Discussion
Behcet’s disease (BD) is a rare, systemic disorder initially described
by Hulusi Behcet, a Turkish dermatologist, as a triad of uveitis and
recurrent oral and genital ulcers. It is prevalent in people of
Mediterranean and Middle East countries and less frequent in the Indian
subcontinent. The disease usually manifests during the 3rd and 4th
decade of their life with male predominance (7,8). We present a case
report of a male serving soldier from Nepal in his fourth decade of
life. Testosterone may play a role in neutrophil and Th-1 cell
activation. This could explain why male patients have a more severe case
of BD(9).
The disease raises the mortality rate, particularly in young male
patients. Large vessel involvement (pulmonary artery aneurysm),
neurological involvement, gastrointestinal system involvement, and
cardiac involvement are the most common causes of mortality(10). The
exact cause of Behcet’s Disease is unknown and is thought to be
multifactorial. The MHC class I region, which includes HLA-B*51,
contains the strongest genetic risk factor for BD. There is a 5. 78-fold
higher chance of getting BD for individuals with the HLA-B*51/B5 allele
than those who did not have this gene (11). Other potential factors can
be microbial factors as oral aphthous ulcer typically precede the
systemic presentations and occurs before every recurrence of the
disease. This case was positive for HLA-B51, which demonstrated probable
genetic cause for the occurrence of the disease. Although the
significance of HLA-B*51 is well established, it is found to be positive
in roughly 60% of patients with Bechet disease. HLA-B*51’s role in the
genetic predisposition to the Behcet disease is around 12–19% (12).
There is no confirmatory test for diagnosing Behcet’s Disease as the
history and clinical picture are often sufficient for the diagnosis.
However, diagnostic criteria proposed by an International Study Group
are used for research purposes and clinical purposes too. According to
International Study Group criteria for Behcet’s Disease (13), there must
be Recurrent oral ulcerations (Minor aphthous, major aphthous, or
herpetiform ulcerations which recurred at least 3 times in 12 month
period). In addition, two of the following criteria must be met:
Recurrent genital Ulcerations, Eye lesions( Uveitis, cells in vitreous
on slit-lamp examination, or retinal vasculitis), Skin lesions (Erythema
nodosum, pseudofolliculitis, papulopustular lesions), and Positive
Pathergy test.
The International Criteria for Behcet’s Disease(ICBD)(14) are proposed
to assist earlier diagnosis as ISG clinical diagnosis has low
sensitivity. According to signs and symptoms, the International Criteria
for Behcet’s Disease has a scoring system; 2 points each for ocular
lesions, genital apthosis, and oral apthosis. Each point for skin
lesions, neurological manifestations, and vascular manifestations, and
Positive pathergy test. A score of more than or equals to 4 indicates
Behcet’s diagnosis. Our patient had ocular lesions, genital apthosis,
oral apthosis, skin lesions, vascular manifestations ( fundal
arteritis), and a positive pathergy test; hence ICBD score was
calculated to be 8, which is strongly suggestive of Behcet’s Disease.
BD responds well to steroids. When vital organs are affected, a
combination of corticosteroids and immunosuppressant medications is
recommended. Our patient responded very well to the steroids. Due to
relapsing ocular symptoms, he was placed on adalimumab (TNF-α
antagonist). TNF-α, a pro-inflammatory cytokine, is involved in the
autoimmune response, inflammation induction, and maintenance. Therefore,
it becomes a crucial target molecule in the disease’s treatment(15).
Adalimumab was linked to a reduced risk of uveitis aggravation or visual
impairment in non-infectious active intermediate, posterior uveitis, and
panuveitis in a placebo-controlled phase 3 research involving patients
with BD(16).