anomalies and curiosities of medicine-第206部分

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　affection；　and　may　be　quite　absent。　The　atrophic　type　of　scleroderma　is　preceded　by　an　edema；　and　from　pressure…atrophy　of　the　fat　and　muscles　the　skin　of　the　face　is　strained　over　the　bones；　the　lips　are　shortened；　the　gums　shrink　from　the　teeth　and　lead　to　caries；　and　the　nostrils　are　compressed。　The　strained　skin　and　the　emotionless　features　（relieved　only　by　telangiectatic　striae）　give　the　countenance　a　ghastly；　corpse…like　aspect。　The　etiology　and　pathology　of　this　disease　are　quite　obscure。　Happily　the　prognosis　is　good；　as　there　is　a　tendency　to　spontaneous　recovery；　although　the　convalescence　may　be　extended。

Although　regarded　by　many　as　a　disease　distinct　from　scleroderma；　morphea　is　best　described　as　a　circumscribed　scleroderma；　and　presents　itself　in　two　clinical　aspects：　patches　and　bands；　the　patches　being　the　more　common。

Scleroderma　neonatorum　is　an　induration　of　the　skin；　congenital　and　occurring　soon　after　birth；　and　is　invariably　fatal。　A　disease　somewhat　analogous　is　edema　neonatorum；　which　is　a　subcutaneous　edema　with　induration　affecting　the　new…born。　If　complete　it　is　invariably　fatal；　but　in　a　few　cases　in　which　the　process　has　been　incomplete　recovery　has　occurred。　Gerard　reports　recovery　from　a　case　of　sclerema　neonatorum　in　an　infant　five　weeks　old；　which　seemed　in　perfect　health　but　for　this　skin…affection。　The　back　presented　a　remarkable　induration　which　involved　the　entire　dorsal　aspect；　including　the　deltoid　regions；　the　upper　arms；　the　buttocks；　and　the　thighs；　down　to　and　involving　the　popliteal　spaces。　The　edges　of　the　indurated　skin　were　sharply　defined；　irregular；　and　map…like。　The　affected　skin　was　stretched；　but　not　shiny；　and　exhibited　a　pink　mottling；　it　could　not　be　pinched　between　the　fingers；　pressure　produced　no　pitting；　but　rendered　the　surface　pale　for　a　time。　The　induration　upon　the　buttocks　had　been　noticed　immediately　after　birth；　and　the　region　was　at　first　of　a　deep　pink　color。　During　the　first　nine　days　the　trouble　had　extended　to　the　thighs；　but　only　shortly　before　the　examination　had　it　attacked　the　arms。　Inunctions　of　codliver　oil　were　at　first　used；　but　with　little　improvement。　Blue　ointment　was　substituted；　and　improvement　commenced。　As　the　induration　cleared　up；　outlying　patches　of　the　affected　skin　were　left　surrounded　by　normal　integument。　No　pitting　could　be　produced　even　after　the　tension　of　the　skin　had　decreased　during　recovery。　The　lowest　rectal　temperature　was　98　degrees　F。　In　a　little　more　than　four　months　the　skin　became　normal。　The　treatment　with　mercurial　ointment　was　stopped　some　time　before　recovery。

Possibly　the　most　interesting　of　the　examples　of　skin…anomaly　was　the　〃elephant…man〃　of　London。　His　real　name　was　Merrick。　He　was　born　at　Leicester；　and　gave　an　elaborate　account　of　shock　experienced　by　his　mother　shortly　before　his　birth；　when　she　was　knocked　down　by　an　elephant　at　a　circus；　to　this　circumstance　he　attributed　his　unfortunate　condition。　He　derived　his　name　from　a　proboscis…like　projection　of　his　nose　and　lips；　together　with　a　peculiar　deformity　of　the　forehead。　He　was　victimized　by　showmen　during　his　early　life；　and　for　a　time　was　shown　in　Whitechapel　Road；　where　his　exhibition　was　stopped　by　the　police。　He　was　afterward　shown　in　Belgium；　and　was　there　plundered　of　all　his　savings。　The　gruesome　spectacle　he　presented　ostracized　him　from　the　pleasures　of　friendship　and　society；　and　sometimes　interfered　with　his　travels。　On　one　occasion　a　steamboat　captain　refused　to　take　him　as　a　passenger。　Treves　exhibited　him　twice　before　the　Pathological　Society　of　London。　His　affection　was　not　elephantiasis；　but　a　complication　of　congenital　hypertrophy　of　certain　bones　and　pachydermatocele　and　papilloma　of　the　skin。　From　his　youth　he　suffered　from　a　disease　of　the　left　hip…joint。　The　papillary　masses　developed　on　the　skin　of　the　back；　buttock；　and　occiput。　In　the　right　pectoral　and　posterior　aspect　of　the　right　axillary　region；　and　over　the　buttocks；　the　affected　skin　hung　in　heavy　pendulous　flaps。　His　left　arm　was　free　from　disease。　His　head　grew　so　heavy　that　at　length　he　had　great　difficulty　in　holding　it　up。　He　slept　in　a　sitting　or　crouching　position；　with　his　hands　clasped　over　his　legs；　and　his　head　on　his　knees。　If　he　lay　down　flat；　the　heavy　head　showed　a　tendency　to　fall　back　and　produce　a　sense　of　suffocation。　For　a　long　time　he　was　an　inmate　of　the　London　Hospital；　where　special　quarters　were　provided　for　him；　and　it　was　there　that　he　was　found　dead；　April　11；　1890；　while　in　bed　his　ponderous　head　had　fallen　backward　and　dislocated　his　neck。

Ainhum　may　be　defined　as　a　pathologic　process；　the　ultimate　result　of　which　is　a　spontaneous　amputation　of　the　little　toe。　It　is　confined　almost　exclusively　to　negroes；　chiefly　males；　and　of　African　descent。　In　Brazil　it　is　called　〃ainham〃　or　〃quigila。〃　〃Ainham〃　literally　means　to　saw；　and　is　doubtless　a　colloquial　name　derived　from　a　supposed　slow；　sawing　process。　The　Hindoo　name　for　it　is　〃sukha　pakla；〃　meaning　dry　suppuration。

In　1866　da　Silva　Lima　of　Bahia；　at　the　Misericordia　Hospital；　gave　the　first　reports　of　this　curious　disease；　and　for　quite　a　period　it　was　supposed　to　be　confined　to　Brazilian　territory。　Since　then；　however；　it　has　been　reported　from　nearly　every　quarter　of　the　globe。　Relative　to　its　geographic　distribution；　Pyle　states　that　da　Silva　Lima　and　Seixas　of　Bahia　have　reported　numerous　cases　in　Brazil；　as　have　Figueredo；　Pereira；　Pirovano；　Alpin；　and　Guimares。　Toppin　reports　it　in　Pernambuco。　Mr。　Milton　reports　a　case　from　Cairo；　and　Dr。　Creswell　at　Suez；　both　in　slaves。　E。　A。　G。　Doyle　reports　several　cases　at　the　Fernando　Hospital；　Trinidad。　Digby　reports　its　prevalence　on　the　west　coast　of　Africa；　particularly　among　a　race　of　negroes　called　Krumens。　Messum　reports　it　in　the　South　African　Republic；　and　speaks　of　its　prevalence　among　the　Kaffirs。　Eyles　reports　it　on　the　Gold　Coast。　It　has　also　been　seen　in　Algiers　and　Madagascar。　Through　the　able　efforts　of　Her　Majesty's　surgeons　in　India　the　presence　of　ainhum　has　been　shown　in　India；　and　considerable　investigation　made　as　to　its　etiology；　pathologic　histology；　etc。　Wise　at　Dacca；　Smyth　and　Crombie　at　Calcutta；　Henderson　at　Bombay；　and　Warden；　Sen；　Crawford；　and　Cooper　in　other　portions　of　Southern　India　have　all　rendered　assistance　in　the　investigation　of　ainhum。　In　China　a　case　has　been　seen；　and　British　surgeons　speak　of　it　as　occurring　in　Ceylon。　Von　Winckler　presents　an　admirable　report　of　20　cases　at　Georgetown；　British　Guiana。　Dr。　Potoppidan　sends　a　report　of　a　case　in　a　negress　on　St。　Thomas　Island。　The　disease　has　several　times　been　observed　in　Polynesia。

Dr。　Hornaday　reports　a　case　in　a　negress　from　North　Carolina；　and；　curious　to　relate；　Horwitz　of　Philadelphia　and　Shepherd　of　Canada　found　cases　in　negroes　both　of　North　Carolina　antecedents。　Dr。　James　Evans　reports　a　case　in　a　negro　seventy…four　years　of　age；　at　Darlington；　S。C。　Dr。　R。　H。　Days　of　Baton　Rouge；　La。；　had　a　case　in　a　negress；　and　Dr。　J。　L。　Deslates；　also　of　Louisiana；　reports　four　cases　in　St。　James　Parish。　Pyle　has　seen　a　case　in　a　negress　aged　fifty　years；　at　the　Emergency　Hospital　in　Washington。

So　prevalent　is　the　disease　in　India　that　Crawford　found　a　case　in　every　2500　surgical　cases　at　the　Indian　hospitals。　The　absence　of　pain　or　inconvenience　in　many　instances　doubtless　keeps　the　number　of　cases　reported　few；　and　again　we　must　take　into　consideration　the　fact　that　the　class　of　persons　afflicted　with　ainhum　are　seldom　brought　in　contact　with　medical　men。

The　disease　usually　affects　the　5th　phalanx　at　the　interphalangeal　joint。　Cases　of　the　4th　and　other　phalanges　have　been　reported。　Cooper　speaks　of　a　young　Brahman　who　lost　his　left　great　toe　by　this　process。　Crombie　speaks　of　a　simultaneous　amputation　of　both　fourth　toes。　Potoppidan　reports　a　similar　case　in　a　negress　on　St。　Thomas　Island。　Sen　reports　a　case　in　a　supernumerary　digit　in　a　child；　whose　father；　a　Hindoo；　lost　a　toe　by　ainhum。　Eyles　reports　a　case　in　a　negro　in　whom　the　second　finger　was　affected。　Mirault；　at　Angiers；　speaks　of　a　case　in　which　two　fingers　were　lost　in　fifteen　days；　a　fact　which　makes　his　diagnosis　dubious。　Beranger…Ferraud　has　seen　all　the　toes　amputated；　and　there　is　a　wax　model　by　Baretta；　Paris；　in　the　Army　Medical　Museum　at　Washington；　in　which　all　the　toes　of　the　right　foot　have　been　amputated；　and　the　process　is　fast　making　progress　at　the　middle　third　of　the　leg。

Ainhum　is　much　more　common　in　males　than　in　females；　it　is；　in　fact；　distinctly　rare　in　the　latter。　Of　von　Winckler's　20　cases　all　were　males。

It　may　occur　at　any　age；　but　is　most　common　between　thirty　and　thirty…five。　It　has　been　reported　in　utero　by　Guyot；　and　was　seen　to　extend　up　to　the　thigh；　a　statement　that　is　most　likely　fallacious。　However；　there　are　well…　authenticated　cases　in　infants；　and　again　in　persons　over　seventy　years　of　age。

In　some　few　cases　the　metatarso…phalangeal　joint　is　affected；　but　no　case　has　been　seen　at　the　base　of　the　ungual　phalanx。　The　duration　of　the　disease　is　between　two　and　four　years；　but　Dr。　Evans's　case　had　been　in　progress　fifty　years。　It　rarely　runs　its　full　course　before　a　year。

Ainhum　begins　as　a　small　furrow　or　crack；　such　as　soldiers　often　experience；　at　the　digito…plantar　fold；　seen　first　on　the　inner　side。　This　process　of　furrowing　never　advances　in　soldiers；　and　has　been　given　a　name　more　expressive　than　elegant。　In　ainhum　the　toe　will　swell　in　a　few　days；　and　a　pain；　burning　or　shooting　in　nature；　may　be　experienced　in　the　foot　and　leg　affected。　Pain；　however；　is　not　constant。　There　may　be　an　erythematous　eruption　accompanying　the　swelling。　The　furrow　increases　laterally　and　in　depth；　and　meets　on　the　dorsal　aspect　of　the　toe；　giving　the　toe　the　appearance　of　being　constricted　by　a　piece　of　fine　cord。　As　the　furrow　deepens　the　distal　end　of　the　toe　becomes　ovoid；　and　soon　an　appearance　as　of　a　marble　attached　to　the　toe　by　a　fibrous　pedicle　presents　itself。　By　this　time　the　swelling；　if　any；　has　subsided。　The　distal　end　of　the　toe　bends　under　the　foot；　and　becomes　twisted　when　walking；　and　causes　inconvenience；　and；　unfortunately；　says　Eyles；　it　is　in　this　last　stage　only　that　the　Fanti　presents　himself。　There　is　in　the　majority　of　cases　a