Scribd Logo
Upload

Welcome to Scribd!

  • Lupus Eritematos

    Uploaded by

    Florin Bujoreanu
    13 views18 pages
    LES

    Copyright

    © © All Rights Reserved

    Available Formats

    PDF or read online from Scribd

    Did you find this document useful?

    Is this content inappropriate?

    Report this Document
    LES

    Copyright:

    © All Rights Reserved
    Download as PDF or read online from Scribd
    Flag for inappropriate content
    13 views18 pages

    Lupus Eritematos

    Uploaded by

    Florin Bujoreanu
    LES

    Copyright:

    © All Rights Reserved
    Download as PDF or read online from Scribd
    Flag for inappropriate content
    of 18
    SECTION 7 RHEUMATOLOGIC DERMATOLOGY 1 Lupus Erythematosus Lela A. Lee and Victoria P. Werth Synonymsivariants: «Disco lupus erythematosus: subset of ‘chronic cutaneous lupus erythematosus = LupUs profundus a variant ‘of lupus panniculs = Lipus erythematosus tumidus:tumid lupus Key features 1 There re several variants of utneous ups defied in pat by {helbcaon and depth of te nflarmetory inflate {8 Acute craneouslpes involves primarly the epidermis and upper Germs ans usualy asoiates wth acve systemic dense 1 Subacite cutaneous ypu involves prima the epidermis nd “pper demi and most patent have photoseniiy ard Smt SA autoontbodies during the course of tha dese the ‘maory do nt have slgrfeat systemic movement ena CS ‘rcaopalmenay) Disco esos of lupus into the epidermis, upper and lower ‘demi, and edna stuctrs and they ean sar the mojnty oF fens donot have cay inca systemic disease 1 Lupus enthematoss tunis ils the dermis but thr no ‘prominent edema volvement and esors donot scar ‘Lupus panniculitis iveves the subcutaneous ise and may renin dafguringSepresed sas due a poatopy INTRODUCTION {Tupus erythematosus is a multisystem disorder that often affects the sin, Cutaneous lesions can be 2 source of disability and, on many ‘eeasions, an indicator of internal disease HISTORY | The term ha = A ATT ne © we E= roca Bo x e- Pesmacie A / + ve osmosis Ade cnotines Aka ore ‘A. cremations Fig. 41.1 Pattegeness of lupus erythematosus. n photosensitive cutaneous ula radiation tigges coke and chemokine production nat mat ‘reins NElar# of neuropil nd apoptoss ofc fading toeeare of DNA. Abehenoi save reaction 1 the endpoint of compl cascate that UE, Setvation of dendrite cel elase of nereron GF, production of chemokines and action of T als CTL, yotoncT mphacyte CXL chemokine ‘oti hgonds GAGs, Syeotaminogveans GBPT, Guarfatc binding pret: iE graye B FOL high moby group box I verelg MT lS inva natal tile cls L37, athens MOC, yells dena cl: MNP matt mtalprsenases, POC plasmecytd deni call RNP : ‘bonaleopote Th, T elper cel TL, Tl ke receptor Tr, tumor necro factor, TRAICAY, mor neces Tar feated apoptoindcing gana ee ‘seed ah Phy so eons Th 72 yest plasmacytold dendritic cells, which are potent producers nef, hus forming an amplification loop™. Increased numbers Netestid (CD1234 and myeloid dendrite calls have boen found mrineous LE lesions", Epidermal and dermal dendrite ells re Heratinocytederivod antigens and prime CD" T ells to Suigens within the skin-draining lmmph nodes. Comborating forthe role of IFN ais provide by the induction afevtancas dees cats receiving IFN-@ for other medical conditions, The LEEW signature seen in SLEhas als been detested in peripheral eMononulea cells fom patents with DLE and SCLE, but not ith LE comida? tse aN. opteaulated chemokines can recruit CXCR3-positive CD4" Weg" Tools to the skin, aswell as immature plasmacytoid den- Cis coneributng othe characteristic Interface inflate of cua LEH, In addition, via the production of IFN-o, plasmacytoid Bes els drive the activation and expansion of T calls. Thete 1 Beidence forthe presence of granzyme B and TIAL (poly/A}-binding bor two cytotaxlegranule-associated proteins involved in apopto: he skit ofall subsets of cutaneous LE, although somewhat less ISCLE,stggesting that there are fewer C8" T eels in SCLES. In ors with divominated, scaring DLE, high numbers of czculting Hef cyte T cells were detected, One study found decreased ‘of Foxps"CD4°CD25" T regulatory cals inthe skin, but not feel subsets of eutancous LE show in ig 41.1. In the proposed pa, a response to UVR tigers cytokine, chemokine, and antima bil peptide production by keratinocytes as well as endothelial cell Hsio, there inating the immune response. In the context of picand cavironmental isk factors, a complex cascade ensues thet es activation of dendritic cells, sclease of FN, activation of T snd pation of chemokines apostieetbckloop ukmatly in a lichenold tissue reaction. ification ost commonly used classification of cutaneous lesions in LE Is De, James Gillam. He segregated skin lesions into those that pete and thove that are not specie based upon whether the histopathology demonstated an interface dermatitis or not, respec- tively: Within the category of specific cutaneous lesions, he subdivided these into acute cutaneous LE, subseute cutaneous LE, and chronic futancous LE (Fig, 41.2). This choice of terms was based upon the fbservation that thre distince pes of cutaneous LE are commonly Seen: the often wansient lesions of soute cutaneous LE (ACLE) typified by malar erythema, the frequent long-lived, intensely inflammatory iscoid LE (DLE} lesions which can lea to permanent disfiguring scars, land a photosensitive eruption characteristically more longlasting than ‘ACLE but without the potential fr atrophy ot scarring, fr which the term subscute cutaneous LE [SCLE} was coined. Typically groupe ‘within the chronic cutaneous LE category ae che less common disor- Gers LE tumids, lupus panniculitis, and chilblain lupus (se Fig. 41.2), esite the observation that characteristic lesions frequenty lack an interface dermatitis, especially LE cums Formal studies have not been dane comparing duration of disease activity forthe various types of cutaneous LE, and there isa consider able range in the duration of activity within each subtype. Patents with SCLE lesions may have a chronic relapsing course, while DLE or LE tumidus lesions may havea relavely short duration due co resolution ‘oreffective disease contol Infact, some authorities have proposed that EE tami should be temoved from the chronic eatery and be given its own category, intermittent cutaneous LES. These: controversies ‘sie, Gilliam’ lasifiation schema has proven uli inthe ovganig tion of the various types of cutaneous LE and it isthe basis for the tlassilcation herein, accompanied by a focus on the morphology and histopathology of lesions [Fi 41.3) The three major forms of cutane- ‘us LE will be discussed Art, followed by the remaining entities eut lined in ig 41.2 Discoid lupus erythematosus Discoid lesions represent one ofthe most common skin manifestations of lupus and they are most frequently found on the face, scalp and eas (Fig. 41.4), but may be present in a more widespread distribution (Fig, 413), Te unusual for discoid lesions to be present beloe the neck ‘without lesions also being present above the neck. Occasionally, discoid lesions develop on mucosal surfaces, including the lips, nasal mucoss, conjunctive, and genial mucosa Some patients with discoid lesions exhibit a photedistibution, and sun expose appears 0 have 4 role in lesional development loweves, many patients have discoid lesions on sun-proteted skin, fand there is no clear association between sun exposure and their evelopment. Fig. 41.2 Classification of ‘taneous lupus erythematosus (UE sed upon the dsieation ‘stam riginaly proposed by iam and Sonthemer —oo a vy ye ‘esaepameevere heroes rand “ae 418) Wager sttronc tm [Ee ast taneriktareytc dance Ents "edu panos, rg indices SCLE shout be consis, TE arRSUE] [Svante xmpaua i] [ Cron oneoue ‘sete ‘eater can = 1 ae i Vv tar erthema Anew Diecole pus (OLE)| | Balous asians SLE! Pretadenboa | | Peoacegaons] | toatnt Reverie [Conbevideraa| | Nooo | reer iS agno reqs encopathlogi comet, Fig, 41. Predominant actions of iflarmatory infirates subset of cutaneous lupus erythematosus. The types of cutaneous pus entherstows ae ‘cite taneous lupus eythematosus ACLE, subacute cutaneous bps evthematoss (SLE), scold kop ertheratosus OLE, ups eythematosi {LET and lupus pannicolis (LEP the ater hee ae forms of evn cutaneous lupus erythematosus (se Fg #12 The pimary locations of te rates {ols superclass ACLE apd SCLE super pur deep dermis and peiadnxal DLE: superlal and deep ders ET and subc.tanecu tL Tt ‘chron cutaneous LE [ie emae [Blues paneate Hi cnoain ups Fig. 41.4 Character sites of volvement forthe tree major forms of cutaneous pus erythematosus (LE. Disco lesions have the potential for scaring, and, over time, a substantial proportion of patients develop disfiguring scarcing. Active lesions are intensely inflammatory, with a pronounced superficial and dep dermal inflammatory infiltrate As result, on palpation, active lesions epically fel thicker and firmer than sutrounding uninvolved skin, The adnexa are prominently involved, ith follicular plugging and Scaring alopecia commonly observed, Dyspigmentation is 2 common ‘esquela noted in longstanding lesions, typically with hypopigmentation in the central ares and hyperpgmentaton atthe periphery [Fg 41.6), but sometimes with viligo-like depigmentation, Rarely, squamous cell carcinoma develops in «longstanding discod lesion Patients who present with discord lesions may have associated anthalgia, but, ver ime, only ~10-20% of theae patents eventually meet the classification criteria for SLE see below), Many ofthese patients meet criteria based primarily upon mucocutaneous disease rather than serious internal disease, and the majority of those who progress do so within five years. The risk may be higher inpatients ‘with widespread (dleseminated) discoid lesions’, ‘An unusual variant of DLE is hypertrophic DLE, characterized Bf ‘hick scaling overlying che discoid lesion or occurring atthe pes othe dscoid lesion, The intensely hyperkeratotic lesions ase ft prominent on th extensor ams see Pg 45H, but the face and UPR {unk may lao be involved, Frequent there are eypical discoid esta ‘resent in thes locations. ‘Subacute cutaneous lupus erythematosus Patients with SCLE typically note photosensitivity. Tei esions most frequently occur on sun-exposed skin. Tt is notable thatthe mila shan i sully spaved, while che sides of the face, upper tink ‘extensor aspeets Of the upper extremities are commonly involved {A17, ace Fig 4.4)". n Some patients, che disease may be mild, Wi ‘only few small scaly patches appearing after sun exposure, ‘esions of SCLE may have an annular configuration, with 1 pinkored borders and central clearing (Fig, 41,8), o7 a papas presentation with # chronic psoiasorm or eczematous p= ECLE lesions characteisealy have a relatively spare, =0pet6al Pmatoryinsltrate, and, consequently, they are minimally palpa lone often resule in dyepigmentation, patculalyhypoplkmen: PGE depigmentation, but develop neither searing aor deena a ‘of patients with SCLE, the disease is linked to meen 2] The frse drug associated with the develepment of #9 hydrachloothiazide, but at least 100 agents have been Fig. 41.5 Various presentations of sce lesions of upus erytematos ‘Rise Lesions vic tava te head and Inadaion fo hyperpgmentato ‘eal € The ea Tes can be arto or erative In tenen planus The patients it ypetonh reat hyperkera reported subsoquently to induce or exacerbate SCLES. A population bused study from Sweden identified in decreasing onde) terbinaf "TNF inhibitors, antkepleptics, and proton pump inhibitors as the ost frequently associated medications", The cutaneous lesions may fr may not clear once the medication is discontinued ‘proximately one-third to onehall of patients vith SCLE full 4 of ore classification criteria for SLES. An oceasional Bs pete stan pus erythematosus (CAB The Lupus frythematosus, reste fered 035 “bute as may be variable (A, edema I eI The prevence oral exons can odin the livelier diagnos SicATIONS ASSOCIATED WITH DRUGANOUCED SUBACUTE (CUTANEOUS LUPUS ERYTHEMATOSUS. onrsus SCLEN ACE angiotensin conervng enya. 3, including nepia st wich Sige syne Jono sigiican sucena de SA sutoanidies are ast SLE, i is pot surpeising thie some patients ave festures nos, and some say have eros etal manifesto vulrome wich ss punonary ot neueolie disease jure of SCL, fom the stonulpint oF unersta asin ofp, i Tul 5 Bunn Bs ‘Ch. 0), Although nvestaby thi opin revalense of and-S8A/Ro sunnah die in SCLE ulcintal waionity af patents with dis enn |-70 Series, Teporead age of 60-1004) have an ARO te cutaneous lupus erythematosus (ACLE) I isons of ACLE are exemplified by the development of bilateral lt entiewna (buttedlyeash", Fig, 41.9). These lesions tend to be het, follow sun exponure, and resolve without scaring (but some with dyspigmentaion|, An association with anti-

    You might also like