Pemphigus refers to a group of chronic autoimmune blistering diseases of the skin. This cutaneous disorder is characterized by bullae in the skin and mucous membranes (mouth, upper airway, genitalia). Two histopathologically and clinically different types of pemphigus have been recognized. They are pemphigus vulgaris (PV) and pemphigus foliaceus (PF). PF is characterized by more superficial blister formation compared with PV, with acantholysis occurring just above the basal layer of the epidermis. Although PF tends to take a milder clinical course than PV, both can have fatal outcomes if left untreated [1-5]. Literature discussing the safety and potential complications of anesthesia for patients with pemphigus is scarce [2-4], and none pertaining to cesarean section was found. This case report illustrates anesthesia administered without untoward effect to a case of PF for cesarean section.
The patient was a 24-yr-old woman weighing 116 kg. She was gravida 7, para 2 at 38 weeks of gestation. She had a 2-yr history of PF lesions involving the arms, back, chest, and abdomen. She was diagnosed with PF after the delivery of her second child, based on clinical manifestations, immunofluorescence of skin, and serological studies. She was treated with large doses of prednisone and hydroxychloroquine sulfate (Plaquenil[R], Safoni, Winthrop, NY). Unfortunately, she was neither compliant with medication nor with her regular follow-up visits, even for prenatal care. Her PF titer was 1:160 during pregnancy and increased to 1:640 2 wk prior to delivery. At time of delivery, she presented with multiple PF lesions and multiple impetiginous plaques on her face and trunk (anterior and posterior).
Preanesthetic assessment revealed an obese woman with Class II airway with no buccal lesions and unremarkable cardiovascular and respiratory systems. After infusion of 1000 ml lactated Ringer's solution under aseptic conditions with the patient in the sitting position and using a 25-gauge Whitcare needle, lumbar puncture was performed at a lesion-free area (L2-3) without local anesthetic infiltration. Spinal anesthesia was performed with 10.5 mg bupivacaine, 200 micro g epinephrine, and 200 micro g preservative-free morphine . The sensory level using pinprick sensation was at the T3-4 level. Prophylactic ephedrine infusion (a total of 45 mg) was administered . A male neonate (2630 gm) with PF and Apgar scores of 8 and 9 at 1 and 5 min, respectively, was delivered. The neonate showed the characteristic skin lesions of PF, and the diagnosis was confirmed by a high titer at the time of delivery (1:80). Tubal ligation was performed, and the rest of the operation and the postoperative period were unremarkable. The patient was discharged home with her baby on the fourth postoperative day.
PF, both the endemic and nonendemic varieties, have previously been reported with pregnancy [1,8,9], and the neonatal condition has also been previously described . However, details of anesthesia, when required, were lacking. Pregnancy can precipitate and/or aggravate PF, as with other bullous diseases, especially during the first and second trimesters. The increasing endogenous corticosteroid produced by the chorion can improve the clinical picture in the third trimester. As in other autoimmune diseases, postpartum flare-up of PF can occur [3,4].
PF lesions can appear in the buccal and upper airway mucosa. Airway management in the presence of these lesions may be difficult and dangerous. Airway manipulation (laryngoscopy and intubation) can lead to acute bullae formation, severe upper airway obstruction, and bleeding [2-4]. In three case reports describing anesthesia for surgery with PF patients [2-4], epidural anesthesia was used in the first , intravenous administration of a ketamine-diazepam mixture in the second , and continuous spinal anesthesia in the third , all of which avoided airway manipulation. Despite absence of airway bullae, we also chose regional anesthesia to avoid the possibility of precipitating such lesions. To avoid airway trauma, should endotracheal intubation be performed (electively or on emergent basis), the laryngoscope, endotracheal tube, and the face and lips should be liberally lubricated with 1% hydrocortisone cream with gel . Airway trauma can be further reduced by using a MacIntosh rather than a Miller laryngoscope and a smaller endotracheal tube. The tube is secured by using a cloth bandage rather than adhesive tape to avoid friction-induced trauma. Direct laryngoscopy before extubation is unnecessary and may induce further trauma. Tracheal extubation should be gentle, and the patient should be observed postoperatively for airway obstruction.
Skin lesions can also predispose a patient to fluid and electrolyte abnormalities (dehydration and hypokalemia). Sepsis and skin infection at the site of local anesthesia injection is possible . Infiltration with local anesthesia is better avoided because of risk of skin sloughing and bullae formation at the injection site. A choice of an area devoid of skin lesions is recommended and considered safe for performing a lumbar puncture .
Management can be further complicated by drug therapy for PF. Preoperative steroid therapy can produce hypothalamic pituitary adrenocortical axis insufficiency. This may require perioperative steroid coverage to avoid addisonian crisis. Other immunesuppressive therapy can predispose the patient to infection, bone-marrow suppression, and cardiorespiratory side effects .
Another important issue is the association of other autoimmune diseases with PF, including myasthenia gravis, systemic lupus erythematosus, Graves' disease, and immune thrombocytopenic purpura . Our patient had no associated disease. Since she had stopped using steroids more than 18 months prior to admission, we elected not to give any steroids preoperatively, but intravenous and topical antibiotic prophylaxis were administered .
This was the first reported case of neonatal PF detected at birth . Spontaneous healing of the infant's skin lesions with PF occurred within six weeks; therefore, no specific therapy was needed apart from usual skin care.
In conclusion, we chose regional anesthesia to avoid general anesthesia, airway manipulation, and possible trauma. We elected single spinal anesthesia rather than continuous epidural anesthesia to minimize skin trauma by the large-bore epidural needle and the need for skin infiltration at the site of insertion. Moreover, with single spinal block, we avoided the application of tape to secure the epidural catheter to a large area of the skin to lessen the possibility of friction trauma and infection. Although the skin lesions with multiple impetiginized plaques may deter the anesthesiologist from using regional anesthesia, it should be realized that in PF, the pathological lesion is not of infectious origin but of autoimmunological origin. With the needle inserted through a lesion-free area, regional anesthesia, as exemplified here by subarachnoid block, is not contraindicated in patients with PF undergoing anesthesia for cesarean section.
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