Aggressive Refractory Pemphigus Vulgaris That Responded to Plasmapheresis

A Case Report

Hiba Hasan Khaddour; Diana Zaher; Triak Kassem; Ahmad Hasan


J Med Case Reports. 2020;14(109) 

In This Article

Case Presentation

Patient Information

A 14-year-old Asian girl came to our hospital with blisters and erosions all over her body and in the oral mucosa. She had been diagnosed with PV by a skin biopsy about 3 months before hospitalization and had been treated with 1 mg/kg/day of oral prednisone. She had no history of diabetes mellitus, cardiovascular diseases, or psoriasis; no psychosocial history; and no familial history of pemphigus diseases. Her parents are not biologically related. The severity of the disease led to the following life-threatening issues: dehydration and electrolyte imbalance secondary to excess fluid loss from the skin wounds, as well as sepsis secondary to infection of the exposed wounds. Due to progressively worsening symptoms, extensive lesions, and high susceptibility to further infections, the patient was admitted to the intensive care unit. The following immediate actions were taken: urinary catheterization and resuscitation with intravenous fluids to correct the circulatory and electrolyte imbalance, early management of sepsis with intravenous antibiotics, and proper pain relief with opioids. The differential diagnosis included allergy eruption,[4] staphylococcal scalded skin syndrome, and Stevens-Johnson syndrome.

General Physical Examination on Admission

The patient was depressed and slightly dull. Her vital signs were as follows; temperature 38.5 °C, blood pressure 90/60 mmHg, heart rate 115 beats/minute, and respiratory rate 24 breaths/minute. Her mucosae were pale. Her capillary refill time was < 2 seconds. Her musculoskeletal system was normal. Her lymph node examination revealed no findings. Her cardiac examination revealed tachycardia with no murmur. Her lung examination revealed no rales or wheeze. Her mental status (consciousness) was slightly obtunded. The result of her cranial nerve examination was normal. Her pupils were equal with normal direct and indirect pupillary light reflexes. Her motor examination revealed muscle bulk and tone were normal. Her strength was full bilaterally. Her reflexes were normal and symmetric at the biceps, triceps, knees, and ankles. Her plantar responses were flexor. Her sensory examination revealed that her light touch, pinprick, position sense, and vibration sense were normal in her fingers and toes.

Clinical Cutaneous Findings

The patient was febrile with numerous flaccid blisters and erosions involving the oral mucosa and more than 75% of the body surface area (face, trunk, and limbs), some covered with slough (Figures 1 and 2). She had oozing from ulceration and hemorrhagic excoriation with the peeling of the skin. Her perilesional Nikolsky sign was positive. The rest of her physical examination was normal.

Figure 1.

Flaccid blisters and erosions involving more than 75% of the body surface area (face, trunk, and limbs)

Figure 2.

Flaccid blisters on the legs and oozing from skin ulcerations

Diagnostic Testing

Skin biopsy confirmed the diagnosis of PV. Histology showed separated keratinocytes (acantholytic cells) just above the basal layer of the epidermis (Figure 3).

Figure 3.

Histopathology confirmed acantholysis in the epidermis with intraepithelial blistering

Blood Analysis

The patient's complete blood count revealed the following values: white blood cells, 18,000 C/UL 18 x 109/L; neutrophils, 86%; lymphocyte count, 10%; hemoglobin, 10 mg/dl 100g/L; hematocrit, 30.3% 0.303 proportion of 1; mean corpuscular volume, 79.5 fl; mean corpuscular hemoglobin, 22.8 pg; platelets, 150 C/UL 150 x 109/L; alanine aminotransferase, 16.3 U/L; aspartate aminotransferase, 53.9 U/L; total bilirubin, 4.3 μmol/L; lactate dehydrogenase, 408 U/L; erythrocyte sedimentation rate, 27 mm/hour; C-reactive protein, 48 mg/L; blood urea nitrogen, 2.8 mmol/L; creatinine, 50.8 μmol/L; potassium, 3.5 mmol/L; and sodium, 136.4 mmol/L. Urinalysis showed no infection. Serum antibodies could not be measured, because the patient could not afford the test.


At the time of admission, two blood cultures and many swabs from the erosions on the patient's trunk and limbs were collected for culture. The cultures were processed in the laboratory as per standard microbiological techniques and were cultured for aerobic and anaerobic bacteria. The organism was an encapsulated, gram-negative, rod-shaped bacterium. The isolates were identified as Pseudomonas aeruginosa. Blood culture and the pus culture from the erosions revealed the presence of P. aeruginosa. The organism was found to be sensitive to polymyxin B or colistin (colistimethate sodium).

Therapeutic Intervention

The patient was treated with oral prednisolone at a dose of 1 mg/kg/day with oral azathioprine at a dose of 150 mg/day (3 mg/kg/day) on admission for 7 days and intravenous ceftazidime 1 g three times per day + oral levofloxacin 500 mg once per day. Treatment with these antibiotics continued until the results of blood and pus cultures were obtained. The bullous skin lesions had breakdown, leaving painful, eroded areas of skin with a similar appearance to the type of wounds found on burn victims. Early assessment by the dermatologists and plastic surgery team resulted in the placement of appropriate dressings to protect the skin wounds and relieve the patient's distress. One week after the treatment, she did not show any improvement, and she developed fever spikes every day. Blood culture and pus culture from the erosions revealed the presence of P. aeruginosa. On the basis of sensitivity reports, she was started on intravenous colistin (colistimethate sodium) 9 mIU/day in three divided doses for 10 days. Because the disease remained active, the prednisolone dose was increased to 1.5 mg/kg/day 3 days after the start of antibiotics. Ten days after that, the erosions persisted but showed slight evidence of re-epithelialization. The patient's Nikolsky sign was still positive, and she continued to develop a few new blisters. She also developed edema of the genitalia. Because the disease remained active and she was in sepsis with persistent fever, we could not consider giving her intravenous corticosteroids. Considering all the above factors, she was given four sessions of plasmapheresis over 10 days with a gap of 2 days between the sessions. Two liters of plasma were removed using a plasma filter attached to the dialysis machine and replaced with 1 L of normal saline and 1 L of fresh frozen plasma. The procedure was done by a nephrologist in the nephrology department of Tishreen Hospital. After the fourth session of plasmapheresis, the patient's Nikolsky sign became negative, and no new blisters appeared. The erosions showed re-epithelialization. Serum antibody levels before and after plasmapheresis sessions could not be measured, because the patient could not afford the test.


Around 90% of the erosions healed after 1 month of plasmapheresis (Figure 4), and oral prednisolone was gradually tapered over 3 months. The patient was maintained on daily oral prednisolone at a dose 0.5 mg/kg/day, and no new blisters formed (Figure 5).

Figure 4.

Follow-up: around 90% of erosions healed after 1 month of plasmapheresis

Figure 5.