Vol.13  No. 1,  October 2003

INCIDENCE AND PATTERN OF OCULAR CHANGES IN 
IMMUNOLOGICAL DISEASES OF SKIN

 Dr. R. K. Grewal,  Dr. Sudhir Salhotra, Dr. Nidhi S. Mittal

INTRODUCTION

Immunology has always been involved in Ophthalmology. Skin and eye are closely connected as skin, mucous membranes and corneal epithelium are derived from common embryological ancestry.  Juxtaposition between surface of the eye and edge of the eyelids can result in ocular damage when a particular disorder is affecting the skin of the eyelids up to the lid margin.  Certain immune disorders, such as cicatricial pemphigoid may result in an autoimmune attack of both ocular structures and skin.  The importance of these shared properties between the eye and skin is relatively under appreciated by ophthalmologists, particularly the ability to diagnose systemic disease on the basis of its ocular manifestations.

MATERIAL AND METHODS

The present study was a prospective observational study of 100 patients between July 2000 to April 2002, diagnosed as having immunological disease of the skin by Department of Dermatology and were referred randomly to Department of Ophthalmology, for ocular involvement.  Diagnosis of immunological disease was made clinically and if required was confirmed by biopsy or collagen profile.

For study purposes, immunological diseases of skin were broadly classified into two types:-

A.    Immunobullous disorders:-

It is further classified into:-

1. Pemphigus

2. Pemphigoid

3. Stevens-Johnson syndrome

4. Epidermolysis bullosa

5. Dermatitis herpetiformis

B.    Connective tissue disorders:-

It is further classified into:-

1.    Lupus erythematosus

• Discoid lupus erythematosus

• Subacute cutaneous lupus erythematosus

• Systemic lupus erythematosus

2.    Systemic sclerosis
3.    Dermatomyositis and polymyositis
4.    Rheumatoid arthritis
5.    Sjogren's syndrome

EXCLUSION CRITERIA

Patients who were currently taking corticosteroids were excluded from the present study.

PATIENT EVALUATION

Each patient was thoroughly examined for ocular involvement. Distant and near visual acuity was assessed by standard Snellen's and Jaeger's chart.  Anterior segment examination was done with torch light and slit lamp and in immobile patients with high magnifications lens (corneal loupe -10X).  Patients of connective tissue disease were screened for evidence of keratoconjunctivits sicca by Schirmer's tear using standardized filter paper strips.  All patients with reduced tear flow had a drop of Rose Bengal 1% instilled into eye , to look for the presence of dead or dying cells and excess mucus.  Intra ocular pressure was measured with applantation / Schiotz's tonometers.  Fundus examination was done with both direct and indirect ophthalmoscope. The details of each patient were recorded on proforma and the data was compiled, tabulated and analysed.

OBSERVATIONS AND RESULTS

Out of 100 patients studied, 69 were females and 31 were males, the mean age being 39.90±5.41 years.

Table 1

INCIDENCE OF OCULAR INVOLVEMENT ACCORDING TO DERMATOLOGICAL DIAGNOSIS

Dermatological Diagnosis	No. of patients of various skin diseases	No. of patients with various ocular lesions	%age
Pemphigus vulgaris (PV) Pemphigus foliaceous (PR) Bullous pemphigoid (BP) Cicatricial pemphigoid (CP) Stevens-Johnson Syndrome (SJS) Toxic epidermal necrolysis (TEN) Epidermolysis bullosa (EB) Dermatitis herpetiformis (DH) Discoid lupus erythematosus (DLE) Systemic lupus erythematosus (SLE) Systemic sclerosis (SS) Polymyositis (PY) Rheumatoid Arthritis ((RA) Sjogren's syndrome (SJ)	15 06 03 04 15 03 03 02 04 13 14 02 14 02	08 04 01 02 14 00 01 00 00 00 10 00 06 02	53.33 66.67 33.33 50.00 93.33 00.00 33.33 00.00 00.00 00.00 71.43 00.00 42.86 100.00

 Out of 100  patients of immunological diseases of skin, 48 had ocular lesions giving the incidence of 48%.  Although various studies regarding ocular involvement in specific immunological diseases of skin have been reported but no study has given the overall incidence of ocular involvement in immunological diseases of the skin.

PATTERN OF OCULAR LESIONS

The spectrum of the ocular changes in each disease has been depicted in Table - II

Table - II

OCULAR LESIONS OBSERVED IN PEMPHIGUS VULGARIS

Ocular lesions	Male	Female	Total	%age
Erosion of lid margins	-	02	02	13.33
Pterygium	-	01	01	06.67
Symblepharon	-	02	02	13.33
Conjunctivitis	02	06	08	53.33

OCULAR LESIONS OBSERVED IN PEMPHIGUS FOLIACEOUS

Ocular lesions	Male	Female	Total	%age
Erosion of lid margins	01	02	03	50.00
Chalazion	01	-	01	16.67
Symblepharon	-	02	02	33.33
Conjunctivitis	01	02	03	50.00

Ocular lesions were found in 53.33% of patients with pemphigus vulgaris (PV) and in 66.67% of patients with pemphigus foliaceus (PF). Most common lesion was purulent conjunctivitis in both types of pemphigus, 53.33% in PV and 50% in PF, which has also been reported as to be the most typical lesion by Michael et al¹ and Venu Gopal and Sam Raj².

OCULAR LESIONS IN BULLOUS PEMPHIGOID

Ocular lesions	Male	Female	Total	%age
Entropion	-	01	01	33.33
Shallow fornix	-	01	01	33.33
Corneal Opacity	-	01	01	33.33
Cataract	-	01	01	33.33

OCULAR LESIONS IN CICATRICIAL PEMPHIGOID

Ocular lesions	Male	Female	Total	%age
Entropion	-	01	01	25.00
Shallow fornix	-	02	02	50.00
Corneal opacity	-	02	02	50.00

Ocular lesions were found in 33.33% of patients with bullous pemphigoid (BP) and in 50% of patients with cicatricial pemphigoid (CP), thereby being more common in CP than in BP as also seen in the study of Peggy et al³.  Shallow fornix was seen in 50% of patients with CP and in 33.33% of patients with BP.  Mark et al⁴ had reported shallow fornix in 59% of cases and in 43% by Peggy has reported in 43% et al³.  Corneal opacity was seen in 50% of patients with CP and in 33.33% of patients with BP.

OCULAR LESIONS IN STEVENS JOHNSON SYNDROME

Ocular lesions	Male	Female	Total	%age
Erosion of lid margins	05	01	06	40.00
Symblepharon	03	01	04	26.67
Corneal ulcer	01	-	01	06.67
Cataract	-	02	02	13.33
Conjunctivitis	09	04	13	86.67
Purulent	07	03	10	66.67
Pseudomembranous	02	01	03	20.00

Ocular lesions were found in 93.33% of patients with Stevens- Johnson syndrome (SJS). Most common ocular lesion was found to be conjunctivitis ((86.67%) with SJS.  Arnall Patz⁶ reported ocular involvement in 91.36% of patients and conjunctivitis in 77.8% of patients.  Arstikaitis MJ⁷, reported ocular involvement in 100% as well as conjunctivitis in 100% of SJS patients.

EPIDERMOLYSIS BULLOSA

VARIOUS OCULAR LESIONS IN EPIDERMOLYSIS BULLOSA

Ocular lesions	Male	Female	Total	%age
Corneal opacity	-	01	01	33.33
Cataract	-	01	01	33.33

OCULAR LESIONS IN SYSTEMIC SCLEROSIS

Ocular lesions	Male	Female	Total	%age
Tightening of lids	02	07	09	64.29
Pterygium	-	01	01	07.14
Shallow fornix	01	01	02	14.29
Keratoconjunctivitis sicca	-	03	03	21.43
Cataract	-	01	01	07.14

Ocular lesions were found in 71.43% of patients with systemic sclerosis (SS).

Tightening of lids were seen in 64.29% of patients while Horna⁸ reported in 65.22% of patients and West and Barnet⁹ in 28.9% of patients.  Keratoconjunctivitis sicca was found in 21.43% of patients.  Horna C⁸ found keratoconjunctivitis in 30.43%, West and Barnet⁹ in 36.84% of his patients and Reena¹⁰ found it in 30% of cases.

RHEUMATOID ARTHRITIS

OCULAR LESIONS IN RHEUMATOID ARTHRITIS

Ocular lesions	Male	Female	Total	%age
Secleritis	01	-	01	07.14
Keratoconjunctivitis sicca	-	04	04	28.57
Uveitis	01	-	01	7.14
Cataract	-	02	02	14.29

Ocular lesions were found in 42.86% of patients with rheumatoid arthritis (RA), which is comparable with studies by Reddy  et al¹¹, who found the incidence to be 39% and Williamson¹²,  who found the incidence to be 20.8%.

Most common ocular lesion was keratoconjunctivitis sicca seen in 28.57% of cases.  Reddy et al¹¹, found the incidence to be 29%.  Incidence of uveitis was seen in 7.14% of patients.  Reddy et al¹¹, found the incidence of uveitis to be 4%.  Incidence of scleritis was found in 7.14% of patients, which is comparable to a study by Jayson and Jones¹³, who found the incidence to be 6.3% in patients of RA.

OCULAR LESIONS IN SJOGREN'S SYNDROME

Ocular lesions	Male	Female	Total	%age
Keratoconjunctivitis sicca	-	02	02	100.00

Ocular lesion ( Keratoconjunctivitis sicca ) was found in both the patients of Sjogren's syndrome (SJ) examined, thus the incidence was 100%, which is comparable to the studies by Erica et al¹⁴ and Kruize AA¹⁵.

No ocular lesions were seen in the patients of Toxic Epidermal Necrolysis, Dermatitis Herpetiformis, discoid lupus erythematosus and Polymyositis.

CONCLUSIONS

A significant number of patients having immunological diseases of skin had ocular involvement (48%).  Ocular involvement in these cases depends upon the severity of disease, being more common in severe and hospitalized patients, and also on type of immunological disease of skin.  Since the importance of shared properties between the eye and skin is relatively under appreciated by both Ophthalmologists and Dermatologists, so it is important to emphasize this connection and its importance, particularly the ability to diagnose systemic disease on the basis of its ocular manifestations. Hence, every patient having immunological disease skin should be carefully examined for any ocular involvement at the earliest so that timely management is instituted and ocular complications prevented.

 REFERENCES

1. Michael E,  Nelson and Lan G Rennie.  Symmetrical lid margin erosions  A conditions specific to   pemphigus vulgaris.  Arch Ophthalmol 1988; 106: 1652.

2. Venu Gopal N S and Sam Raj D. Ocular manifestations in bullous dermatoses. Ind J Ophthalmol 1997; 25: 13.

3. Peggy A F, Venning V A ,Wojnarowska F et al .  Conjunctival involvement in cicatricial and bullous pemphigoid A clinical and immunopathological study.  Br J Ophthalmol 1989; 73 : 52.

4. Mark J, Elder Wolfgang B,  Jonathan L,  John KG Dart.  Progression of disease in ocular cicatricial pemphigoid.  Br J Ophthalmol 1996; 80: 292-296.

5. Arnd H,  Jorg S et al.  Eosinophil granule protein expressed in ocular cicatricial pemphigoid.  Br J Ophthalmol 1998;  82: 312.

6. Arnall P. Ocular involvement in erythema multiforme.  Arch Ophthalmol 1949; 9:244.

7. Arstikaitis MJ.  Ocular aftermath of Stevens  Johnson syndrome Review of 33 cases.  Arch Ophthalmol 1973; 90: 376-379.

8. Horna EC. Ophthalmic manifestations of progressive systemic sclerosis.  Br J Ophthalmol 1969; 53 : 388.

9. West RH and Barnett AJ. Ocular involvement in scleroderma. Br J Ophthalmol 1985; 63: 845. 

10. Reena A. Ocular involvement in a case of scleroderma. Ind J Ophthalmol 1985; 33: 71-72.

11. Reddy SC, Gupta SD , Deodhar SD. Ocular manifestations of rheumatoid arthritis 1977; 25: 20-26.

12. Williamson J. Incidence of eye disease in cases of connective tissue disease.  Trans Ophthalmol Soc UK 1974; 94: 742.

13. Jayson MIV, Jones DEP. Scleritis and rheumatoid arthritis.  Ann Rheum Dis 1971; 30: 343

14. Erica M, Anne J et al. Conjunctival biopsy in scleroderma and primary Sjogren's syndrome.  Am J Ophthalmol 1993; 115: 792.

15. Kruize AA, Hene RJ  et al.  Long term course of tear gland function in patients with keratoconjunctivitis sicca and Sjogren's syndrome.  Br J Ophthalmol 1997; 81: 435-438.

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Department of Ophthalmology,
Dayanand Medical College, Ludhiana.

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