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Introduction
Pemphigus vulgaris , bullous pemphigoid and other bullous
disorders may have eye involvement in various range of severity.
Patients of pemphigus vulgaris have skin and mucous membrane
involvement. Acantholysis is present in suprabasilar layer of
epithelium i.e. intraepithelial. On Biopsy, IgG can be confrmed by
immunoforesecence and in circulation antiepithelial autoantibodies
are found. There is a correlation with MHC II class genes. Majority
of Ashkenazi Jews having a diagnosis of pemphigus vulgaris have
DRB1 allelle. Pemphigus vulgaris is caused by auto-antibodies
against desmoglein(a glycoprotein that is present in desmosomes
,tight junctions between epithelial cells). Desmoglein has also been
found in the mucous membranes epithelial cell junction that describes
the involvement of eye in pemphigus vulgaris.
The initial and common presentation is infammation as seen in all
autoimmune phenomena. As pemphigus vulgaris is an autoimmune
and infammatory disease, it responds well to immunosuppressive
medication. Steroids are given intravenously/oral depending upon
condition of patient and severity of the disease. There are various
other immunosuppressive medicines e.g, azathioprine, cyclosporine,
cyclophosphamide mycophenolate mofetil etc. Medical literature
was sparse concerning ocular involvement in PV. In cooperation with
the International Pemphigus Foundation a survey was undertaken to
determine the prevalence and extent of ocular involvement in this rare
disease.
Methods
After taking written informed consent hundred patients fulflling
the inclusion criteria were included in the study and were questioned
and examined according to the International Pemphigus Questionnaire
that appeared in the Foundation’s website, discussion group and
quarterly newsletter. A total of 158 responses were collected in 1999.
Of these, 134 were from patients with PV and only those responses
were considered.
1–7
Results
This study included 100 pemphigus vulgaris patients who got
admitted in the Indoor of Dermatology department, Mayo hospital
during the year 2017 and 2018 upto month of May. The study was
conducted based upon a questionnaire that included history of disease,
its confrmation on biopsy and tzank smear and clinical examination
of all mucosal surfaces to establish prevalence and pattern of eye
involvement in the patients of pemphigus vulgaris. Eye involvement
was found in 32 (32%) patients of which the most common symptom
was found to be conjunctival redness which was found in 18 patients
(18%). Eyelid skin involvement was also quite common in 17 patients
(17%). Conjunctival discharge was found in 15% of patients. Blurring
of vision was found in only 4(4%) of patients. Pain was quite common
and noted in about 21 patients (21%).Eye involvement was found
bilaterally in 27 patients (90% of patients with eye involvement)
with 3 patients having unilateral eye involvement. Photophobia was
present in 7 (7%) patients. Bulbar conjunctival erosions were found
in 2 patients (2%).These all discussed problems were present during
the course of pemphigus vulgaris and not present before or during the
remission of the disease. These eye problems gradually got improved
as systemic disease got improved during the treatment with systemic
corticosteroids, immunosuppressants, local symptomatic treatment
and eye care (Table 1).
J Dermat Cosmetol. 2019;3(5):138‒140. 138
©2019 Butt et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which
permits unrestricted use, distribution, and build upon your work non-commercially.
Prevalence of ocular involvement in pemphigus
vulgaris patients in the dermatology department of
Mayo Hospital Lahore
Volume 3 Issue 5 - 2019
Ghazala Butt, M Arslan Ibrahim, Ahmad Raza,
Uzair Wazir, Umara Siddique, Uzma Malik
Department of Dermatology, Mayo Hospital, Pakistan
Correspondence: Ghazala Butt, Assistant professor of
Dermatology, Mayo Hospital, Pakistan,
Email
Received: February 21, 2019 | Published: October 31, 2019
Abstract
Pemphigus vulgaris (PV) is a bullous disease. It has autoimmune etiology. It mainly affects
skin along with mucous membranes. The frequency and pattern of involvement of eyes in
this disease is not clearly defned till now. We decided to conduct a study of hundred patients
with diagnosis of PV in a tertiary care hospital in Pakistan. The result showed a signifcant
number of patients with eye involvement, 32 out of 100 cases in the indoor department of
hospital i.e. (32%) showed eye involvement in a variable range of severity and presentation.
Most common symptom was conjunctivitis followed by palpebral conjunctival erosions.
Discharge was present in few patients that was either watery or mucoid due to the secondary
infection.
Keywords: pemphigus vulgaris, bullous disease, conjunctivitis, discharge
Journal of Dermatology & Cosmetology
Research Article
Open Access