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Sehgal Et Al. (2004) - Erythroderma :Exfoliative Dermatitis- A Synopsis

Erythroderma - Exfoliative dermatitis
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   © 2004 The International Society of DermatologyInternational Journal of Dermatology   2004, 43  , 39–47  39  BlackwellPublishingLtd.Oxford,UKIJDInternationalJournalofDermatology0011-9059BlackwellPublishingLtd,200345  Education  Erythroderma/exfoliativedermatitisSehgal,Srivastava,andSardana  Erythroderma/exfoliative dermatitis: a synopsis  Virendra N. Sehgal, MD  , Govind Srivastava, MD  , and Kabir Sardana, MD, MNAMS  From the Dermato-Venereology (Skin/VD) Centre, Sehgal Nursing Home, Panchwati, Azadpur, Delhi, Skin Institute and School of Dermatology, Greater Kailash, New Delhi, and Department of Dermatology and STD, Lady Hardinge Medical College, New Delhi, India Correspondence  Virendra N. Sehgal, MD  , Dermato-Venereology (Skin/VD) Centre, Sehgal Nursing Home, A/6, Panchwati, Delhi 110 033, IndiaE-mail:  Introduction  Exfoliative dermatitis is an extreme state of skin irritationresulting in extensive erythema and/or scaling of the body.Several skin disorders may ultimately culminate in the pres-entation of erythroderma/exfoliative dermatitis. Largely, it isa secondary process; therefore, it is mandatory to establish itsetiopathology in order to facilitate its precise management.   1  An increasing number of new drugs have been incriminated inthe recent past.   2–9  Its clinical pattern is fascinating and hasbeen the subject of detailed studies.   4,8,10–16  Its changing sce-nario in various age groups,   17–19  its presentation postopera-tively,   20–22  and its occurrence in human immunodeficiencyvirus (HIV)-positive individuals   23–25  are vivid indicators.Several factors may be responsible for the causation of thisextensive skin disorder. Subtle/sudden generalization of pre-existing dermatoses may be an intriguing dilemma, and mayreflect an individual variation. A detailed outline of apatient’s history to elicit possible triggering events, namelyinfections, drug ingestion, topical application of medica-ments, sun/ultraviolet light exposure, and other factors,   16  may be imperative. It is also challenging to manage the con-dition, because the intricate process puts an extensive strainon an already compromised body system. Furthermore, it isprobable that the srcinal dermatosis may be masked byextensive erythema/scaling, thus making it difficult to obtaina clear-cut diagnosis. This synopsis is an endeavor to revisitand update the information available on erythroderma/ exfoliative dermatitis.  Definition  Erythroderma and exfoliative dermatitis are largely synony-mous; however, erythroderma is the preferred term   1,13,14  andis currently in vogue. Nonetheless, it is imperative to definethese conditions. The former is characterized by extensiveand pronounced erythema coupled with perceptible scaling,whereas the latter is conspicuous by the presence of wide-spread erythema and marked scaling. Accordingly, ≥  90%skin surface involvement is considered as a salient prerequisiteto make a clinical diagnosis of exfoliative dermatitis.   1,16,26–28  Incidence  It is hard to obtain a precise incidence for erythroderma/ exfoliative dermatitis as most reports are retrospective, and donot address the issue of overall incidence.   8  This aspect wasdealt with for the first time by Sehgal and Srivastava   16  in alarge prospective study from the Indian subcontinent, where theincidence was recorded as 35 per 100,000 dermatologic out-patients. In another survey from The Netherlands, the annualincidence was recorded as 0.9 per 100,000 inhabitants.   29  Astudy based on an analysis of 138 consecutive erythrodermapatients from South Africa found that 75% were black,22.5% Indian and 2.5% white.   24  In addition, a large numberof patients were HIV positive, and a drug reaction was themost common cause of erythroderma. Furthermore, maleswere affected 2–3 times more frequently, with an averagemale to female ratio of 2.3 : 1.   8  In another study from Spain,   International Journal of Dermatology   2004, 43  , 39–47© 2004 The International Society of Dermatology   40Education  Erythroderma/exfoliative dermatitis  Sehgal, Srivastava, and Sardana   the male to female ratio was found to be as high as 4 : 1.   14  Theincidence as a function of age is usually variable, and any agegroup may be affected; however, affected patients are usuallyover 45 years of age   1  (excluding hereditary disorders/atopicdermatitis), with an average age of onset at 55 years. On theIndian subcontinent, the age of onset was found to be slightlylower.   16,30  Erythroderma in children has a mean age of onsetof 3.3 years, with a male to female ratio of 0.89 : 1.   18  Etiology  Erythroderma/exfoliative dermatitis may embrace or becaused by any pre-existing dermatosis, drug-induced reac-tion, malignancy, and miscellaneous or idiopathic disorder.   Pre-existing dermatoses  Several dermatologic disorders  per se  , or their therapy, canresult in exfoliative dermatitis (Table 1). This is the singlemost common cause of adult exfoliative dermatitis in themajority of studies.   1,11,12,14,16,31–36  Psoriasis is the most common cause of exfoliative dermati-tis amongst the dermatologic disorders;   12,16,30,36  however, inchildren, psoriasis is the second most common cause of thedisease, with drugs as the main culprit.   18   Drugs  Topical and systemic medications are notorious for precipi-tating erythroderma/exfoliative dermatitis. An apparentincrease in the incidence of the disease may be directly pro-portional to the introduction of new drugs.   8  Apart from thewell-known allopathic medicines, homeopathic, unani,ayurvedic, herbal, and common home remedies have beenincriminated.   16  Many drug eruptions that commonly presentas morbilliform, lichenoid, or urticarial forms may oftenprogress to extensive erythema and exfoliation.   8  The inventory of drugs causing erythroderma/exfoliativedermatitis is increasing;   2–9,37–47  however, the most commonare shown in Table 2.It is therefore pertinent to provide a inventory of drugs fre-quently responsible for causing such an episode.Drug-induced erythroderma due to dapsone/antileprosydrug hypersensitivity may often mimic cutaneous T-cell lym-phoma in terms of both clinical features and histopathology.Fortunately, it resolves after withdrawal of the offendingdrug(s) and the administration of supportive therapy.   8   Malignancies  Erythema/exfoliative dermatitis may ultimately be one of theclinical expressions of reticuloendothelial neoplasms andinternal blood vessel malignancies. The latter invariablyaffect older individuals, and erythema/exfoliative dermatitisis considered to be a salient cutaneous marker of internalmalignancy. Its incidence is around 1%.   8  Lymphomas in gen-eral and T-cell lymphoma [comprising mycosis fungoides andSezary syndrome (its leukemia variant, in particular)] areoften reported to present as exfoliative dermatitis. They con-stitute over 25–40% of cases of malignancy-related erythro-dermas.   8,26–28,32,48–51  Exfoliative dermatitis may precede,accompany, or follow T-cell lymphomas, and its appearancemay be identical to that of benign erythroderma.   8  An immu-nophenotypic study with the use of advanced antibody panelsmay be required to distinguish it from the benign form.   26,52  Reticular cell sarcoma, acute and chronic leukemia, andmalignant histiocytosis are a few other implicated condi-tions.   8,50,53  Carcinoma of the colon, lung, prostate, thyroid,fallopian tubes, larynx, and esophagus have also been allegedto cause the condition.   8,54–57  An insidious, debilitating, pro-gressive course, absence of a history of a previous skin dis-order, and recalcitrant nature may warrant an exploration of the possibility of an underlying malignancy.   4,9,54   Miscellaneous/idiopathic disorders  Hepatitis, irradiation, acquired immunodeficiency syndrome Table 1 Dermatoses frequently resulting in exfoliative dermatitis   Table 2 Common drugs causing exfoliative dermatitis 8   CommonUncommon PsoriasisCandidiasisAirborne contact dermatitisDermatophytosisSeborrheic dermatitisMastocytosisAtopic dermatitisLichen planusStaphylococcal scalded skin syndromeReiter’s syndromePhytophotodermatitisToxic epidermal necrolysisPhotosensitive dermatitisDiffuse/erythrodermic mastocytosisPityriasis rubra pilarisSarcoidosisPemphigus foliaceusPemphigoidStasis dermatitisLupus erythematosusIchthyosiform erythrodermaCrusted (Norwegian) scabiesAcetaminophenMinocyclineActinomycin-DNitrofurantoinAllopurinolOmeprazoleArsenicPara-amino salicylic AcidBarbituratesPenicillinCaptoprilPhenothiazineChloroquine diphosphatePhenytoinChlorpromazineQuinidineCemetidineRifampicinDapsoneStreptomycinGoldSulfadiazineHydantoin sodiumSulfonyl ureaInterferonTetracyclineIsoniazid/isonicotinic hydrazideThalidomideIsotretinoinTolbutamideLithiumVancomycinMercurials   © 2004 The International Society of DermatologyInternational Journal of Dermatology   2004, 43  , 39–47  41  Sehgal, Srivastava, and Sardana   Erythroderma/exfoliative dermatitis  Education  (AIDS), graft-vs.-host disease, Ofuji papuloerythroderma, andOmenns’ syndrome can also cause the condition.   8,12,14,51,58–61  Despite the best endeavors, a small proportion of patientsremain in whom no clear-cut etiology can be defined andthese are classified under idiopathic disorders. Sustainedefforts during the course of follow-up may lead to the precisedefinition of the etiology.   8  Pathogenesis  The pathogenesis of erythroderma/exfoliative dermatitis isunclear. Currently, it is believed that the condition is second-ary to an intricate interaction of cytokines and cellular adhe-sion molecules, including interleukins-1, -2, and -8,intercellular adhesion molecule-1 (ICAM-1), and tumornecrosis factor (TNF).   15  These interactions result in a dra-matic increase in the epidermal turnover rate, causing ahigher than normal mitotic rate and an increase in the abso-lute number of germinative skin cells. Furthermore, the timerequired for cells to mature and travel through the epidermisis decreased, and is manifested as an increased loss of epider-mal material, together with a significant loss of protein andfolate.   62  In contrast, the exfoliation of normal epidermis ismuch less and contains very little important viable material,such as nucleic acids, soluble proteins, or amino acids.   63  Abel et al   .   26  studied the immunophenotypic characteristicsof benign (psoriasis, dermatitis, drug-induced) and malignant(Sezary syndrome, mycosis fungoides) forms of erythro-derma, and found them to be similar. In immunohistochemi-cal studies conducted by Sigurdsson et al   .,   64,65  the dermalinfiltrate in patients with Sezary syndrome mainly showed aT-helper-2 cytokine profile, while benign reactive erythro-derma showed a T-helper-1 cytokine profile, indicating that,although clinically similar, they have different underlyingpathogenic mechanisms.  Clinical presentation  Exfoliative dermatitis starts as patch(es) of erythema accom-panied by pruritus. The patch(es) enlarge and coalesce toform extensive areas of erythema which eventually spread tocover whole/most of the skin surface. Exfoliative dermatitisis also associated with profuse scaling, which has its onset2–6 days after erythema with individual variations.   1,16,63  Theacute form is heralded by the formation of large scales, whilstthe chronic form is recognized by small scales.   8  The skin isconspicuously bright red, dry, scaly, hot, and indurated (Fig. 1).Mild to severe pruritus is usually present. Lichen simplexchronicus may be its ultimate expression (Fig. 2). In addition,the nails become thick, lusterless, dry, brittle, and show ridg-ing of the nail plate. Periorbital skin inflammation and edemacause ectropion and epiphora. Lymphadenopathy, hepat-osplenomegaly, edema of the feet/ankles, and gynecomastiamay also be observed. In black people, a widespread loss of pigmentation is usual. The basal metabolic rate is increasedand a catabolic state causes significant weight loss overtime.   1,8  At times, patients can slip into an irreversible hypo- orhyperthermia. The former may result in ventricular bradycar-dia and hypotension. An increased peripheral blood flow mayresult in high-output cardiac failure. All body systems may beaffected by these manifestations.   8  The general picture is modified accordingly to the nature of the underlying disorder, whose etiology and prompt treat-ment should be addressed. Generalized dermatitis usuallyoccurs in the sixth or seventh decades of life; however, atopicdermatitis may occur at any age. Pruritus is at its worst in oldage. Frenk et al   .   66  reported senile erythrodermic patches withincreased serum immunoglobulin E (IgE) and lactic dehydro-genase levels together with eosinophilia.Psoriasis is the most common underlying disorder, and itsfeatures may be present until the whole body develops exfo-liative dermatitis. In a few cases, generalized pustular psoriasismay also be present. There may be a history of precedingplaque(s), treatment with tar, potent steroids, or psoralen Figure 1 Erythroderma/exfoliative dermatitis, whole of the skin surface was bright red, dry, scaly, hot and indurated   International Journal of Dermatology   2004, 43  , 39–47© 2004 The International Society of Dermatology   42Education  Erythroderma/exfoliative dermatitis  Sehgal, Srivastava, and Sardana   plus UVA (PUVA) therapy, intermittent infections, or emo-tional stress.   1,4,8,63  A history of drugs for certain dermatoses/ systemic disorders may be elicited prior to the onset of exfo-liative dermatitis. Erythema is acute in onset and progressesto generalized exfoliation, which may resolve over the courseof 2–6 weeks.   1  Erythroderma following pityriasis rubra pilaris is fairlydiagnostic, as it usually starts in childhood or adulthood andthe lesions occupy the hair follicle in the form of papules and/ or plaques with “islands of sparing.”Other uncommon causes are lichen planus, pemphigusfoliaceus (Fig. 3), dermatophytosis, ichthyosiform erythro-derma, crusted (Norwegian) scabies, graft-vs.-host disease,and irradiation.   1,12,14,16,59,67–69  Postoperative erythroderma, atype of graft-vs.-host disease appearing several days aftersurgery along with blood transfusion, is characterized byerythroderma, fever, pancytopenia, hepatic insufficiency, anddiarrhea, and may prove fatal.   21,22,70  Exfoliative dermatitismay also develop during seroconversion in HIV-infectedpatients with florid manifestations.   23,24,71  Exfoliative dermatitis associated with lymphoma mayshow the classical features; however, it may fail to respond tocorticosteroid therapy. It may continue to progress, even if repeated investigations over months or years fail to pinpointany convincing etiology. Efforts should be continued to makea diagnosis as, sooner or later, lymphoma will be revealed.Sezary cells/immature neutrophils are diagnostic pointers.Hodgkin’s disease may also show unexplained eosinophilia.   1  Hemodynamic/metabolic disturbances  The disease may cause an enormous aberration of bodymetabolism. The increased skin blood flow may cause hypo-thermia and profound heat loss. Compensatory hyperme-tabolism and an increased basal metabolic rate without anyprimary increase in thyroid activity may ensue. Excessiveprotein loss through scaling and leaking through skin, hemo-dilution due to the increased plasma volume, andhypermetabolism may contribute to hyperalbuminemia andsevere edema. Furthermore, high-output cardiac failure mayoccur at any time. 1,72–75  Histopathology  The histopathology of exfoliative dermatitis often reveals anonspecific picture consisting of orthokeratosis (hyperkera-tosis, parakeratosis), acanthosis, and a chronic perivascularinflammatory infiltrate with or without eosinophilia. Botella-Estradas et al   .   14  observed that the clinicopathologic correla-tion in erythroderma is difficult, because the specific featuresof the dermatosis are masked by the nonspecific features of  Figure 2 Erythroderma/exfoliative dermatitis, Lichen simplex chronicus was conspicuous and was characterized by pigmentation, thickening of the skin and exaggerated skin marking and marked by pruritis Figure 3 Erythema/exfoliative dermatitis in pemphigus foliaceus
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