Pemphigus Foliaceous: A Rare Case Report Abstract
Pemphigus Foliaceous: A Rare Case Report Abstract
Pemphigus Foliaceous: A Rare Case Report Abstract
ABSTRACT:
Pemphigus foliaceus (PF) which is a rare autoimmune blistering disease , presents in endemic
and sporadic forms with the typical presentation of seborrheic distribution of recurrent shallow
erosions.Here we present a case of a 62-years-old male with PF who was successfully treated
with a combination of oral corticosteroids and azathioprine .
INTRODUCTION:
The term pemphigus is derived from the Greek word pemphix meaning blister or bubble. The
incidence of pemphigus ranges from 0.76 to 5 cases per 0ne million per year (1).
Pemphigus is further divided into 2 major forms on the basis of blister location: pemphigus
vulgaris (PV) and pemphigus foliaceus (PF). A variant of PV is Pemphigus vegetans and
variants of PF are pemphigus erythematosus (PE) and fogo selvagem. Uncommon forms of
pemphigus have been described during past 3 decades , which includes pemphigus herpe-
tiformis, immunoglobulin A (IgA) pemphigus, and paraneoplastic pemphigus (2).
CASE REPORT:
A 62-year-old male presented with a 4-month history of superficial erosions on the upper back
in 2011. He denied any photosensitivity, and his medical history was otherwise unremarkable.
On examination, the erosions were in the form of painless vesicles, which bursted in 2 days
discharging clear watery fluid. The patient was started on low dose Prednisolone (20 mg) for 1
month but he did not improve; rather the lesions spread on the entire body from scalp to legs.
Two punch biopsies (one lesional and one perilesional) were performed. Routine histology
results showed intraepidermal vesicles in the upper granular layer containing acantholytic cells.
Results of direct immunofluorescence demonstrated IgG and complement 3 (C3) in the
intercellular spaces in the epidermal cell surface. Also, antinuclear antibody and anti–double-
stranded DNA were negative. These findings were consistent with our clinical diagnosis of
Pemphigus Foliaceous. The patient was then started on high dose Prednisolone(40 mg ) for 4
weeks when new lesions ceased to appear; then the medication was slowly tapered off.
Azathioprine(50 mg) was also added to his regimen. Shelcal in a single morning dose containing
500 mg calcium and 250 IU vitamin D3 also was added to the regimen. He was seen at regular
follow-up visits every month. As the patient improved clinically, oral prednisone taper continued
slowly over a total of 18 months. No lesions were present for the last 5 monthly visits.
CASE DISCUSSION:
Pemphigus is a blistering disease affecting the skin and mucous membrane. The disease is
characterized by acantholysis ; which means loss of adhesion between keratinocytes
histologically, and immuno-pathologically charecterised by the presence of antibodies directed
towards the cell surface of keratinocytes (3) . Indirect immunofluorescence study
revealed that circulating antibodies are mainly IgG1 and IgG4 in pemphigus faliaceous and the deposited
antibodies are mainly IgG type , with predominantely IgG1 and IgG4 types of antibodies (4).
In order to understand the pathogenesis of Pemphigus it is essential to have a
basic knowledge of the desmosome. Desmosomes also known as maculae adherens are
organelles responsible for cell-to-cell adhesion in keratinocytes. Desmoglea which is the extra
cellular part, is composed of transmembrane adhesion glycoproteins which belongs to the
cadherin superfamily, including desmogleins and desmocollins. Desmosomal plaques which are
the intracellular part, has two groups of proteins : a)The plakin family (desmoplakins,
envoplakin, periplakin, plectin) which binds to cytokeratin filaments and b) The plakoglobin
and plakophilin which binds to the intracellular domain of cadherins. The pemphigus antibodies
bind to the antigens in the desmosome which results in acantholysis. (4)
PF clinically presents with recurrent shallow erosions associated with
erythema, scaling, and crusting (5). Lesions usually are found in a seborrheic distribution
(central face, neck, chest, or upper back) (6). The onset of disease may be slow, starting with
only a few transient scattered crusted lesions . In general, patients are not severely ill but often
complain of burning and pain associated with the skin lesions (7). The condition may then stay
localized for years or progress into generalized involvement, sometimes resulting in an
exfoliative erythroderma (8). In our case the patient presented with painless superficial erosions
first on upper back and later on it gradually spread to other parts of body .
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