ACTA SCIENTIFIC DENTAL SCIENCES (ISSN: 2581-4893)

Volume 3 Issue 2 February 2019

Case Report

Self - Limiting Cutaneous Pemphigus Preceding the Oral Lesions - A Case Report

Aparna Aggarwal* and Saurabh Jain

Oral Medicine and Radiology, Vitaldent Dental Clinic, Faridabad, India

*Corresponding Author: Aparna Aggarwal, Oral Medicine and Radiology, Vitaldent Dental Clinic, Faridabad, India.
Received: November 29, 2018; Published: January 17, 2019

Abstract
Pemphigus is a group of potentially fatal autoimmune, muco-cutaneous disease, characterized by appearance of vesicle or bullae
that rupture to form large ulcers. The oral manifestations often precedes the skin lesions by many months, or may remain as the
only symptoms of the disease. It is therefore, important that its manifestations to be detected early to make a proper diagnosis, and
initiate timely treatment This care report presents an uncommon case of female patient reporting to our dental hospital with skin
lesions preceding the oral lesions of Pemphigus Vulgaris. This case emphasises on pivotal role of the dentist in the early detection and
following multidisciplinary treatment approach for better management of Pemphigus Vulgaris.
Keywords: Pemphigus Vulgaris; Skin Lesions; Bullous Lesions; Nikolsky’s Sign; Autoimmune Disorders

Abbreviation tions in immunofluroscence [5]. Management of Pemphigus has to

PV: Pemphigus vulgaris be vigorous including oral and systemic corticosteroids, and other
steroid sparing drugs like methotrexate, cyclophosphamide, etc.
Introduction Patient with skin involvement are treated in hospital, as extensive
lesions can be life threatening due to dehydration, loss of protein
Pemphigus is a rare, chronic, potentially life-threatening, auto-
or secondary infection [2].
immune bullous disease of the skin and the mucous membranes.
The two major subtypes of pemphigus are pemphigus vulgaris
Before the introduction of corticosteroids, around 75% of pa-
(PV) and pemphigus foliaceus [1]. It is a rare disease reported in
tients died within the first year of appearance of lesion. Although,
0.1-0.5/100,000 population/year [2], with peak incidence in the
Matinez., et al. had reported a drastic fall in mortality to less than
fifth or sixth decade of life [1,2]. Pemphigus vulgaris (PV) is the
10% currently [2]. Precise and early diagnosis increases the effi-
most common type affecting the oral cavity, and sometimes with
ciency and efficacy of treatment strategy [6].
lesions on skin as pemphigus vegetans. Oral lesions usually pre-
cede skin lesions in 60% of the cases, and skin involvement may As the oral presentation of the disease is often the first indica-
not be seen at all [3,4]. Oral lesions appear as flaccid bullae, which tor, it is very critical for the dental practitioner to recognize the
easily rupture leaving behind painful erosions. These lesions occur lesions at a sufficiently early stage to initiate further investigations
on lips, tongue, buccal mucosa, palate, and often along with des- and treatment. This case report presents a rather unusual case
quamative gingivitis [5]. The skin involvement can be seen similar of PV, reporting of self-limiting skin lesions preceding the oral le-
to oral lesions on normal or erythematous skin of neck, abdomen sions.
and back commonly.

Case Report
Pemphigus is diagnosed based on clinical appearance, positive
nikolsky’s sign, tzank cells and supra-basilar split in histopatholo- A 53-year-old female farmer reported to, with a complaint of
gy, and demonstration of immunoglobulins in the spinous cell junc- ulcers and bleeding from mouth for 3 days. These ulcers were

Citation: Aparna Aggarwal and Saurabh Jain. “Self - Limiting Cutaneous Pemphigus Preceding the Oral Lesions - A Case Report”. Acta Scientific Dental
Sciences 3.2 (2019): 59-63.
Self - Limiting Cutaneous Pemphigus Preceding the Oral Lesions - A Case Report

60

preceded by bullae formation, which bled on rupturing. She also

complaint of soreness of her entire gingiva and difficulty in swal-
lowing. On further history taking, she also admitted of having simi-
lar lesions on her neck from past 2 weeks. However, that was not
her primary concern, as no new lesions had formed in past 5 days.
Her medical history was unremarkable. Patient was not taking any
medication. Her family history and past medical history was non-
contributory. She had no known oral abusive habits.

On general examination, the patient was lean built with signs of

pallor. The extra- oral examination revealed a wide spread lesion
on her neck anteriorly, starting from below the chin superiorly to
mid neck inferiorly, and extending to submandibular space bilater-
ally (Figure 1). The irregular area was pinkish red, inflamed, tender
and covered with dried brownish scab superiorly. A recently rup-
tured bulla was present just below the chin. There was bloody frus-
tration on her lower lip and dried blood on both her lips (Figure 2).
There was no involvement of the eyes or any other part of the skin.

Figure 2: Blood on the lips.

Intraorally, ruptured linear erosions with irregular borders, and

macerated tissue tags were present in bilateral posterior buccal
mucosa along the line of occlusion, and lower labial mucosa (Fig-
ure 3-5). Desquamative gingivitis was seen confined to both upper
and lower anterior gingiva (Figure 6). Nikolsky’s sign was positive
in buccal mucosa. General oral hygiene of the patient was poor
with extensive deposits of calculus.

Based on history and clinical appearance a provisional

diagnosis of pemphigus vulgaris involving skin and mucosa was
drawn. A differential diagnosis of para-neoplastic pemphigus was
given. Patient was advised to get a biopsy done for confirmation
of the diagnosis, but patient refused to undergo the same. She was
put on soft diet and topical steroid ointment (0.1% triamcinolone
acetonide) and topical analgesic (mucopain) for oral use.

She was referred to a hospital for evaluation and further

management by a dermatologist. She was admitted and started
on IV dexamethasone 100 mg for 3 days along with 500 mg of
cyclophosphamide (Pulse therapy). Before that, biopsy from
Figure 1: Skin Lesions. both skin and oral lesions was taken. Histopathology confirmed
diagnosis of PV for both the specimens. Two more cycles of this
regime at intervals of 4 weeks each were repeated. The patient was
prescribed prednisolone 30 mg/day during the interim 4-week
period. Patient had undergone complete remission by the end of
therapy.

Citation: Aparna Aggarwal and Saurabh Jain. “Self - Limiting Cutaneous Pemphigus Preceding the Oral Lesions - A Case Report”. Acta Scientific Dental
Sciences 3.2 (2019): 59-63.
Self - Limiting Cutaneous Pemphigus Preceding the Oral Lesions - A Case Report

61

Figure 5: Erosions on lower labial mucosa.

Figure 3: Ruptured Bulla on right buccal mucosa.

Figure 6: Ruptured Bulla on right buccal mucosa.

Figure 4: Ruptured Bulla on left buccal mucosa.

Citation: Aparna Aggarwal and Saurabh Jain. “Self - Limiting Cutaneous Pemphigus Preceding the Oral Lesions - A Case Report”. Acta Scientific Dental
Sciences 3.2 (2019): 59-63.
Self - Limiting Cutaneous Pemphigus Preceding the Oral Lesions - A Case Report

62

Discussion
Vesico - bullous lesions have varied presentation, with autoim- Oral lesions can range from superficial ulcers, to small vesicles,
mune aetiology in most cases. Pemphigus is an autoimmune muco- or flaccid bullae. The primary lesion is usually a thin-walled or flac-
cutaneous disease characterized by intra-epidermal bulla forma- cid bulla, several centimetres in size, containing clear fluid. The
tion with reported incidence of 0.1-0.5/100,000 population/year most common sites of oral involvement include the buccal mucosa,
[2], to 0.42 to 1.62 cases per 100,000 [6]. The word Pemphigus is soft palate, labial mucosa, and gingiva, although any oral site may
derived from the Greek word “pemphix”, meaning bubble or blis- be affected [6]. Nikolsky’s phenomenon- detachment of a large area
ter. It predominantly affects adults of both genders equally, with a of the surface with the formation of blisters can occur by exerting
mean age of 50 years [6]. Pemphigus is common amongst certain a slight pressure on the apparently normal epithelium of these pa-
races like Ashkenazi Jews, Mediterranean’s, and Asians (especial- tients, is the key to clinical diagnosis [2,7]. The oral lesions of PV
ly Indians and Japanese) [2]. A relationship has been found with are extremely painful, resulting in hyper salivation, halitosis, dif-
HLA, especially with certain HLA class II alleles, with implication ficulty in swallowing and difficulty in phonation [6,7]. Healing is
of HLA-DR4 (DRB1*0402) in Ashkenazi Jews and of HLA-DRw14 very slow but without scars formation (which is typical of bullous
(DRB1*1041) and HLA-DQB1*0503 in Mediterranean and Asiatic pemphigoid).
peoples [2,6].
Pemphigus skin lesions can arise as simple rashes to erosions,
Pemphigus has many variants like Pemphigus Vulgaris, Pemphi- vesicles, blisters, or ulcers, affecting predominantly the trunk and
gus Foliaceous, Pemphigus Vegetans, Pemphigus Erythematoses, the limbs, especially large areas of bending folds such as the neck
and rarely Paraneoplastic Pemphigus. PV is the most common form [9], as seen in our case. The other mucosae covered by pavement
(80%) seen affecting the oral cavity and sometimes, it occurs in epithelium, e.g., oropharyngeal, genital or conjunctival mucosae,
conjunction with Pemphigus Vegetans on the skin [3]. Mucosal le- can also be affected [7].
sions may be the sole sign for months before skin lesions develop,
or they may be the sole manifestation of the disease [3,7]. In our The diagnosis of PV is based on clinical features, histology, and
case, the skin lesions had preceded the oral lesions by 2 weeks. Al- positive immunological tests. For a definitive diagnosis, an inci-
tun E., et al. had reported of skin lesions preceding oral lesions in sional biopsy of a peri-lesional tissue should be performed. Histo-
18.4% of 49 of their patients, and both mucosal and skin involve- pathological examination typically shows acantholysis, which is the
ments in 38 (77.6%) patients [8]. loss of coherence of epidermal cells and their subsequent detach-
ment. This further leads to, intra-epithelial vesicle above the basal
The autoimmune response may be triggered in susceptible in- cell layer causing supra – basilar split. On cytological examination,
dividuals due to various medicines (especially thiol-containing typical acantholytic cells contains detached rounded keratinocytes
drugs, e.g., penicillamine and ACE-inhibitors), diet, stress, physical with swollen hyperchromatic nuclei called Tzanck cells are seen
or viral agents, vaccines or neoplasms [2,6,7,9]. In our case, use of [6,7]. Direct Immunofluorescence study shows the typical “fish-
fertilizers or excessive sun exposure in her work place may have net” pattern of IgG and complement C3 deposits in the spinous
triggered PV. The blisters occur in the epithelium where the IgG layer [5]. The most frequent differential diagnoses of PV with oral
autoantibodies are produced in response to triggering factors tar- lesions are recurrent aphthous stomatitis, Bullous pemphigoid, Be-
get desmoglein 3, which leads to epithelial cell separation, by trig- hçet disease, erythema multiforme, erosive lichen planus, and oral
gering complement activity or plasminogen plasmin system. Thin candidiasis [2].
separation at the desmosomal region triggers the acantholysis and
suprabasal spilt. D Kuriachan., et al. have reported of new pemphi- Oral lesions are challenging, since their response to treatment
gus antigen Desmoglein 4 and other non-Desmoglein antigens like is much slower in comparison to cutaneous lesions [2]. For local
human α-9-acetylcholine receptor that regulates keratinocyte ad- lesions of the oral mucosa, mouthwashes or topical corticosteroids
hesion and keratinocyte annexin like molecules binding acetylcho- ointments, (0.1% triamcinolone acetonide, 0.05% fluocinolone
line termed pemphaxin and catenin that are thought to play a role acetonide, or 0.05% clobetasol propionate) or intralesional injec-
in its etio-pathogenesis [5]. tion (triamcinolone acetonide (20 µg/L) or paramethasone every

Citation: Aparna Aggarwal and Saurabh Jain. “Self - Limiting Cutaneous Pemphigus Preceding the Oral Lesions - A Case Report”. Acta Scientific Dental
Sciences 3.2 (2019): 59-63.
Self - Limiting Cutaneous Pemphigus Preceding the Oral Lesions - A Case Report

63

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Conclusion
The appearance of skin lesions of pemphigus prior to oral le-
sions is rather uncommon. Whenever such a case is seen, referral
to an oral medicine specialist or dermatologist should be done
Volume 3 Issue 2 February 2019
without further delay. Early management of pemphigus lesions
© All rights are reserved by Aparna Aggarwal and
particularly skin lesions, can have better control of the disease, and
Saurabh Jain.
save patient from life-threatening dehydration and secondary in-
fections.

Citation: Aparna Aggarwal and Saurabh Jain. “Self - Limiting Cutaneous Pemphigus Preceding the Oral Lesions - A Case Report”. Acta Scientific Dental
Sciences 3.2 (2019): 59-63.