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Ocular Pathology in Malignant Atrophic Papulosis
OCULAR PATHOLOGY IN MALIGNANT ATROPHIC
PAPULOSIS
DEGOS' DISEASE P A U L H E N K I N D , M.D.
AND W I L L I A M
E.
CLARK, II,
M.D.
New York Malignant atrophic papulosis was first de scribed in detail by Degos and his co-workjrs 1 in 1942. I n this r a r e and distinctive en:ity of unknown etiology, pathognomonic skin lesions develop, and weeks to years a t e r death usually occurs from perforation }f the intestine. T h e cutaneous lesions begin is papules scattered over the t r u n k and e x tremities, soon becoming umbilicated with depressed centers of a porcelain-white tint. 2 In addition to the lesions of the skin and gastrointestinal tract there may be involve ment of the central nervous system, 3 - 4 heart, 4 m d bladder. 5 T h e pathologic findings appear :o result from a characteristic endovasculitis af small caliber vessels with secondary :hrombosis and slow necrosis of the sur rounding tissue. Ocular abnormalities have been noted in several cases; conjunctival microaneurysms ind papules of the lid, 6 episcleral plaques, 3 third nerve palsy, 7 papilledema, 3 optic itrophy, 7 pigmentary choroiditis, 8 and a gray ivascular area in the fundus. 9 I n this report we document the finding on histopathologic sxamination of typical vascular lesions in the episclera, choroid, and retina of a patient who died of Degos' disease (malignant itrophic papulosis). Other ocular abnormali ties were also present but may be unrelated to the disease.
after birth she began to develop a maculopapular eruption involving the trunk and extremities. The lesions appeared one or two at a time progressively for three years, intermittently thereafter, and healed with depressed scarring. Biopsy showed focal epidermal atrophy, fibrosis, patchy lymphoid infiltration, and a few small calcium deposits. At age four years, right leg pain developed, and several months later the patient had a staggering gait, headaches, nausea and vomiting. By age nine years she had urinary tract disease and widespread inter mittent pains. New skin lesions appeared and the older ones remained as pitted circular scars. By age 12 years, on her final admission, the patient was chronically ill, unable to move her legs, and had mul tiple central nervous system abnormalities. EYE HISTORY
At two years of age the patient's left eye en larged, turned inward, and felt hard. Examination revealed congenital glaucoma, and an iridencleisis was performed. At that time a large coloboma in volving the right macula was noted. Two years later the left eye was blind, the right eye had 20/200 vision with constriction of the nasal field, and large areas of chorioretinitis with marginal pigment deposits noted bilaterally; there was optic atrophy of the left eye. Skin tests for tuberculosis, histoplasmosis, coccidiodomycosis, and a Sabin dye test for toxoplasmosis were all negative. At age 12 the ocular tension was normal in both eyes (con trolled by pilocarpine), there was burned-out cho rioretinitis, and conjunctival telangiectasis. PATHOLOGY
T h e nonocular pathologic findings will be published elsewhere. Of interest, however, was the fact that the diagnosis in this case was made post mortem. E Y E PATHOLOGY
C A S E REPORT
A 12^-year-old girl had had multisystemic dis ease since three or four weeks of age. Three weeks From the Department of Ophthalmology, New tfork University School of Medicine, and the De partment of Pathology, Hartford Hospital, Hart ford, Connecticut. This study was supported in part by Public Health Service Research Grant NB-05059-04 from the National Institute of Neurological Diseases and Blindness, National Institutes of Health.
T h e eyes were removed seven hours after death, placed in glutaraldehyde for one month, and then sectioned. N o report of a gross examination of the eyes was available. Only one hematoxylin- and eosin-stained slide of each eye was available for examina tion. N o worthwhile sections could be cut from the remaining block, and wet tissue was unavailable for embedding. 164
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Fig. 1 (Henkind and Clark). A marked anterior extension of the ciliary body and an atrophic iris root make up the posterior wall of the angle. The angle is open and the ciliary muscle seems retroplaced. ('Hematoxylin-eosin, Xl25.) MICROSCOPIC EXAMINATION
Right eye. The corneal epithelium was ir regular, but the cornea was otherwise nor mal. The anterior chamber was deep and the angle open; however, the ciliary muscle seemed to insert more posteriorly into the trabecular meshwork than usual, giving the angle an abnormal appearance (fig. 1). No abnormalities were noted in the vessels of the conjunctiva, iris, or ciliary body. There were several small cysts of the pigment epi thelium of the ciliary body. A large coloboma occupied the macular re
gion, with the retina and choroid either to tally absent in this area or reduced to a layer one cell thick (fig. 2 ) . At the nasal edge of the coloboma, where the retina was start ing to thin, there was a nodule containing proliferated pigment and thickened choroid. The retina between the coloboma and the disc had the normal layered configuration, but on the temporal side the retina was thinner than normal, with apparent intermingling of of the inner nuclear and ganglion cell layers. On the opposite side of the eye near the equator there was an area of thickened ret ina which appeared to contain a retinal ro-
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Fig. 2 (Henkind and Clark). Coloboma of the macula region in the right eye. The retina and choroid are a thin irregular mem brane. Note the pigment-capped choroidal nodule at the right, just posterior to the coloboma. (Hema toxylin-eosin, x50.)
sette. No gross vascular abnormalities were present in the retina, choroid, or sclera. The diagnoses in this eye were (1) coloboma of the retina and choroid (macular region), (2) abnormal retinal tissue with rosette, and (3) possible angle anomaly. Left eye. An old, healed, perforating corneoscleral limbus wound lined by uveal tis sue was present on one side. There was par tial absence of the corneal epithelium and thinning of the central stroma (possibly arti fact). The iris leaf opposite to the site of op eration showed marked atrophy of the stroma, particularly centrally. There was atrophy of the ciliary body, with chronic in flammatory cells present in the iris root.
Fig. 3 (Henkind and Clark). Three anterior episcleral vessels in the left eye (arrows), showing marked subendothelial fibrosis and narrowed irregular lumens. The folds are sectioning artifacts. ('Hematoxylin-eosin, xSO.)
Peripheral anterior synechiae occluded the trabecular meshwork on one side; the angle on the other side appeared similar in appear ance to that seen in the right eye. The ante rior episcleral vessels on the side of the wound showed marked subendothelial fi brosis and narrowed lumens, but relatively normal muscular and adventitial coats (fig. 3 ) . Fibrinoid necrotic material was present in the proliferated wall of one vessel (fig. 4). Though the posterior half of the globe was partially folded on one side, making in terpretation difficult, the retina and choroid appeared fused in one area, suggesting ei ther choroiditis or a coloboma. The inner ret-
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Fig. 4 (Henkind and Clark). A higher-powered view of an anterior episcleral vessel shown in Figure 3. There is marked subendothelial fibrosis and fibrinoid necrosis (ar row). (Hematoxylin-eosin, X200.)
inal layers were atrophic, with glial tissue replacing the normal cellular structure. A number of retinal vessels had thickened walls which appeared to be due to suben dothelial thickening (fig. 5). In the wall of the cupped optic nervehead was a vessel with proliferated endothelium and luminal nar rowing, closely resembling the abnormal ves sels seen in the episclera (fig. 6 ) . One active choroidal vascular lesion with fibrinoid de posit was seen about 0.5 cm from the optic nerve (fig. 7). Optic atrophy with gliosis, hypercellularity and questionable atrophy of the extraocular muscles, and possibly some obliteration of subconjunctival vessels were noted. The diagnoses in this eye were (1) congen ital glaucoma, due to angle anomaly, (2) iri-
Fig. 6 (Henkind and Clark). Markedly thickened wall of (?) arteriole (arrow) in cupped optic nervehead. The lumen appears narrowed and there appears to be subendothelial proliferation of fibrous tissue. Hematoxylin-eosin, X320.) dencleisis, (3) coloboma of the choroid and retina, and (4) vascular changes of the epi sclera, choroid, and retina compatible with the vascular changes seen in Degos' disease. DISCUSSION
Fig. 5 (Henkind and Clark). Abnormally thick ened wall of a retinal vessel (? arteriole). There appears to be subendothelialfibrosis.(Hematoxylineosin, X320.)
Malignant atrophic papulosis appears to be a rare multisystemic disorder with only 20 cases reported to date.9 Although the ma jority of patients have been young, white
168
AMERICAN JOURNAL OF OPHTHALMOLOGY
Fig. 7 (Henkind and Clark). Choroidal vascular lesion with fibrinoid deposits (arrow) in left eye. Interestingly, the pigment epithelium is intact and presumably nourished by patent choriocapillaris. (Hematoxylin-eosin, X12S.) males in the second or third decades of life, older individuals, Negroes, and females can be affected. The patient herein presented is the youngest known case of malignant atrophic papulosis. Diagnosis is made by the appearance of typical papules which erupt in crops on the skin, although the disease is not limited to skin involvement. Occlusion of small caliber vessels, primarily arterioles, but also capillaries and venules, usually leads to intestinal necrosis and perforation or, less commonly, to severe central nervous system disease which culminates in death. Possibly patients suffer internal manifestations without concomitant skin lesions, and a few patients with typical skin eruption have sur vived without systemic disease.8 Laboratory values tend to be normal when the skin solely is involved. Biopsy of the skin lesions helps to establish the diagnosis. In our case and that presented by Nomland and Layton,4 the correct diagnosis was established in re trospect only after postmortem examination. Apparently, treatment is of little value in this condition. Vascular lesions appear to account for the various manifestations of malignant atrophic papulosis. Usually there is endovasculitis in volving small arteries, with proliferation of the endothelium and deposition of fibrous
FEBRUARY, 1968
tissue between the endothelium and internal elastic lamina, often with sparing of the media or adventitia, or both. A chronic in flammatory cell response surrounding in volved vessels is sometimes noted. Secon dary thrombosis apparently results from the narrowing of the vascular lumina, with the tissue surrounding the vessel undergoing slow necrosis. Though the condition may histologically resemble the vascular involve ment seen in thromboangiitis obliterans or the endovasculitis or scleroderma, it is clini cally unlike either of these disorders. 3 Ocular findings have been stressed by Feuerman, 6 who noted prominent microaneurysms of the bulbar conjunctiva vessels in a 46-year-old white man with malignant atrophic papulosis. The patient also had a typical papule on his right upper lid. Feuer man feels that the conjunctival vessel abnor malities are probably related to the disorder and should be searched for in other patients with malignant atrophic papulosis. The pa tient of Strole, Clark, and Isselbacher,9 a 21-year-old white man, had typical vascular lesions of the conjunctiva demonstrable his tologically but not noted clinically. Both of the cases reported by Winkelmann and his colleagues3 had ocular abnormalities. The first patient, a 20-year-old white man with central nervous system complications, had glial blurring of his fundi which later devel oped into definite papilledema. The second patient, a 36-year-old white man, developed unusual whitish plaques on the sclera be tween the cornea and inner canthus of each eye, more marked on the right side. These plaques were surrounded by telangectasia. The patient of Culicchia, Gol, and Erickson,7 a 34-year-old white man, devel oped total external ophthalmoplegia and bilat eral optic atrophy during the terminal phase of his disease. Sidi and co-workers8 describe their patient, a 30-year-old white man, as having a small plaque of atrophic choroiditis on the right lateral f undus. In our patient, congenital glaucoma of the left eye and a coloboma of the right macula
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were noted at age two years. At a later age, patches of choroiditis were seen bilaterally but tests for tuberculosis, histoplasmosis, and toxoplasmosis were negative. Endovasculitis with marked subendothelial fibrosis in volving the anterior episcleral vessels of the left eye was noted on pathologic examina tion. Similar vascular changes were seen in several of the larger retinal vessels, and a choroidal arteriole. These changes were identical with lesions found elsewhere in the body, including the brain, and would thus appear to be directly related to malignant atrophic papulosis. The relationship of the posterior pole coloboma and possible anter ior chamber abnormality to malignant atrophic papulosis is unknown, and probably fortuitous. However, choroidoretinal lesions could well result from slow obliteration of choroidal vessels, if these are involved. Per haps a coloboma of the macula could develop in infancy if choroidal vessels beneath the macula are obliterated by the disease pro cess. There is little question that this intriguing disease needs further ophthalmic study from both a clinical and a pathologic standpoint. 550 First Avenue
(10016)
169
ACKNOWLEDGMENTS
We wish to thank Dr. George Kurz for review ing this paper, and Miss Doris Pagan and Mr. Henrick Malpica for secretarial and photographic as sistance. REFERENCES
1. Degos, R., Delort, J., and Tricot, R.: Dermatite papulo-squameuse atrophiante. Bull. Soc. Franc. Derm. Syph. 49:148,1942. 2. Degos, R.: Malignant atrophic papulosis: A fatal cutaneointestinal syndrome. Brit. J. Dermat. 66:304,1954. 3. Winkelmann, R. K., Howard, F. M., Jr., Perry, H. O., and Miller, R. H.: Malignant papu losis of skin and cerebrum: A syndrome of vascu lar thrombosis. Arch. Derm. 87:54, 1963. 4. Nomland, R, and Lay ton, J. M.: Malignant Papulosis atrophicans maligna (Degos' disease). neointestinal syndrome. Arch. Derm. 81:181, 1960. 5. Naylor, D., Mullins, J. F., and Gilmore, J. F.: Papulosis atrophicans maligna (Degos' disease). Arch. Derm. 81:189, 1960. 6. Feuerman, E. J.: Papulosis atrophicans ma ligna Degos: With microaneurysms of the conjunc tiva. Arch. Derm. 94:440, 1966. 7. Culicchia, C. F., Gol, A., and Erickson, E. E.: Diffuse central nervous system involvement in pa pulosis atrophicans maligna. Neurology 12:503, 1962. 8. Sidi, E., Reinberg, A., Spinasse, J. B., and Hincky, M.: Lethal cutaneous and gastrointestinal arteriolar thrombosis (malignant atrophying papu losis of Degos). JAMA 174:1170, 1960. 9. Strole, W. E., Jr., Clark, W. H , Jr., and Isselbacher, K. J.: Progressive arterial occlusive dis ease (Kohlmeier-Degos). A frequently fatal cutaneo-systemic disorder. New England J. Med. 276:195,1967.
PAPULOSIS
DEGOS' DISEASE P A U L H E N K I N D , M.D.
AND W I L L I A M
E.
CLARK, II,
M.D.
New York Malignant atrophic papulosis was first de scribed in detail by Degos and his co-workjrs 1 in 1942. I n this r a r e and distinctive en:ity of unknown etiology, pathognomonic skin lesions develop, and weeks to years a t e r death usually occurs from perforation }f the intestine. T h e cutaneous lesions begin is papules scattered over the t r u n k and e x tremities, soon becoming umbilicated with depressed centers of a porcelain-white tint. 2 In addition to the lesions of the skin and gastrointestinal tract there may be involve ment of the central nervous system, 3 - 4 heart, 4 m d bladder. 5 T h e pathologic findings appear :o result from a characteristic endovasculitis af small caliber vessels with secondary :hrombosis and slow necrosis of the sur rounding tissue. Ocular abnormalities have been noted in several cases; conjunctival microaneurysms ind papules of the lid, 6 episcleral plaques, 3 third nerve palsy, 7 papilledema, 3 optic itrophy, 7 pigmentary choroiditis, 8 and a gray ivascular area in the fundus. 9 I n this report we document the finding on histopathologic sxamination of typical vascular lesions in the episclera, choroid, and retina of a patient who died of Degos' disease (malignant itrophic papulosis). Other ocular abnormali ties were also present but may be unrelated to the disease.
after birth she began to develop a maculopapular eruption involving the trunk and extremities. The lesions appeared one or two at a time progressively for three years, intermittently thereafter, and healed with depressed scarring. Biopsy showed focal epidermal atrophy, fibrosis, patchy lymphoid infiltration, and a few small calcium deposits. At age four years, right leg pain developed, and several months later the patient had a staggering gait, headaches, nausea and vomiting. By age nine years she had urinary tract disease and widespread inter mittent pains. New skin lesions appeared and the older ones remained as pitted circular scars. By age 12 years, on her final admission, the patient was chronically ill, unable to move her legs, and had mul tiple central nervous system abnormalities. EYE HISTORY
At two years of age the patient's left eye en larged, turned inward, and felt hard. Examination revealed congenital glaucoma, and an iridencleisis was performed. At that time a large coloboma in volving the right macula was noted. Two years later the left eye was blind, the right eye had 20/200 vision with constriction of the nasal field, and large areas of chorioretinitis with marginal pigment deposits noted bilaterally; there was optic atrophy of the left eye. Skin tests for tuberculosis, histoplasmosis, coccidiodomycosis, and a Sabin dye test for toxoplasmosis were all negative. At age 12 the ocular tension was normal in both eyes (con trolled by pilocarpine), there was burned-out cho rioretinitis, and conjunctival telangiectasis. PATHOLOGY
T h e nonocular pathologic findings will be published elsewhere. Of interest, however, was the fact that the diagnosis in this case was made post mortem. E Y E PATHOLOGY
C A S E REPORT
A 12^-year-old girl had had multisystemic dis ease since three or four weeks of age. Three weeks From the Department of Ophthalmology, New tfork University School of Medicine, and the De partment of Pathology, Hartford Hospital, Hart ford, Connecticut. This study was supported in part by Public Health Service Research Grant NB-05059-04 from the National Institute of Neurological Diseases and Blindness, National Institutes of Health.
T h e eyes were removed seven hours after death, placed in glutaraldehyde for one month, and then sectioned. N o report of a gross examination of the eyes was available. Only one hematoxylin- and eosin-stained slide of each eye was available for examina tion. N o worthwhile sections could be cut from the remaining block, and wet tissue was unavailable for embedding. 164
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Fig. 1 (Henkind and Clark). A marked anterior extension of the ciliary body and an atrophic iris root make up the posterior wall of the angle. The angle is open and the ciliary muscle seems retroplaced. ('Hematoxylin-eosin, Xl25.) MICROSCOPIC EXAMINATION
Right eye. The corneal epithelium was ir regular, but the cornea was otherwise nor mal. The anterior chamber was deep and the angle open; however, the ciliary muscle seemed to insert more posteriorly into the trabecular meshwork than usual, giving the angle an abnormal appearance (fig. 1). No abnormalities were noted in the vessels of the conjunctiva, iris, or ciliary body. There were several small cysts of the pigment epi thelium of the ciliary body. A large coloboma occupied the macular re
gion, with the retina and choroid either to tally absent in this area or reduced to a layer one cell thick (fig. 2 ) . At the nasal edge of the coloboma, where the retina was start ing to thin, there was a nodule containing proliferated pigment and thickened choroid. The retina between the coloboma and the disc had the normal layered configuration, but on the temporal side the retina was thinner than normal, with apparent intermingling of of the inner nuclear and ganglion cell layers. On the opposite side of the eye near the equator there was an area of thickened ret ina which appeared to contain a retinal ro-
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FEBRUARY, 1968
Fig. 2 (Henkind and Clark). Coloboma of the macula region in the right eye. The retina and choroid are a thin irregular mem brane. Note the pigment-capped choroidal nodule at the right, just posterior to the coloboma. (Hema toxylin-eosin, x50.)
sette. No gross vascular abnormalities were present in the retina, choroid, or sclera. The diagnoses in this eye were (1) coloboma of the retina and choroid (macular region), (2) abnormal retinal tissue with rosette, and (3) possible angle anomaly. Left eye. An old, healed, perforating corneoscleral limbus wound lined by uveal tis sue was present on one side. There was par tial absence of the corneal epithelium and thinning of the central stroma (possibly arti fact). The iris leaf opposite to the site of op eration showed marked atrophy of the stroma, particularly centrally. There was atrophy of the ciliary body, with chronic in flammatory cells present in the iris root.
Fig. 3 (Henkind and Clark). Three anterior episcleral vessels in the left eye (arrows), showing marked subendothelial fibrosis and narrowed irregular lumens. The folds are sectioning artifacts. ('Hematoxylin-eosin, xSO.)
Peripheral anterior synechiae occluded the trabecular meshwork on one side; the angle on the other side appeared similar in appear ance to that seen in the right eye. The ante rior episcleral vessels on the side of the wound showed marked subendothelial fi brosis and narrowed lumens, but relatively normal muscular and adventitial coats (fig. 3 ) . Fibrinoid necrotic material was present in the proliferated wall of one vessel (fig. 4). Though the posterior half of the globe was partially folded on one side, making in terpretation difficult, the retina and choroid appeared fused in one area, suggesting ei ther choroiditis or a coloboma. The inner ret-
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167
Fig. 4 (Henkind and Clark). A higher-powered view of an anterior episcleral vessel shown in Figure 3. There is marked subendothelial fibrosis and fibrinoid necrosis (ar row). (Hematoxylin-eosin, X200.)
inal layers were atrophic, with glial tissue replacing the normal cellular structure. A number of retinal vessels had thickened walls which appeared to be due to suben dothelial thickening (fig. 5). In the wall of the cupped optic nervehead was a vessel with proliferated endothelium and luminal nar rowing, closely resembling the abnormal ves sels seen in the episclera (fig. 6 ) . One active choroidal vascular lesion with fibrinoid de posit was seen about 0.5 cm from the optic nerve (fig. 7). Optic atrophy with gliosis, hypercellularity and questionable atrophy of the extraocular muscles, and possibly some obliteration of subconjunctival vessels were noted. The diagnoses in this eye were (1) congen ital glaucoma, due to angle anomaly, (2) iri-
Fig. 6 (Henkind and Clark). Markedly thickened wall of (?) arteriole (arrow) in cupped optic nervehead. The lumen appears narrowed and there appears to be subendothelial proliferation of fibrous tissue. Hematoxylin-eosin, X320.) dencleisis, (3) coloboma of the choroid and retina, and (4) vascular changes of the epi sclera, choroid, and retina compatible with the vascular changes seen in Degos' disease. DISCUSSION
Fig. 5 (Henkind and Clark). Abnormally thick ened wall of a retinal vessel (? arteriole). There appears to be subendothelialfibrosis.(Hematoxylineosin, X320.)
Malignant atrophic papulosis appears to be a rare multisystemic disorder with only 20 cases reported to date.9 Although the ma jority of patients have been young, white
168
AMERICAN JOURNAL OF OPHTHALMOLOGY
Fig. 7 (Henkind and Clark). Choroidal vascular lesion with fibrinoid deposits (arrow) in left eye. Interestingly, the pigment epithelium is intact and presumably nourished by patent choriocapillaris. (Hematoxylin-eosin, X12S.) males in the second or third decades of life, older individuals, Negroes, and females can be affected. The patient herein presented is the youngest known case of malignant atrophic papulosis. Diagnosis is made by the appearance of typical papules which erupt in crops on the skin, although the disease is not limited to skin involvement. Occlusion of small caliber vessels, primarily arterioles, but also capillaries and venules, usually leads to intestinal necrosis and perforation or, less commonly, to severe central nervous system disease which culminates in death. Possibly patients suffer internal manifestations without concomitant skin lesions, and a few patients with typical skin eruption have sur vived without systemic disease.8 Laboratory values tend to be normal when the skin solely is involved. Biopsy of the skin lesions helps to establish the diagnosis. In our case and that presented by Nomland and Layton,4 the correct diagnosis was established in re trospect only after postmortem examination. Apparently, treatment is of little value in this condition. Vascular lesions appear to account for the various manifestations of malignant atrophic papulosis. Usually there is endovasculitis in volving small arteries, with proliferation of the endothelium and deposition of fibrous
FEBRUARY, 1968
tissue between the endothelium and internal elastic lamina, often with sparing of the media or adventitia, or both. A chronic in flammatory cell response surrounding in volved vessels is sometimes noted. Secon dary thrombosis apparently results from the narrowing of the vascular lumina, with the tissue surrounding the vessel undergoing slow necrosis. Though the condition may histologically resemble the vascular involve ment seen in thromboangiitis obliterans or the endovasculitis or scleroderma, it is clini cally unlike either of these disorders. 3 Ocular findings have been stressed by Feuerman, 6 who noted prominent microaneurysms of the bulbar conjunctiva vessels in a 46-year-old white man with malignant atrophic papulosis. The patient also had a typical papule on his right upper lid. Feuer man feels that the conjunctival vessel abnor malities are probably related to the disorder and should be searched for in other patients with malignant atrophic papulosis. The pa tient of Strole, Clark, and Isselbacher,9 a 21-year-old white man, had typical vascular lesions of the conjunctiva demonstrable his tologically but not noted clinically. Both of the cases reported by Winkelmann and his colleagues3 had ocular abnormalities. The first patient, a 20-year-old white man with central nervous system complications, had glial blurring of his fundi which later devel oped into definite papilledema. The second patient, a 36-year-old white man, developed unusual whitish plaques on the sclera be tween the cornea and inner canthus of each eye, more marked on the right side. These plaques were surrounded by telangectasia. The patient of Culicchia, Gol, and Erickson,7 a 34-year-old white man, devel oped total external ophthalmoplegia and bilat eral optic atrophy during the terminal phase of his disease. Sidi and co-workers8 describe their patient, a 30-year-old white man, as having a small plaque of atrophic choroiditis on the right lateral f undus. In our patient, congenital glaucoma of the left eye and a coloboma of the right macula
VOL. 65, NO. 2
MALIGNANT ATROPHIC PAPULOSIS
were noted at age two years. At a later age, patches of choroiditis were seen bilaterally but tests for tuberculosis, histoplasmosis, and toxoplasmosis were negative. Endovasculitis with marked subendothelial fibrosis in volving the anterior episcleral vessels of the left eye was noted on pathologic examina tion. Similar vascular changes were seen in several of the larger retinal vessels, and a choroidal arteriole. These changes were identical with lesions found elsewhere in the body, including the brain, and would thus appear to be directly related to malignant atrophic papulosis. The relationship of the posterior pole coloboma and possible anter ior chamber abnormality to malignant atrophic papulosis is unknown, and probably fortuitous. However, choroidoretinal lesions could well result from slow obliteration of choroidal vessels, if these are involved. Per haps a coloboma of the macula could develop in infancy if choroidal vessels beneath the macula are obliterated by the disease pro cess. There is little question that this intriguing disease needs further ophthalmic study from both a clinical and a pathologic standpoint. 550 First Avenue
(10016)
169
ACKNOWLEDGMENTS
We wish to thank Dr. George Kurz for review ing this paper, and Miss Doris Pagan and Mr. Henrick Malpica for secretarial and photographic as sistance. REFERENCES
1. Degos, R., Delort, J., and Tricot, R.: Dermatite papulo-squameuse atrophiante. Bull. Soc. Franc. Derm. Syph. 49:148,1942. 2. Degos, R.: Malignant atrophic papulosis: A fatal cutaneointestinal syndrome. Brit. J. Dermat. 66:304,1954. 3. Winkelmann, R. K., Howard, F. M., Jr., Perry, H. O., and Miller, R. H.: Malignant papu losis of skin and cerebrum: A syndrome of vascu lar thrombosis. Arch. Derm. 87:54, 1963. 4. Nomland, R, and Lay ton, J. M.: Malignant Papulosis atrophicans maligna (Degos' disease). neointestinal syndrome. Arch. Derm. 81:181, 1960. 5. Naylor, D., Mullins, J. F., and Gilmore, J. F.: Papulosis atrophicans maligna (Degos' disease). Arch. Derm. 81:189, 1960. 6. Feuerman, E. J.: Papulosis atrophicans ma ligna Degos: With microaneurysms of the conjunc tiva. Arch. Derm. 94:440, 1966. 7. Culicchia, C. F., Gol, A., and Erickson, E. E.: Diffuse central nervous system involvement in pa pulosis atrophicans maligna. Neurology 12:503, 1962. 8. Sidi, E., Reinberg, A., Spinasse, J. B., and Hincky, M.: Lethal cutaneous and gastrointestinal arteriolar thrombosis (malignant atrophying papu losis of Degos). JAMA 174:1170, 1960. 9. Strole, W. E., Jr., Clark, W. H , Jr., and Isselbacher, K. J.: Progressive arterial occlusive dis ease (Kohlmeier-Degos). A frequently fatal cutaneo-systemic disorder. New England J. Med. 276:195,1967.