An 18 year old female was referred from a medical physician at Klawer hospital telephonically. He had informed me of his patient, an 18 year old female who presented with oral ulcerations for over 1 year which he and a dermatologist colleague had struggled to resolve. He described the lesions and forwarded pictures.
Figure 1: Picture set from referring doctor
He had indicated that the patient was otherwise healthy and all blood tests conducted were normal. They had tried to treat the lesions with chlorhexidine mouthrinses, nyastatin and several courses of systemic acyclovir without success. He had indicated that no skin or genital lesions were present at the time.
After reviewing the photographs, I had asked for an immediate referral as the lesions appeared to resemble an immune mediated aetiology such as:
- Erosive lichen planus
- Erythema multiforme
- Pemphigus vulgaris
- Pemphigoid etc.
As the patient lived more than two hours’ drive away from Tygerberg hospital, transport from the Klawer was arranged and she presented at the oral medicine clinic two weeks later.
Clinical examination:
A single extra-oral lesion was apparent on the right cheek as well-defined circular erosion with central erythema measuring approximately 2.5cm and surrounded by a pale halo. The lesion appeared within the last few days prior to her visit and she indicated the presence of a blister which had since burst. The lesions on the lips were extremely painful with surface crusting visible. New intra-oral pictures were not taken as she indicated that the lesions bleed and cause great discomfort. The erosive lesions involved the labial and buccal mucosa bilaterally as well as partial mandibular gingival involvement as a desquamative gingivitis. The soft palate was profoundly erythematous with some shallow erosion. There no signs of reticular striation. She was once again asked if there were any other lesions affecting the skin which she replied in the negative. Questions investigating the possibility of medication or food allergies were asked. The patient had found it very difficult to eat and had been on a cold soft diet devoid of spices. She had stopped all medications since we informed the doctor of a possibility of allergic aetiology. Only diluted chlorhexidine mouthrinse was used twice a day and the occasional Paracetamol to reduce her discomfort.
Figure 2: Extra-oral presentation at TBH
Figure 3: Extra oral lesion
Differential diagnosis:
- Erythema multiforme
- Lupus erythematosus
- Bullous pemphigoid
- Paraneoplastic pemphigus
- Linear IgA bullous dermatosis
- Erosive lichen planus
Management:
Following local anaesthesia, two incisional biopsies from the least affected sites of the left buccal mucosa were sent for histopathology and direct immunofluorescence. An immediate referral to dermatology was arranged following the biopsy in order to assess the skin for possible early lesions. A prescription for prednisone 1mg/kg for 7 days was provided and to be retrieved from pharmacy after the dermatology examination. Dermatology at that stage found no evidence of skin lesions and advised not to take the prednisone as their protocol prevents all medications in suspected cases of erythema multiforme. She was advised to keep the prescription so that the medication could be retrieved from her local clinic after the diagnosis is confirmed. A follow up examination was scheduled for the following week.
Oral biopsy was provisionally reported within a week and indicative of pemphigus vulgaris but indirect immunofluorescence was advised.
Figure 4: Oral biopsy result
Special investigations:
The Meningitis Screen Multiplex PCR was performed and tested negative:
Herpes Panel Result Negative
Enterovirus Panel Result Negative
The screening test includes:
The Herpes Panel includes the following viruses:
- Cytomegalovirus, Epstein-Barr virus, Herpes Simplex virus type 1, Herpes Simplex virus type 2, Human Herpes virus type 6, and Varicella Zoster virus.
The Enterovirus Panel includes Human Enterovirus and Mumps virus.
Haematology flags:
Creatinine 42 L umol/L 49 – 90
Alanine transaminase (ALT) 84 H U/L 7 – 35
MCHC 29.8 L g/dL 32.7 – 34.9
Red Cell Distribution Width 17.9 H % 12.4 – 17.3
6 days later following initial visit:
The patient called as she was on her way to TBH. She complained that multiple blisters began to form on her back, chest and abdomen. An emergency dermatologic visit was then arranged and the patient was admitted. Since the oral pathology results were not yet available thus the definitive diagnosis was still unknown. Dermatologic treatment involved their protocol for pemphigus vulgaris and skin biosy was taken at that stage.
Management remains with dermatology.
Case 2: Pemphigus vulgaris with ophthalmologic involvement
Main complaint and medical history:
A 54 year old otherwise healthy female visited the oral medicine clinic at TBH while on holiday from Johannesburg. She complained of a persistent, painful recurrent oral ulceration for the past 12 months. She visited her GP who prescribed numerous mouthwashes and antibiotics. However, no treatment has so far provided any relief of the symptoms or prevented recurrence of the ulcers. The patient also reported that during the past month she has experienced discomfort in her eyes “which feel dry, scratchy and appear red at times”. She has used numerous over the counter eye-drops to manage the symptoms.
Clinical examination
The extra-oral examination revealed bilateral submandibular lymphadenopathy. The patient’s right eye appeared to be red clinically. The patient reported no lesions on her skin or genital areas.
Intra-orally she had generalized bilateral buccal mucosal erosions with patches localized to areas on the hard and soft palate. The central zone of the erosive lesions displayed areas of central necrosis at the right buccal. White striations were present radiating from lesions in the left cheek. The gingiva presented with areas of desquamative gingivitis particularly severe in the posterior mandibular segments. In the floor of the mouth, ulceration was associated with the sublingual ducts and mandibular lingual frenum. The tongue and lips remained uninvolved.
Figure 5): Bilateral ulceration and erosion of the buccal mucosa
Figure 6): Erosions and ulceration extending onto the hard and soft palate
Figure 7): Ulceration associated with the sublingual ducts
Figure 8): Desquamative gingivitis quadrant in the 4th
Figure 9): Erosions and ulceration extending onto the hard and soft palate
The differential diagnosis included:
- Pemphigus vulgaris
- Erosive lichen planus
- Pemphigoid
- Beçhet’s disease
- SLE
- CUS etc.
The patient was placed on a course of systemic steroids (Prednisolone 1mg/kg) for 7 days and an arrangement was made with ophthalmology and dermatology. Ophthalmology performed their assessment and provided steroid and lubricating drops to be applied twice daily.
The oral lesions did demonstrate some improvement after 7 days of treatment and it was decided to continue the systemic steroids and to provide additional topical steroids for the lesions in the buccal mucosa (namely Advantan). Dermatology had detected a rash on the lower extremities and provided medications. As the patient was to return home, referral letters were provided to attend here regional hospital (university of Witwatersrand).