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Conjunctival dermoid cyst in an infant
784
Journal of the American Academy of Dermatology May 1997
Brief communications
bility to toxic epidermal necrolysis. Arch Dermatol 1987; 123:1171-3. 9. Chan SH, Tan T. HLA and aUopurinol drug eruption. Dermatologica 1989;179:32-3. 10. Hertl M, Bohlen H, Jugert F, et al. Predominance of epidermal CD8÷ T lymphocytes with bullous cutaneous reactions caused by beta-lactam antibiotics. J Invest Dermatol 1993;101:794-9. 11. Bell SJ, Pichler WJ. Penicillin-allergic patients react to penicillin-modified "self." Allergy 1989;44:199-203.
12. Class FI-IJ, Rumia-van Nieuwkoop R, van den Berge W, et al. Interaction of penicillin with HLA-A and B antigens. Hum Imrnunol 1982;5:83-90. 13. Shiohara T. What is new in fixed drug eruption? Dermatology 1995;191:185-7. 14. Hertl M, Merk HF. Lymphocyte activation in cutaneous drug reactions. J Invest Dermatol 1995;105:95-8S. 15. Kalish RS. Antigen processing: the gateway to the immune response. J Am Acad Dermatol 1995;32:64052.
Conjunctival dermoid cyst in an infant Yoshiko Kudo, M D , a K a z u m o t o Katagiri, M D , a Yoshiteru Ishii, M D , a Hiromi Shibuya, MD, a Hiroto Terashi, M D , a Hiroyuki Hashimoto, M D , a Susumu Takayasu, M D , a and Kenji Matsuo, M D b Oita, Japan
Orbital dermoid cysts are usually lined b y keratinizing squamous epithelium with adnexal structures. 1-3 W e describe an infant with an orbital conjunctival dermoid cyst that was lined mainly with nonkeratinlzing epithelium that contained goblet cells.
CASE REPORT A 7-month-old Japanese girl had a soft, elastic, bluered, subcutaneous nodule on her lower left eyelid (Fig. 1). Although she could hardly open that eye, her visual acuity and motility of the eye appeared to be normal. We initially diagnosed a cavernous hemangioma and, to prevent amblyopia, attempted to reduce its size with betamethasone, 0.5 mg daily for 36 days. However, there was no response. When the patient was 1 year of age, we injected a mixture of betamethasone, 6 rag, and methylprednisolone, 40 mg, into the lesion. This treatment significantly reduced its size within a few days. But during the next 5 months, the mass enlarged and virtually closed the eye. The lesion was reinjected with corticosteroids several times. Computed tomographic scans
From the Departments of Dermatologya and Ophthalmology? Oita Medical University. Reprint requests: Yoshiko Kudo, MD, Department of Dermatology, Oita Medical University, 1-1 Idaigaoka Hasama-machi, Oita 879-55, Japan. J Am Acad Dermatol 1997;36:784-5. Copyright © 1997 by the American Academy of Dermatology, Inc. 0190-9622/97/$5.00 + 0 16/54/79673
Fig. 1. Marked swelling of lower eyefid at age 7 months.
showed a cystic mass of low density, which suggested a dermoid cyst. When the patient was 27 months of age, we performed
Journal of the American Academy of Dermatology Volume 36, Number 5, Part 1
A
Fig. 2. A, Both keratinizing (left) and nonkeratinizing epithelia (right) are present. B, Nonkeratinizing epithelium contains numerous goblet cells. (A and B, Hematoxylin-eosin stain; original magnifications: A, xl00; B, x200.)
an orbitotomy and removed a conjunctival 20 x 30 mm derrnoid cyst. Histologic examination revealed that the cyst was lined mainly with nonkerafinizing epithelium and partly with keratinizing epithelium (Fig. 2, A). The nonkeratinizing epithelium contained numerous goblet cells (Fig. 2, B). The stroma of the cyst wall contained several sebaceous glands and sweat glands around the keratiuizing epithelium. During 2 years of follow-up, there were no postoperative complications or recurrences, and the child's visual acuity is normal. DISCUSSION
Orbital dermoid cysts can usually be differentiated clinically from other lesions by their superorbital location, cystic nature, and relation to b o n e ) In our patient, however, because the tumor was soft, bluered, and was present in an unusual site, we initially diagnosed a cavernous hemangioma. The marked improvement observed after the first intralesional injection of corticosteroids was probably due to the
Brief communications
785
antiinflammatory effect on the granulomatous tissue that surrounded the cyst. Dermoid cysts are benign developmental choristomas that are usually classified as superficial (simple) and deep (complicated) types. 1, 3 The superficial type is usually discovered in infancy as a painless subcutaneous nodule commonly located superolaterally beneath the brow. The deep type usually occurs in adults. It is associated with proptosis or displacement of the globe and tends to produce bony destruction. Jakobiec, Bonanno, and Sigelman 4 described a subgroup of epidermoid and dermoid cysts that were lined with conjunctival epithelium and called them conjtmctival cysts, or conjunctival dermoid cysts, according to the absence or presence of appendages. To our knowledge, only 11 cases of conjunctival cysts and 11 cases of conjunctival dermoid cysts, including the present case, have been reported in the literature. 4-8 Both conjuncfival and conjunctival dermoid cysts are usually located superonasally in the orbit and do not affect the bone. 4-8 Conjunctival dermoid cysts often occur in the second decade of life. 4 Only one case was reported to appear in infancy. 8 Conjunctival dermoid cysts are thought to represent embryologic sequestrations of the inner layer of ectoderm that normally forms the conjunctiva, rather than sequestrations of the surface epidermis. 4
REFERENCES
1. Grove JrAS. Orbital disorders: diagnosis and management. In: McCord CD Jr, editor. Oculoplasticsurgery. New York: Raven Press, 1981:257-84. 2. Shields JA, Bakewell B, AugsburgerJJ, et al. Classification and incidence of space-occupyinglesions of the orbit: a survey of 645 biopsies. Arch Ophthalmol 1984;102:160611. 3. Sherman RP, Rootman J, Lapointe JS. Orbital dermoids: clinical presentation and management. Br J Ophthalmol 1984;68:642-52. 4. Jakobiec FA, Bonanno PA, Sigelman J. Conjunctival adnexal cysts and dermoids. Arch Ophthalmol 1978;96: 1404-9. 5. Fiander D, Brownstein S, Nicolle D, et al. Mucosal cyst of lacrimal gland fossa simulating lacrimal gland neoplasm. Can J Ophthalmol 1980;15:87-90. 6. Shields JA, Augsburger JJ, Donoso LA. Orbital dermoid cysts of conjunctival origin. Am J Ophthalrnol 1986;101: 726-9. 7. McCollough ML, Glover AT, Grabski WJ, et al. Orbital dermoid cysts showing conjunctivalepithelium. Am J Dermatopathol 1991;13:611-5. 8. Boynton JR, Searl SS, Ferry AP, et al. Primary nonkeratinized epithelial('conjunctival') orbital cysts. Arch Ophthaltool 1992;110:1238-42.
Journal of the American Academy of Dermatology May 1997
Brief communications
bility to toxic epidermal necrolysis. Arch Dermatol 1987; 123:1171-3. 9. Chan SH, Tan T. HLA and aUopurinol drug eruption. Dermatologica 1989;179:32-3. 10. Hertl M, Bohlen H, Jugert F, et al. Predominance of epidermal CD8÷ T lymphocytes with bullous cutaneous reactions caused by beta-lactam antibiotics. J Invest Dermatol 1993;101:794-9. 11. Bell SJ, Pichler WJ. Penicillin-allergic patients react to penicillin-modified "self." Allergy 1989;44:199-203.
12. Class FI-IJ, Rumia-van Nieuwkoop R, van den Berge W, et al. Interaction of penicillin with HLA-A and B antigens. Hum Imrnunol 1982;5:83-90. 13. Shiohara T. What is new in fixed drug eruption? Dermatology 1995;191:185-7. 14. Hertl M, Merk HF. Lymphocyte activation in cutaneous drug reactions. J Invest Dermatol 1995;105:95-8S. 15. Kalish RS. Antigen processing: the gateway to the immune response. J Am Acad Dermatol 1995;32:64052.
Conjunctival dermoid cyst in an infant Yoshiko Kudo, M D , a K a z u m o t o Katagiri, M D , a Yoshiteru Ishii, M D , a Hiromi Shibuya, MD, a Hiroto Terashi, M D , a Hiroyuki Hashimoto, M D , a Susumu Takayasu, M D , a and Kenji Matsuo, M D b Oita, Japan
Orbital dermoid cysts are usually lined b y keratinizing squamous epithelium with adnexal structures. 1-3 W e describe an infant with an orbital conjunctival dermoid cyst that was lined mainly with nonkeratinlzing epithelium that contained goblet cells.
CASE REPORT A 7-month-old Japanese girl had a soft, elastic, bluered, subcutaneous nodule on her lower left eyelid (Fig. 1). Although she could hardly open that eye, her visual acuity and motility of the eye appeared to be normal. We initially diagnosed a cavernous hemangioma and, to prevent amblyopia, attempted to reduce its size with betamethasone, 0.5 mg daily for 36 days. However, there was no response. When the patient was 1 year of age, we injected a mixture of betamethasone, 6 rag, and methylprednisolone, 40 mg, into the lesion. This treatment significantly reduced its size within a few days. But during the next 5 months, the mass enlarged and virtually closed the eye. The lesion was reinjected with corticosteroids several times. Computed tomographic scans
From the Departments of Dermatologya and Ophthalmology? Oita Medical University. Reprint requests: Yoshiko Kudo, MD, Department of Dermatology, Oita Medical University, 1-1 Idaigaoka Hasama-machi, Oita 879-55, Japan. J Am Acad Dermatol 1997;36:784-5. Copyright © 1997 by the American Academy of Dermatology, Inc. 0190-9622/97/$5.00 + 0 16/54/79673
Fig. 1. Marked swelling of lower eyefid at age 7 months.
showed a cystic mass of low density, which suggested a dermoid cyst. When the patient was 27 months of age, we performed
Journal of the American Academy of Dermatology Volume 36, Number 5, Part 1
A
Fig. 2. A, Both keratinizing (left) and nonkeratinizing epithelia (right) are present. B, Nonkeratinizing epithelium contains numerous goblet cells. (A and B, Hematoxylin-eosin stain; original magnifications: A, xl00; B, x200.)
an orbitotomy and removed a conjunctival 20 x 30 mm derrnoid cyst. Histologic examination revealed that the cyst was lined mainly with nonkerafinizing epithelium and partly with keratinizing epithelium (Fig. 2, A). The nonkeratinizing epithelium contained numerous goblet cells (Fig. 2, B). The stroma of the cyst wall contained several sebaceous glands and sweat glands around the keratiuizing epithelium. During 2 years of follow-up, there were no postoperative complications or recurrences, and the child's visual acuity is normal. DISCUSSION
Orbital dermoid cysts can usually be differentiated clinically from other lesions by their superorbital location, cystic nature, and relation to b o n e ) In our patient, however, because the tumor was soft, bluered, and was present in an unusual site, we initially diagnosed a cavernous hemangioma. The marked improvement observed after the first intralesional injection of corticosteroids was probably due to the
Brief communications
785
antiinflammatory effect on the granulomatous tissue that surrounded the cyst. Dermoid cysts are benign developmental choristomas that are usually classified as superficial (simple) and deep (complicated) types. 1, 3 The superficial type is usually discovered in infancy as a painless subcutaneous nodule commonly located superolaterally beneath the brow. The deep type usually occurs in adults. It is associated with proptosis or displacement of the globe and tends to produce bony destruction. Jakobiec, Bonanno, and Sigelman 4 described a subgroup of epidermoid and dermoid cysts that were lined with conjunctival epithelium and called them conjtmctival cysts, or conjunctival dermoid cysts, according to the absence or presence of appendages. To our knowledge, only 11 cases of conjunctival cysts and 11 cases of conjunctival dermoid cysts, including the present case, have been reported in the literature. 4-8 Both conjuncfival and conjunctival dermoid cysts are usually located superonasally in the orbit and do not affect the bone. 4-8 Conjunctival dermoid cysts often occur in the second decade of life. 4 Only one case was reported to appear in infancy. 8 Conjunctival dermoid cysts are thought to represent embryologic sequestrations of the inner layer of ectoderm that normally forms the conjunctiva, rather than sequestrations of the surface epidermis. 4
REFERENCES
1. Grove JrAS. Orbital disorders: diagnosis and management. In: McCord CD Jr, editor. Oculoplasticsurgery. New York: Raven Press, 1981:257-84. 2. Shields JA, Bakewell B, AugsburgerJJ, et al. Classification and incidence of space-occupyinglesions of the orbit: a survey of 645 biopsies. Arch Ophthalmol 1984;102:160611. 3. Sherman RP, Rootman J, Lapointe JS. Orbital dermoids: clinical presentation and management. Br J Ophthalmol 1984;68:642-52. 4. Jakobiec FA, Bonanno PA, Sigelman J. Conjunctival adnexal cysts and dermoids. Arch Ophthalmol 1978;96: 1404-9. 5. Fiander D, Brownstein S, Nicolle D, et al. Mucosal cyst of lacrimal gland fossa simulating lacrimal gland neoplasm. Can J Ophthalmol 1980;15:87-90. 6. Shields JA, Augsburger JJ, Donoso LA. Orbital dermoid cysts of conjunctival origin. Am J Ophthalrnol 1986;101: 726-9. 7. McCollough ML, Glover AT, Grabski WJ, et al. Orbital dermoid cysts showing conjunctivalepithelium. Am J Dermatopathol 1991;13:611-5. 8. Boynton JR, Searl SS, Ferry AP, et al. Primary nonkeratinized epithelial('conjunctival') orbital cysts. Arch Ophthaltool 1992;110:1238-42.