Acquired poikiloderma: Proposed classificationand diagnostic approach

Ahmad Nofal, MD, and Eman Salah, MScZagazig, Egypt

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‘‘Poikiloderma’’ is a morphologic and descriptive term referring to a combination of cutaneous atrophy,telangiectasia, and varied macular pigmentary changes that result in a mottled skin appearance. Its etiologyincludes both congenital and acquired causes. Many studies have reported different causes of acquiredpoikiloderma; however, no single well-defined classification has been explored to date. Herein, we analyzeall the possible causes of acquired poikiloderma and propose an etiological classification that, hopefully,will lead to better characterization for this ill-defined condition. Moreover, this study presents a step-by-step approach to the management of patients with acquired poikiloderma and summarizes the keydifferentiating features for each individual cause, which may help in easy and precise diagnosis of differentcauses of acquired poikiloderma. ( J Am Acad Dermatol 2013;69:e129-40.)

Key words: mottled pigmentation; parapsoriasis; poikiloderma; poikiloderma of Civatte; poikilodermavasculare atrophicans; poikilodermatous mycosis fungoides.

‘‘Poikiloderma’’ is a descriptive term referringto a combination of cutaneous atrophy,telangiectasia, and macular pigmentary

changes that result in a mottled skin appearance.Miliary lichenoid papules, fine scaling, and smallpetechial hemorrhages are variable features thathave been considered as secondary or associatedfeatures by some authors.1 The distribution of poiki-loderma varies according to the cause, as shown in adiagrammatic representation (Fig 1).

Poikiloderma includes both congenital and ac-quired types. Congenital poikiloderma occurs incertain genetically determined and well-defined syn-dromes such as RothmundeThomson syndrome.1

On the other hand, acquired poikiloderma has beenattributed to multiple and variable causes; however,a single well-defined classification for this divergentcondition has not been explored to date.

Herein, we review the different aspects of ac-quired poikiloderma, proposing a new etiologicalclassification (Table I), presenting a step-by-stepapproach for a claimed case of acquired poikilo-derma (Fig 2), and summarizing the key differenti-ating features for each individual cause (Table II).

Dermatology and Venereology, Faculty of Medicine, Zagazig

niversity, Zagazig, Egypt.

ing sources: None.

licts of interest: None declared.

int requests: Eman Salah, Dermatology and Venereology,

culty of Medicine, Zagazig University, Zagazig 11456, Egypt.

mail: connexin1980@yahoo.com.

HISTOPATHOLOGYHistopathological findings (Fig 3) include com-

mon features in any case of poikiloderma such asthinning of the stratum malpighii, hydropic degen-eration of the basal cell layer, presence of melano-phages in the papillary dermis, and dilatation of thepapillary dermal capillaries.2,3 Characteristic featuresin relation to some specific causes such as epider-motropism in case of poikilodermatous mycosisfungoides (MF) have also been reported.4

CLASSIFICATIONInfections

Borrelia burgdorferi: Acrodermatitis chron-ica atrophicans of Lyme disease. Lyme diseaseis a tick-borne zoonosis caused by strains of theGram-negative spirochete Borrelia burgdorferisensu lato.5 Acrodermatitis chronica atrophicans isthe late stigma of Lyme disease6 with an insidiousonset after the initial tick bite.7 The sun-exposedextensors of the lower extremities are the mostcommon predilection site, however, lesions canalso affect the trunk and rarely the face.5 In a matterof years, poikilodermatous skin may be the end

Published online July 30, 2012.

0190-9622/$36.00

� 2012 by the American Academy of Dermatology, Inc.

http://dx.doi.org/10.1016/j.jaad.2012.06.015

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result of the untreated plaques of acrodermatitischronica atrophicans.8

In addition to the common histopathologicalfeatures of poikiloderma, acrodermatitis chronicaatrophicans is characterized by destruction of epi-dermal appendages, a subepidermal zone of degen-erated connective tissue, and aprominent plasma cell

CAPSULE SUMMARY

d Different causes of acquiredpoikiloderma have been reported in theliterature; however, no single well-defined classification has beenestablished to date.

d The study suggests an etiologicalclassification, presents a step-by-stepapproach, and summarizes the keydifferentiating features of acquiredpoikiloderma.

d The proposed classification anddiagnostic approach would serve as apractical tool for proper diagnosis,differentiation, and management ofacquired poikiloderma.

dermal infiltrate. Moreover,the spirochete may be iden-tified byWarthin-Starry stain9

andmay be cultured from theatrophic skin in some cases.10

InflammatoryAtopic dermatitis. Poi-

kiloderma is frequently en-countered on the neck insevere cases of adult atopicdermatitis. This may be at-tributed to chronic inflamma-tion and delayed woundhealing induced by the pro-longed topical corticosteroidapplication. The histopatho-logical findings include thoseof poikiloderma and spongi-otic dermatitis.11

Chronic graft-versus-

host disease. Chronic graft-versus-host diseasemay occur within 3 years after allogeneic hemato-poietic cell transplantation. Poikiloderma is one ofthe diagnostic cutaneous signs for chronic graft-versus-host disease and is often present on the faceand trunk during sclerodermoid reaction.12 In addi-tion to the common histologic features of poikilo-derma, the sclerodermoid phase is characterized bydermal fibrosis with destruction of adnexalstructures.13

Lichen planuseinduced poikiloderma. Thisis classic lichen planus that is rarely followed, withinmonths or years, by a network of generalizedsmall erythematous papules and poikilodermatouslesions. Histopathologically, the lesions show fea-tures suggestive of both lichen planus andpoikiloderma.14

MetabolicAmyloidosis: Poikiloderma-like cutaneous

amyloidosis. Poikiloderma-like cutaneous amyloi-dosis combines cutaneous poikilodermatouschanges with amyloid deposits.15 Two differentclinical forms were described: ordinary and syn-dromic.16 The ordinary poikiloderma-like cutaneousamyloidosis appears around the fifth decade oflife and is characterized by the presence of

poikilodermatous lesions at sun-exposed or sun-protected sites (Fig 4), lichenoid papules, and blis-ters, especially on the limbs.17

On the other hand, the term ‘‘poikiloderma-likecutaneous amyloidosis syndrome’’ was proposed forpatients with poikilodermatous lesions, lichenoidpapules, blisters, photosensitivity, palmoplantar

keratoses, short stature, andonset early in life.15,16

The histopathologicalfindings in routine sectionsinclude the common poiki-lodermatous changes andeosinophilic amyloid de-posits. Moreover, sectionsstain positive for Congo redstain and fluoresce withapple-green birefringenceunder polarized light.15,17

Connective tissuediseasesLupuserythematosus. In

systemic lupus erythemato-sus, poikiloderma has beenreported mainly as a featureof advanced disease in whichacute erythematous lesions

can proceed to poikilodermatous lesions on sun-exposed sites.18 More surprisingly, in 1964 Tuffanelliet al19 described generalized poikiloderma occurringin an identical twin sister of a patient with systemiclupus erythematosus.

Cases of poikilodermatous subacute cutaneouslupus erythematosus are rarely reported.1 In onecase report from Bulgaria, the patient developedpoikilodermatous subacute cutaneous lupus eryth-ematosus after an episode of sunburn, suggesting arole for ultraviolet radiation in the development ofthis poikiloderma.20

In discoid lupus erythematosus, an extremely rarevariant consisting of purplish plaques or blotchyreticulate telangiectasia is called the ‘‘telangiectoidvariant’’ that may develop on many sites, particularlythe face and neck.21,22 This variant is mostly associ-ated with other chronic discoid lupus erythematosuslesions or can replace active inflammation in patientswith classic discoid lupus erythematosus. Cases oflupus panniculitis that clear with poikilodermatousfeatures have also been reported.23

Regarding neonatal lupus erythematosus, thetypical skin lesions usually clear in the first year oflife but may leave areas of atrophy, hypopigmenta-tion, and telangiectasia, resulting in a poikiloderm-atous appearance.24

Fig 1. Diagrammatic representation of distribution of acquired poikiloderma. ACA, Acroder-matitis chronic atrophicans; AD, atopic dermatitis; EAI, erythema ab igne; MF, mycosisfungoides.

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Crowley and Frieden25 reported a patient withneonatal lupus erythematosus and an unusual pre-sentation in the form of generalized poikilodermaassociated with extensive skin erosions, patchyscalp alopecia, absent eyebrows and eyelashes,absent neonatal lupus erythematosus classic skinlesions, and negative Ro and La antibodies associ-ated with a strongly positive antinuclear antibodytiter with a speckled pattern in both mother andinfant.

Dermatomyositis. Poikiloderma in dermato-myositis is often a late finding and it may occur onsun-exposed skin such as the V-shaped area of theneck and/or sun-protected skin such as the upperaspect of the back (shawl sign) (Fig 5) and the upper-lateral aspect of thighs (holster sign).26-28 Sometimesthe poikiloderma in dermatomyositis is more gener-alized and this may resemble a poikilodermic cuta-neous T-cell lymphoma.29 The histopathologicalalternations found in poikilodermatomyositis (Fig2) are just those of classic poikilodermatouschanges.26

Systemic sclerosis. In systemic sclerosis, themost characteristic pigmentary change is loss ofpigment in a large patch with perifollicular pigmentretention. The affected areas become hairless andatrophic.30 Mat-like telangiectasia is found mainly on

the face but may extend as far as the upper aspect ofthighs. Pigmentation occurs also most frequently onthe face and to a lesser extent on the legs, thighs, andlower aspect of abdomen.9 Therefore, we believethat a mixture of these findings can result in apoikilodermic appearance.

EnvironmentalSolar radiation. Poikiloderma of Civatte. Poi-

kiloderma of Civatte is a common condition with aslowly progressive and irreversible course.31 Accord-ing to the predominance of 1 or more of the com-ponents of poikiloderma, some classifypoikiloderma of Civatte into erythematotelangiec-tatic, pigmented and mixed types.32

The classic distribution of the lesions symmetri-cally involves the side of the face and neck and theupper aspect of the chest (Fig 6). Solar radiation hasbeen proposed as the main culprit besides otherfactors such as genetics, low estrogen, and photo-toxic or photoallergic reactions to chemicals infragrances or cosmetics.32-34 The most prominentand constant histopathological feature, besides theclassic poikilodermic changes, is solar elastosis of thepapillary dermis.35

Photoaging ‘‘dermatoheliosis’’. Repeated solarinjuries can ultimately result in photoaging or what

Table I. Proposed etiological classification foracquired poikiloderma

Infectionsd Borrelia burgdorferi: acrodermatitis chronica atrophi-cans of Lyme disease

Inflammatoryd Atopic dermatitisd Chronic graft-versus-host diseased Lichen planuseinduced poikiloderma

Metabolicd Amyloidosis: poikiloderma-like cutaneousamyloidosis

Connective tissue diseasesd Lupus erythematosusd Dermatomyositisd Systemic sclerosis

Environmentald Solar radiation: poikiloderma of Civatte and photo-aging (dermatoheliosis)

d Heat and infrared radiation: erythema ab igned Chemical intoxication: sulfur mustard-inducedpoikiloderma

Iatrogenicd Drugs

CorticosteroidsHydroxyurea

d RadiotherapyChronic radiation dermatitis

Neoplasticd Mycosis fungoides

Poikilodermatous variantGranulomatous variant

d Poikiloderma vasculare atrophicansd Poikilodermatous parapsoriasis

Fig 2. Histopathology of acquired poikiloderma in case ofdermatomyositis showing thinning of stratum malpighii,hydropic degeneration of basal cell layer, and dilatation ofpapillary dermal capillaries.

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is called ‘‘dermatoheliosis.’’ This happens more inpatients with fair skin, especially outdoor workers.Themost common sites affected are the sun-exposedareas, particularly face and scalp in bald men.36

Cutaneous manifestations of dermatoheliosis(Fig 7) show a combination of atrophy, telangiecta-ses, purpura, spotty depigmentation, hyperpigmen-tation solar lentigines, and actinic keratoses.36

Heat and infrared radiation: Erythema abigne. Erythema ab igne is characterized by localizedmottled and reticulated cutaneous hyperpigmenta-tion with variable telangiectasia and skin atrophyresulting from repeated or prolonged exposure toheat and infrared radiation. Therefore poikilodermais considered the end stage of the cumulativechanges caused by repeated and prolonged expo-sure to infrared radiation.9

Erythema ab igne can occur at all ages includingchildhood37 but it was reported as a common con-dition on the shins among the elderly using heatersor fireplaces.38 The distribution of the lesions

depends on the direction of the incident radiation,the contour of the skin, and the interposition ofclothing.9

In the early stages, epidermal atrophy, dermalpigmentation, and capillary dilatation are evident.Basophilic degeneration of the connective tissue,focal hyperkeratosis, and epithelial cellular atypiaoccur later.39

Chemical intoxication: Sulfur mustardein-duced poikiloderma. Sulfur mustard is a chemicalweapon that was first used by the German Army in191740 and was widely used in the 1980s during theIran-Iraq conflict. Based on the lipophilic propertiesof this gas, it easily penetrates the skin and mucosalsurfaces causing several acute and chronic effects onthe skin, eye, and respiratory system.41

In 2011, Emadi et al42 proposed a diagnosis ofsulfur mustardeinduced poikiloderma in a 41-year-old man who was injured with sulfur mustard duringan Iraq chemical attack in 1988. Their diagnosiswas based on the clinical findings of atrophy,pigmentation, and telangiectasia on the upper aspectof the trunk, back of the hands, and genitalia asso-ciated with relevant histopathological features ofpoikiloderma.

IatrogenicDrugs. Corticosteroids. Corticosteroids are ac-

cused of many undesirable side effects includingatrophy, telangiectasia, and cutaneous dyspigmen-tation.43 Therefore, it can be expected that a con-stellation of these features will eventually result in apoikilodermic skin appearance.

Hydroxyurea(hydroxycarbamide). Hydroxyureais commonly used for myeloproliferative disordersand rarely for severe recalcitrant psoriasis.44 Majoradverse reactions of hydroxyurea include both

Table II. Key differentiating points of acquired poikiloderma

Causes History Site

Other symptoms

and signs topathology Comments

Achrodermatitis chronicaatrophicans

Months or yearsafter initial tick bite

Extensor surfaces oflower limbs

Arthritis, morphea-likeor lichen sclerosusatrophicuselikelesions

Promin t plasmacell rmal infiltrateand struction ofappe ages

Spiroch es may bedete d by Warthin-Starr tain

Positive serology and PCRfor Borrelia can confirmdiagnosis

Culture from atrophic skinmay reveal organism

Chemoprophylaxis andvaccination for Lymedisease may preventcondition in endemicregions

Atopic dermatitis Mostly in adults Sides of neck Itching and otheratopic manifestations

Spong ic dermatitisand ikiloderma

Prolonged corticosteroidapplication maycontribute to condition

Chronic GVHD Usually within 3 ypost-HCT

Face and trunk duringsclerodermoid reaction

Lichen planuselike,morphea-like, lichensclerosus atrophicuselike lesions

Other systemicmanifestations, eg,esophageal web,bronchiolitisobliterans, and jointstiffness

Sclerod moid phase ischar erized byderm fibrosis anddest tion ofadne l structures

_

Lichen planuseinducedpoikiloderma

Years after onset ofclassic lichen planuslesions

May be generalized Classic lichen planuslesions

Itching

Histolo changes ofboth chen planusand ikiloderma

Only 1 case is reported

Poikiloderma-likecutaneous amyloidosis

Around fifth decadeof life

Sun-exposed or sun-protected skin

Lichen amyloidosisand cutaneousblisters

Derma myloiddepo ts andposi e Congo red

Apple- enbiref gencewith larized light

Disappointing therapeuticoptions

Continued

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Table II. Cont’d

Causes History Site

Other symptoms

and signs Histopathology Comments

Lupus erythematosus Patients with SLEand NLE

With SCLE aftersunburn

After clearinglesions of lupuspanniculitis

On sun-exposed sites,may be generalized

SLE and NLE: othercutaneous andsystemicmanifestations

SCLE: other classicskin lesions

Patchy lymphocyticinfiltrate of LE pluspoikilodermatouschanges

Poikiloderma indicatesadvanced SLE

Serologic tests can help toconfirm diagnosis

Anti-Ro and La werein the reported caseabout NLE andpoikiloderma

Dermatomyositis Usually late findingduring diseasecourse

Arm extensors, V-areaof neck, upper aspectsof back and thighs orgeneralized

Photosensitivity, myositisDiagnostic signs ofdermatomyositis, eg,Gottron sign andpapules

Poikilodermatous changes EMG and muscle biopsyspecimen showinginflammatory myopathy

Elevated CK, AST, ALT

Systemic sclerosis Progressive Usually on face Other systemicmanifestation

Dermal fibrosis _

Poikiloderma of Civatte After repeated UVexposure

Slowly progressive andirreversible

Sun-exposed side of faceand neck, upper aspectof chest with sparing ofsubmental area

Usually asymptomaticMay be itching, burning,and flushing

Poikilodermatous changesplus solar elastosis

Patch test to diagnosecases induced by certainallergens

IPL may helpDermatoheliosis Chronic sun exposure

especially in fair skinSun-exposed, eg, baldscalp in men

Actinic keratoses areinvariably present

Solar elastosis pluspoikilodermatouschanges

Proper photoprotectioncan limit occurrence

EAI EAI starts 2 wk to severalmonths after heatexposure

Any heat-exposed site canbe affected butcommonly on shins

Usually asymptomaticSome reported itching andburning sensation

Poikiloderma withconnective tissuebasophilic degeneration

Later, epithelial cell atypiacan be found

Sulfur mustardeinducedpoikiloderma

Years after exposure towarfare agent withhistory of blistering afteracute exposure

Upper aspect of trunk,back of hands, genitalia

Eyes and respiratorysystem

Poikilodermatous changes Was widely used in 1980sduring Iran-Iraq conflict

Corticosteroids Depends on potency, site,and presence orabsence of occlusion

Sites of prolonged steroidapplication

Other side effects, eg,acneiform eruptions,hypertrichosis

Poikilodermatous changes Following safe guidelinesfor corticosteroid usecan prevent thispoikiloderma

Hydroxyurea Usually in patients withmyeloproliferativedisorders onhydroxyurea therapy

More on sun-exposedsites

Other cutaneous adverseeffects, eg, leg ulcersand dermatomyositis-like eruption

Poikilodermatouschanges

Other nondermatologicside effects can befound, eg, bone-marrowsuppression

Chronic radiationdermatitis

History of exposure toionizing radiationsource

At radiation-exposedsite

Other adverse reactions,eg, alopecia, nail loss,and tissue fibrosis

Poikilodermatouschanges and dermalfibrosis

Pulsed dye laser canimprove telangiectasia

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Poikilo

derm

atousMF

Itslowly

progresseslike

patch

stag

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MFbutfew

cases

proceedto

tumor

stag

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Mainly

onbreasts

and

hips

Otherpatchesan

dplaquesofclassicMF

may

befound

Typ

ical

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gesofMF

andpoikilo

derm

aCD4+butrarely

CD8+

phenotypecanbe

found

GranulomatousMF

Itisrare

typewithnodistinct

clinical

presentation

Histopathological

featuresareessential

foritsdiagnosis

Granulomatous

reactionswith

epiderm

otropism

Itindicatespoorprognosis

Poikilo

derm

avasculare

atrophican

sSlowly

progressive

Trunkan

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areas

6Itching

Like

MFplus

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atous

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isconsideredas

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Poikilo

derm

atous

parap

soriasis

Slowly

progressive

Loweraspect

of

trunk

andupper

aspect

ofthighs

6Itching

Usually

showsmild

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atitis

Confusingterm

that

may

bejust

prelymphomatousor

trueMF

ALT,Alanineam

inotran

sferase;AST,aspartate

aminotran

sferase;CK,creatinekinase;

EAI,erythemaab

igne;EM

G,electromyo

graphy;

GVHD,graft-versus-host

disease;HCT,

hematopoieticcell

tran

splantation;IPL,intense

pulsedlig

ht;MF,mycosisfungoides;NLE,n

eonatallupuserythematosus;PCR,p

olymerase

chainreaction;SCLE,subacute

cutaneouslupuserythematosus;SLE,systemic

lupuserythematosus;UV,ultraviolet.

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nondermatologic effects such as bone-marrow sup-pression, and dermatologic effects such as poikilo-derma, ichthyosis, Gottron-like eruptions, nailabnormalities, oral ulceration and pigmentation,leg ulcers, actinic keratoses, and squamous cellcarcinoma.45-47

Radiotherapy. Chronic radiation dermati-tis. Chronic radiation dermatitis occurs after expo-sure to ionizing radiation used in tumorradiotherapy, and during interventional proceduressuch as fluoroscopic imaging.48 In chronic radiationdermatitis, persistent poikilodermatous changes areindicative of significant cutaneous injury.49,50

NeoplasticMycosis fungoides. Poikilodermatous var-

iant. Poikilodermatous MF is predominantly lo-cated on the breast (Fig 8), hips, and buttocks.These lesions may be associated with other patchesand plaques of classic MF.4 Rarely, patients may haveextensive poikiloderma as a feature of erythrodermicdisease.9 The progression of poikilodermatous MFmay be similar to that of the patch stage of classic MF,although a greater proportion of cases tend to showspontaneous regression and fewer patients proceedto tumor stage.51

A skin biopsy specimen shows histologic findingssimilar to those seen in long-standing patch- orplaque-stage MF lesions, in addition to the typicalhistopathological changes of poikiloderma.52

Immunophenotypic studies typically demonstrate aCD21, CD31, CD41, CD45RO1, CD8�, and CD7�

pattern that is similar to that observed in classic MF.Rarely, cases of poikilodermatous MF are character-ized by a CD81 cytotoxic T-cell phenotype.53

Granulomatous variant. Granulomatous MF is arare type of cutaneous T-cell lymphoma that indi-cates a poor prognosis.54 It can only be diagnosed bythe histopathological demonstration of granuloma-tous reaction because of the lack of a distinct clinicalpresentation.55

In 2008, Morihara et al55 reported a case granu-lomatous MF presenting clinically with poikilo-derma, ichthyosis, and erythematous scalyplaques, and histologically by a granulomatousreaction with giant cells and epidermotropic atyp-ical lymphocytes.

Poikiloderma vasculare atrophicans. Poikil-oderma vasculare atrophicans has been consideredas poikilodermic MF56 and, according to Kreuteret al57 in 2005, the term ‘‘poikilodermatous variant ofcutaneous T-cell lymphoma’’ has mostly replaced‘‘poikiloderma vasculare atrophicans.’’ However,poikiloderma vasculare atrophicans has been

Fig 3. Step-by-step approach to the management of the patient with acquired poikiloderma.ACA, Acrodermatitis chronica atrophicans; AD, atopic dermatitis; B burgdorferi, Borreliaburgdorferi; DM, dermatomyositis; EAI, erythema ab igne; GVHD, graft-versus-host disease;HCT, hematopoietic cell transplantation; H&E, hematoxylin-eosin; IPL, intense pulsed light; LD,Lyme disease; LSA, lichen sclerosus atrophicus; MF, mycosis fungoides; NLE, neonatal lupuserythematosus; PC, poikiloderma of Civatte; PDL, pulsed dye laser; PLCA, poikiloderma-likecutaneous amyloidosis; PVA, poikiloderma vasculare atrophicans; SLE, systemic lupuserythematosus; SS, systemic sclerosis.

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known to show a benign course, without progres-sion to the tumor stage of MF.58

Besides poikiloderma, lesions of poikilodermavasculare atrophicans usually manifest as asymp-tomatic or mildly pruritic flat-topped, scaly papulescoalescing into retiform patterns or zebra-like distri-butions and associated with telangiectasia. The typ-ical sites of predilection are the trunk and flexuralareas.59

The histologic findings include those of poikilo-derma and the classic histopathological and immu-nohistochemical findings of long-standing patch orplaque lesions of the classic MF.55,57

Poikilodermatous (large-plaque) parapsoria-sis. The term ‘‘parapsoriasis’’ has caused muchconfusion since its introduction in 1902.9 Two typesof parapsoriasis en plaque have been described:large-plaque parapsoriasis and small-plaque

Fig 4. Poikiloderma-like cutaneous amyloidosis of breast.

Fig 5. Poikiloderma of back in patient with dermatomy-ositis ‘‘shawl sign.’’

Fig 6. Poikiloderma of Civatte at V-area of neck.

Fig 7. Dermatoheliosis: poikiloderma affecting bald areasof scalp in farmer frequently exposed to sun.

Fig 8. Poikilodermatous mycosis fungoides (MF) ofbreasts and multiple patches of classic MF affecting trunk.

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parapsoriasis.60 Large-plaque parapsoriasis (or poi-kilodermatous parapsoriasis) is considered a pre-lymphomatous skin condition because of its biologicbehavior and the presence of clinical, histologic,and/or genotypical alterations that overlap earlyMF.61 In a large series of patients with poikiloderm-atous parapsoriasis, 11% developed definite MF butwhether these cases were MF from the start or notremains unclear.62

Clinically, patients present with persistent large,flat, red or brown, scaly atrophic patches and thin

plaques.63 Lesions appear finely wrinkled as a resultof epidermal atrophy, then telangiectasia and mot-tled pigmentation can be observed, hence the syn-onym ‘‘poikilodermatous parapsoriasis.’’64 Commonsites include the lower aspect of the trunk, upperaspect of thighs, and major flexural surfaces.63

Lesions at the breast and buttock should suggestMF.62

Most skin biopsy specimens only show a milddermatitis65 and a superficial bandlike dermal lym-phocytic infiltrate beneath atrophic epidermis.66

Single, rarely atypical, lymphocytes withoutPautrier microabscesses can be observed in theepidermis in the absence of spongiosis.67

TREATMENTTreatment of acquired poikiloderma includes the

use of a specific therapy, cosmetic management, orboth. Moreover, some causes can be prevented such

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as the use of chemoprophylaxis and vaccination inLyme diseaseeendemic regions,68 proper photo-protection and patch testing for poikiloderma ofCivatte,69 avoidance of prolonged heat or infraredcontact for erythema ab igne,38 and following thesafe guidelines for topical corticosteroid use.70

Topical and intralesional corticosteroid, hydro-colloid dressings, topical dimethylsulfoxide, calci-neurin inhibitors, acitretin, cyclophosphamide,dermabrasion, phototherapy, and pulsed-dye laserhave been all used for poikiloderma-like cutaneousamyloidosis, but are usually disappointing.71

Laser therapy using argon or pulsed dye laser canimprove poikilodermatomyositis,72 whereas the re-sults in poikiloderma of Civatte are unsatisfactory.Also, hydroquinones, chemical peels,38 electrosur-gery, and cryotherapy all failed in treating poikilo-derma of Civatte.73 The new intense pulsed lightsources are more promising with clearance of morethan 75% of telangiectasias and hyperpigmentationin different studies.34

Topically applied retinoids may reverse some ofthe changes of dermatoheliosis.36 In chronic radia-tion dermatitis, hyperbaric oxygen can reduce painand erythema74 but it has no effect on telangiecta-sia75 for which pulsed dye laser may be beneficial.76

The standard treatment for early poikiloderma-tous MF includes topical steroids, topical chemo-therapy, psoralen plus ultraviolet A, narrowbandultraviolet B, interferon alfa-2a, and oral retinoids.Systemic chemotherapies, denileukin diftitox, anti-CD52 antibody, and interleukin-12 are reserved foradvanced stages.77

Therapeutic options for poikilodermatous para-psoriasis include high-potency topical steroids, pho-totherapy, and topical nitrogen mustard.64

CONCLUSIONAcquired poikiloderma is a divergent condition

that has been attributed to different etiological fac-tors. We hope that our proposed classification anddiagnostic approach, including the key differentiat-ing points, may help in easy and precise diagnosis ofpoikiloderma and its underlying causes. Althoughthe histopathological features are nonspecific, aprecise evaluation can lead to the diagnosis ofserious diseases as is the case with poikilodermatousMF. Treatment of poikiloderma is that of the causeand laser therapy may be beneficial in some cases;however, the results are usually unsatisfactory.

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