- Email: [email protected]
Hairy leukoplakia with involvement of the buccal mucosa
I
II
Hairy leukoplakia with involvement of the buccal mucosa G. Ficarra, MD, P. Romagnoli, MD, S. Piluso, DDS, D. Milo, MD, and K. Adler-Storthz, PhD Florence, Italy, and Houston, Texas Oral hairy leukoplalda occurs mainIy on the tongue of human immunodeficiency virus (HIV)-infected persons. An HIV-infeeted patient with hairy leukoplakia involving the tongue and buccal mucosa was studied by light and electron microscopic methods, in situ hybridization, and polymerase chain reaction. Our findings indicate that hairy leukoplakia may involve the buccal mucosa and should be considered in the differential diagnosis of white oral lesions in HIV-positive patients. Epstein-Barr virus particles were found in the epithelial cells of both buccal and tongue mucosa. (J AM ACADDERMATOL1992;27:855-8.)
Oral hairy leukoplakia ( O H L ) occurs mainly in patients who are infected with human immunodeficiency virus (HIV), 1"4 although it has also recently been describbd in patients with other forms of immunosuppression.5, 6 O H L appears predominantly on the lateral borders of the tongue, although a few cases of involvement of the buccal mucosa have been reported. 1, 2, 7 Greenspan et al. 1 described 2 of 37 patients with O H L of the tongue who also had lesions on the buccal mucosa. Eversole et al. 2 observed five patients who had O H L lesions with extension to other mucosal sites. Recently Kabani et al. 7 reported two cases of O H L with extensive involvement of the palate, floor of the mouth, vestibular mucosa, and pharynx. We describe a woman who was HIV-1 seropositire, abused drugs intravenously, and developed O H L of the tongue and buccal mucosa. CASE REPORT A 25-year-old HIV-infected woman with a history of intravenous drug abuse had a white Iesion of the buccal mucosa and the tongue. The patient had been treated with antifungal medications and acyclovir and had only a parFrom the Institute of Stomatology,Departmentof HumanAnatomy and Histologyand DivisionofInfectiousDiseases,UniversityofFlorenceandUSL I0/D, and the DepartmentofMicrobiology,University ofTexas. Supportedin part by grant DE-08061from the National Instituteof Health and by the AmericanFundfor Dental Health. Reprint requests: GiuseppeFicarra, MD, Institute of Stomatology, Universityof Florence,VialeMorgagni 85~50134Florence,Italy. 16/4/35798
tial, transient response. Examination revealed white keratotic patches with a smooth surface on both sides of the vestibular mucosa (Fig. 1). The lateral margins of the tongue had vertical white striae (Fig. 2). A clinical diagnosis of OHL with possible involvement of the buccal mucosa was made. The patient's CD4 T Iymphocyte level was 46 cells/mm 3 (6%), the CD8 T lymphocyte level was 292 cells/mm 3 (38%), and the CD4/CD8 ratio was 0.16. Treatment with oral acyclovir was unsuccessful. Material and methods Punch biopsy specimens were taken from the right border of the tongue and the right buccal mucosa for routine histopathologic and electron microscopic examination. In situ hybridization was used to detect EpsteinBarr virus (EBV) DNA in formalin-fixed tissues. A biotin-labeled EBV DNA probe, which consisted of the BamHI"V" fragment of the EBV genome cloned into the plasmid pBR322 (Enzo Biochem, Inc., New York, N.Y.) was used. Biotin-labeled plasmid pBR322 DNAwas used as a negative control. Details of the technique have been described previously.8 The polymerase chain reaction (PCR) and Southern blot hybridization were used to test for the presence of human papillomavirus (HPV) type 6, 11, 16, and 18 in the tissues. Details of this technique have been reported elsewhere.9,10
Results Light and electron microscopic studies. Microscopic examination of biopsy tissue from the tongue showed hairlike surface projections, hyperparakeratosis, and vacuolated koilocytoid cells in thespinous and granular layer. Tissue from the buccal mucosa showed moderate acanthosis and hyperparakeratosis with ballooning cells in the upper layers (Fig. 3). The histopathologic findings were 855
856
Journal of the American Academy of Dermatology
Ficarra el al.
Fig. 1. White keratotic patches on both sides of buccal mucosa.
Fig. 2. Hairy leukoplakia of the tongue.
consistent with those described for OHL and suggested a viral cause. Ultrastructural studies demonstrated the presence of intracellular herpes-type viral particles within the koilocytoid cells in tissues from both the tongue and buccal mucosa (Fig. 4). In situ hybridization and PCR. Positive EBV DNA hybridization, as evidenced by dark brown deposits of the reaction product, was observed in the nuclei of both koilocytoid and nonkoilocytoid cells in the upper spinous and granular layers of tissues of both the tongue and buccal mucosa. No HPV DNA was found in the OHL tissues by PCR and Southern blot hybridization. DISCUSSION 0 H L with extralingual involvement is infrequently observed in HIV-infected patients. Although O H L of the tongue has a characteristic appearance that makes it easily recognizable, 0 H L of the buccal mucosa may be confused with other white lesions. In patients with H I V infection differential diagnosis includes candidiasis, idiopathic or smoking-related leukoplakia, frictional keratosis, carci-
noma, idiopathic lichen planus, and drug-related lichenoid reactions. 7, 11 Candidiasis can be differentiated by routine laboratory studies. Leukoplakia, frictional keratosis, lichen planus, and lichenoid reactions may require a biopsy to confirm the clinical diagnosis. Our clinical and histopathologic findings and the results of the electron microscopic and in situ hybridization studies are consistent with those previously reported. Although we have diagnosed more than 150 cases of O H L of the tongue, this is the first time we have observed a patient with O H L of the buccal mucosa, which, in addition, did not respond to treatment with acyclovir. Evidence from several studies has indicated that EBV is involved in the cause of OHL, and demonstration of this virus has been considered mandatory for its definitive diagnosis, l, 4, 12, t3 However, a recent study reported the presence of EBV D N A and HPV D N A in both O H L and normal buccal mucosa of HIV-infected patients. 9
Volume 27 Number 5, Part 2 November 1992
Hairy leukoplakia and buccal mucosa
857
Fig. 4. Electron microscopic view of keratinocytes from biopsy specimen of buccal mueosa, which contain intranuclear immature virions of herpes type. Mature virions of the same type are free in the intercellular space. (Uranyl acetate and bismuth tartrate; •
Fig. 3. Tissue from buccal mucosa shows moderate acanthosis and hyperparakeratosis with ballooning cells in upper layers. (Hematoxylin and eosin stain; X 10.)
The development of O H L on the buecal mucosa raises several questions in relation to the underlying pathogenetic mechanisms. It is not understood why O H L appears almost always on the tongue and is so uncommon on the buccal mucosa. EBV is a herpes virus that causes subclinical infection of the oral cavity in most of the human population. Replicating EBV has been found in desquamative epithelial cells of pharyngeal washings from both normal persons and patients with mononucleosis. 14, 15 In HIV-infected patients EBV has been detected in both O H L and normal oral epithelium and is shed in saliva. 9, 16 Thus a higher number of cases of O H L on the buccal mucosa might be expected. This may imply that because EBV is ubiquitious in immunosuppressed persons, it is present by chance in O H L or that other local cofactors are necessary for the genesis of OHL. Riccardi et al. 17 recently reported that the depletion
of nonlymphoid accessory cells in the buccal mucosa of patients with H I V infection m a y facilitate the appearance of OHL. The pathogenesis of O H L has been strongly linked to the replication of EBV in the lesional epithelium.18 Given this finding, why does EBV replicate only in the tongue mucosa and give rise to O H L only on the tongue? Also, what is the mechanism that keeps the replication of EBV under control in the remaining buccal mucosa? The situation appears even more complicated when the findings of Sugihara et al. 19 are considered. They reported that replicative EBV particles can be found in scrapings of clinically normal mucosa of HIV-infected patients. REFERENCES
1. GreenspanD, Greenspan JS, Conant M, et al. Oral"hairy" leukoplakia in male homosexuals: evidence of association with both papillomavirus and a herpes-group virus. Lancet 1984;2:831-4. 2. EversoleLR, JacobsenP, Stone CE, et al. Oral condyloma planus (hairy leukoplalda) among homosexual men: a clinicopathologic study of thirty-six cases. Oral Surg 1986;61:249-55. 3. Greenspan D, Hollander H, Friedman-Kien A, et al. Oral hairy leukoplakia in two women, a hemophiliac and a transfusion recipient [Letter], Lancet 1986;2:378. 4. Ficarra G, Barone R, Gaglioti D, et al, Oral hairy leukoplakia among intravenous drug abusers: a clinicopathologic and ultrastructural study. Oral Surg 1988;65:421-6. 5. Itin P, Rufli T, Rudlinger R, et al. Oral hairy leukoplalda in a HIV-negativerenal transplant patient: a marker for immunosuppression? Demaatologica 1988;177:126-8.
Journal of the American Academy of Dermatology
Ficarra et al. 6. Ficarra G, Miliani A, Adler-Storthz K, et al. Recurrent oral condylomata acuminata and hairy leukoplakia: an early sign of myeledisplastic syndrome in a HIV-seronegative patient. J Oral Pathol Med 1991;20:398-402. 7. KabaniS, Greenspan D, De Souza Y, et aL Oral hairy leukoplakia with extensive oral mucosal involvement. Oral Surg 1989;67:4ll-5. 8. Adler-Storthz K, Newland JR, Tessin BA, et al. Identification of human papillomavirus types in oral verruca vulgaris. J Oral Pathol Med 1986;15:230-3. 9. Adler-Storthz K, Ficarra G, Woods-Francis K, et al. Prevalence of Epstein-Barr virus and human papillomavirus in oral mucosa of HIV-infected patients. J Oral Pathol Meal (submitted for publication). 10. Ting Y, Manos MM. Detection and typing of genital human papillomaviruses. In: Innis MA, Gelfand DH, Snislay J, et al., eds. PCR protocols: a guide to methods and applications. New York: Academic Press, 1990:356. 11. Ficarra G, Romagnoli P, Flaitz CM, et at. Oral lichenoid reactions in patients with human immunodefieiency virus infection. J Oral Pathol Med (submitted for publication). 12. Greenspan JS, Greenspan D, Lennette ET, et al. Replication of Epstein-Barr virus within the epithelial cells of oral
13. 14. 15. 16.
17. 18. 19.
"hairy" leukoplakia, an AIDS-associated lesion. New Engl J Med 1985;313:1564-71. Greenspan JS, Greenspan D. Oral hairy leukoplakia: diagnosis and management. Oral Surg 1989;67:396-403. Morgan D, Miller G, Niederman J, et al. Site of EpsteinBarr replication in the oropharynx. Lancet 1979; 1:1154-65. Diaz-Mitoma F, Preiksaitis JK, Leung W-C, et al. DNADNA dot hybridization to detect Epstein-Barr virus in throat washings. J Infect Dis 1987;155:297-303. Diaz-Mitoma F, Ruiz A, Flowerdew G, et al. High levels of Epstein-Barr virus in the oropharynx: a predictor of diseases progression in human immunodefieiency virus infection. J Med Virol 1990;31:69-75. Riccardi R, Pimpinelli N, Ficarra G, et al. Morphology and membrane antigens of nonlymphoid accessory cells in oral hairy leukoplakia. Hum Pathol 1990;21:897-904. Greenspan JS, Greenspan D. Oral hairy leukoplakia: diagnosis and management. Oral Surg 1989;67:396-403. Sugihara K, Reupke H, Schimidt-Westhausen A, et al. Negative staining EM for the detection of Epstein-Barr virus in oral hairy leukoplakia. J Oral Pathol Med 1990; 19:367-70.
Epidermolysis bullosa acquisita in a 31A-year-old girl Hugues Roger, MD, a Paulo Machado, MD, c Jean-Franpois Nicolas, MD, c Michel D'Incan, MD, a Catherine Prost, MD, b Claude Chevenet, MD, a Jean Kanitakis, MD, c Marie-Christine Ferrier, MD, a Brigitte Chouvet, MD, c and Pierre Souteyrand, MD a Clermont-Ferrand, Crbteil, and Lyon, France A 31A-year-old girl had a subepidermal bullous eruption with immunopathologic features that were consistent with epidermolysis buUosa acquisita or bullous systemic lupus erythematosus. This report highlights the difficulty encountered in distinguishing between epidermolysis bullosa acquisita and other buUous disorders that involve the dermoepidermal junction and the need for modern immunologic investigations in the diagnosis of bullous diseases in children. (J AM ACAD DERMATOL 1992;27:858-62.)
Immunobullous diseases in children are rare. We describe an unusual chronic bullous eruption in a child with features of epidermolysis bullosa acquisita (EBA) and systemic lupus erythematosus (SLE).
From the Department of Dermatology, H6tel-Dieu,n Inserm U.312, H6pital Henri-Mnndor,b and the Department of Dermatologyand Inserm U.209, H6pital Edouard-Flerrlot.e Reprintrequests:Pr, Pierre Souteyrand,MD, Servicede Dermato[ogieu HStel-Dieu, BP 69, 63003 Clermont-FerrandCedex, France. 16/4136060
858
CASE R E P O R T A 31A-year-old girl had a recurrent bullous disorder for 8 months. Her eruption consisted of slightly pruritic vesicles and bullae arising on erythematous skin; most were taut and sometimes exhibited a "string-of-pearls" arrangement. Bullae occurred on the face, nape of the neck, and back of the hands. No cutaneous fragility was observed, and Nikolsky's sign was absent. Scarring, milia, and nail dystrophy did not appear at this time. The eruption worsened in sun-exposed areas after a few days at high altitude (Fig. 1), with bullae on the flexural areas and oral and vulvar mucosae. Prednisolone (1 m g / kg/day) was begun, and the skin lesions cleared within 8 weeks. The bullae healed with formation of milia and
II
Hairy leukoplakia with involvement of the buccal mucosa G. Ficarra, MD, P. Romagnoli, MD, S. Piluso, DDS, D. Milo, MD, and K. Adler-Storthz, PhD Florence, Italy, and Houston, Texas Oral hairy leukoplalda occurs mainIy on the tongue of human immunodeficiency virus (HIV)-infected persons. An HIV-infeeted patient with hairy leukoplakia involving the tongue and buccal mucosa was studied by light and electron microscopic methods, in situ hybridization, and polymerase chain reaction. Our findings indicate that hairy leukoplakia may involve the buccal mucosa and should be considered in the differential diagnosis of white oral lesions in HIV-positive patients. Epstein-Barr virus particles were found in the epithelial cells of both buccal and tongue mucosa. (J AM ACADDERMATOL1992;27:855-8.)
Oral hairy leukoplakia ( O H L ) occurs mainly in patients who are infected with human immunodeficiency virus (HIV), 1"4 although it has also recently been describbd in patients with other forms of immunosuppression.5, 6 O H L appears predominantly on the lateral borders of the tongue, although a few cases of involvement of the buccal mucosa have been reported. 1, 2, 7 Greenspan et al. 1 described 2 of 37 patients with O H L of the tongue who also had lesions on the buccal mucosa. Eversole et al. 2 observed five patients who had O H L lesions with extension to other mucosal sites. Recently Kabani et al. 7 reported two cases of O H L with extensive involvement of the palate, floor of the mouth, vestibular mucosa, and pharynx. We describe a woman who was HIV-1 seropositire, abused drugs intravenously, and developed O H L of the tongue and buccal mucosa. CASE REPORT A 25-year-old HIV-infected woman with a history of intravenous drug abuse had a white Iesion of the buccal mucosa and the tongue. The patient had been treated with antifungal medications and acyclovir and had only a parFrom the Institute of Stomatology,Departmentof HumanAnatomy and Histologyand DivisionofInfectiousDiseases,UniversityofFlorenceandUSL I0/D, and the DepartmentofMicrobiology,University ofTexas. Supportedin part by grant DE-08061from the National Instituteof Health and by the AmericanFundfor Dental Health. Reprint requests: GiuseppeFicarra, MD, Institute of Stomatology, Universityof Florence,VialeMorgagni 85~50134Florence,Italy. 16/4/35798
tial, transient response. Examination revealed white keratotic patches with a smooth surface on both sides of the vestibular mucosa (Fig. 1). The lateral margins of the tongue had vertical white striae (Fig. 2). A clinical diagnosis of OHL with possible involvement of the buccal mucosa was made. The patient's CD4 T Iymphocyte level was 46 cells/mm 3 (6%), the CD8 T lymphocyte level was 292 cells/mm 3 (38%), and the CD4/CD8 ratio was 0.16. Treatment with oral acyclovir was unsuccessful. Material and methods Punch biopsy specimens were taken from the right border of the tongue and the right buccal mucosa for routine histopathologic and electron microscopic examination. In situ hybridization was used to detect EpsteinBarr virus (EBV) DNA in formalin-fixed tissues. A biotin-labeled EBV DNA probe, which consisted of the BamHI"V" fragment of the EBV genome cloned into the plasmid pBR322 (Enzo Biochem, Inc., New York, N.Y.) was used. Biotin-labeled plasmid pBR322 DNAwas used as a negative control. Details of the technique have been described previously.8 The polymerase chain reaction (PCR) and Southern blot hybridization were used to test for the presence of human papillomavirus (HPV) type 6, 11, 16, and 18 in the tissues. Details of this technique have been reported elsewhere.9,10
Results Light and electron microscopic studies. Microscopic examination of biopsy tissue from the tongue showed hairlike surface projections, hyperparakeratosis, and vacuolated koilocytoid cells in thespinous and granular layer. Tissue from the buccal mucosa showed moderate acanthosis and hyperparakeratosis with ballooning cells in the upper layers (Fig. 3). The histopathologic findings were 855
856
Journal of the American Academy of Dermatology
Ficarra el al.
Fig. 1. White keratotic patches on both sides of buccal mucosa.
Fig. 2. Hairy leukoplakia of the tongue.
consistent with those described for OHL and suggested a viral cause. Ultrastructural studies demonstrated the presence of intracellular herpes-type viral particles within the koilocytoid cells in tissues from both the tongue and buccal mucosa (Fig. 4). In situ hybridization and PCR. Positive EBV DNA hybridization, as evidenced by dark brown deposits of the reaction product, was observed in the nuclei of both koilocytoid and nonkoilocytoid cells in the upper spinous and granular layers of tissues of both the tongue and buccal mucosa. No HPV DNA was found in the OHL tissues by PCR and Southern blot hybridization. DISCUSSION 0 H L with extralingual involvement is infrequently observed in HIV-infected patients. Although O H L of the tongue has a characteristic appearance that makes it easily recognizable, 0 H L of the buccal mucosa may be confused with other white lesions. In patients with H I V infection differential diagnosis includes candidiasis, idiopathic or smoking-related leukoplakia, frictional keratosis, carci-
noma, idiopathic lichen planus, and drug-related lichenoid reactions. 7, 11 Candidiasis can be differentiated by routine laboratory studies. Leukoplakia, frictional keratosis, lichen planus, and lichenoid reactions may require a biopsy to confirm the clinical diagnosis. Our clinical and histopathologic findings and the results of the electron microscopic and in situ hybridization studies are consistent with those previously reported. Although we have diagnosed more than 150 cases of O H L of the tongue, this is the first time we have observed a patient with O H L of the buccal mucosa, which, in addition, did not respond to treatment with acyclovir. Evidence from several studies has indicated that EBV is involved in the cause of OHL, and demonstration of this virus has been considered mandatory for its definitive diagnosis, l, 4, 12, t3 However, a recent study reported the presence of EBV D N A and HPV D N A in both O H L and normal buccal mucosa of HIV-infected patients. 9
Volume 27 Number 5, Part 2 November 1992
Hairy leukoplakia and buccal mucosa
857
Fig. 4. Electron microscopic view of keratinocytes from biopsy specimen of buccal mueosa, which contain intranuclear immature virions of herpes type. Mature virions of the same type are free in the intercellular space. (Uranyl acetate and bismuth tartrate; •
Fig. 3. Tissue from buccal mucosa shows moderate acanthosis and hyperparakeratosis with ballooning cells in upper layers. (Hematoxylin and eosin stain; X 10.)
The development of O H L on the buecal mucosa raises several questions in relation to the underlying pathogenetic mechanisms. It is not understood why O H L appears almost always on the tongue and is so uncommon on the buccal mucosa. EBV is a herpes virus that causes subclinical infection of the oral cavity in most of the human population. Replicating EBV has been found in desquamative epithelial cells of pharyngeal washings from both normal persons and patients with mononucleosis. 14, 15 In HIV-infected patients EBV has been detected in both O H L and normal oral epithelium and is shed in saliva. 9, 16 Thus a higher number of cases of O H L on the buccal mucosa might be expected. This may imply that because EBV is ubiquitious in immunosuppressed persons, it is present by chance in O H L or that other local cofactors are necessary for the genesis of OHL. Riccardi et al. 17 recently reported that the depletion
of nonlymphoid accessory cells in the buccal mucosa of patients with H I V infection m a y facilitate the appearance of OHL. The pathogenesis of O H L has been strongly linked to the replication of EBV in the lesional epithelium.18 Given this finding, why does EBV replicate only in the tongue mucosa and give rise to O H L only on the tongue? Also, what is the mechanism that keeps the replication of EBV under control in the remaining buccal mucosa? The situation appears even more complicated when the findings of Sugihara et al. 19 are considered. They reported that replicative EBV particles can be found in scrapings of clinically normal mucosa of HIV-infected patients. REFERENCES
1. GreenspanD, Greenspan JS, Conant M, et al. Oral"hairy" leukoplakia in male homosexuals: evidence of association with both papillomavirus and a herpes-group virus. Lancet 1984;2:831-4. 2. EversoleLR, JacobsenP, Stone CE, et al. Oral condyloma planus (hairy leukoplalda) among homosexual men: a clinicopathologic study of thirty-six cases. Oral Surg 1986;61:249-55. 3. Greenspan D, Hollander H, Friedman-Kien A, et al. Oral hairy leukoplakia in two women, a hemophiliac and a transfusion recipient [Letter], Lancet 1986;2:378. 4. Ficarra G, Barone R, Gaglioti D, et al, Oral hairy leukoplakia among intravenous drug abusers: a clinicopathologic and ultrastructural study. Oral Surg 1988;65:421-6. 5. Itin P, Rufli T, Rudlinger R, et al. Oral hairy leukoplalda in a HIV-negativerenal transplant patient: a marker for immunosuppression? Demaatologica 1988;177:126-8.
Journal of the American Academy of Dermatology
Ficarra et al. 6. Ficarra G, Miliani A, Adler-Storthz K, et al. Recurrent oral condylomata acuminata and hairy leukoplakia: an early sign of myeledisplastic syndrome in a HIV-seronegative patient. J Oral Pathol Med 1991;20:398-402. 7. KabaniS, Greenspan D, De Souza Y, et aL Oral hairy leukoplakia with extensive oral mucosal involvement. Oral Surg 1989;67:4ll-5. 8. Adler-Storthz K, Newland JR, Tessin BA, et al. Identification of human papillomavirus types in oral verruca vulgaris. J Oral Pathol Med 1986;15:230-3. 9. Adler-Storthz K, Ficarra G, Woods-Francis K, et al. Prevalence of Epstein-Barr virus and human papillomavirus in oral mucosa of HIV-infected patients. J Oral Pathol Meal (submitted for publication). 10. Ting Y, Manos MM. Detection and typing of genital human papillomaviruses. In: Innis MA, Gelfand DH, Snislay J, et al., eds. PCR protocols: a guide to methods and applications. New York: Academic Press, 1990:356. 11. Ficarra G, Romagnoli P, Flaitz CM, et at. Oral lichenoid reactions in patients with human immunodefieiency virus infection. J Oral Pathol Med (submitted for publication). 12. Greenspan JS, Greenspan D, Lennette ET, et al. Replication of Epstein-Barr virus within the epithelial cells of oral
13. 14. 15. 16.
17. 18. 19.
"hairy" leukoplakia, an AIDS-associated lesion. New Engl J Med 1985;313:1564-71. Greenspan JS, Greenspan D. Oral hairy leukoplakia: diagnosis and management. Oral Surg 1989;67:396-403. Morgan D, Miller G, Niederman J, et al. Site of EpsteinBarr replication in the oropharynx. Lancet 1979; 1:1154-65. Diaz-Mitoma F, Preiksaitis JK, Leung W-C, et al. DNADNA dot hybridization to detect Epstein-Barr virus in throat washings. J Infect Dis 1987;155:297-303. Diaz-Mitoma F, Ruiz A, Flowerdew G, et al. High levels of Epstein-Barr virus in the oropharynx: a predictor of diseases progression in human immunodefieiency virus infection. J Med Virol 1990;31:69-75. Riccardi R, Pimpinelli N, Ficarra G, et al. Morphology and membrane antigens of nonlymphoid accessory cells in oral hairy leukoplakia. Hum Pathol 1990;21:897-904. Greenspan JS, Greenspan D. Oral hairy leukoplakia: diagnosis and management. Oral Surg 1989;67:396-403. Sugihara K, Reupke H, Schimidt-Westhausen A, et al. Negative staining EM for the detection of Epstein-Barr virus in oral hairy leukoplakia. J Oral Pathol Med 1990; 19:367-70.
Epidermolysis bullosa acquisita in a 31A-year-old girl Hugues Roger, MD, a Paulo Machado, MD, c Jean-Franpois Nicolas, MD, c Michel D'Incan, MD, a Catherine Prost, MD, b Claude Chevenet, MD, a Jean Kanitakis, MD, c Marie-Christine Ferrier, MD, a Brigitte Chouvet, MD, c and Pierre Souteyrand, MD a Clermont-Ferrand, Crbteil, and Lyon, France A 31A-year-old girl had a subepidermal bullous eruption with immunopathologic features that were consistent with epidermolysis buUosa acquisita or bullous systemic lupus erythematosus. This report highlights the difficulty encountered in distinguishing between epidermolysis bullosa acquisita and other buUous disorders that involve the dermoepidermal junction and the need for modern immunologic investigations in the diagnosis of bullous diseases in children. (J AM ACAD DERMATOL 1992;27:858-62.)
Immunobullous diseases in children are rare. We describe an unusual chronic bullous eruption in a child with features of epidermolysis bullosa acquisita (EBA) and systemic lupus erythematosus (SLE).
From the Department of Dermatology, H6tel-Dieu,n Inserm U.312, H6pital Henri-Mnndor,b and the Department of Dermatologyand Inserm U.209, H6pital Edouard-Flerrlot.e Reprintrequests:Pr, Pierre Souteyrand,MD, Servicede Dermato[ogieu HStel-Dieu, BP 69, 63003 Clermont-FerrandCedex, France. 16/4136060
858
CASE R E P O R T A 31A-year-old girl had a recurrent bullous disorder for 8 months. Her eruption consisted of slightly pruritic vesicles and bullae arising on erythematous skin; most were taut and sometimes exhibited a "string-of-pearls" arrangement. Bullae occurred on the face, nape of the neck, and back of the hands. No cutaneous fragility was observed, and Nikolsky's sign was absent. Scarring, milia, and nail dystrophy did not appear at this time. The eruption worsened in sun-exposed areas after a few days at high altitude (Fig. 1), with bullae on the flexural areas and oral and vulvar mucosae. Prednisolone (1 m g / kg/day) was begun, and the skin lesions cleared within 8 weeks. The bullae healed with formation of milia and