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AlluringNitrogen

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Andre Basset, Bernard Lioufaud, Bassirou Ndinye, Andrew Pembroke

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dermatology skin diseases black skin medical textbook

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This pocket atlas focuses on the clinical signs of skin diseases in Black patients, highlighting significant differences from those in White patients. It emphasizes the importance of studying dermatology in diverse populations, especially in tropical regions.

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'gena ket al.. ---mma* ~ kin at Flll ((t-g. mcasic: I systc n &eases, sarc is). A 3 10 ieprosy and its lesions of Black Skin Andre Basset Honorary Professor, Faculty of Medicine,...

'gena ket al.. ---mma* ~ kin at Flll ((t-g. mcasic: I systc n &eases, sarc is). A 3 10 ieprosy and its lesions of Black Skin Andre Basset Honorary Professor, Faculty of Medicine, S~rasbourg,France Bernard Lioufaud Professor of Dermatology, Port-ou-Pr~nce,Hoit~ Bassirou Ndinye Professor of Dermatology, Dakar, Senegal Tranrlaied by Andrew Pembroke Consultant Dermatologist, King'sCollege Hospiial, London, UK Oxford University Press 1986 Contents Pigmentary Disorders Other Characteristics of Black Skin hema Psoriasis Liehen Planus Collagen Diseases Bdous Dermatoses Geoodermatoses Acne Leprosy Benign Tumours Malignant Tumours Kaposi's Sarcoma ~Usneous Index I This pocket atlas is not just intended for dermatologists but for all medicaI and para-medical stafflooking after Black patients. Its obiect is to desaibethe clinical signs of skindiseases in lack patients, which arc signific&tly different form thc classical text hook doscriptions based on Whitc ~aticnts.This is whv we have concentrated on the majoiskin diseases in thii work, although the &Id of Black dennatologyis much wider than this. We have deliberately omitted infections and parasitic diseases, as these-mvolveother disciplines and as their cutaneous I features would merit a separate volume. We have made an exception for leprosy because of its obvious importancein Black patients. It is worth stresing the importanceof studying dermatology in bla~kskin. For one thing, the number of patients is very large. Most Black people live in tropical countries where 30% of wnsultations are for skin disease. In these wuntries the dermatologist sees all the conditions met in temperate climates, plus certain serious endemic diseases such as leprosy and various malignancies. Moreover, the physical signsin bla& s k i are different to those in white skin and here dermatologyin black skin forms a study in its own right, which this Atlasis intended to assist. PhysicalSignsin Black Skin Melanin pfgmutMion. This is the most obvious difference of black skin. The reasons for the difference are now well known. White and Black people have the same melanin and the same number of melanocytes per unit surface area. The melanhcytes are more active 4 Black peopie, but more important is the absorption of melanin by the Malpighian layer; in White people melanin does not get past the first few layers of the epidermis, while in Blacks melaninis not reabsorbed and reaches thestratum corneum. This is easily 1 demo~~strated: cotton wool soaked in ether becomes dark if rubkd o n the skin of a 13lack subject even if he is smpulously clean. In black skin a whole ranpe of dermatological terms such as etythema, erythrodenna, pityriasis rosea or mbra cannat be takenliterally. ETythema is black or a dark violet; the 'homme rouge' of Hallopeau is slate-grey. It is &cult far theunpractised eye to diagnose measles, and a macular syphilideis Cvenmore difficult. The melanin pigment protects against ultra-violetrays, and Blacks very rarely get solar keratoses or basal-cell carcinomas. On the other hand, melanin does not orotect axdnst infra-red rays, and Blacks are more liable ihan whiiees to heatdstroke: despite a rather 1 better-developed superficial cukmeons vaseulature. I Other features. ln manv dermatoses. the Dlimarv leslons are larger and more prc;nounced than in h t e s. ' Fia. 2 H~stoloov of blackskm Same stam The oiument 1s seen ~ ~ For instance, in lichen planus, Wickham's striae, a fine l h ~ ~ ~ n o ~ l l n ~ a~, aayerl p.ana g h n\.mero~s"&nent garLles are white network on the surface of each papule, may not be srrcr ever1.n Itlf! sqll:lmls r)t lhe slratim correum. readily visible id Caucasians, whereas it is readily visible Flg. 3 Measles to the naked eye in Blacks. Pityriasis rosea is another exanthem, acute good example. phase Theskln 1s not red, I! is con- oested and has a k e granularsur- face. The d~agnosis IS dlff~cultfor the In experienced despite the presence offever, catarrh and K ~ l l k ' s spots as In Caucas~ans. I Fig. 1 n sloogy w the SK,? irom a n.dncllc CG2nsan s..h,ec Stalnea wath sllver lo silo& lhc mela.1.n The plgrnenr 1s p1om.nelt in I~ICoasal layer and :he lirsl four luyers ot tile M u I p ~ y l ~laycr. k r ~ lrlere S no !racc of plgrnent at !he sLrlace. m.4 Inthephase of resolution. measles leavesa fine desquamation which is much more conspiclous than in I darrna k offen related - to a Caucasians. &~~hom'ain the I Blackit isnot red but slategrey. -5 Maculer syphiiide(roseola). rnisearlyand characterisfic sign 07 secondarysyphilis is sean in.the Caucasian as an eruptionof discrete 'peach-blossan' macules on the flanks in the Black it appearsas small hypopigmented macules. Fig.7 Prtyriass rosea in a Fig.8 P~iynaslsrosea in & Caucasian Thts eruption of pink Black, illustrating well the medallicnseach bordered bya d~fferencesIn physicalsrgns the fine collarehe of scale occurs on eruotion is orevish rather than the trunk and~.the oroximal oarts ~ ~ ~. otni the- mzdil~ons - - -~ - are ~ ~ - laroer ~- d k e 1.mos: 11clears spon-' ihescales thicker. ~everthG&. tanewsly m 2-3 weeks. A vlral thecourseof the disease is the aebology has been suspected Same but not proved. - Pigmentary Disorders emption, lichen planus and lichenifcation. In scaly parakeratotic dermatoses, on the other hand, there may be hypopigmentation as the accelerated keratinisation does not allow the melanocytes to inject their 1 melanommes into the keratlnocytw. This is seen in psoriasis and seborrhceiceaema. Many other mechanisms may account for pigmentary disturbances, I such as nerve involvement in tuberculoid leprosy, products secreted by certain fungi, spirochaetes, cancers, scarring in lupus erythematosus, cosmetics, or developmental conditions such as the Mongolian spots. Fig. S Vitiiigo. The loss of pigment is usually complete. The first case is the commonest type which is symmetrical over the hands and forearms. In the second case, the zosteriform pattern suggests a neural mechanism. The treatment of vitiligo is disappointing: topical or oral 8- These are similar to those in Caucasians but more methoxypsoralen is - not always helpful conspicuous in a black skin. Black people are more and may be sensitive to any variation in skin colour, and are dangerous. especially womed by loss of pigment because of the fear of leprosy. Syndromes of total depigmentation such as vitiligo and albinism occur. A special form of vitiligo with incomplete loss of pigment is common and corresponds to the 'vitiligo minor'of Fitzpatrick. Vitiligo is caused by 7 theloss of melanocytes. Albinism, on theather hand, is due to a genetic defect such as tyrosinase deficiency. Oculocutaneous albinism, which has an autosomal recessive inheritance, is characterised by total loss of I pigment of the skin, hair and eyes, which are affected with nystagmus and photophobia. This condition is serious in a tropical climate, as the skin has lost its melanin protection against sunlight and exhibits an early tendency to keratoses and skin cancers. Acquired pigmentary disturbances are of various types. In dermatoseswhere there is damage to the basal layer, the pigment enters the upper dermis and remains there for a long time; this happens in the fixed drug.. Fig. 10 Vitiligo minor. This in- complete loss of pigment is common in Black oatients. - -----, reveals meianocyte abnomlalities similar tothose in classical vitiligo. *la 1 Fig.11 ocuro- FiwddNg cutaneousalblnism eruption. in the first Thffiboy demon- case, thw & a stralesdepigmen- slngle lesion dueto tation of the skm, anmidopyme hair and eyes, with derivative Inthe second,multpte I dipiopia and pale blue irises. Note the lesionslctlatadto I stellate freckles on teking suiphone- the face. and in the mides. Thess second picturethe lesiansare severe actinic persistem they chedbs. By courtesy become inRamed of Dr Caussade, and m y w e n blister WetyfiineW offending d u g is taken:eliirn M of the drug is cu%We. I Flg.13 Sebor- Flg. 18 Tiea due I rhoaic denatitis of to Tr;ohqohymrl the face. Thii rubrum. Thefungus infeelionincreases &unman. hi thep~gmentation of I presenceoffine. ' scaJingand lack of sensorydisturbance theskin. Treatment: a topicel imidamle, excludethediw nosis of leprosy. Flg.14 Pityriasis versicolor_w$h hypopigmentation: small lesions witha fine scale scattered llke confeni over the trunkand the prox~maicatsof the Ilrnbs, and some- limes even involving the face in atropical climate.Thecause :s a Pitymsporon Fig. 17 Discoid whtch secretes lupus erythema- azelaic ac~a. loxc lo tosus. The central act.ve melanocytes. area is depigmented Tnts explains tne and scarred. The 10.8 of the sun.n tne periphery on the appearanceof other hand is active hypopgmentation and deeply pig- - Treahnent: a toplca? mented because of irniWole or Selsun. the inflammatory reaction. Flg. 113 Laucwnelanorle~~a pintoides. Thislsa ~anrfestammof yawsand bej4 jeln in Africa, similar to pinta.The imlons fwm a oharacteri$~ctriangleonthe fomarm:iheyareti~~lour&the palm is pinkand hypopigmentedthe forearm lesions araviolacews, and there are black islands of parsistent pismentation. Theyare due to trspanmatain the epidermis. Treatment peniullin, Fig,l!3 M4anodermiaparaneapI~bmcsinananaf50wim~r &the bladder;it is a m p a n b d by slight hyperkereWto. - I I Fig2 l Monadipnsp4ts.Theae(trerwnde#pigmented macules , ! e r e p m t f r m BM on theMockssnsl backand&eppsw Thrrir~almdstunb~MIh nfti~.l"nevarefdlwm 12 - Other Characteristics of Black Skin FoIliculi@iof the beard may produce monsiroqskeloids full of purulent sinuses. Apparmtly printary kehids are *soally seen in the paternal areain the form of parallel bansverse bars. In fact they are the result of a minor Wculitis which escapedattention. True entemive primrry idiopathic keloids are quite rare; these have a m a d raised overhangingedge surrounded by a faint Halo of eqthema. The histology shows non-specific =&#lammation. The treatment of keloids is difficult. They should never be surgically excised as this results in an even larger keloid. The best @eatmeatis to give intralesional injections of cortiwsteroids by syringe a r Hyperkeratosis. Black skin is in general smooth anu delicate; it is very sensitive to mechanical and chemical irritants, wh~eheasily produce a reactive hyperkeratosis. This is seen especially on the soles of people who walk bare-foot. 'Keratoderma sulcatum' is a severe form of massive hyperkeratosis with furrows; Castellani blamed an infective agent, Actinornyces keratolyticur, whichin fact is a saprophyte. Sometimes there are unusual forms of hyperkeratosis such as the 'prayer nodules' on the foreheads of devout Muslims. Oil acne appears as keratoticpapules which are difficult to recogmse as follicularin origin. Keratodermas are also seenin the treponematoses such as yaws and bejel. Finally, Blacks like other races may present with congenital palmaplantar keratodermas. Keloids. These are fibrotic dermal tumours to which Black people are notoriously prone. Hence plastic surgery is hazardous, and even the risks of a skln blopsy should be considered carefully. Several sorts of keloids -22 Plantar keratoderma This 'hoplcalkeratoderm' ISvery may be distinguished. Secondary kmoids appear after m m o n In Afrcca inpeople who walk bare-footon roughground,for examplefi8hwmenfrom the Salourn blands in Senegal where the injuries, surgery, vaccinations, biopsies, burns etc, and ground Is covered in broken shells in the course of infections, acne, leprosy or tuberculosis., 14 I 15 1oa Plantar ratp pa ma in a Isedbeiel. The sharp demarcation of the keratodemta On@medidpartof thafmtandthe w s e desqua- ation golnt to a %gomatosi8 11th wascon- firmedsemlogicelly. Peniallm sth he treabnentof choice. rayer mdu1e.A looalisedksratodermaon the forehead of rho prostrstes himself five tfimes a day at prsyer. I FIg.24 'Kerato- derma suicatum: Thisgross, furrow6 keratodermacan be disabl~wand just- lfiestheUse of oral aromatic rstimids cTieaspn): CBS*nrb bory that it @dueto an actinomycate is unpmvw. e Fig. Is Oil acne in amatalvvorkerfrom%negd. Thefollicular arifioesaleoccluded by kerBtoiIic papules. Thi+isusualIydue t. armng01h I7 Fig. 27 Mai de Meleda in a 7year- old child The first two picturesshow the hand and foot lesions, and the third the knee invofve- ment: this dffitri- bufin confirms the atagnosis and excludes omer keratodermassuch as tylosis. Mal de 3.28 Keloid of the arm after smallpoxvaccination. Meledahas an auto- soma! recessive in- heritance. In severe cases, oral treat- ment with aromatic retlno~ds (T~gason) can be considered n. 29 Kelold of the olnnadue to o a r a i l e~-~ lineia~wWef~ ~~ ider of the helix, a tkdihonai practice in young African w a n m tl s not due to the wearlng 01earrings. flg.32 True primary keloids m w e extenslva keioids are thtck plaqueswith a sl~ghtlydepressed Gentreandan over- hangrngrnargm.At the endof the keio~d there is a faint ery- thematnus halo WhlCh becOmeS p~gmented and ind~catesan tnflam- matow prgcess, the histology only shows anon-speaf~cIn- flarnrnatbn and the GaUSe I$ ~nkn0wll These prlmaryor Fig. 30 Aeloid acne 0' the uack Thc solid Ics'ons or1the bacn are idiopathic keloids nor suppl.ra1 ng hut firm ana clastic. Thctace is rlardly aflcclcd. On ate seen in Aktca toe antenor aspect of the cnest there s some act:vc acrle TII s appearance~ouldsuggest \eprornatousleprosy Fig. 31 ApparPrMy pnrnary kelo~ds These arevery common ~nAfnca, and present as parallel barson the front of thechest Between these lesionsthereISan inconqplcuous folliculitrswh~ch1s responsrblefarthe kellolds Fiu.33 Dermabtis in a houaew~fe ~~~ ~~due to sensitisation ~ ~~.~ to.~ cleanina - - - rnseriaa, especially b eacn The ~nvolvmentof the forearms raQses the susplclon of a superimpmad allergy In n ohher QloveS. Eczema is an inflammationof the skin eharacterised by a poiymorphouseruption of erythema, vesicles, crusfs and &ales; usually Gsociated &th itcbing. Contrary to the traditional view black skin is sensitised as easilv as white. In Africa, 30% of dermatologicalcansulta60ns are for emema. Types of ewema include contact dermatitis, (irritant or allergic], infmve dermatitis, atopiceczema and ~bborfhoeicdannatitis.AU these types tend to become infected in the tropics, and chronic cases may become Lichenified. Topical treatment should avoidgreasy ointments in a hot climate but may include lotions, baths ot sprays M h copper sulphate, potassium permangmate and aniline dyes such as gentian VioIet andmethyleneblue. TopicaI &IC inorigin. A mixed picture is mmmon inocctipatilonal steroidsare useful in moderation and sysPemic steroids dermstil~s. are not required. Oral antibiotics are often needed especially in children. 22 Flg.37 Ths acute. cedematous erys~pelas-l~ke eczema of the face 1s dueto allergyto hair dye cantatnmg paiaphenylene dmine. Despitethe dramahc svmdoms u' [Ilc fcet aliu inc r symmetry suggests h snoc a erg, 11ins case o-e 13 o as! c srlocs, urn cn was corftrmea oy r,ai~ti:esr nq Tqe sharp dernarcatlon of the lesions w~tha cellarette 1s a stgnbf secondary ~nfection Fig. 38 This eczemao?theface and upper chest is due to anallergy to the antfingal agent Mywsalyl It 16 resoking wdh des- quamation I Flg. 36 Th~suesbulareczemaaflheneskI9dweto&I rgy tothe d patisnt'snecklme Theellergen is nickel, usedto ma cheap iwellery 24 Flg.90 This subacute eczenla mainlvmthe fO0ei. I headihom @ si& resolutionwith desauamation%t?d I p~g&ntarmn A halr lollon - - - was the pro Ible cause. Fig. 42 lnfative Fig.44 This eczema o i M ~nfect~ve eczema of nipple, the whole leg, 91ll characterlsed by called infective vesicopustules a d eczematad excoriabons It dermatltls, 1s chara- I arises, as in Europe, after scab~esand in women with Inverted cterlsed by large vesicopustules, scale-crusts and nipples ulcers Such eczemas are common among farm-workers and herdsmen and seem due to contact wlth earih and plants Fig. 43 Infected eczemaof the ankle wnh pustules and erostons, its edge is clearly demarcated by a cellarette. Such eczemas are en couraged by clrcula tory disorders and often provoked by insect-bltes tile atopic ecremq, common in well-nourished ba mithecheeks andsprsads to thellmbs. In somecases itmay ma transient igA deficlenoyandclears by 18rnanthsin many 29 I FN.48 Etythro- dermlc exacerbabon of atop~ceczema dueto infection Secondary lnfectlon is common in this type I Fig. 46 Llchenifled eczemaof the ante cubttal and popitteal fossae This appearance is palhognomon~cofatoplc eczemain older children and wen adults Fig. 47 Atoplc Fig. 49 Sebor- eczema in a little g ~ r i rhoeic eczema note Pigmented erythe the hypopigmen- matous and vesl- tatlon. The fine cular les~onson the scallng and normal forearms and right Sensation drstin th~ghNote the gush it from macular involvementof the lesions of leprosy antecubital fossae d 5. 5 0 Piiriasis alba in a little boy. This is one d the eczemagroup: it is often associated wift an upper res- piratory infection or threadworms.This t w must be distin- guished from leprosy. Flg.51 Lichen simplexchronicus of theiimbs: it is characterised by a central area of Psoriasis is one of the most prevalent dermatological atrophic depig- conditions in the world but its cause is unknown. The mented scarring physical signs are scaly erythematous plaques, similar in surrounded by a black and white skin. Parakeratosis, the typical lichenifiedzone with histological feature of psoriasis, is associated with thickening d the epidermis, some- hyper$gmentation. 1%clinical diagnosis is wnfiyed times with some hv scratchine the lesion liehtlv. which reveals the three lichenoid papules at cfassical sta&s describedYby B ~ O C ~first : a waxy surface, the edge. This very then loose flaky scales, then erythema with fine blegding itchy condition may follow an eczema or points. Thirty years ago, psoriasis was unusual in Africa and the tropics generally; more recently, it has appeared prurigo. Similar lesionsare seen in onchocerciasis. - -- in the laree towns. This sueeests that usorasis could be a disease of civilisation. At present psoriasis is more widespread and is even seen in the bush. Psoriasis is generally a harmless disease. Its only complication is arthropathy. Treatment is largely based on ultra-violet light. In Africa, the sun makes ultra-violet lamps unnecessary, and psoralens are dangerous and best avoided. It is sufficient to de-scale the lesions with 5 % salicylic acid ointment to allow the rays to penetrate. Dithranol therapy is very effective and inexpensive, but is not appropriate for most outpatients because of its ocular toxicity. 33 Fig.= Exterrswg Fia. 54 Discoid and psoriasis of the olass1cA type The l&ons afemainly on the extensor aspects of the limbs;a6 well prognostic signi 8s large p~wue*, ficance, on the other there are some gtrt hand, the d~fferenhal tate lesiinsmthe d~agnos~s ~ncludes chest. tlnea, lupus ery thernatosus and leprosy, wh~ch would be assoc~ated with sensory changes. typ~caland may be the anEysign of m r i & a m rmld cases PFOna6ls oan Iccur at a ~ age. y I m.67 m a 6 i s -..65 Psonasisof the scalp. This ste 1 inversa psaiasr, mmt-d of togetherwlthnail inwivemeinia typioal of psariesls. snaving the head sllowstreatment~ I 'I affectingthe &ensor asgectsof the limb, mvolvesthe fiexures and the palms and Soles. In sunlight. thisyoung woman. ~SOWIS was mis- takenalfimfora hrwl 1nfecDonand was very reS)stBntto treatment wah sariliffi acid gntrnent. Flp.56 PSOriasCSOf the eni is. Gen~tal I lesionsare notun- wrnrnon in both sexes. There may be afunctianaldistur- bance. snd tungal andsex~lly tram m W infectlorn should becmd- deredin the differen 1 tial diagnosis. F$.W pc+r,na&srthmpathica. ArIhmpalhy due to psoriasis is rare in it can be serious and needs to be d l s t ~ n g ufrom l ~ iM chronrcfonns of pdyarthritis. It can bevery dlsabllng, and now&- ment is m i ~ effecbve y %y~ U - Y Syd Caussade.. Lichen Pianus m.99Uohen ~anwsatthebilok. me% snail shiny papule5m b r * Ihese&remin @ a u a b u t they antbFa2Rfalner manvid8ae6LES. iI Tbisdermafosisisvs:rgmmmoninBfacks.Itismen boUli~tdaaaadco~andataUa$cs,.e~enb childhoodi whereas it i s m u a l i n maDpean6Wm. jasiiy seen. Lichen @musis &srackwkd by shiny flat-top@ itehp paptlles, wm a n y k on the M y batespeddy on theUmbs:it mayaffectthe mucous membranes&a@ mouth a d geni-m, but less kequently in ~laclc-than Whites.The histology is characteristic, with h ~ & aand~ottbkmtw4s s wm a densa I iymphvytk?in@i@aimhattachin tl3e basal lay@. ~ i o b e n p l a m ?6ften~oridin ~ ~ 1 & , with cxlggei;stlon of €hephygieal si@s,so that w ~ w s s%riaesre teadilp vidble and there is &tendencyr9 ~ & e # t m i da w l h k m W t a Q B.Thr w-ogy of lichenp1wusis unkno~vaSidlar eandftions sewen m a @on to gold and m s i c eftex bn6-msutow transplants, which suggest an immunological mechanism. Stres CPI)easilv be blamed. This relativelv' 1 I b e n i g o ~ m ~ n & t o k ~ ~ w l y. ~ ~ ~ t i l s ~ ~ r i a t ~ ~ s t e r o ,i ~ ~ i p~eparatioBand mitdsedatives. ! I 38 39 m.81 Annular 19.63 Corn~fred Iiobnplanusina ~nnularlichen young woman: tb~s danus This hyper- acwnrnon form, keratot~cform IS Notethe centfal pecul~artothe Mack pl~mentafionand the cDnspicuars \hr~clham'sstriae Fig. 64 The Kobner 1henomenon.Thi61s.ornmon In actwe a e s ofpsortasrs tnd lichen planus dinor superflcral ljurtes produce- zslons In lichen ,lanus m 6 :aucasians, these lppear as fine linear =Ions, but gross m d s are seen in he blackskin.This !xaggerahon isone ~fthe special eatures of black I%-62 Psbr~asikrml i c b n planua m a60 yw+ld m,Scratehin~ t h a l e ~ ~ n s a histobgy nd confirm tnedagnvsts, Zlibugh lrchen p h u s may be difficult to drstlngui* frompsartaeis 40 Fig. 65 General- ised lichen planus in an 8 year-old girl. Lichen planus in children is fairly common in Africa. but unusual in Europe.The pmg- nosis remains good. Fig. 67 Llcncr pCLlluS :If!I(! Lrdl m.cosa O r tnc I macular pigment- Fig. 66 Linear [ ation anteriorly. On lichen planus in an the Inner aspect of adult The lesions the buccal mucosa began at the heel m the second patlent and followed the there IS a lacv whlte cu-rsc of :rle sclat c network anda~so IICl\C J2 W U l C UJ: rnacular plgrnent- tot* S ~ c furms n are atlon, demonstrat~ng corrmm n At'ca I the pigmenting ten- and must bedlf- , dency of these, ferentiatedfrom the separate condition ,....: , I cases of lichen pianus of iichen st0atus. %. ~ , FgW 6 8 n g 1 atrophic depigment- ins lickn planus on the penis. The small papulesof lichen DlanUsonthe backs of the hands confirm Uledlagnosls. Thls sencus form of lichen planus inter- feres with funct~on and malignant chanae can occur Rg-89 tichen plaIlU6Ofthe SGCilR ina young woman. It scars mthls%te and resultsina p~rma- nentwatring alopecia.The mesence of nurnwrousfypicrrl ,"tes0fli planus 6n the dorsum Of the hand snd wristoonrirmme diagnosis. ' Thi$outdated term covers a nrUnber of wnditiom which are now thought to be ayto-immune. 1t is l~gjcal to goup them togetherin this chapter*aa thereissome overlap between the diseases. The main collagen diseases are lupus ervthematosw, -. F@SO Uchen i3ematomyositi~andthe sclero&&as. - -~- planus pigrnentosus Lupus erythematurrrs. ?he W i s e d form is common in ina youngm a n. Blacks south of the Sahara. The lesions are nrovoked bv P~pnwionfo~~ow+ mg lichen planus 1s sunlight and are commonest on the e ~ p m d p a a s , seen inWhke especially the face but also the scalp ahd lowerlip. The [email protected] iA more only importance of this disease is hmm a cosmetic point pronouncadand ofview: it leaves disfguringscaxs, evith permanenfloss persistentin Blacks of hair on the d o. Its immunoloeical aetiolorrvis ltappeamaethe pmCess resolves supported by the &ding ofimrnu&globbulin d&osits at andlastsformany the basement membrane zone in involved skis, The months, permittinga usual treatment is with antimalarials, such as (etrospFxilve chloroquine, which are effective but do not prevent diagnosis. relapse.. Systadc lupus erythemataws is rarer, hut one doessee somevery severe casesas in Whites. The i m m u n o l o ~ abnormalities include circulatingautodbodies, especially DNA, and immunoglobnlin deposits at the basement membrane zone in both involved and uninvolvedskin. Apart from antimalaxkls, it can be I bution in a young treated with steroids and immnno-suppressives. ' w m ~ Thisisa n more chronic case: the border is Still Dermatornyosith, as its name implies, affects the skin well-defined, but the and muscles. The prognosis depends on the myositis centre IS no longer which mainly affects the proximal muscles and can affect homogeneous. the myocardium and oesophagus in severe cases. As in There are deQig- Whites, there is a subacute oedematousform and a mented, scarred chronicpoikilodermatousform, but there are also areas. Notethe 1 small plaque on the special features in Blacks: scaly erythemas in the forehead. subacute',oedematousform and irregular pigmented plaques in the poikilodermatous form. The usual treatment is with steroids. The prognosis is fairly good, except when there is an underlying malignancy. Selerodermasare characterisedby an increase of dermal collagen with inflammation. All forms are seen in Blacks. Among the systemic sclerodermas, as well as the classicalprogressive systemic sclerosiswith sclerodactyly, thereis also a special form in Africans with sclerodermat~myositis.It begins with scleroderma of the cape area which becomes generalised and is associated with muscle involvement. Its prognosis is perhaps not so bad as that of progressive systemic sclerosis. We have obtained some improvement with iodide therapy. The physical signs of scleroderma in the black skin are very characteristic, with speckled black pigment on a depigmented background. Rg.71 Discoid 1 iupuserythematosus in the class~cal 'butterfly' or 'bat- wing pattern. Nots me well-defined, actlve edge, the I pigmentede w e matous centre and the conspicuous adherent scale There are also oenoiai lmions wlth Flg. 73 D~sco~d lupds erylhematosus around the muulh n a woman Dn y the nark sca y postenor ooraer.s ac1.ve. Tne rest 1s oeplg- nented an0 scarreo These lessonsare pnrqanent 'I ug.74 Lupus erythematosus turn~dusThis form of dlscoid lupus 16 characterwedby the oedema of the leslons, wh~chcan Present ddficulties n dlagnos~s and even slrnulate a retl- 19.76 Discoid rpus erythematosus Ia man, which is nusual as the iseaseis much ornrnoner vl romen. There fs ermanent scarring nd depigmcntation: IS disfiaurerrent is fie w a t >f the Fig. 76 -.nLs eylnema:dsus of me the! I p Tn s sre s commonly.nvoveo 'n tnls dsease. 11can be Iso.ateo an0 nems tc be aist,ngoisned from anlnlc chelit:s, seen in the second pddre. -77 Discoid '10.79 Systemic lupus e ~ m a t o s u s 1 ~e ~ m s of the scalp in a latosus. In the first young woman Note ase, the cuaneous the large plaqueof :%ionsare relatively alo~ez~ wlth a two 'krete on me nose zories: the active nd scalo. There erythematous pew ,as a systenrc ah- phery and spread- css w ~ l hrcnal In- ing central po~ntwnh ulvemenr and s complete dep~g- os.1Ive LL ce I test mentation. Such ha~r 1the second case. I loss is permanent otethe br~lliant and enters the eryrnema w m In- differential dlagnos~s ofscarrlngalopeca, wlth peudepdade, I vdvernrnl of :tie lops and everlthe evelrds s indermato-. llchen planus and lyosltts Th~scase favus. Note mci- ad renal involve- dentally the small lent and an assoc- wsmetic scar& ited iymphwna. cations d t h e helix of ear Fig. 78 Disseminated lupus elythematosus ma 20 year-old girl. There aremultiple disseminatedsmall discoid lesions, a wmrnon Ranern in Blacks.This is a case of svstemic ,~ luous with visceral ~ involvement and apos tive LE cell lest, the prognosis should Lo guardeo despite treatment. I Dena- tomyositis ha young m. A mixture of me'buaetfiy patternem of lupus erythe- matosus and the periorb'tai pettern of dmatomyositis Note sl.gnt atrophy :.,. , ! of the right deltoid r.. A. 5.82 D e w tompait~s,po~kllo d ~ o u form s in a little girl. The erup tim oonsisb of ir- regular pigmented macules wh~chin someareas, , especially the abdomen, waiesce to forma retloular O pattern. Theohlid died from resptratory ~nvolvement. Fig. 80 Demtomyosihs, oedematousform in ayoUng w a Note thenedema ofthefakewith awemaand scaling, m d lupus-Uke lesionswl the chest. On the arms and forearms the erythema is coveredin t h ~ o k Wch e ~ Wwld bevery uWsW in aWhite patienl.There are ~lsosmaliiasionsonthe b& of the hands and fingers. Fia. 83 Scleroderm. showma ttiesoecial ohvsoal , , slons ~ ~ In black - ~. skTn Tnn nKlr s III~II~~ICUas.ibVhd& b ~ tssdrtace t isnypo mgrnented and speckled wlln 0 a& dots slanos of rne~anir I lhe'oll cles. [~erswtirlg R g W Sclero- derma wrth cedema oftheface in a young girl Th~s1s usuaily the start of sderodermato- myosIt@,which 1s common in Afrfnca It can affectthe Young F&. 65 Sctwodemaln a 'cape' dlstributlon, on the shoulders, chest and upper back This is usuallythe second phase of sclero- dermatomyosrt~sand 1s follow6d by generaltsed ~nvoivemsntspread- Ing downwards. Fig.= Acw Bnlloun Dermatoses sciefosis. SystWc scieroais occurs in BlackwmdWhites. mereare emasof complete lossof I pigmenton the 1 extremities. even thekcalp.As -..disease tna -.. evolves. me black spedding 1 fades, and is replaced by total."*",".-.,-,... 1 Such cases have This term includes many different eondirions with rn@ma1imo'vement. 1 nothing in common but their primary lesion, the bulla. This may be defined as a localised fluid-filled blister, I larger than a veside. We distinguish: 1.Bdoasimpetigo. Lmpetigo isusually pustular but may be bullow; this formis sometimes called tropical impetigo. The aetiology is bacterial, either streptococcal I I or staphylomccal. Treatment is with local antiseptics, AIEBB %RSr combinedwith oral anribiotin inthe streptococcal forms variant of sclero- dermain a ywng to avoid renal eampli'cations. woman. This type 2. Bollow drug eruptions. The most usual type is the hascalnnosi5Gufls and aless rapid I fireddrug eruption which is only bullous in the acute pmgresion The attacks. The most ~ w f o m ithetoxic s epidermal skin signs are the same wim black wecldhg, < necrolysis of L el!: this produces massive bullow skin loss and affects he mucous membrane8 and the eyes. Any drug can produce a drug eruption but in our experience long-acting sulphonamidssand drug I combinations are the commonest. The ammosis o f LyeII'ssyndrome is serious and the conditi6n is often fatal; treatment requires resuscitation under sterile conditions, which are seldom available in the tropics. 3. Erythema multifom is characterised by target lesions 6. EpidermoIy& bullosa is a group of genetic conditions and often by involvement of the mucbus membranes. present from infancy. The dystropkc forms have a poor There are various causes including drug reactions and prognosis. virusinfections. The Stevens Johnson mdrome is c l ~ l ~ e l a t eThese but tend to relapse. a d. conditions have good prognosis 4. The Pemphigus Group. The comnion feature of these conditions is t i e presence of large flaccid blisters arising on clinicallv normal skin. The histoloev shows - -- intraepide& blisterswith amntholf&s, and the immunofluorescence reveals antibodies to the intercellular substance ofthe epidermis. AU types of pemphigus, vulgaris, vegcians and foliaceus, &e seen in Blacks: the commonest in Aliica is a form of suoerficial pemph&us resembling ~razilianpemphigus. Tdis can o a r in young people and even children. At different stages, it can resemble the Senear-Usher syndrome or pemphigus foliaceus, and thereis also a vermcous form, where small blisters are concealed by a warty hyperkeratosis. The pemphigus group are serious diseases, and may need large doses of steroids (e.g. 2 mgiKg), as well as immuno-suppresives. The superficial form has a better prognosis, as a remission induced by steroids may be maintained with sulphones. 5. Bullous pemphigoid and related disorders. This group is characterised clinically by tense blisters, associated with a rash resembling urticaria or eczema, and histologically by sub-epidermal blisters. This group is now divided into several disease entities including bullous pemphigoid and dermatitis herpetiformis. Bullourpemphigoid is manifested by large tense blisters usually with other skin lesions. The blister is sub-epidermal and immunofluorescence reveals antibodies to the basement membrane zone. This is a serious disease affecting the elderly. However, some cases resolve completely after 2 years of treatment with steroids and immuno-suppressives. Dermatitis herpetifomlr is characterised clinically by small blisters or vesicles with a rash resembling eczema or urticaria. D i c t immunofluorescence reveals IgA deposits at the basement membrane. A gluten enteropathy is associated in many cases. Fy1.89 Bullous Impellgo. These arc plirulcnl blisters cuntan~ng Dermatitis herpetiformis is seen at d ages; it is pyngenlc mccl, StleptococCl or staphyfowccl The blisters collapse lo leave crusts. The trealment cnns~stsof clean ng ofl tne crush and subject to exacerbation and responds to sulphones. applying antibacterials. Fig. W Fueddrug 1 F@SZ TMc eruption of the face. epldemralnecroiysis Repeatedepisodes (Lyell's syndrome). I occur with bilstering, in the first case. note associated w~th fhe presence of taklng thedrug res- 1 blistersw m n e c w ~ s ponsible Many of theepidermis only drugs can produce in the pre-ifwrack this reactton, butthe region. in the commonest InAfr~ca second case eoi- aresulphonamides dermal necrosis is and mild analgesics almsltotal, giving such as the appearan- of a noramidopyrine. scalded patient. Note also them- volvement ofthe mouth and eyes. I fhe f~rstcase, the drug responsible was a tongacting sulphonamide ('Fanasd'), lnthe second case it was thiacetazone, an antituberculous went uxnrnoniy used in Haih FIB. si General- lsed bliienng toxic erythema The pig- mentation 1sin favour of a drug reaction m e necrows of ths roofs of the bullae suggests a rebtion to Lyell's syndrome; theduo reswnsibie was a l&g-ic!ing suiphonarnide. 6 Erythema -. 5. M Mnmn hultiformewffh Faempmgus: this ypical tamet

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