Acute Generalized Pustular Psoriasis presenting with Erythroderma Associated with Shock and Acute Renal Failure Abstract lear/n Takes/al MD lCUflhl )‘inianiio unPin’ All) Takes/n MD akin Health S stems. Family Practice. Residency Prooram, HO0ILOJ H. Asscsate CiLOcai Professor, University of Hawaii. John A. Earns Sciool of tfedicine c’rr H n niF’ but c.iangercvis fArm of osoriasis wiTh a systemic orm sentation. Acute exacaarbatien, an early picture of ac.,fe e”c”alrreu csusrj’are.sc’aass lAGER.). ca he hera rhore’cre’. ea’h.’tc-s.’oarrira’uarra.svstemic the rape s craca; I; s ar. cayocr:anr wfte’rner/a/ raagncs.v at erythroderma. Epidemiology, etiology, diagnosis, and treatment options are discussed in fhi.s paper. introduction Psoriasis is a common skin disease and may mani test with various clinical pictures. Acute generalized pus tular psoriasis ( AGPP Is recognized as an uncommon form of psoriasis that can he tatal if not treated in a timely manner, Initial clinical presentations may mimic other diseases that presents with erythroderma. Somm times its clinical picture resembles those of acute generalized exanthematous pustulosis AGEPi. We experienced a patient with a histor\ of p50051515 that initially’ presented with ery’throderma, fever and hy potension. The patient subsequently developed gener alized pustulosis and desquamation. which lead us to the diagnosis. Although ,AGPP may not be common, primary care phasic ians must he aware of this disease as one of the differential diagnoses of er throderma. Case Presentation A previously healthy’ 67yeawold man presented with prof u nd tatinue and generalized i tchi ii ess m his hack. The patient snake Lip ns ith acute onset of itchi ness and redness an his hack. Suhscqucnll\. he den el oped chills, tactile Layer, diarrhea. lighLOheadedrtess, tighiness of chest, and proknmLl weakness. On esarni nation in the office, the patient had Uencralitcd eryth ianificant orthostatic hvpotenvion and 5 roslernia and , wa.s i.mmediateiy admitted to the. h.ospital. Past ine.d.i cal historn included coronars bypass surcerv, appen dectomv. peptic ulcer disease, and psoriasis. lIe took icsoopathr hut 5 sS.\Il) otcrmitent3s or cain cat ra to food rnr mcdic.ations. rec.e.n.t de.nied any aile.r ingestion ot uncooked food, or recent foreign travel, On ‘xritolnti the patient sea’- is nt ‘denote dis ‘a mtar ss ith temperature JOeL’, ‘esp!J’atorv 2751 nun, blood pressure I 2216t HuinHa ni supine and 78i 48mmHg in standing position. and heartrate 72/mm in supine and 84 /min in standing position. There was generalized ervthema on his entire bod\ including head. trunk, and extremities without blisters. There were well demarcated scaly round plaques, approxu matelv 4x 2cm on right anterior chest and left upper hack. No Is inphadenopaths Sn as noted. Heart sounds were normal w ithout muritiurs. Lungs were clear hilatcralB Bowel sounds were hyperactis c, hut there was no tenderness on abdominal palpation. Occult blood was posuive. There nn crc several thickened dvstrophic toenails without pittins. Laboratory evaluation res ealed hemoglobin I 5.4g! dL, WBC count I 4,4x I 09/L with 2 1% hands. Cheim istr\ abnormalities included B UN 5$ mg/dL and crea— tinine 5.Hmg/dL. Urinalysis revealed punoint I +± specific gras it’s I .030. sediment 0-5 RBC and I 0 12 WBC/hpf: few to moderate hacteria/hpf, and 5 no 1(3 hval ne and granular east lIp1. Chest X-ra\ was unre . markable. The patient nn as treated sn ith rigorous fluid replace ment for presumed dehydration from recurrent diam rhea and len er. Renal unction rapidly improved fol loss ne rehvdratu n. Tosic Shock Syndrome TSS i at utykn( wn origin nvos suspected initially hecause at the suspicious skin lesions and the patient was started empiricails on cefazol in. On the 2nd hi rnpital Jar, numerous pustnles. 2 to 3 mm m diameter. erupted on hotli l’lank and thighs Figure I). These pustules pro gress ive.iy spread c.ove.nr.g 7fu80 hf of the. trunk and 5//P of c\tremities. The lesions became confluent i th ius’rnked di schars a’ and r ml itig of sLi hepiderma I pus The (iram stain at the ecudatc had rare anal the. culture did not show si.gnificant haLcte.riaI orgamsms. Blood and stool cultures were neganive. stcniie antlisiotic treatment. he cuntin Desptte the iCS er and watery diai rh’si. tfpoi spicing has e to ati.ent revealed h.istorv v.f an the. further interview, treated with methotr soriass of acute exacerhation to thi’- episode. vearpi’iei ‘ac pftrntlnsoar’ earate and iiUI,’io’r ‘eoiiis,’nlcrla t C’on’-ultLiii dernlatot.og\ for possible pusmlar psoriasis. Soon after the mediea— non started, the affected skin became dry and desqua— mated and systemic s’s mptoms resols ed. The punch biopsy of the lesion showed psoriasis. The patient was discharged on 5th hospital da in stable condition. Discussion Epidemiology Psoriisis is a common. chronic, recurrent. inliamma— tory skin disease and its pies alence is estimated to be approximately I .0% to 2,0% of people in the US,’ Its annual incidence ssas estimated to be 60.4/I (it (.00(1 154.3 bar men. 00.2 for women).- The incidence varies in ethnic origins: it is low in South American Andes (0%). American Samoa (0% :and high in Norway Denmark t2.9% and Faroe Island (2.5% I. Both sexes are equaIl predisposed and all age groups are af%cted. The average age of onset is usually in the 20 s. raeing from birth to 5th or 9th decade. \iorc females has e earlier onset before the age of 30 than males: Faroe Island series indicated a milder course in later onset (alter 25) group. Onset before age 10 is likely to have a more se’s crc course: although the onset of the disease is less common in the very ‘soung and elderly. 5 The incidence of acute generalized pus tular psoriasis 1 AGPP is generally much lower but a reliable data is not ax ailablc, Clinical manifestations There are many arie ties of lesions, and arious de scriptive terms have been applied to diverse appear ances of psoriasis. The classic form tpieallv presents xs ith well—defined ervthematous plaques with sharp borders and silvery gray scaling on the surface. The Koebner phenomenon. precipitated by the trauma, is a well—know n lesion. The lesions on hands and feet are generally less er\ thematous. hut well demarcated and have white scales. The lesion on scalp and in skin folds maw mimic sehorrheic dermatitis although they usu— ally lack typical silver scale. Guttate type is another form of psoriasis. which manifests as small crythema— tous papules with fine scale, and is frequently gener alized and occasionalls des elops into an explosive eruption of teardrop—shaped lesions prtmaril on trunk, As for pustular psoriasis, it can be divided into tsvo groups: [ion—acute l’orm and acute cc’ neralized pustu— lar psoriasis I von Zumbu’.c h: AGPP. Ihe ox o form’ have different mortality rates. The former includes generalized pustular psoriasis of pregnancy. circinate and annular pustular psorias is. usenilc and infantile pusutular psoriasis, and localized form, This entity carries better prognosis: Typical AGPP starts with er\ dieuia and subsequentl torm’ lakes of Pu’ periunguall and at the edges oF psoriatic plaques. Generalized erythema and more pustules usually fol sv. Pruritus and intense burning cause extreme dis comfort and the patient ina he sex erel ill because of concomitant constitutional symptoms such as fever, general malaise. arthiargia. and m algia. The pustules dry tip to farm yellow-brown crusts over a reddish brown, and shiny surface, In the absence of effective treatment, it can he fatal because of serious complica tions such as cardiac failure, respiratory fat lure, hv— poalbuminemia, hypocalcemia. acute renal tubular necrosis, and pulmonary embolism due to deep vein thrombosis. InfIammator po1arthritis is also com— monlv seen. Etiology and predisposing factors The alteration of keratinocvie differentiation such as epidermal h’s perprolitiration. altered maturation of skin cells, vascular changes and inflammation are associated with the paihoecnests of psonasis. But genetic and precipitating laciors are more compli cated than was previously suspected. 5 Psoriasis can he induced by many drugs such as beta bIocker. lithium, and anti malaria age mits among others. \Iorc recent — studies revealed terbinafine, calcium channel blockers. eaptopril. glyhuride. and the lipid-lowering drugs such as gcmfihro,m I ma\ also induce this condi tion. S stenlic steroids or short—term c’s closporin therapy is well known to cause rebound. 5 In acute GPP. precipitating factors include strongl irritating topical thei-ap prenanc\ sunlichi. hvpoc— . 279 . alcemia h)llo\s ing accidental parathyroidectomv and numberoidrues including salicvlates, iodine, lithium. phenvlhntatone. oxsphcnhutaione. tra,odone. terhinafine and penicillin as s\ elI as \s ithdrasval at eyclosporin treatment: Table 1 —Differential Diagnoses of Etythroderma Primary Cutaneous Disorders Atopic dermatitis Contact dermatitis Fencal dseases lchthvosifcrm dermatoses Lichen planus Mycosis fungoides Pemg-hous foliaceus Photosensitivity reacton Pityriasis rosea Pityriasis rubra pilaris Pscnasis Pyoderma with reaction Scabies Seborrheic dermatitis Stass dermatitis Systemic Diseases Coion carcinoma HIV infection Leukemia CTCL Lymphoma Multiple myeloma Reitef s syndrome Sezary syndrome Systemic lupus erythematosus Toxic epidermal necrolysis — Staphylococcal scaled skin syndrome Drugs Sultonamides and Sultones Penicillins Cephaiosporins Anticonvulsants NSAID s Codeine Heady metals NH Qardine CatcnriI maine Antimalanas Phencthaznes Metbotrexate t)iJjere,itial i)iagnosic The clinical manifestations of psoriasis are usually characteristic enough to establish the diagnosis and skin hiopy is not necessar Histopatholoic I indines or laboratory tests are not specific and ss ill not estah lish the diagnosis with certainty, Once it changes its hehas ior and become eruptis C. pusiular or er\thema tons. establ ishine the diagnosis becomes more dilli— cult. This case presented ss oh shock, diarrhea. ervthro derma svi h I aht raroiv data sug cesi i e of deteriorati tic retail function and handemia. This clinical septicemia picture directed us to four possible infectious process. Causes 01’ ers.throderma are shossii in Table 1 In our case, in! iall toxic shock s\ ndron te appeared to he most likely until he developed pustulosis on the 2nd hospital day. The dtffereniial diagnoses of pustulosis are listed in Table 2. In this case the hisior of psoriasis ma he adequate to make the diagnosis of AGPP. But acute generalited exanthematous pustulosis (AGEP) \s:liich is also known to have similar clinical picture. We from AGPP. may be dilticult to differentiate following the of because concluded this case as AGPP factors (i ) hislorr of psoriasis (ii 1 good response to acitretin treatment (iii) no known prccipimatinu factor’ such as medications to cause AGEP iv) AGEP usually present with more polymorphic lesions including pseudo—ervthema and multi form pruritic lesions v mdi associated edema. ) This case didn’t appear to has e a self-limiting course until the commencement of acitretin. AGEP should he spontaneously resols inc and could has e resolved more rapidl Although the histological findings obtained from the classic plaque was consistent with psoriasis. it might not reflect os erall clinical pictures. V e lortu— natelv obtained a timely consult from a dermatologist and the patient had a favorable outcome, A though development of A(PP from classical plaque—t pe poriasis ulgaris is rare. this case reminds us to he iss are of this lik thre iten1n condition js One of the differential diagnosi’. of ervthroderma. Treatment and outcome There arc ses eral modes of treatment for AGPP [loss ever. cho sine appropriate mcasure is challeng ing, lithe patient is not under immediate litd—threaten— ing condition, the initial management should he con sers ative. But once the pati’nt mnariIcsts s steinic symptoms. we should consider s\ ‘temtc treatment. The choices of systemic drugs for AGPP include methotrexate i MTX c closporinc. and retmnoid. . M2DiC6 JOURNAi,,, JO [. 62, E2CEME3iR202J. 280 Table 2W—Differential Diagnoses of Generalized Pustulosis Scute generalized pustuiar psoriasis AGPP Acute generalized exanthematous pustulosis AGEP> Acute pemphigus toliaceus Banal staphylooerma Bampant candidasis Pustular eruption due to iodine or bromide Gram negative or Other septicemia \lT X has been the inainsla\ ut treatment fur acute (PP since the late 50s. The niu$ dangerous potential side effect is acute invelosupression especially in the elderl\ and patients with renal impairment. Cvcluspurin is effective for evere plaque—type psortasis and i’ also pros ed to he eflective in ervthrudermic and generalized pust ular psoriasis. It is nephrotoxic. hut reversible aher drug with drawal. The combination with PUVA therapy is also reported to have a good response. Reti noids, the derivative of itamin A and etretinate used formerly. was replaced h acitretin. Its letratogenecit\ restricts the use for the patients in cli ldhearina age. Conclusion e epei ienced a case ss ith psortasis. ss hich des elopcd acute generaltied pustular psoriasis. Fscn its clinical piettnc of inititI ersthroderma is tspical for acute uc craliicd pustular psoriasis sse should consider caily consultation ot d rmatolu cist and obtaining tssuc dui tim act t phasc in otder to nitke a dcl mitts diagnosis u d ruling out acute genLraliled e’.anthematous pustulosi s. I arl teLoOniti( n and mntedtate ss stem ic treatment is crttial ni Ac PP. 1 Acknowledgements Aopreciaucn and thank to: Douglas Jotinson. MD who gave us suggestion for treatment and encouragement to wnte thi manuscript. References PS *1 281