A study on ocular manifestations of skin disorders - At a tertiary care centre

Neurofibromatosis

Neurofibromatosis is a disorder that primarily affects the cell growth of neural tissues. Eyelid neurofibromas tend to develp early in life. When it involves the upper lid, it frequently causes mechanical ptosis. Plexiform neurofibromas causes an S” shaped deformity of the upper lid.

Psoriasis

It is a common, chronic, disfiguring, inflammatory and proliferative condition of the skin. In which genetic and environmental influences have a critical role. It is equally common in males and females.1 It is more likely to appear between the ages of 15030 yrs.

Ocular lesions such as blepharitis and mucoid conjunctivitis are common. Keratitis in the form of raised, marginal, corneal infilterates is knwn to occur. Symblepheron and trichiasis have been reported.Of all the ocular features, conjunctivitis is the commonest ocular manifestation. In majority of the patients conjunctivitis follows the onset of psoriasis. In few cases, it is known to precede the disease.

Stevens- Johnson Syndrome

It is acute life threatening muco cutaneous reactions characterized by extensive necrosis and detatchment of the epidermis.

Herpes Zoster

Vericella zoster virus is member of the herpes virus family.2 During the course of varicella, vericella zoster virus passes into the contiguous endings of sensory nerves and is transported centripetaaly up the sensory fibres to the sensory ganglia. In the ganglia, the virus establishes a latent infection that persists for life.

Ocular cicatrical pemphigoid

Cicatrical pemphigoid is a rare chronic autoimmune sub epithelial blistering disease characterized by erosive lesons of the mucous membrane and skin that results in scarring.3

Patients typically describes the onset of painful, erosive, and/or blistering lesions on one or more mucosal surfaces. The two most frequently involved sites in these patients are the oral and conjuctival mucosae.4, 5

Besides, the ophthalmologist plays a crucial role in identifying the ocular manifestations and administering prompt treatment as, in several situations these (dermatological diseases may resolve but the ophthalmic complications that develop may persist and cause severe visual loss and blindness.

Study type

It was a observational Prospective study

Study population & duaration of the study

The materials for this study were collected from outpatients and inpatients of ophthalmology and dermatology departments from January 2019 to December 2019. 4 patients who were referred to the Department of ophthalmology from the department of dermatology and venereology were accepted. Different types of skin diseases were seen, five were studied in detail.

Methodology A preliminary ophthalmic examination was carried out with oblique illumination and slit lamp to assess the ocular involvement. External deformities and adnexal involvement were noted. Extraocular movements were assessed in all patients followed by a detailed check using slit lamp. Examination comprised of evaluation of the anterior segment, vitreous and posterior segment. Indirect ophthalmoscopy and bio microscopy was performed on all patients to examine for retinal involvement. Intraocular tension was recorded with Goldmann applanation tonometer. Other investigations like corneal staining and gonioscopy was done wherever situation demanded. Fundus photographs were taken if retinal involvement was present.

Conservative (medical) management for the patients with ocular involvement was advised. Conservative management included topical cycloplegics, topical steroids, topical antibiotics artificial tears and taping of lids.

Neurofabromatosis Type 1

Of 12 patients with NEUROFABROMATOSIS TYPE 1,5(41.67%) were males and 7(58.33%) were females (Table 2)

Among the 12 patients with NEUROFABROMATOSIS TYPE 1,6(50%) patients belonged to the age group of <20 years, 2(16.67%) patients belonged to the age group of 41-60 and 1(8.3%) patient was>60 years of age (Table 3)

Of the 12 patients with NEUROFABROMATOSIS TYPE 1 in our study we found that 1(8.3%) patient had plexiform neurofibroma and nodular lesions were found in 11 (91.7%) patients.

Figure 1

Distribution of neurofibromas

Psoriasis

Out of the 10 patients with psoriasis, 6(60%) patients were males males and 4(40%) patients were females (Table 4)

Of the 10 patients with psoriasis, the commonest age group affected was 21-40 years (Table 5).

Among the 10 patients with psoriasis, blepharonjunctivitis was found to be the most common manifestation seen in (50%) patients. 3 (30%) patients had keratoconjunctivitis sicca and2 (20%) patients had chronic anterior uveitis (Figure 2)

Stevens-Johnson Syndrome

Out of 8 patients with Stevens-Johnson Syndrome, 5(62.5%) patients were males and 3 (37.5%) patients were females (Table 6).

The following table shows the age distribution of patients with STEVENS-JOHNSON SYNDROME (Table 7).

All patients with Stevens-Johnson Syndrome had bilateral involvement. Bilateral symmetrical involvement was seen in 7 (87.5%) patients and bilateral,asymmetrical involvement was found in 1(12.5%) patient (chart 4)

Figure 2

In our study, the most common etiological agent for Stevens-Johnson Syndrome was drugs,found in 5 (62.5%) patients .2(25%) patients gave a previous history of viral fever with no history of drug injection. No definitive cause was ascertained in 1(12.5%) patient (Table 8).

On slit-lamp examination ,the lid, conjunctiva and corneal complications were seen in 5(62.5%).2(25%) and 1(12.5%), respectively aong the 8 patients with Stevens-Johnson Syndrome.

Herpes zoster ophthalmicus

Of the 6 patients with Herpes Zoster Ophthalmicus, 4 (66.67%) patients were males and 2(33.33%) patients were females (Table 9).

The table below shows the age distribution of patients with HZO in our study (Table 10).

In our study, pain was the commonest prodromal symptom noticed in 3(50%) patients,followed by burning sensatiom in 2(33.33%) patients and only 1 (16.67%) patient experienced fever.

All patients with HERPES ZOSTER OPHTHALMICUS had unilateral involvement. No definite bilaterality was noticed in our study.

Ocular cicatricl pemphigoid

Of 4 patients with Ocular Cicatricl Pemphigoid, 3(75%) patients were females and 1(25%) patient was male (Table 11).

The following table shows the age distribution of patients with ocular cicatricial pemphigoid in our study (Table 12).

Among 4 patients with ocular cicatricial pemphigoid we found that 2 (50%) presented with conjunctivitis, 1(25%) patient presented with entropion and 1 (25%) patient presented with entropion and trichiasis .

Neurofibromatosis type-1

Of the 12 patients with Neurofibromatosis type-1, 5(41.67) were males and 7 (58.33%) were females. In a study done by Huson sm and Harper, there was a slight female preponderance.6 This is in contrast to a study conducted by Odebode, in which there was a definite male preponderance affecting 60 males and 38 females with a total of 98 patients.7

Among the 12 patients with neurofibromatosis type - I in our study, 6 (50%) patients belonged to the age group of 20-40 years, 3 (25%) patients belonged to the age group of < = years, 2 (16.67%) patients belonged to the age group of 41-60 years and 1(8. 3%) patient was> 60 years of age. The commonest age group with which patients presented to us was between 20-40 years. On comparing this with the study done by Genet et al, the commonest age group affected was > 20 years of age.8 Of the 12 patients .with Neurofibromatosis type-1, in our study we found that 1 (8.3%) patient had plexiform neurofibroma and nodular lesions were found in 11(91.7%) patients. However, a study was conducted by ALO and Massobrio showed that 20% of the patients had plexiform neurofibromas.9

Lisch nodules were found to be the commonest manifestation among all patients with neurofibromatosis type 1, in our study. This finding was supported by the studies done by Huson et al.10 Riccardi 11 and by Goeker^`12 rare findings such as conjuctival freckles and sphenoid wing dysplasia were seen In 1 patient as found earlier in a study conducted by premalatha.13 A similar study was done by Mirowitz et al and it confirmed the above findings.14

Psoriasis

Of the 10 patients with Psoriasis, 6 (60%) patients were males and 4 (40%) were females giving a male to female ratio of 1.5:1. This finding was found to be in concordance with a German study done by Henseler et al,15 where the ratio 1.74:1. Smith AE also noted the male preponderance.16 The age of the patients ranged from 16 years to 64 years. The commonest age group affected was 21 — 40 years followed by 41 — 60 years. Farber and Nall 1 found that the average age of onset was 28 years, while in the study on psoriasis done in China by YuiYie,17 the average age onset was 36 years. in a recent U.K based study done by Nevitt and Hutchinson.18 the mean age of onset was 3 years with the mode in the second decade, This is in contrast to a study done by LomhoIt19 who reported the average age of onset as 12 years.

Among the 10 people with psoriasis, in our study we found blepharoconjuctivitis to be the most common manifestation seen in 5 (50%) patients followed by 3(30%) patients with keratoconjuctivitis sicc. Only 2 (20%) patients had chronic anterior uvetis. Catsaru-Catsari 20 found that blephariconjuctivitis and keratoconjuctivitis sicca were the most common ocular manifestations of psoriasis however. offers et al found in their study that uveitis and blepharitis are the most common ocular manifestation of psoriasis. However, Yamamoto21 et al found in their study that uveitis and blepharitus are the most common ocular manifestation of psoriasis followed dry eye.

Stevens- Johnson syndrome

in our study, out of 8 patients with Stevens- Johnson syndrome. 5 (62.5%) patients were males 3 (33.5%) patients Were females. We found a male preponderance in our study.This is in concordance with the study done by Letko et al 22 in 2005 in which they noted a clear male dominance.

However, this is an contrast to a study done on ophthalmic complications and management of Stevens Johnsons Syndrome by Kompella, 23 in which they found a female predominance.

The common age group affected with Stevens-Johnson syndrome is between 21-40 years our study. The study done by Kompella also found that majority of the Patients (55.78%) were between 20 and 40 years of age.23 All patients with Stevens-johnson syndrome had bilateral involvement. Bilateral, symmetflcal was CCIi m 7 (87.5%) patients and bilateral, asymmetrical involovement was found in 1 (12.5) Patient. In the study done by Kompella all the patients with Stevens Johnson syndrome had bilateral involvement and most had bilateral, symmetrical presentation. 23 a study done by Wilkins J and Morrison L also showed similar findings.24

In our study, the most common etiological agent for Stevens- Johnson syndrome was drugs found in 5(62.6%) patients.2(25%) patients gave a previous history of viral fever with no history of drug intake, no definitive cause was ascertained in 1(12.5%) patient. The most commonly identified possible causative factor was drugs in 52 (51.89%) patients, 3(3.15%) patients had a history of viral fever preceding the onset and no definitive causative factor was identified in 37 (38.84%) patients according to the study done by Kompella 23 Drugs were found to be the most common etiological agent in the study by letko E et al and Wilkins J and Morrison also.24

In our study, the patients with Steven Johnson syndrome on slit lamp examination; the complications seen on the lids (62.5%) were crusting, entropion. and trichiasis. The conjunctival complications (25%) found were conjunctivitis and symblepharon cratopathy was seen in 12.5% due to entropion and tnchiasis. In the study done by Kompella.23 the lid abnormalities were observed in 87 (91.51%), conjunctival abnormalities in 92 (6.84%) and corneal complications in 93 (97.89%).23

Herpes zoster ophthalmicus

Of the 6 patients with Herpes Zoster Ophthalmicus, 4 (66.67%) patients were male and 2 (33.33%) were female giving a male to female ratio of 2:1. This found to be in concordance with a study done by Dubey et al in 2005 in South India where the ratio was l.84:I. 25 Chaudhary, Sehgal, Nigarn and Mathur also noted this, male preponderance.26, 27, 28, 29 The commonest age of presentation among the patients with herpes zoster ophthalmicus in our study, was found to be between 21-40 years. 3 (50%) presented within 21-40 years of age followed by 2 (33.33%) patients who presented within 41-60 years of age and 1 (1 6.67%) was less than 20 years of age. In the study by Dubey et al, the mean age of presentation was 37.65 years with a range of 2-77 years in their study.25 Chaudhary found a high incidence in the 2nd and 3rd decade,26 whereas Sehgal found a high incidence in the 4^th and 5^th decade.27 In our study, pain was the commonest prodromal symptom noticed in 3 (50%) patients, followed by burning sensation in 2 (33.33%) patients and only 1 (16.67%) patient experienced fever before the eruptions. Dubey et al found 90.65% to have pain m the prodromal period followed by paresthesia in 23.36% and itching in 19.62%63. No definite laterality was noted in our study. This is in concordance to most of the prevailing studies.25, 26, 27, 28

Ocular cicatricial pemphigoid

Of the 4 patients with OCULAR CICATRICIAL PEMPHIGOID, 3 (75%) patients sere ftma1es and 1 (25%) patient was male giving a female to male ratio of 3:1. Similar findings were seen in a study done by Egan CA and Yancey KB in which they had found a female to male ratio of 2.6:1.4 This is in contrast to a study done by John Chang & Peter in 2005, in which there was only a slight female prepondance.30

The commonest age group in which the patient ocular cicatrical pemphigoid presents was >60 years of age. Similar findings were noted by John H chang & peter J mc Cluskey in their study.30 Among 4 patients with ocular cicatricial pemphigoid. We found that 2 (50%) patients presented with conhjuctivitus, 1 (25%) patient presented with entropion and 1 (25%) patient presented with entropion trichiasis. Similar findings were observed in the study done by Stephen foster in which he observed that conjuctival abnormalities were the most common manifestation among the patients presenting with ocular cicatricial pemphigoid. Chang and Peter found that lid abnormalities (62.5%)were more common.30