Document Gmj4mJaw7EkO76mKgNg9VNOzn

RECEIVED JUL 1 a 1983 R. N. Wheeler, Jr. Cutaneous-Lesions 224 Arch Derm/Vol 106. Aug 1972 T Lawrrnct B. Meytrwon. MD, and Gtrhard C. Mtitr, MD, Fort Dig, NJ A patient with Idiopathic aeroosteolyala had unique papular akin lesiona. To our knowledge, aimilar leaiona have been previoualy mentioned only In connection with acrooeteolyale aeon in vinyl chloride processing, end a do* tailed description of theae haa net been The term acrooeteolyais refera to lytic changes of the shafts of the distal phalanges, with preservation of the tufts and bases. In most cases, other bones are also involved. Three types have been described in the literature: (1) familial,'-* (2) idio pathic or nonfamilial,1* and (3) thoee associated with vinyl chloride proces sing.'* We are describing a case of idio pathic acrooeteolyais, manifesting unique skin lesions. Similar lesions have been described only briefly in some cases associated with industrial vinyl chloride processing. To our knowledge, these cutaneous findings have not been discussed in the der matologic literature. Report of a Case In August 1969, a 36-year-old white woman was examined at US Walaon Army Hospital. Her condition had appar ently started in 1960. During cold weather, she began to develop symptoms Accepted for publication Jan 17.1972. From the Dermatology Service and Departmerit or Medicine, Walton Army Hoapital, Fort Dii. NJ. Dr. Meyemon it now in practice in trving, Tex, and Dr. Meiar it in practice in New. port. Ri. Reprint requeue to Irving Medical end Profcuional Villnge, 3101 MacArthur Bivd, Irving, Tex 78061 iDr. Meyerwni, Fig I.-Note ihort, thick fingertips and nail changes. eig.,.-PS MJHiU* jm i | '\ 1 : !. l {j ' }; i- ' . . I Acroosteolysis/Meyerson A Meier ucc 049234 Arch Oerm/Vol 106, Aug 1972 Fi( 4.--X-ray film of right hand showing acroosteolysi*. Fig 5.-Thickcned dermis with enlarged collagan fibers (separation of fibers is an artifact). Nota preservation of eeenne sweat gland and duct (hematoxyiin-eosin,x40). Fig 6.-Increase in elastic fibers, with fray ing and fragmentation (elastic stain,*400). Acroosteolysis/Meyerson & Meier 223 ucc 049235 typical of Raynaud'i phenomenon. Early in 1961. the right fifth finger became painful, slowly shortened* end the fingernail became shorter and thicker. At the tame time, cutaneous lesion* ap peared on both wrists. By May 1961. most of the lingers had become similarly in volved. and the cutaneous lesion* spread to involve the arms, axillae, neck and trunk. The patient was hospitalized at that time, and x-ray film* of the hands showed destruction of all distal pha langes, with preservation of the tufts. A diagnosis of scleroderma was made. Despite the administration of predni sone from 1961 to 1966, the changes in the distal fingers and nails gradually progressed, and the skin lesions enlarged and spread. The finding* of a skin biopsy obtained in 1965 were reported to be con sistent with the diagnosis of scleroderma, and the patient was placed on a regimen of potassium aminobenzoate with no efiect. From 1966 on, the patient de veloped progressive difficulty in the use of her jaw. with pain involving the temporo mandibular joints, leading to progreaaive malocclusion of the teeth. X-ray films of the jaws in 1968 revested osteolysis of the articular surfaces of the mandible. This condition progressed, and in 1970 she developed a pathologic fracture through the neck of the right mandibular condyle. Therapy for this aspect of her condition was conservative with the use of various dental appliances for support and partial correction. In 1968. vitamin E was prescribed, and in the patient's opinion, the akin lesion* decreased somewhat in size. However, no inprov*"n`nt was noted ii the otter symptom* or on subsequent x-rey films. The patient denied any sensory change* in the hands or feet. She was never em ployed in the processing of vinyl chloride or any other chemical. She primarily worked in offices of various financial in stitutions. There is no family hietory of a similar condition. Her father, aged 68, has arthri tis end a "straight spine," and her moth er, aged 69. has rheumatoid arthritis. Her maternal great-great-grandfathers were closely related, but it it not certain whe ther they were brothers or first cousins. Physical examination revealed shorten ing of all the distal phalanges of the hands and short, thickened fingernails (Fig 1). Similar changes were noted on the fourth toe of the right foot. The fingertips were broad with no tapering or ulcerations. Skin of the hands and arms was somewhat tighter than normal, but there appeared to be normal range of mo tion in the joints. The facial skin similarly was tighter than normal. The most striking Abnormality of the skin was the presence of multiple yellow ish, cutaneous papules, 2 to 4 mm in size, appesring in linear distribution or in patches, and in areas becoming confluent. The most involved area was the skin of the forearms, primarily the flexor sur faces. Here, the papules were large and tended to form linear bands extending up the arms iFig 2). Similar, though 1cm prominent, papule* surrounded both axil lae, but spared the vaults (Fig 3). Patches of small papules were also found on the anterior and posterior aspect* of the neck, the upper part* of the chest and back, bendath both breasts, around the waist, and the upper thigh*. No involvement of the face, lower part of the back, abdomen or lower part of the leg* was noted. The petient was seen in consultation by an oral surgeon who noted an inability of the mandible to return into a normocentric, occlusal relationship with the maxil la. Due to this malocclusion, the brunt of the occlusal force* was borne by the poste rior teeth which resulted in an anterior open bite. The remainder of the physical examina tion showed no abnormalities. The results of the following laboratory tudies showed values within normal lim its: complete blood cell count, sedimenta tion rate, VDRL test for syphilis, blood glucose, bilirubin, total protein and pro tein electrophoresis, calcium, phosphorus, alkaline phosphatase, uric arid, serum glutamic oxaloacetic transaminase, se rum glutamic pyruvic transaminase, lac tic dehydrogenase, blood urea nitrogen, rheumatoid rrthriti i latex fixation, cryo globulins, antinuclear antibodies, LE prep aration, and urinalysis. X-ray film* showed acrooateolyai* of all distal phalanges of the hands (Fig 4) and of the right fourth and fifth and th* left second toes. X-ray film* of the mandible showed a marked reiorptive process of the articular surfaces of the condyle* and a complete pathologic fracture through the neck of the right condyle. X-ray films of the chest, skull, spine, and long bones were sll normal. Pathological Findings.--Biopsies tak en from the right axilla and the right wrist revealed identical findings. Histo logic examination showed flattening of the epidermis with loss of the rate ridges. The dermis was thickened with enlarged collegen fibers and decreased cellularity (Fig 5). The adnexal structures appeared intact, and there was no inflammatory reaction. An elastic tissue stain showed an increase in the number of elastic fibers with fraying and fragmentation (Fig 6). The results of stain tests for mucopulysaccharides were negative. Comment Familial acroosteolysis11 is proba bly autosomal dominant and occurs more commonly in males. The dis ease usually begins in early child hood or adolescence with a slightly painful ulceration on the sole of the foot. The ulcer slowly grows over several weeks or months, discharges bony fragments, and then heals. The process continues with recurrent epi sodes associated with slight fever. The feet gradually become deformed, swollen, and mutilated. Sensory dis turbances of both the upper and lower extremities are always associ ated. X-ray films show osteomyelitic changes in the areas of ulceration as well as acroosteolysia of the pha langes. Idiopathic, nonfamilial acroosteolysis*^ usually starts in early adult hood. The hands are more severely involved than the feet, and there is no associated neurologic abnor mality. Raynaud's phanomenon has been reported in some cases. No ulcerations appear, and no consis tent, cutaneous lesions have been described. Radiologic findings in clude acroosteolysia of the phalanges, defects of the metatarsal-phalangeal joints, and resorption of the alveolar process in the jiwr. Changes have also been reported in the skull, spine, and other bones. Classification of these first two types is by no means consistent or totally accepted in the literature. Some reported cases of acroosteolysia have been secondary to osteomalaria*'1* or hyperparathyroidism." Giaccai* mentioned a sporadic ''non familial type of neurogenic acroosteolysis." Cheney'* described a family' in which several members had fea tures of both familial and idiopathic acroosteolysis. A new type of acroosteolysis was first reported in the English litera ture by Wilson et al* in 1967. They described 31 cases involving the hands of workmen engaged in vinyl chloride polymerization. (Vinyl chlo ride is made into polyvinyl chloride, a widely used synthetic resin.) The 226 Arch Derm/Vol 106, Aug 1972 Acroosteolysis/Meyerson & Meier UCC 049236 t ..(imon is apparently specifically .. n-iaii'rf with the hand cleaning of Sint-mers/ 11 A survey of more _n 1,000 persons who handled the -.islicd resin, or processed it into .,`.,rnc products, failed to find any . ;^r,ional cases/ '! he great majority of patients de. riL'c-d by Wilson et al had Ray _uds phenomenon and all had ac- Ti-teolysis of the distal phalanges of hands. According to a written - rr.munication from R. H. Wilson, VD. nn Jan 21, 1970, three or four of M ir 31 cases "had external skin le. >-< on the dorsal surfaces of the mds and forearms, with a rope:c appearance resembling changes ..incomes seen in scleroderma."7 ;'.irns and Adams" described two .scs associated with vinyl chloride . -no.-sing. One had "puffinoss of the cc and thickening of the skin of the `..mds, fingers, and left forearm," and * .t condition had previously been : agnosed as scleroderma. The other .orient had "raised nodules in the -..in. described as 'xanthomatous\c patches" around his wrists. The skin lesions described in these patients are almost identical with those found in our case. Further more, the histopathologic findings reported by Harris and Adams are essentially the same as in our pa tient. We reviewed the slides from two cases of Wilson et al and found these too to have the same histopath ologic evidence. All had thickened collagen fibers and fragmented elas tic fibers in the dermis. The adnexae were normal, and there was no significant inflammation present. Because of the similarities to our patient and because we could not find such skin lesions described in other types of acroosteolysis, we carefully questioned our patient about any possible contact with vinyl chloride processing. She denied any such contact and has never been employed in any type of chemical factory. Thus, our patient apparently represents the first reported case of idiopathic acroosteoiysts to manifest these peculiar skin lesions. The cause of this disease ie ob scure. Some of the industrial cases were initially thought to represent atypical scleroderma,"-'* and our pa tient had this diagnosis for some ti me. However, the skin lesions do not really resemble scleroderma, and the histopathological findings are also different. Furthermore, the x-ray film changes in scleroderma almost always show destruction of the phal angeal tufts, and not acroosteolysis, which has relative sparing of the tufts. Only a small fraction of workers engaged in cleaning polymerizers develop the disease. Therefore, there must be some predisposition in these people which is brought out by con tact with certain chemicals. Similar ly, our patient may have had such a predisposition and had her disease triggered by some unknown factor or factors. John Crttch, MD* km us th* pathological slid** from th* aw of Wilton *t al. Lonia J. Brunei!* mdr the photographs and photomicrographs. Nonproprietary and Trade Names of Drug Potassium sminobenzoate--Potato. ' Harm I: Cber die familiar* Akrootteo- Fm-uahr Ruentgtnttr 80-.7Z7.732,1984. Ciaocai U Familial and tpondic neuro aie acnmteolyti*. Acta Radiol 38:17.29. : 'I. I llamasch Hi Die Akrwataolyiit. ein netit* ..jnkheitahild. Forirehr Rorntgennr 72:352- 1.19*0. Wicland II: Ein Beitrag zur Kenntni* der 1 -ueieolyae, Fontchr Rorntgrnttr 77:193-198, l. ' Cmnberg BE. Street DM: Idiopathic non-- ilial .icrnoateolyiia. Radiology 69:259-262, Pvpavaailiou CG, Gargano FP, Waite Wlc References Idiopathic noniasilial acro-oateolysia associ ated with othtr bona abnonnalitia*. Amtr J Roentgen 83:6874191, i960. 7. Wilaon RH. McCormick WE, Tetu* CF, et al; Occupational acmottaolyaia. JAMA 201:577881,1967. 8. Harris DK, Adama WGF: Acro-o*t*olyi* occurring in men engaged in the polymerization of vinyl chloride. Srtr Mta'J 3:712-714,1967, 9. Kleinaorg# H: Akmoeteolytiache Enchainungen der Oateonialacie. Fortnehr Rorntgtnrtr 73:471-475. 1950. 10. Wassner L'J: Ein weiteter Fall einer Akio-oataolyna: Zugleich ein Beitrag (u ihter differential Ditgnoe*. Foruehr Rotmgtnsir SO: 186-191.1964. 11. Jeeaner H: Zuan Erscheinungibild der Akrooateolya*. FarUehr Rotnigtnttr 77:645552,1962. 12. Cheney WD: Acra-oateolyata. Amtr J Rotnlgen 94:596-607.1965. 13. Dinman BD. Cook U'A. Whitehoua* WM. et al: Occupational acronrieolyiia: I. Epidemiol ogical ttudy. Anh Enitron Htalth 22:61-73, 1971. 14. Sudu I, Drtjman I. Valaakal M: Contributii la atudiul imbolnavinlor pmduae d* do- run de vinil. Mtd Inttm 15:967-978,1963. *"h Drrm/Vol 106, Aug 1972 y *" _ '* --ft . ' 1 - ' .Tf . Acroosteolysis/Meyerson & Meier 227 Occ w*t*&^*p*^- - ..*9237 ...................