Acquired poikiloderma: Proposed classification and diagnostic approach

REVIEW

Acquired poikiloderma: Proposed classification and diagnostic approach Ahmad Nofal, MD, and Eman Salah, MSc Zagazig, Egypt ‘‘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 etiology includes both congenital and acquired causes. Many studies have reported different causes of acquired poikiloderma; however, no single well-defined classification has been explored to date. Herein, we analyze all 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-bystep approach to the management of patients with acquired poikiloderma and summarizes the key differentiating features for each individual cause, which may help in easy and precise diagnosis of different causes of acquired poikiloderma. ( J Am Acad Dermatol 10.1016/j.jaad.2012.06.015.) Key words: mottled pigmentation; parapsoriasis; poikiloderma; poikiloderma of Civatte; poikiloderma vasculare atrophicans; poikilodermatous mycosis fungoides.

‘P

oikiloderma’’ is a descriptive term referring to a combination of cutaneous atrophy, telangiectasia, and macular pigmentary changes that result in a mottled skin appearance. Miliary lichenoid papules, fine scaling, and small petechial hemorrhages are variable features that have been considered as secondary or associated features by some authors.1 The distribution of poikiloderma varies according to the cause, as shown in a diagrammatic representation (Fig 1). Poikiloderma includes both congenital and acquired types. Congenital poikiloderma occurs in certain genetically determined and well-defined syndromes such as RothmundeThomson syndrome.1 On the other hand, acquired poikiloderma has been attributed to multiple and variable causes; however, a single well-defined classification for this divergent condition has not been explored to date. Herein, we review the different aspects of acquired poikiloderma, proposing a new etiological classification (Table I), presenting a step-by-step approach for a claimed case of acquired poikiloderma (Fig 2), and summarizing the key differentiating features for each individual cause (Table II).

From Dermatology and Venereology, Faculty of Medicine, Zagazig University, Zagazig, Egypt. Funding sources: None. Conflicts of interest: None declared. Reprint requests: Eman Salah, Dermatology and Venereology, Faculty of Medicine, Zagazig University, Zagazig 11456, Egypt. E-mail: [email protected].

HISTOPATHOLOGY Histopathological findings (Fig 3) include common features in any case of poikiloderma such as thinning of the stratum malpighii, hydropic degeneration of the basal cell layer, presence of melanophages in the papillary dermis, and dilatation of the papillary dermal capillaries.2,3 Characteristic features in relation to some specific causes such as epidermotropism in case of poikilodermatous mycosis fungoides (MF) have also been reported.4

CLASSIFICATION Infections Borrelia burgdorferi: Acrodermatitis chronica atrophicans of Lyme disease. Lyme disease is a tick-borne zoonosis caused by strains of the Gram-negative spirochete Borrelia burgdorferi sensu lato.5 Acrodermatitis chronica atrophicans is the late stigma of Lyme disease6 with an insidious onset after the initial tick bite.7 The sun-exposed extensors of the lower extremities are the most common predilection site, however, lesions can also affect the trunk and rarely the face.5 In a matter of years, poikilodermatous skin may be the end

Published online July 28, 2012. 0190-9622/$36.00 Ó 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 acrodermatitis poikilodermatous lesions at sun-exposed or sunchronica atrophicans.8 protected sites (Fig 4), lichenoid papules, and blisIn addition to the common histopathological ters, especially on the limbs.17 features of poikiloderma, acrodermatitis chronica On the other hand, the term ‘‘poikiloderma-like atrophicans is characterized by destruction of epicutaneous amyloidosis syndrome’’ was proposed for dermal appendages, a subepidermal zone of degenpatients with poikilodermatous lesions, lichenoid erated connective tissue, and a prominent plasma cell papules, blisters, photosensitivity, palmoplantar dermal infiltrate. Moreover, keratoses, short stature, and the spirochete may be idenonset early in life.15,16 9 CAPSULE SUMMARY tified by Warthin-Starry stain The histopathological findings in routine sections and may be cultured from the Different causes of acquired include the common poikiatrophic skin in some cases.10 poikiloderma have been reported in the lodermatous changes and literature; however, no single welleosinophilic amyloid deInflammatory defined classification has been posits. Moreover, sections Atopic dermatitis. Poiestablished to date. stain positive for Congo red kiloderma is frequently enThe study suggests an etiological stain and fluoresce with countered on the neck in classification, presents a step-by-step apple-green birefringence severe cases of adult atopic approach, and summarizes the key under polarized light.15,17 dermatitis. This may be atdifferentiating features of acquired tributed to chronic inflammapoikiloderma. tion and delayed wound Connective tissue healing induced by the prodiseases The proposed classification and longed topical corticosteroid Lupus erythematosus. In diagnostic approach would serve as a application. The histopathosystemic lupus erythematopractical tool for proper diagnosis, logical findings include those sus, poikiloderma has been differentiation, and management of of poikiloderma and spongireported mainly as a feature acquired poikiloderma. otic dermatitis.11 of advanced disease in which Chronic graft-versusacute erythematous lesions host disease. Chronic graft-versus-host disease can proceed to poikilodermatous lesions on sunmay occur within 3 years after allogeneic hematoexposed sites.18 More surprisingly, in 1964 Tuffanelli poietic cell transplantation. Poikiloderma is one of et al19 described generalized poikiloderma occurring the diagnostic cutaneous signs for chronic graftin an identical twin sister of a patient with systemic versus-host disease and is often present on the face lupus erythematosus. and trunk during sclerodermoid reaction.12 In addiCases of poikilodermatous subacute cutaneous tion to the common histologic features of poikilolupus erythematosus are rarely reported.1 In one derma, the sclerodermoid phase is characterized by case report from Bulgaria, the patient developed dermal fibrosis with destruction of adnexal poikilodermatous subacute cutaneous lupus erythstructures.13 ematosus after an episode of sunburn, suggesting a Lichen planuseinduced poikiloderma. This role for ultraviolet radiation in the development of is classic lichen planus that is rarely followed, within this poikiloderma.20 months or years, by a network of generalized small In discoid lupus erythematosus, an extremely rare erythematous papules and poikilodermatous levariant consisting of purplish plaques or blotchy sions. Histopathologically, the lesions show features reticulate telangiectasia is called the ‘‘telangiectoid suggestive of both lichen planus and variant’’ that may develop on many sites, particularly poikiloderma.14 the face and neck.21,22 This variant is mostly associated with other chronic discoid lupus erythematosus lesions or can replace active inflammation in patients Metabolic with classic discoid lupus erythematosus. Cases of Amyloidosis: Poikiloderma-like cutaneous lupus panniculitis that clear with poikilodermatous amyloidosis. Poikiloderma-like cutaneous amyloifeatures have also been reported.23 dosis combines cutaneous poikilodermatous 15 changes with amyloid deposits. Two different Regarding neonatal lupus erythematosus, the clinical forms were described: ordinary and syntypical skin lesions usually clear in the first year of dromic.16 The ordinary poikiloderma-like cutaneous life but may leave areas of atrophy, hypopigmentation, and telangiectasia, resulting in a poikilodermamyloidosis appears around the fifth decade of atous appearance.24 life and is characterized by the presence of d

d

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Fig 1. Diagrammatic representation of distribution of acquired poikiloderma. ACA, Acrodermatitis chronic atrophicans; AD, atopic dermatitis; EAI, erythema ab igne; MF, mycosis fungoides.

Crowley and Frieden25 reported a patient with neonatal lupus erythematosus and an unusual presentation in the form of generalized poikiloderma associated with extensive skin erosions, patchy scalp alopecia, absent eyebrows and eyelashes, absent neonatal lupus erythematosus classic skin lesions, and negative Ro and La antibodies associated with a strongly positive antinuclear antibody titer with a speckled pattern in both mother and infant. Dermatomyositis. Poikiloderma in dermatomyositis is often a late finding and it may occur on sun-exposed skin such as the V-shaped area of the neck and/or sun-protected skin such as the upper aspect of the back (shawl sign) (Fig 5) and the upperlateral aspect of thighs (holster sign).26-28 Sometimes the poikiloderma in dermatomyositis is more generalized and this may resemble a poikilodermic cutaneous T-cell lymphoma.29 The histopathological alternations found in poikilodermatomyositis (Fig 2) are just those of classic poikilodermatous changes.26 Systemic sclerosis. In systemic sclerosis, the most characteristic pigmentary change is loss of pigment in a large patch with perifollicular pigment retention. The affected areas become hairless and atrophic.30 Mat-like telangiectasia is found mainly on

the face but may extend as far as the upper aspect of thighs. Pigmentation occurs also most frequently on the face and to a lesser extent on the legs, thighs, and lower aspect of abdomen.9 Therefore, we believe that a mixture of these findings can result in a poikilodermic appearance. Environmental Solar radiation. Poikiloderma of Civatte. Poikiloderma of Civatte is a common condition with a slowly progressive and irreversible course.31 According to the predominance of 1 or more of the components of poikiloderma, some classify poikiloderma of Civatte into erythematotelangiectatic, pigmented and mixed types.32 The classic distribution of the lesions symmetrically involves the side of the face and neck and the upper aspect of the chest (Fig 6). Solar radiation has been proposed as the main culprit besides other factors such as genetics, low estrogen, and phototoxic or photoallergic reactions to chemicals in fragrances or cosmetics.32-34 The most prominent and constant histopathological feature, besides the classic poikilodermic changes, is solar elastosis of the papillary dermis.35 Photoaging ‘‘dermatoheliosis’’. Repeated solar injuries can ultimately result in photoaging or what

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Table I. Proposed etiological classification for acquired poikiloderma Infections d Borrelia burgdorferi: acrodermatitis chronica atrophicans of Lyme disease Inflammatory d Atopic dermatitis d Chronic graft-versus-host disease d Lichen planuseinduced poikiloderma Metabolic d Amyloidosis: poikiloderma-like cutaneous amyloidosis Connective tissue diseases d Lupus erythematosus d Dermatomyositis d Systemic sclerosis Environmental d Solar radiation: poikiloderma of Civatte and photoaging (dermatoheliosis) d Heat and infrared radiation: erythema ab igne d Chemical intoxication: sulfur mustard-induced poikiloderma Iatrogenic d Drugs Corticosteroids Hydroxyurea d Radiotherapy Chronic radiation dermatitis Neoplastic d Mycosis fungoides Poikilodermatous variant Granulomatous variant d Poikiloderma vasculare atrophicans d Poikilodermatous parapsoriasis

is called ‘‘dermatoheliosis.’’ This happens more in patients with fair skin, especially outdoor workers. The most common sites affected are the sun-exposed areas, particularly face and scalp in bald men.36 Cutaneous manifestations of dermatoheliosis (Fig 7) show a combination of atrophy, telangiectases, purpura, spotty depigmentation, hyperpigmentation solar lentigines, and actinic keratoses.36 Heat and infrared radiation: Erythema ab igne. Erythema ab igne is characterized by localized mottled and reticulated cutaneous hyperpigmentation with variable telangiectasia and skin atrophy resulting from repeated or prolonged exposure to heat and infrared radiation. Therefore poikiloderma is considered the end stage of the cumulative changes caused by repeated and prolonged exposure to infrared radiation.9 Erythema ab igne can occur at all ages including childhood37 but it was reported as a common condition on the shins among the elderly using heaters or fireplaces.38 The distribution of the lesions

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

depends on the direction of the incident radiation, the contour of the skin, and the interposition of clothing.9 In the early stages, epidermal atrophy, dermal pigmentation, and capillary dilatation are evident. Basophilic degeneration of the connective tissue, focal hyperkeratosis, and epithelial cellular atypia occur later.39 Chemical intoxication: Sulfur mustardeinduced poikiloderma. Sulfur mustard is a chemical weapon that was first used by the German Army in 191740 and was widely used in the 1980s during the Iran-Iraq conflict. Based on the lipophilic properties of this gas, it easily penetrates the skin and mucosal surfaces causing several acute and chronic effects on the skin, eye, and respiratory system.41 In 2011, Emadi et al42 proposed a diagnosis of sulfur mustardeinduced poikiloderma in a 41-yearold man who was injured with sulfur mustard during an Iraq chemical attack in 1988. Their diagnosis was based on the clinical findings of atrophy, pigmentation, and telangiectasia on the upper aspect of the trunk, back of the hands, and genitalia associated with relevant histopathological features of poikiloderma. Iatrogenic Drugs. Corticosteroids. Corticosteroids are accused of many undesirable side effects including atrophy, telangiectasia, and cutaneous dyspigmentation.43 Therefore, it can be expected that a constellation of these features will eventually result in a poikilodermic skin appearance. Hydroxyurea(hydroxycarbamide). Hydroxyurea is commonly used for myeloproliferative disorders and rarely for severe recalcitrant psoriasis.44 Major adverse reactions of hydroxyurea include both

Causes

History

Site

Other symptoms and signs

Histopathology

Achrodermatitis chronica atrophicans

Months or years after initial tick bite

Extensor surfaces of lower limbs

Arthritis, morphea-like or lichen sclerosus atrophicuselike lesions

Prominent plasma cell dermal infiltrate and destruction of appendages Spirochetes may be detected by WarthinStarry stain

Atopic dermatitis

Mostly in adults

Sides of neck

Itching and other atopic manifestations

Spongiotic dermatitis and poikiloderma

Chronic GVHD

Usually within 3 y post-HCT

Face and trunk during sclerodermoid reaction

Sclerodermoid phase is characterized by dermal fibrosis and destruction of adnexal structures

Lichen planuseinduced poikiloderma

Years after onset of classic lichen planus lesions Around fifth decade of life

May be generalized

Lichen planuselike, morphea-like, lichen sclerosus atrophicuse like lesions Other systemic manifestations, eg, esophageal web, bronchiolitis obliterans, and joint stiffness Classic lichen planus lesions Itching Lichen amyloidosis and cutaneous blisters

Poikiloderma-like cutaneous amyloidosis

Sun-exposed or sunprotected skin

Positive serology and PCR for Borrelia can confirm diagnosis Culture from atrophic skin may reveal organism Chemoprophylaxis and vaccination for Lyme disease may prevent condition in endemic regions Prolonged corticosteroid application may contribute to condition _

Only 1 case is reported

Disappointing therapeutic options

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Histologic changes of both lichen planus and poikiloderma Dermal amyloid deposits and positive Congo red Apple-green birefringence with polarized light

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Table II. Key differentiating points of acquired poikiloderma

Causes

History

Site

Other symptoms and signs

Histopathology

Lupus erythematosus

Patients with SLE and NLE With SCLE after sunburn After clearing lesions of lupus panniculitis

On sun-exposed sites, may be generalized

SLE and NLE: other cutaneous and systemic manifestations SCLE: other classic skin lesions

Patchy lymphocytic infiltrate of LE plus poikilodermatous changes

Dermatomyositis

Usually late finding during disease course

Arm extensors, V-area of neck, upper aspects of back and thighs or generalized

Poikilodermatous changes

Systemic sclerosis

Progressive

Usually on face

Poikiloderma of Civatte

After repeated UV exposure Slowly progressive and irreversible Chronic sun exposure especially in fair skin

Sun-exposed side of face and neck, upper aspect of chest with sparing of submental area Sun-exposed, eg, bald scalp in men

Photosensitivity, myositis Diagnostic signs of dermatomyositis, eg, Gottron sign and papules Other systemic manifestation Usually asymptomatic May be itching, burning, and flushing

Dermatoheliosis

EAI

Sulfur mustardeinduced poikiloderma

Corticosteroids

Chronic radiation dermatitis

Usually in patients with myeloproliferative disorders on hydroxyurea therapy History of exposure to ionizing radiation source

More on sun-exposed sites

At radiation-exposed site

Poikilodermatous changes plus solar elastosis

Actinic keratoses are invariably present

Solar elastosis plus poikilodermatous changes Usually asymptomatic Poikiloderma with Some reported itching and connective tissue burning sensation basophilic degeneration Eyes and respiratory Poikilodermatous changes system

Other side effects, eg, acneiform eruptions, hypertrichosis

Poikilodermatous changes

Other cutaneous adverse effects, eg, leg ulcers and dermatomyositislike eruption Other adverse reactions, eg, alopecia, nail loss, and tissue fibrosis

Poikilodermatous changes

Poikilodermatous changes and dermal fibrosis

Poikiloderma indicates advanced SLE Serologic tests can help to confirm diagnosis Anti-Ro and La were in the reported case about NLE and poikiloderma EMG and muscle biopsy specimen showing inflammatory myopathy Elevated CK, AST, ALT _ Patch test to diagnose cases induced by certain allergens IPL may help Proper photoprotection can limit occurrence Later, epithelial cell atypia can be found Was widely used in 1980s during Iran-Iraq conflict

Following safe guidelines for corticosteroid use can prevent this poikiloderma Other nondermatologic side effects can be found, eg, bone-marrow suppression Pulsed dye laser can improve telangiectasia

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Hydroxyurea

EAI starts 2 wk to several Any heat-exposed site can months after heat be affected but exposure commonly on shins Upper aspect of trunk, Years after exposure to back of hands, genitalia warfare agent with history of blistering after acute exposure Depends on potency, site, Sites of prolonged steroid and presence or application absence of occlusion

Dermal fibrosis

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Table II. Cont’d

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ALT, Alanine aminotransferase; AST, aspartate aminotransferase; CK, creatine kinase; EAI, erythema ab igne; EMG, electromyography; GVHD, graft-versus-host disease; HCT, hematopoietic cell transplantation; IPL, intense pulsed light; MF, mycosis fungoides; NLE, neonatal lupus erythematosus; PCR, polymerase chain reaction; SCLE, subacute cutaneous lupus erythematosus; SLE, systemic lupus erythematosus; UV, ultraviolet.

Usually shows mild dermatitis 6Itching Slowly progressive Poikilodermatous parapsoriasis

Trunk and flexural areas Slowly progressive Poikiloderma vasculare atrophicans

Lower aspect of trunk and upper aspect of thighs

It indicates poor prognosis

Granulomatous reactions with epidermotropism Like MF plus poikilodermatous changes Granulomatous MF

6Itching

CD4+ but rarely CD8+ phenotype can be found Poikilodermatous MF

Mainly on breasts and Other patches and It slowly progresses like hips plaques of classic MF patch stage of classic may be found MF but few cases proceed to tumor stage It is rare type with no distinct clinical presentation Histopathological features are essential for its diagnosis

Typical changes of MF and poikiloderma

Debatable term that is considered as poikilodermatous MF by many authors Confusing term that may be just prelymphomatous or true MF

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nondermatologic effects such as bone-marrow suppression, and dermatologic effects such as poikiloderma, ichthyosis, Gottron-like eruptions, nail abnormalities, oral ulceration and pigmentation, leg ulcers, actinic keratoses, and squamous cell carcinoma.45-47 Radiotherapy. Chronic radiation dermatitis. Chronic radiation dermatitis occurs after exposure to ionizing radiation used in tumor radiotherapy, and during interventional procedures such as fluoroscopic imaging.48 In chronic radiation dermatitis, persistent poikilodermatous changes are indicative of significant cutaneous injury.49,50

Neoplastic Mycosis fungoides. Poikilodermatous variant. Poikilodermatous MF is predominantly located on the breast (Fig 8), hips, and buttocks. These lesions may be associated with other patches and plaques of classic MF.4 Rarely, patients may have extensive poikiloderma as a feature of erythrodermic disease.9 The progression of poikilodermatous MF may be similar to that of the patch stage of classic MF, although a greater proportion of cases tend to show spontaneous regression and fewer patients proceed to tumor stage.51 A skin biopsy specimen shows histologic findings similar to those seen in long-standing patch- or plaque-stage MF lesions, in addition to the typical histopathological changes of poikiloderma.52 Immunophenotypic studies typically demonstrate a CD21, CD31, CD41, CD45RO1, CD8 , and CD7 pattern that is similar to that observed in classic MF. Rarely, cases of poikilodermatous MF are characterized by a CD81 cytotoxic T-cell phenotype.53 Granulomatous variant. Granulomatous MF is a rare type of cutaneous T-cell lymphoma that indicates a poor prognosis.54 It can only be diagnosed by the histopathological demonstration of granulomatous reaction because of the lack of a distinct clinical presentation.55 In 2008, Morihara et al55 reported a case granulomatous MF presenting clinically with poikiloderma, ichthyosis, and erythematous scaly plaques, and histologically by a granulomatous reaction with giant cells and epidermotropic atypical lymphocytes. Poikiloderma vasculare atrophicans. Poikiloderma vasculare atrophicans has been considered as poikilodermic MF56 and, according to Kreuter et al57 in 2005, the term ‘‘poikilodermatous variant of cutaneous T-cell lymphoma’’ has mostly replaced ‘‘poikiloderma vasculare atrophicans.’’ However, poikiloderma vasculare atrophicans has been

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Fig 3. Step-by-step approach to the management of the patient with acquired poikiloderma. ACA, Acrodermatitis chronica atrophicans; AD, atopic dermatitis; B burgdorferi, Borrelia burgdorferi; 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 lupus erythematosus; PC, poikiloderma of Civatte; PDL, pulsed dye laser; PLCA, poikiloderma-like cutaneous amyloidosis; PVA, poikiloderma vasculare atrophicans; SLE, systemic lupus erythematosus; SS, systemic sclerosis.

known to show a benign course, without progression to the tumor stage of MF.58 Besides poikiloderma, lesions of poikiloderma vasculare atrophicans usually manifest as asymptomatic or mildly pruritic flat-topped, scaly papules coalescing into retiform patterns or zebra-like distributions and associated with telangiectasia. The typical sites of predilection are the trunk and flexural areas.59

The histologic findings include those of poikiloderma and the classic histopathological and immunohistochemical findings of long-standing patch or plaque lesions of the classic MF.55,57 Poikilodermatous (large-plaque) parapsoriasis. The term ‘‘parapsoriasis’’ has caused much confusion since its introduction in 1902.9 Two types of parapsoriasis en plaque have been described: large-plaque parapsoriasis and small-plaque

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Fig 4. Poikiloderma-like cutaneous amyloidosis of breast.

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

Fig 5. Poikiloderma of back in patient with dermatomyositis ‘‘shawl sign.’’

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

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

parapsoriasis.60 Large-plaque parapsoriasis (or poikilodermatous parapsoriasis) is considered a prelymphomatous skin condition because of its biologic behavior and the presence of clinical, histologic, and/or genotypical alterations that overlap early MF.61 In a large series of patients with poikilodermatous parapsoriasis, 11% developed definite MF but whether these cases were MF from the start or not remains 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 result of epidermal atrophy, then telangiectasia and mottled pigmentation can be observed, hence the synonym ‘‘poikilodermatous parapsoriasis.’’64 Common sites include the lower aspect of the trunk, upper aspect of thighs, and major flexural surfaces.63 Lesions at the breast and buttock should suggest MF.62 Most skin biopsy specimens only show a mild dermatitis65 and a superficial bandlike dermal lymphocytic infiltrate beneath atrophic epidermis.66 Single, rarely atypical, lymphocytes without Pautrier microabscesses can be observed in the epidermis in the absence of spongiosis.67

TREATMENT Treatment of acquired poikiloderma includes the use of a specific therapy, cosmetic management, or both. Moreover, some causes can be prevented such

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as the use of chemoprophylaxis and vaccination in Lyme diseaseeendemic regions,68 proper photoprotection and patch testing for poikiloderma of Civatte,69 avoidance of prolonged heat or infrared contact for erythema ab igne,38 and following the safe guidelines for topical corticosteroid use.70 Topical and intralesional corticosteroid, hydrocolloid dressings, topical dimethylsulfoxide, calcineurin inhibitors, acitretin, cyclophosphamide, dermabrasion, phototherapy, and pulsed-dye laser have been all used for poikiloderma-like cutaneous amyloidosis, but are usually disappointing.71 Laser therapy using argon or pulsed dye laser can improve poikilodermatomyositis,72 whereas the results in poikiloderma of Civatte are unsatisfactory. Also, hydroquinones, chemical peels,38 electrosurgery, and cryotherapy all failed in treating poikiloderma of Civatte.73 The new intense pulsed light sources are more promising with clearance of more than 75% of telangiectasias and hyperpigmentation in different studies.34 Topically applied retinoids may reverse some of the changes of dermatoheliosis.36 In chronic radiation dermatitis, hyperbaric oxygen can reduce pain and erythema74 but it has no effect on telangiectasia75 for which pulsed dye laser may be beneficial.76 The standard treatment for early poikilodermatous MF includes topical steroids, topical chemotherapy, psoralen plus ultraviolet A, narrowband ultraviolet B, interferon alfa-2a, and oral retinoids. Systemic chemotherapies, denileukin diftitox, antiCD52 antibody, and interleukin-12 are reserved for advanced stages.77 Therapeutic options for poikilodermatous parapsoriasis include high-potency topical steroids, phototherapy, and topical nitrogen mustard.64

CONCLUSION Acquired poikiloderma is a divergent condition that has been attributed to different etiological factors. We hope that our proposed classification and diagnostic approach, including the key differentiating points, may help in easy and precise diagnosis of poikiloderma and its underlying causes. Although the histopathological features are nonspecific, a precise evaluation can lead to the diagnosis of serious diseases as is the case with poikilodermatous MF. Treatment of poikiloderma is that of the cause and laser therapy may be beneficial in some cases; however, the results are usually unsatisfactory. REFERENCES 1. Hughes R, Loftus B, Kirby B. Subacute cutaneous lupus erythematosus presenting as poikiloderma. Clin Exp Dermatol 2009;34:e859-61.

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