A 18-year-old lady presented with recurrent painful oral ulcer and multiple blisters

DR. Azmiree Binte Aslam

Particulars of the patients:

• Name: Asma• Age: 18 years• Sex: Female• Occupation: Student• Religion: Islam• Marital Status: Unmarried• Address: Kaliganj, Satkhira• Date of Admission: 16.07.16

Presenting complaints:

1. Recurrent painful oral ulcer for 5 years2. Recurrent multiple blisters involving

different parts of the body for same duration

3. Recurrent appearance of purpuric spots on the lower legs, chest and back for 1 year.

History of Present Illness

According to the statement of the patient, she was reasonably well 5 and half years back. Then she developed tingling and burning sensation of the hand followed by weakness of right side of the body and fall on the ground. For this complaint she was treated with a medication but she could not mention the name. After taking the drug for six months, she developed painful oral ulceration involving tongue, buccal mucosa and lips. She also developed few pruritic, flaccid blister on her hand, that ruptured spontaneously and healed with post inflammatory hyperpigmentation.

Contd. With these complaints, she was admitted in a

hospital and diagnosed as a case of Steven Jhonson syndrome. She was treated with injectable dexamethasone and ceftriaxone for 7 days with withdrawal of the offending drug. Her oral ulcer and skin lesion were healed in 10 days. After discharge, she was advised to take acetalopram for her neurological symptoms by local doctor which she took 2 months and was symptom free for 3 years. Then she again developed neurological symptoms and then she was treated with sodium valproate for 6 month. Since then, she is symptom free.

Contd.

4 years back, she again developed painful oral ulcer and blisters involving extrimities and trunk for which she was treated with antibiotic in nearby hospital with healing of the lesions in 2 weeks. Since then she has been suffering from relapsing and remitting attack of few flaccid skin blisters and painful oral ulcer that heals in 10-14 days with or without medication occuring 4-5 times per year up to 3 years. In the course of the disease she took some antibiotics by herself.

Contd.

Last year she had got admitted in hospital due to development of multiple pruritic flaccid blisters in the extrimities, face and back with painful oral ulcers. At that episode, she also developed multiple purpuric spots in her chest and lower legs. She was diagnosed as a case of probable case of Systemic lupus erythematosus and treated with deflazocort and hydroxychloroquine along with some topical medications. Her purpuric spots resolved but the oral ulcer and bullous lesions continued to appear.

Contd.

She has no history of joint pain, fever, photosensitivity, alopecia or any other mucosal site involvement. Her bladder and bowel habit is normal. With these complaints she is admitted in BSMMU for better management.

History of past Illness:

• She was hospitalized for four times for the same illness.• No history of accident• No history of blood transfusion

Drug history:

Name Of The Drug Dosage Date Started

Duration Of Treatment

Inj. Dexamethasone 1 vial 8 hourly

5 yrs back 7 days

Inj. Ceftriaxone 1 gm 1 vial daily 5 years back

7 days

Tab. Acetalopram 5mg 5mg daily 5 years back

2 months

Tab. Na valproate 300mg 12 hrly

2 years back

6 months

Tab.Hydroxychloroquine 200 mg/day 1 year back 7 monthsTab.Daflazacort 12 mg/day 1 year back Tappered

in 2 months

Personal history:

• Nonsmoker• No history of betel nut chewing• Nonalcoholic • No history of exposure.

Family History:

• Nothing significant

Socio economic condition:

She belongs to a lower middle class family.• House: Semi- pacca house • Drinking water: Tube well water

Immunization History:

Immunized as per EPI schedule.

Menstrual History:

• Menarche: 13 yrs• Menstrual period: 3 days• Menstrual cycle: 60-70days

General Examination

•Appearance: Ill looking.•Body build: Below average.•Co-operative: Co-operative.•Decubitus: On choice.•Anaemia: Mild.• Jaundice: Absent.•Cyanosis: Absent.•Clubbing: Absent.

Contd.

• Koilonychia:Absent.• Leukonychia: Absent.• Edema: Absent.• Dehydration: Absent.• JVP: Not raised• Lymph nodes: Not palpable.• Thyroid gland: Not enlarged• Neck vein: Not engorged

Contd.

• Temperature: 98° F• Pulse: 80 beats/min• Blood pressure: 110/70 mm of Hg.• Respiratory rate: 16 breaths /min• Body weight: 39 kg

Systemic Examination

Integumentary system:• Skin:

Flaccid vesicle some containing haemorrhagic fluid and some clear fluid present over erythematous base on the forearm.

Ruptured bulla covered by crust present on the trunk.

Palpable painless purpura present over chest and back.

Nikolsky sign: Negative. Asboe -Hansen sign: Negative.

Hemorrhagic fluid containing vesicle over erythematous base on flexor surface of forearm

Vesicle over erythematous base containing clear fluid present on the extensor surface of forearm

Ruptured vesicle with erosion covered by crust over erythematous base on the trunk

Hyperpigmented macule and patch present over the right cheek

Painless palpable purpura present over the back of the trunk

Contd.

• Oral mucosa:One painfull whitish shallow ulcer

present over tongue. Multiple petechiae present over soft

palate.

Painless shallow ulcer present over the tip of the tongue

Petechial spots present over the soft palate

Contd.

• Hair and Nail: Normal

Systemic Examination

• Neurological system:Higher psychic function: NormalSensory examination: IntactCranial nerves: IntactMotor examination: Normal

Systemic Examination

• Alimentary system: Normal• Respiratory system: Normal• Cardio-vascular system: Normal• Musculoskletal system: Normal• Genito-urinary system: Normal

Salient features

Miss Asma ,18 years old student, hailing from Satkhira got admitted in BSMMU in 16.7.2016 with the complains of recurrent painful oral ulcer involving tongue, buccal mucosa and lips for last 5 years. She also developed flaccid, spontaneouly ruptered pruritic blisters involving trunk, face extrimity for same duration. 1 year back, she developed purpuric spots over chest, back and legs. She was treated with various topical and systemic medications including Hydroxychloroquine and Daflazacort.

With those treatments her purpuric lesions improved but other lesions remained same. She was hospitalized 4 times for her illness. She has no history of joint pain, photosensitivity, fever, alopecia. On examination: she is mildly anaemic, few flaccid vesicle containing haemorrhagic fluid or clear fluid and ruptured vesicles with erosion covered by crusting present over extrimities and back. Painless palpable purpura present over chest and back. One painfull whitish shallow ulcer present over the tongue. Multiple petechiae present over soft palate.

Diagnostic dilemma

Differential diagnosis:

Investigations:

17.07.16 10.06.15

Hemoglobin 10.7 g/dl 10.7 g/dlESR 85 mm/ 1st hr 102 mm/ 1st

hrRBC 3.85 x

10^12/l 3.5 x 10^12/l

WBC(total count)

9000/cumm 7200/cumm

Platelet count 350 x 10^9/l 248 x 10^9/l

1. Complete Blood Count

17.7.16

2. Peripheral blood film:RBC: Normocytic normochromicWBC: MaturePlatelet: NormalComment: Normocytic normochromic

anaemia.

17.7.16

3. Urine R/M/E:• Protein: Nil• Sugar: Nil• Pus cell: 2-5/ HPF• Epithelial cell: 3-5/HPF• RBC: Nil• Casts: Not found

17.7.16

4. Serum creatinine: 0.67 mg/dl5. S.G.P.T: 18IU/l6. RBS: 5.4 mmol/L7. Serum electrolyte:

Sodium: 141 mmol/lPotassium: 3.1 mmol/lChloride: 103mmol/lCO2: 30.9 mmol/l

8. Anti HSV 1 IgM & AntiHSV 2 IgM: Negative9. Wound swab for C/S: No growth

18.7.16

10. Chest X ray P/A view: Normal study

11. ANA: Negative both in ELISA and Immunofluorescence

21.07. 16

14.06.15

12. Anti-ds DNA: positive ( 32.60IU/ml)

25.07.16

13. Skin biopsy for histopathology:Site: Skin from right forearmGross examination: specimen consists

of a grey white piece of tissue measuring about 0.4 x 0.3 x 0.1 cm. submitted as such.

Microscopic examination: section shows skin. The dermis shows mild peri vascular infiltration of chronic inflammatory cells.

No vasculitis or bullae is seen.Diagnosis: Non-specific changes.