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Palmar-Plantar Hyperkeratosis Associated With Bilateral Keratopathy
280
AMERICAN JOURNAL OF OPHTHALMOLOGY
According to the manufacturer (Cilco), the subepithelial corneal changes have been noted in approximately 0.5% of cases. Of the surgeons involved as investigators, 4.9% have noted the corneal changes. Research has shown that the subepithelial white deposits are probably calcium phosphate precipitates. The calcium phosphate tends to precipitate if BSS+ or sodium citrate is used with the sodium chondroitin. The precipitate is least likely to occur if BSS is used and at least 50% of the sodium chondroitin is aspirated or irrigated from the anterior chamber. Six months to two years may be required for the subepithelial deposits to resolve. The manufacturer recommends no treatment if the deposits are outside the visual axis and do not affect visual acuity. If visual acuity is effected, topical EDTA may be used for chelation.
References 1. Barron, B. A., Busin, M., Page, c., Bergsma, D. R., and Kaufman, H. E.: Comparison of the effects of Viscoat and Healon on postoperative intraocular pressure. Am. J. Ophthalmol. 100:337, 1985. 2. Wilson, M. F. II: Adverse external ocular effects of topical ophthalmic medications. Surv. Ophthalmol. 24:47, 1979.
Palmar-Plantar Hyperkeratosis Associated With Bilateral Keratopathy
August, 1986
with severe corneal epithelial disease and developmental delay. The patient, a 25-month-old girl, was the product of an uneventful full-term pregnancy. When she was first examined at the age of 22 months, it was found that she had had severe photophobia, tearing, and blepharospasm since the age of 6 weeks. The child was being monitored for developmental delay and was taking no medications. The family history was noncontributory for ophthalmic or dermatologic disorders. The child had extreme photophobia and blepharospasm even in room illumination (Fig. 1). The instillation of proparacaine allowed the child to fix and follow objects bilaterally. The eyelids, conjunctiva, lens, fundus, and cycloplegic refraction were normal. A slit-lamp examination showed a symmetric diffuse superficial punctate keratopathy that stained with fluorescein. No corneal vascularization or infiltrates were apparent. Physical findings were normal except for severe hyperkeratotic lesions on her palms and soles (Fig. 2). The lesions were not inflamed or painful. Results of serum amino acid analysis, 24-hour urine for amino acids, and chromosomal analysis were all normal. After two weeks of ocular lubricants every four hours the patient had a marked improvement in her ocular symptoms; the corneal examination showed only mild superficial punctate keratopathy. Palmar-plantar hyperkeratosis is a rare, heterogeneous condition sometimes associated with distinct clinical entities." The RichnerHanhart syndrome (tyrosinemia type II) consists of hyperkeratosis of the hands and feet,
Saul Ullman, M.D., Leonard B. Nelson, M.D., and Ming-liang Lee, M.D.
Department of Pediatric Ophthalmology, Wills Eye Hospital (S.U. and L.B.N.) and Department of Pediatrics, Division of Medical Genetics, Rutgers Medical School (M.L.). Inquiries to Leonard B. Nelson, M.D., Wills Eye Hospital, Ninth and Walnut Sts., Philadelphia, PA 19107.
Hyperkeratosis of the volar aspects of the hands and feet is a rare condition that may be associated with inborn errors of metabolism and ophthalmic disease;' We studied a patient with palmar-plantar hyperkeratosis associated
Fig. 1 (Ullman, Nelson, and Lee). Bilateral blepharospasm, tearing, and photosensitivity before the instillation of proparacaine.
Vol. 102, No. 2
281
Letters to The Journal
Acid-Soluble Protein in Behc;et's Disease Machiko Shimakawa, M.D., and Mitsuko Kogure, M.D.
Fig. 2 (Ullman, Nelson, and Lee). Hyperkeratosis of the palms.
corneal disease, and aberrations of tyrosine metabolism." Any child with bilateral corneal epithelial disease of unknown origin (often initially misdiagnosed as bilateral herpes keratitis) should have a dermatologic examination. If an abnormality of the palms or soles is found, a metabolic evaluation for the Richrier-Hanhart syndrome should be performed. A prompt diagnosis is important since treatment by dietary restriction of phenylalanine and tyrosine usually results in resolution of the skin and corneal lesions and may prevent mental retardation. 4 Our patient's findings were not typical of other syndromes with palmar-plantar hyperkeratosis." The response to ocular lubricants was dramatic. The cause of the superficial punctate keratopathy and its relationship to this dermatologic disorder, however, remain unknown.
References 1. Burns, R. P.: Soluble tyrosine aminotransferase deficiency. An unusual cause of corneal ulcers. Am. J. OphthalmoI. 73:400, 1972. 2. Grayson, M.: Diseases of the Cornea, 2nd ed. St. Louis, C. V. Mosby, 1983, pp. 535-572. 3. Charlton, K. H., Binder, P. S., Wozniak, 1., and Digby, D. J.: Pseudodendritic keratitis and systemic tyrosinemia. Ophthalmology 88:355, 1981. 4. Goldsmith, 1. A., and Reed, J.: Tyrosineinduced eye and skin lesions. A treatable genetic disease. J.A.M.A. 236:383, 1976. 5. Bienfang, D. c., Kuwabara, T., and Pueschel, S. M.: The Richner-Hanhart syndrome. Arch. OphthalmoI. 94:1133, 1976.
Department of Ophthalmology, Tokyo Women's Medical College. Inquiries to Machiko Shimakawa, M.D., Department of Ophthalmology, Tokyo Women's Medical College, 18 Kawada-cho, Shinjuku-ku, Tokyo, 162, Japan. Acid-soluble protein, a component of glycoprotein, is increased in the serum of the patients with a variety of inflammatory diseases." We investigated the serum level of acid-soluble protein in patients with Behcet's disease with special reference to the clinical status. The serum level of acid-soluble protein was quantitatively estimated by the method of Chikakiyo and associates" in 87 patients with Behcet's disease who had ocular or systemic manifestations. The acid-soluble protein levels were also evaluated in 73 patients with uveitis and without Behcet's disease and in 117 normal subjects serving as controls (Figure). The mean (± S.D.) serum acid-soluble pro-
200 (M±S.E.l
........ <::Ii "'0
*, *. ** ** * :I P
150
C)
E .......
a..
« 100
(j)
E ~ l0-
a>
(j)
50
n=73
o
Behcet*
Other uveitis**
n=117
Control***
Figure (Shimakawa and Kogure). Serum acid-
soluble protein levels in patients with Behcets disease and with uveitis and in normal controls.
AMERICAN JOURNAL OF OPHTHALMOLOGY
According to the manufacturer (Cilco), the subepithelial corneal changes have been noted in approximately 0.5% of cases. Of the surgeons involved as investigators, 4.9% have noted the corneal changes. Research has shown that the subepithelial white deposits are probably calcium phosphate precipitates. The calcium phosphate tends to precipitate if BSS+ or sodium citrate is used with the sodium chondroitin. The precipitate is least likely to occur if BSS is used and at least 50% of the sodium chondroitin is aspirated or irrigated from the anterior chamber. Six months to two years may be required for the subepithelial deposits to resolve. The manufacturer recommends no treatment if the deposits are outside the visual axis and do not affect visual acuity. If visual acuity is effected, topical EDTA may be used for chelation.
References 1. Barron, B. A., Busin, M., Page, c., Bergsma, D. R., and Kaufman, H. E.: Comparison of the effects of Viscoat and Healon on postoperative intraocular pressure. Am. J. Ophthalmol. 100:337, 1985. 2. Wilson, M. F. II: Adverse external ocular effects of topical ophthalmic medications. Surv. Ophthalmol. 24:47, 1979.
Palmar-Plantar Hyperkeratosis Associated With Bilateral Keratopathy
August, 1986
with severe corneal epithelial disease and developmental delay. The patient, a 25-month-old girl, was the product of an uneventful full-term pregnancy. When she was first examined at the age of 22 months, it was found that she had had severe photophobia, tearing, and blepharospasm since the age of 6 weeks. The child was being monitored for developmental delay and was taking no medications. The family history was noncontributory for ophthalmic or dermatologic disorders. The child had extreme photophobia and blepharospasm even in room illumination (Fig. 1). The instillation of proparacaine allowed the child to fix and follow objects bilaterally. The eyelids, conjunctiva, lens, fundus, and cycloplegic refraction were normal. A slit-lamp examination showed a symmetric diffuse superficial punctate keratopathy that stained with fluorescein. No corneal vascularization or infiltrates were apparent. Physical findings were normal except for severe hyperkeratotic lesions on her palms and soles (Fig. 2). The lesions were not inflamed or painful. Results of serum amino acid analysis, 24-hour urine for amino acids, and chromosomal analysis were all normal. After two weeks of ocular lubricants every four hours the patient had a marked improvement in her ocular symptoms; the corneal examination showed only mild superficial punctate keratopathy. Palmar-plantar hyperkeratosis is a rare, heterogeneous condition sometimes associated with distinct clinical entities." The RichnerHanhart syndrome (tyrosinemia type II) consists of hyperkeratosis of the hands and feet,
Saul Ullman, M.D., Leonard B. Nelson, M.D., and Ming-liang Lee, M.D.
Department of Pediatric Ophthalmology, Wills Eye Hospital (S.U. and L.B.N.) and Department of Pediatrics, Division of Medical Genetics, Rutgers Medical School (M.L.). Inquiries to Leonard B. Nelson, M.D., Wills Eye Hospital, Ninth and Walnut Sts., Philadelphia, PA 19107.
Hyperkeratosis of the volar aspects of the hands and feet is a rare condition that may be associated with inborn errors of metabolism and ophthalmic disease;' We studied a patient with palmar-plantar hyperkeratosis associated
Fig. 1 (Ullman, Nelson, and Lee). Bilateral blepharospasm, tearing, and photosensitivity before the instillation of proparacaine.
Vol. 102, No. 2
281
Letters to The Journal
Acid-Soluble Protein in Behc;et's Disease Machiko Shimakawa, M.D., and Mitsuko Kogure, M.D.
Fig. 2 (Ullman, Nelson, and Lee). Hyperkeratosis of the palms.
corneal disease, and aberrations of tyrosine metabolism." Any child with bilateral corneal epithelial disease of unknown origin (often initially misdiagnosed as bilateral herpes keratitis) should have a dermatologic examination. If an abnormality of the palms or soles is found, a metabolic evaluation for the Richrier-Hanhart syndrome should be performed. A prompt diagnosis is important since treatment by dietary restriction of phenylalanine and tyrosine usually results in resolution of the skin and corneal lesions and may prevent mental retardation. 4 Our patient's findings were not typical of other syndromes with palmar-plantar hyperkeratosis." The response to ocular lubricants was dramatic. The cause of the superficial punctate keratopathy and its relationship to this dermatologic disorder, however, remain unknown.
References 1. Burns, R. P.: Soluble tyrosine aminotransferase deficiency. An unusual cause of corneal ulcers. Am. J. OphthalmoI. 73:400, 1972. 2. Grayson, M.: Diseases of the Cornea, 2nd ed. St. Louis, C. V. Mosby, 1983, pp. 535-572. 3. Charlton, K. H., Binder, P. S., Wozniak, 1., and Digby, D. J.: Pseudodendritic keratitis and systemic tyrosinemia. Ophthalmology 88:355, 1981. 4. Goldsmith, 1. A., and Reed, J.: Tyrosineinduced eye and skin lesions. A treatable genetic disease. J.A.M.A. 236:383, 1976. 5. Bienfang, D. c., Kuwabara, T., and Pueschel, S. M.: The Richner-Hanhart syndrome. Arch. OphthalmoI. 94:1133, 1976.
Department of Ophthalmology, Tokyo Women's Medical College. Inquiries to Machiko Shimakawa, M.D., Department of Ophthalmology, Tokyo Women's Medical College, 18 Kawada-cho, Shinjuku-ku, Tokyo, 162, Japan. Acid-soluble protein, a component of glycoprotein, is increased in the serum of the patients with a variety of inflammatory diseases." We investigated the serum level of acid-soluble protein in patients with Behcet's disease with special reference to the clinical status. The serum level of acid-soluble protein was quantitatively estimated by the method of Chikakiyo and associates" in 87 patients with Behcet's disease who had ocular or systemic manifestations. The acid-soluble protein levels were also evaluated in 73 patients with uveitis and without Behcet's disease and in 117 normal subjects serving as controls (Figure). The mean (± S.D.) serum acid-soluble pro-
200 (M±S.E.l
........ <::Ii "'0
*, *. ** ** * :I P
150
C)
E .......
a..
« 100
(j)
E ~ l0-
a>
(j)
50
n=73
o
Behcet*
Other uveitis**
n=117
Control***
Figure (Shimakawa and Kogure). Serum acid-
soluble protein levels in patients with Behcets disease and with uveitis and in normal controls.