmann, 1946 Hiner J., Ophthal., 29., 1146-49, Conjunctivitis Associated Unit E.M.F.-1941, |
|3.||Koke, Arch. of Ophthal., 28, 78-88 Conjunctivitis in Erythemo Exsuation Mulliforme. |
|4.||Newell, and Greetham, 1946, Amar J. Ophthal., 29., 1426-39, Pemphigus Conjunctiva. |
|5.||Parsons. Diseases of the Eye 14th Edition Appendix 11, 568. |
[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6], [Figure - 7]