Epidermolysis bullosa dystrophica recessive: Oral surgery and anesthetic considerations

J Oral Maxillofac 40 753-758,

Surg

1982

Epidermolysis Bullosa Dystrophica Recessive: Oral Surgery MICHAEL

and Anesthetic

S. BLOCK,

Considerations

DMD,* AND BOB D. GROSS, DDS, MSt

Epidermolysis bullosa is an intriguing, mechanobullous disease of the skin and was first described by Von Hebra’ in 1870. The patients have clinical lesions resulting from minor frictional trauma to the skin. Epidermolysis may not be a true description of the chronic and noninflammatory lesions found in these patients, because the lesions are subepidermal. Thus, the term dermolytic may be more accurate. There are many subtypes of dermolytic disease, as described by Pearson,* depending on whether the patient has a scarring (dystrophic) or nonscarring reaction to the bullas. Included in the nonscarring groups are epidermolysis simplex, recurrent bullous eruptions of hands and feet (Cockayne’s disease), junctional bullous epidermatosis (also known as epidermolysis bullosa hereditaris letalis, or Herlitz disease), bullous congenital ichthyosiform erythroderma (or epidermodysplasia hysteroides bulloss), acantholytic bullous epidermatosis, acrodermatitis enteropathica, benign familial pemphigus, pachyonychia congenita, and localized abscence of skin with blistering and nail dystrophy. The scarring groups include dermolytic bullous dermatosis or epidermolysis bullosa dystrophica recessive, epidermolysis bullosa acquisita, Mendes Da Costa syndrome (or epidermolysis bullosa dystrophica macular), cutaneous porphyria, and localized collagen dystrophy.* Oral manifestations of the dystrophic type were first reported in 1897 by Fox.~ Many of the early reports were merely descriptive, and only recently have the many subtypes been clinically characterized.*j4 The patient discussed in this report had the recessive dystrophic type, epidermolysis bullosa dystrophica recessive (EBDR).

gestation and was noticed at delivery to have an absence of skin over the medial aspect of both tibias and also smaller areas of denudation over his left arm. lips. and nose. There was also some denudation of the oral mucosa. A skin biopsy at birth was consistent with epidermolysis bullosa dystrophica. The family history revealed that no other family members had disease. The parents are both normal in development, and the patient’s three siblings were alive. One younger sister had juvenile-onset diabetes mellitus. The patient had no known allergies. He had already been hospitalized five times, including once for otitis media when 19 months old, and again for severe anemia when he was 5 years old. His hemoglobin at the latter admission was 7.8 gidl, consistent with iron-deficiency anemia. He was given iron therapy and followed up by the pediatrics department as an outpatient. An esophagram at that time was negative. His third hospital admission was by the oral surgery service when he was 13 years old, for extraction of his carious teeth. Prior to that admission, there had been numerous outpatient visits for multiple skin infections. Severe scarring of the feet and hands, and generalized debilitation. At the time of the third admission, the patient’s hemoglobin was 6.7 g/d1 and his hematocrit was 22.3%. The result of urinalysis was normal except for numerous casts and protein. The patient was hypertensive, with a blood pressure of 1.56/110 mm Hg. He was given a transfusion of two units of packed red blood cells to raise his hematocrit. A complete workup ensued, resulting in a diagnosis of post-streptococcal glomerulonephritis with secondary hypertension confirmed by the laboratory findings. The patient’s blood pressure was controlled after a fourth admission one month later with furosemide, hydralazine, and reserpine. He was followed up by the pediatric department as an outpatient and remained hypertensive, with a blood pressure in the range of 150/100 mm Hg. At age 15 the patient was admitted to the hospital for the fifth time, again for control of his hypertension. His blood pressure was controlled after three weeks with hydrochlorothiazide. 30 mg twice a day, and hydralazine, 20 mg four times a day. On this admission the patient’s hemoglobin was 13.0 gidl and his hematocrit was 39.2%. The patient was sent to the oral surgery clinic for evaluation of his carious dentition and was readmitted approximately three weeks later for extraction of his teeth. Upon admission, the patient appeared to be younger than his stated age. He was very thin and had obvious alopecia, microstomia, dental decay, and abundant scarring of his neck, hands, legs, and feet (Figs. I, 2. and 3). The patient was alert, oriented, and normal in mental development. The blood pressure was 1lo/65 mm Hg. pulse 54 beats/min and regular. respirations 16/min, height, 150 cm, and weight 30 kg. His eyes and ears were normal except for some mild scar formation in his left external

Report of a Case A Idyear-old black boy was admitted to the oral and maxillofacial surgery service for extraction of all of his remaining teeth. The patient was the product of a normal * Resident. t Associate Professor. Received from the Department of Oral and Maxillofacial Surgery, LSU School of Dentistry, 1100 Florida Ave., New Orleans, LA 70119. Address correspondence and reprint requests to Dr. Block.

0278-2391/82/l10010753$01.20 @ American Association of Oral and Maxillofacial

753

Surgeons

754

EPIDERMOLYSIS

BULLOSA

percussion and palpable 2 cm beneath the lower costal margin. The genitals were small; both testes were descended. The patient’s skin was very atrophic and was marked with scars over all extremities, resulting in generalized hair loss. His fingers were flexed; several digits were short and several fingernails were absent (Fig. 2). The small toes on both feet were missing, as were the toenails (Fig. 3). Veins on the arms and legs were difficult to identify. The results of neurologic examination, chest radiograph and electrocardiogram were normal. Laboratory findings indicated a microcytic, hypochromic anemia (hemoglobin 13.1 gdl, hematocrit 40.2%, mean corpuscular volume 76.2 cu pm, mean corpuscular hemoglobin concentration 32.7%, and mean corpuscular hemoglobin 24.9 pg). The electrolytes and liver functions were normal, as were the prothrombin time and the partial thromboplastic time. The results of screening for urine porphyrin and hepatitis B were negative. Consultation with the anesthesiology department helped both the surgical team and the anesthesia team to completely understand the plans for operation and anesthesia. The surgical nurses were requested to obtain the sheepskin and foam packing for the operating room table, to be prepared for cricothyroidotomy and bleeding problems, and to have the room set up early. The patient was well prepared psychologically for his expected operation, and thus his only premedicant was lorazepam, 1 mg by mouth. He was brought into the operating room and positioned himself on the sheepskin. After two unsuccessful attempts at venipuncture, the patient was given 150 mg ketamine intramuscularly in the right gluteal region. He was quiet within one minute. A bite block was placed in his mouth, and his lips and face were covered with Kenalog cream and petroleum jelly. An intravenous line was then started in his left external jugular vein and sutured in place with 4-O silk. A perfusion of 60 mg ketamine per hour was started. A loose

FIGURE 1. Top, Preoperative intraoral view, showing poor oral hygiene and extensive periodontal disease. FIGURE 2. Center, Dorsal view of hands, showing absence of several nails, and newly formed bulla. FIGURE 3. Below, Dorsal view of feet, showing extensive scarring and absence of toes. canal. His mouth was circular, with signs of chronic scarring of the commissures. He had abundant gingival, buccal, and pharyngeal ulcers. There was marked ankyloglossia, and his tongue was smooth and glossy. His teeth were grossly carious, and periapical pathologic conditions were seen on the panoramic radiographs (Fig. 4). His neck was supple; the trachea was small and in the midline; the thyroid was not palpable. There was nontender submandibular adenopathy. The lungs were clear, and the heart was normal in rate and rhythm, and without scarring. The liver was 10 cm in breadth on auditory

FIGURE 4. Above, Panoramic radiograph, showing pathologic dental condition. FIGURE 5. Below, Postoperative view, showing minimal perioral ulceration one week after extractions.

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755

pharyngeal gauze pack was placed in the posterior oral cavity, and inferior alveolar nerve blocks were given, Nasal cannuli were used to deliver a NZO/Oz combination. The teeth in the upper right quadrant were extracted with forceps, and microfibrillar collagen (Avitene) was placed in each extraction socket, resulting in good hemostasis. A surprising amount of secretion was encountered in spite of careful suctioning and the use of 0.2 mg glycopyrrolate intravenously. No respiratory distress was observed. The teeth in the lower right quadrant were extracted in a similar fashion and packed with Avitene. Upon completion of these extractions, saline-dampened gauze was placed over the wounds, a bite block was inserted, and the teeth in the left upper and lower quadrants were removed in the same manner. In order to minimize the amount of ketamine administered, little time was wasted. Toward the end of the procedure, some mild gurgling sounds were heard. Gentle suctioning of the pharynx relieved this mild obstruction; however, it resulted in immediate sloughing of the palatal mucosa and the formation of small blebs on the patient‘s tongue. The total operative time was 56 minutes, and 245 mg of ketamine was used. In spite of meticulous care of the lips and oral cavity, suctioning near the buccal mucosa resulted in immediate sloughing of paper-thin epithelium. The patient was brought to the recovery room on the sheepskin and foam rubber padding in no distress. With the exception of one period of emesis, his postoperative course was uneventful, without emergence delirium. He was discharged on the following afternoon; he did not complain of pain but there was a generalized sloughing of his buccal. palatal, and gingival mucosa, which did not bother him. He said that he had less oral pain one day postoperatively than when the carious teeth had been in his mouth. He had an uneventful recovery. with his gingiva healing more slowly than normal but without evidence of infection (Fig. 5). He was sent home with instructions to take penicillin, 250 mg for five days, and was followed up as an outpatient. Discussion

ETIOLOGY Many mechanisms have been postulated for the etiology of epidermolysis bullosa dystrophica recessive (EBDR). These have included epidermal blood vessel continuity weakness;5,6 cutaneous malfunction and excess fluid collection, along with excess capillary permeability;7***g elastic tissue deficiency ;l”-13 thyroid, adrenal, or gonadal problems;14-22 and excessive protease activity.23 Recently, increased collagenase activity has been implicated in weakening the bonds between the dermis and epidermis, thus resulting in a separation upon relatively minor mechanical trauma.24-27 Ryan and Francis have supported this view in a separate study.28 These patients are treated palliatively because of the lack of obvious cure for this unique disease. Review of the literature shows that certain points are repeatedly emphasized. All of these patients are

dental cripples, and their ability to maintain good oral hygiene is limited despite dedicated attempts to comply with hygiene measures. As a result, multiple periapical pathosis develops. In addition to chronic skin lesions and secondary infections, the oral cavity serves as a source of constant bacteremia. The patient in this report developed a post-streptococcal glomerulonephritis with resultant labile hypertension. Obviously, conservative treatment may be extraction of the teeth to prevent future problems and to enable the patient to eat without odontalgia. CLINICAL MANIFESTATIONS The clinical manifestations of EBDR are specific and represent sequelae of repeated mechanical trauma and resultant scarring. Erosions and blisters are most often present at birth, especially about the feet, hands, and mouth. The early course may be quite variable, both in severity and penetrance. Slight trauma precedes most lesions, but some seem to occur spontaneously. The foot and hand lesions may heal with fusion of the digits and resultant mitten deformities, making ambulation difficult. The digits begin to fuse at different times in each patient’s life, representing that particular person’s course of disease. There may be loss of the nails as well. Gradually, the deep scarring and contractures make separation quite difficult, and the fingers fuse in the flexed position. However, the less severely affected often make remarkable use of their hands.2Y*30 Repeated cycles of blistering with secondary infection and healing leave wide areas of superficial scarring on the body. Blisters heal in two to ten days. A gradual cicatricial alopecia may occur as a result of the scarring, but the ears may be normal in appearance. Dysphagia may result from esophageal webbing secondary to trauma to the membranes of the esophagus.31-34 The perianal and genital mucosa are minimally involved. Early cataract formation as well as an increased incidence of cutaneous porphyria has been reported.’ Intraoral findings include multiple carious teeth, most likely as a result of poor dental hygiene. In other forms of the disease, odontogenic abberations have been found in the enamel.35,36 Ankyloglossia is common. The tongue is atrophic, and without the normal papillae, giving it a smooth glossy appearance. There are abundant gingival, buccal, pharyngeal, and palatal ulcers. Scarring of the commisures of the lips results in severe microstomia with limited opening.37-45 Laboratory findings represent secondary phenomena. Iron-deficiency anemia probably nutri-

EPIDERMOLYSIS

756 tional in origin, is common. Chronic infections of the skin may lead to general debilitation, amyloidosis, meningitis, and post-streptococcal glomerulonephritis. Hypertension secondary to renal problems is common.46 GENERAL TREATMENT Treatment is controversial and palliative for these patients. Cortiocosteroids have been tried in several patients without consistent benefit.46 An empirical trial of vitamin E in various dosages has not proved to reduce the number of bullous lesions.47-49 The hand deformity may be corrected,2g,30 and there can be dilatation of the esophageal webbing.31 Hypertension can be controlled, often by multiple drug regimens. The cataracts may be removed successfully.50 Seizures that may be secondary to meningitis in these children may also be controlled.J6 Dentition has proved to be almost unmanageable because of the gingival trauma resulting from “normal” toothbrushing. Thus, extraction of all teeth may be the resultant treatment. Prostheses produce considerable friction on the oral mucosa, resulting in bleb formation and sores.51 The food intake improves dramatically after the oral cavity is debrided, resulting in resolution of nutritional anemias.52 ANESTHETIC CONSIDERATIONS In reviewing the literature, we found that 28 patients with EBDR were reported to have undergone various anesthetics. Their ages ranged from 2 months to 35 years, with an average of 11.3 years. Seventeen males and 11 females were included. Thirteen patients had dental surgery, including extractions and restorations.52,53-56 Other surgery included hand surgeTy,2s,30*61,63-66 excision of epidermoid carcinoma,52 cataract removal,5o placement of intravenous lines,67 and an emergency cesarian section.68 Most of the patients had microcytic, hypochromic anemia consistent with iron deficiency.46,52,54,57*5s,61,62,65 All patients had a history of chronic infections. Other associated problems included esophageal stricture,2s,52,61,64,68cardiac problems 52,54*5sseizures,68*6s a pre-seizure history of meni;lgitis,53 post-streptococcal glomerulonephritis,61 limb disfigurement with limited range of motion (all patients), and multiple oral lesions. None of the patients had porphyrins in their urine 46,51,52,57,59-62,64,66,70 Preoperative sedation ranged from oral medicines 951,5581rectal suppositories,66 intramuscular injec_ injections, 46,50.53,57a60,63-65~67 and intravenous tions54 to no preoperative medications.52*54*56,58In-

BULLOSA

tramuscular injections were found to be tolerated well by 12 patients, but blebs formed at some of the sites.5*,66 When an intravenous site is available away from scarred regions, a premedicant is easily administered once the child is positioned. Adequate preoperative counseling is necessary so that the minimally premeditated child may move onto the operating table without traumatizing himself. The operating table should be padded to remove areas of stress and pressure.s1,55 Care should be taken to avoid warm wraps, which may increase the patient’s susceptibility to bulla formation.‘jl The operating room personnel should be informed of the patient’s disease and of the precautions to be followed.46 Intravenous lines should not be taped to the skin.46,61,71 They may be sutured in place or wrapped securely around the patient’s arm in nonocclusive gauze.51,54 Precordial stethoscopes have been used,53,56,“4,68 with areas of erythema produced when they are firmly positioned.61 Some authors believe they should be avoided.30,46*51,54,58,61,63,65 Palpation of the radial58 or brachiaF’ pulses to estimate perfusion has been advocated by some. Judicious use of blood pressure cuffs, with the skin padded with lubricated gauze, results in only minor irritation.2g,46,51-54* 56*58*62,63,65 Occlusive tourniquets have been used in hand repairs without significant reported sequelae. The oral manifestations of this disease play a major role in the airway management for these patients. Microstomia is commonplace; openings as small as 11 mm have been reported.53,55*57*5s*61,62 Visualization of the vocal cords is therefore extremely difIicult.46*55,61*62 Tracheostomy may be necessary in acute obstruction. 55 Because of repeated trauma, ankyloglossia is common, but it actually plays a favorable role by resulting in less tongue obstruction to the airway because of limited motion.52*62,68 The formation of bullae intraorally after minimal trauma predisposes these patients to obstructive phenomena, and close postoperative observation is needed.61 In nonfacial surgery, various open mask techniques2s,55,58*60,65,66,68 have been used successfully. A technique using a closed system via a polyethylene bag placed over the patient’s head has also been used.@’ However, many open techniques are not suitable because of exposure of the surgeons, assistants, and anesthesia personnel to the vapors.‘jl Surgical procedures have been performed using natural airways5i*55j61and oral airways.60*65 In spite of meticulous care taken by the anesthesiologist, all of the nasal and oral intubations resulted in lesions secondary to mechanical trauma.46,50*51,57*60,66,6s*72 In two patients, the oral lesions and the severe superficial sloughing necessitated hospitalization for an

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additional four weeks to provide local care.60369In one patient with EBDR, progressive laryngeal stenosis, developed, necessitating multiple laryngeal procedures. 6s Tracheostomy is not a panacea in these patients.46 Intubation should be used sparingly, and each patient should be individually asSessed.'"."O>46

Ideally. anesthesia produced without touching the patient is needed. Ketamine has been used as the sole anesthetic agent in many of these patients di_ with great success. 46,50-54-56~58,63,73 Emergence lerium may be controlled with diazepam prior to completion of the operative procedure. Drying agents can control the excessive salivation seen in many of these patients. 63 The use of a head-down position with gentle pharyngeal suction, careful hemostasis, and the use of a pharyngeal curtain may minimize upper airway obstruction.46,51*55,57*58*60*72 SURGICAL

CONSIDERATIONS

The patient should be medicated preoperatively with antibiotics to prevent postoperative systemic infections from possible bacteremia. These patients may be predisposed to post-streptococcal glomerulonephritis. After consideration of the clinical manifestations and anesthetic concerns of the patient with EBDR, certain special surgical techniques may be required. At the beginning of induction, a bite block should be placed. Meticulous suctioning of secretions and blood is necessary to prevent laryngeal spasm and airway obstruction. The surgical team should be ready to perform its duties immediately in order to minimize the length of ketamine anesthesia. Suction pressure should be decreased to minimize mucosal damage, although mucosal sloughing may be very difficult to prevent. The use of a topical coagulating agent such as Avitene may be advantageous. Gentle extraction techniques, with minimal manipulation of the gingiva, are recommended. Extractions will probably be simple, because of the extensive bone loss associated with the patient’s poor oral hygiene. Constant observation of the patient’s face by all surgical personnel is necessary to insure adequate lubrication and gentle handling of the tissues. Impactions may be left until the teeth erupt, thus avoiding excessive manipulation of the tissues. Summary The patient with EBDR provides a unique challenge to the oral and maxillofacial surgeon and anesthesia team. After review of the literature and familiarization with the preoperative considerations, a successful and relatively atraumatic general anesthetic

can be delivered in such a way that oral-facial surgery is accomplished without compromising the patient’s health. References In arzlicher Bericht des K. K. Allgemeinen Krankenhauses. Vienna. 1870, pp 362--364 2. Pearson R W: The mechanobullous diseases (epidermolysis bullosa). In Fitzpatrick TB: Dermatology in General Medicine. New York, McGraw-Hill Book Co, 1971 3. Fox C: Pemphigus in a woman of nine years duration. Br J Dermatol9:341, 1897 4. Jones RR: Epidermolysis bullosa: Report of a family and discussion of the dominant dystrophic types. Clin Exp Dermatol 4:303. 1979 Goldsheider A: Hereditare Neiging zur Blasenbildung. Mschr Prakt Derm l:l63, 1882 Koebner H: Hereditare Analage zur Blasenbildung. Dtsch Med Wochenschr 12:21. 1886 Blumer C: Hereditare zu traumatischer Blasenbildung. Arch Dermatol Syph 1I: 105. 1892 Elliot GT: A contribution to the histopathology of epidermolysis bullosa (hereditaria). NY State J Med 71:585, 1900 9. Winer MM. Orman JM: Epidermolysis bullosa: A suggestion as to possible causation. Arch Dermatol Syph 52:317. 1945 10. Engman MF. Mook WH: A study of some cases of eoidermolysis bullosa with remarks upon the congenital absence of elastic tissue. J Cutan Pathol 24:55, 19% II. Engman MF. Mook WH: A further contribution to the study of elastic tissue in epidermolysis bullosa. J Cutan Pathol 28:275, 1910 an zwei Familien mit Epider12. Dorn HI: Beobachtungen molysis bullosa hereditaria simplex: II. Biochemischgenetische Betrachtungen zur Epidermolysis buhosa hereditaria and entsprechende theraputische Massnahmem. Z Hautkr 23:40, 316, 1957 CM: Sur un des cas rares et 13. Hodara-Constantinople atypiques d’epidermolyse bulleuse (Kobner). J Dermatol Stud 21:609, 1910 14. Kaftan F: Ein Fall der Epidermolysis bullosa hereditaria mit typischen Erscheinurgen im Munde und and den Zahnen. Dtsch Mschr Zahnheilk 43:165, 1925 15. Beinhauer LG: Treatment of epidermolysis buflosa. Arch Dermatol Syph 32:469, 1935 16. Bonaduce F: Osservazioni comparative sopra diversi casi di pemifgo traumatic0 ed infantile. G Ital Dermatol 67:761. 1926 cutanees (sclerodermie. epider17. Drouet L: Endoctinides molyse bulleuse) chez un myxoedemateux. Bull Sot Franc Dermatol Syph 35:503, 1928 IS. Longo P: Distrotia cronica della pelle a tip di epidermolisi bullosa. Arch ltal Dermatol Vener 2:449. 1927 19. Marcozzi A: Epidermolisis bullosa distrofica con ematoporfurmuira ed alterazione endocrinosimpatica. Arch Ital Derm Vener 4:555, 1929 20. Pasini A: Dystrophie cutanee bulleuse atrophiante et albopapuloide. Ann Dermatol Syph (Paris) 9: 1044 1928 21. Pas&i A: epidermolisi congenital bullosa edo albopapuloide. G Ital Dermatol 73: 125, 1932 22. Stuhmer A: Ueber Epidermolysis bullosa congenita. Arch Dermatol Syph 121:568, l918- 19 23. Plenert W: Studie zur Blasenbildung bei der Epidermolysis bullosa. Z Kinderheilk 78329, 1956 24. Bauer EA. Eisen AZ: Recessive dystrophic epidermolysis bullosa. J Exp Med 148:1378, 1978 25. Lazarus GS: Collagenase: Its physiological and pathological role. Med times, 98:83. 1970 26. Eisen AZ: Human skin collagenase: Localization and distribution in normal human skin. J Invest Dermatol 52:442, I%9

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27. Eisen AZ: Human skin collagenase: Relationship to the pathogenesis of epidermolysis bullosa dystrophica. J Invest Dermatol 52449, 1969 28. Ryan TJ, Francis MJO: Epidermolysis bullosa dystrophica. Proc R Sot Med 68:449, 1975 29. Rees TD, Swinyard CA: Rehabilitative digital surgery in epidermolysis bullosa. Plast Reconstr Surg 40:169, 1967 30. Cuono C, Finseth F: Epidermolysis bullosa: Current concepts and management of the advanced hand deformity. Plast Reconstr Surg 62~280, 1978 31. Hillemeier C, et al: Esophageal web: A previously unrecognized complication of epidermolysis bullosa. Pediatrics 67:678, 1981 32. Ramadass T, Thangavelu TA: Epidermolysis bullosa and its ENT manifestations. J Laryngol Otol 92:441, 1978 33. Nix TE Jr, Christianson HB: Epidermolysis bullosa of the esophagus. South Med J 58:612, 1965 34. Kabakian HA. Dahmash NJ: Pharvngoesoohageal manifestations of epidermolysis bullosa.. Chin Radio: 29:91, 1978 35. Gardner DG, et al: The disturbances in odontogenesis in epidermolysis bullosa hereditaria letalis. Oral Surg 40:483, 1975 36 Arwell T, et al: Epidermolysis bullosa hereditaria: III. A histologic study of changes in teeth in the polydysplasta dystrophic and lethal forms. Oral Surg 19:723, 1965 37. Giansanti JS: Oral nodular excrescences in epidermolysis bullosa. Oral Surg 40:385, 1975 38. Crawford EG Jr, et al: Hereditary epidermolysis bullosa: Oral manifestations and dental therapy. Oral Surg 42:490, 1976 39. Gorlin RJ, Chaudhry AP: Unusual stomadromes, pachyonchia congenita, lipoid proteinosis, Reiter’s syndrome, epidermolysis bullosa, Bonnevie-Ullrich-Turner’s syndrome, and multiple hereditary telangiectasia. Oral Surg 13:257, 1960 40. Arwill T, Bergenholtz A: Epidermolysis bullosa hereditaria: VIII. Growth rate of the dentine in deciduous teeth in epidermolysis bullosa revealed by tetracycline lines. Arch Oral Biol, 13:819, 1968 41. Winstock D: Oral aspects of epidermolysis bullosa. Br J Dermatol 74:431, 1962 42. Farris CD: A case of dystrophic dentition in epidermolysis bullosa dystrophica. Proc R Sot Med, 56:619, 1963 43. Schow SR, Pay JT: Epidermolysis buIlosa dystrophica: Report of two cases. J Oral Surg 26:349, 1968 44. Gorlin RJ: Epidermolysis bullosa. Oral Surg 32:760, 1971 45. Kaslick RJ, Brustein HC: Epidermolysis buUosa: Review of the literature and report of a case. Oral Surg 14: 1315, 1961 46. Zaekheim HS, et al: Skin and bone disorders. In Katz J, Kadis LB: Anesthesia and Uncommon Diseases. Philadelphia, WB Saunders Co, 1973, pp 445-446 47. Unges WP, Nethescott JR: Epidermolysis bullosa dystrophica treated with vitamin E and oral corticosteroids. N Engl J Med 180:1136, 1973 48. Smith EB, Michener WM: Vitamin E treatment of dermolytic bullosa dermatosis. Arch Dermatol 108:254, 1973 49. Ayres S, Mihan R: Vitamin E treatment of dermolytic bullous dermatosis. Arch Dermatol 109:99, 1974 50. Pratilas V, Biezunski A: Epidermolysis bullosa manifested

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and treated during anesthesia. Anesthesiology 43:481, 1975 51. Endruschat AJ, Keenen DA: Anesthetic and dental management of a child with epidermolysis bullosa dystrophica. Oral Surg 36667, 1973 52. Lee C, Nagel EL: Anesthetic management of a patient with recessive epidermolysis bullosa dvstrophica. Anesthes_ iology 43:122, 1975 _ 53. Hubbert CH, Adams JG: Anesthetic management of patients with epidermolvsis bullosa. South Med J 70: 1375. 1977 54. Album MM, et al: Epidermolysis buUosa dystrophica polydysplastica: A case of anesthetic management in oral 55. Gormley JW, Schow CE: Epidermolysis bullosa and associated problems in oral surgical treatment. J Oral Surg 34~45, 1976 56. Morgan WL: Dental anesthetic management of epidermolysis bullosa: A new approach. Oral Surg 40:732, 1975 57. Howden EF, Oldenburg TR: Epidermolysis bullosa dystrophica: Report of two cases. J Am Dent Assoc 85: 1113, 1972 58. Hamann RA, Cohen PJ: Anesthetic management of a patient with epidermolysis bullosa dystrophica. Anesthesiology 34:389, 1971 59. Haas DD: Epidermolysis bullosa dystrophica. Oral Surg 26:291, 1968 60. Marshall BE: A comment on epidermolysis bullosa and its anaesthetic management for dental operations. Br J Anaesth 35:724, 1963 61. Reddy ARR, Wong DHW: Epidermolysis bullosa: A review of anaesthetic problems and case reports. Can Anaesth Sot J 19:536, 1972 62. Boyer HE, Owens RH: Epidermolysis bullosa: A rare disease of dental interest. Oral Surg 14:1170, 1961 63. Loverme SR, Oropollo AT: Ketamine anesthesia in dermolytic bullous dermatosis (epidermolysis bullosa). Anesth Analg 56:398, 1977 64. Petty WL, Gunther RC: Anesthesia for nonfacial surgery in polydisplastic epidermolysis bullosa (dystrophic). Anesth Analg 49:246, 1970 65. Kubota Y, et al: Anesthetic management of patients with epidermolysis bullosa undergoing surgery. Anesth Analg 40:244, 1961 66. Micheels J: Anesthetic management of a child with epidermolysis bullosa. Acta Anesthesiol Belg 29: 141, 1978 67. Kelly AJ: Epidermolysis buUosa dystrophica: Anesthetic management. Anesthesiology 35:659, 1971 68. Berryhill RE, et al: Anesthetic management of emergency cesarean section in a patient with epidermolysis bullosa dystrophica polydysplastica. Anesth Analg 57:281, 1978 69. Cohen SR, et al: Epidermolysis bullosa associated with laryngeal stenosis. Ann Otol Rhino1 Laryngol 87 (Suppl 5): 25, 1978 70. Marshall J: Diseases of the Skin. Edinburgh, Churchill Livingstone, 1960 71. Simon CR: The effect of certain types of trauma in epidermolysis buUosa. J Pediatr 47:750. 1955 72. Wilson F: Epidermolysis buUosa: Arare disease of anesthetic interest. 31:26, 1959 73. Corssen G, et al: Ketamine in the anesthetic management of asthmatic patients. Anesth Analg 51:588, 1972