Vesicular Palmoplantar Eczema is a Term Used to Describe a Group of Diseases Characterized by...
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Vesicular palmoplantar eczema is a term used to describe a group of diseases characterized by vesiculobullous eruption involving mainly the hands and feet. Clinical presentations vary from acute dermatitis to more chronic relapsing and remitting disease patterns. The diversity of presentation has created challenges in classifying hand eczema. A 2011 publication assembled an algorithm for chronic hand eczema based on etiology, morphology and clinical features . [1] Although considerable overlap exists in the various forms of vesicular palmoplantar eczema, the disease can be roughly divided into 4 distinct categories: pompholyx, subacute or chronic relapsing vesiculosquamous eczema, chronic vesiculohyperkeratotic or hyperkeratotic eczema, and id reactions. Pompholyx ("blister" or "bubble" in Greek) may be further subdivided into vesicular and bullous forms, in which patients present with acute severe eruptions of blisters over their palms and, less commonly, the soles. Chronic vesiculosquamous eczema, also called dyshidrotic eczema, was initially thought to be caused by abnormal functioning of the sweat glands. This association has since been disproved, but the term dyshidrotic eczema is still used. Patients with this variant present with small (1-2 mm) vesicles on nonerythematous skin involving the inner sides of the fingers or on the palms and soles. The vesicles are pruritic, last 1-2 weeks, desquamate, and then recur at unpredictable intervals. The chronic hyperkeratotic variety involves mainly the central palms, where it causes thickening and fissures. This category is notoriously the most difficult to treat. An id reaction refers to vesicular eruption of the hands, caused by a distal focus of infection, with fungal infections being the most common. Despite the wide range of clinical presentations, all 4 types of vesicular palmoplantar eczema are histologically characterized by features of dermatitis, such as spongiosis and exocytosis. Pathophysiology Vesicular palmoplantar eczema is often thought to have an unidentified intrinsic cause. Although many etiologic factors are described, the underlying pathology of vesicular palmoplantar eczema is unknown. Similarly, although certain triggers have been associated with the development or worsening of symptoms, how these triggers cause flares has not been elucidated. Vesicular palmoplantar eczema results in histologic evidence of dermatitis, such as spongiosis, which is often accompanied by lymphocytic infiltrates. Epidemiology Frequency United States The frequency of vesicular palmoplantar eczema in the United States is unknown. International The true incidence is unknown, but vesicular palmoplantar eczema is probably responsible for 5-20% of all cases of eczema of the hand. A 2012 study found pompholyx accounted for 14% of all cases of hand eczema. [2] Mortality/Morbidity Patients with mild cases of pompholyx have an excellent prognosis. The more severe chronic hyperkeratotic variety of vesicular palmoplantar eczema often requires lifelong treatment and results in considerable disability. Sex The male-to-female ratio for vesicular palmoplantar eczema is 1:1. Age Pompholyx most commonly occurs in patients aged 20-40 years, but it may occur in individuals of any age. Onset in patients younger than 10 years is unusual. The frequency of recurrent episodes of
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Vesicular palmoplantar eczema is a term used to describe a group of diseases characterized byvesiculobullous eruption involving mainly the hands and feet. Clinical presentations vary from acutedermatitis to more chronic relapsing and remitting disease patterns. The diversity of presentation hascreated challenges in classifying hand eczema. A 2011 publication assembled an algorithm for chronic hand eczema based on etiology, morphology and clinical features.[1]
Although considerable overlap exists in the various forms of vesicular palmoplantar eczema, thedisease can be roughly divided into 4 distinct categories: pompholyx, subacute or chronic relapsingvesiculosquamous eczema, chronic vesiculohyperkeratotic or hyperkeratotic eczema, and idreactions.
Pompholyx ("blister" or "bubble" in Greek) may be further subdivided into vesicular and bullousforms, in which patients present with acute severe eruptions of blisters over their palms and, lesscommonly, the soles.
Chronic vesiculosquamous eczema, also called dyshidrotic eczema, was initially thought to becaused by abnormal functioning of the sweat glands. This association has since been disproved, butthe term dyshidrotic eczema is still used. Patients with this variant present with small (1-2 mm)vesicles on nonerythematous skin involving the inner sides of the fingers or on the palms and soles.The vesicles are pruritic, last 1-2 weeks, desquamate, and then recur at unpredictable intervals.
The chronic hyperkeratotic variety involves mainly the central palms, where it causes thickening andfissures. This category is notoriously the most difficult to treat.
An id reaction refers to vesicular eruption of the hands, caused by a distal focus of infection, withfungal infections being the most common.
Despite the wide range of clinical presentations, all 4 types of vesicular palmoplantar eczema arehistologically characterized by features of dermatitis, such as spongiosis and exocytosis.
PathophysiologyVesicular palmoplantar eczema is often thought to have an unidentified intrinsic cause. Althoughmany etiologic factors are described, the underlying pathology of vesicular palmoplantar eczema isunknown. Similarly, although certain triggers have been associated with the development or worsening of symptoms, how these triggers cause flares has not been elucidated.
Vesicular palmoplantar eczema results in histologic evidence of dermatitis, such as spongiosis, whichis often accompanied by lymphocytic infiltrates.
EpidemiologyFrequency
United States
The frequency of vesicular palmoplantar eczema in the United States is unknown.
International
The true incidence is unknown, but vesicular palmoplantar eczema is probably responsible for 5-20%of all cases of eczema of the hand. A 2012 study found pompholyx accounted for 14% of all cases of
hand eczema.[2]
Mortality/Morbidity
Patients with mild cases of pompholyx have an excellent prognosis. The more severe chronichyperkeratotic variety of vesicular palmoplantar eczema often requires lifelong treatment and resultsin considerable disability.
Sex
The male-to-female ratio for vesicular palmoplantar eczema is 1:1.
Age
Pompholyx most commonly occurs in patients aged 20-40 years, but it may occur in individuals of anyage. Onset in patients younger than 10 years is unusual. The frequency of recurrent episodes of
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pompholyx decreases after middle age, although this is not true of chronic vesicular andhyperkeratotic variants.
HistoryThe severity of vesicular palmoplantar eczema symptoms varies, ranging from mild discomfort toacute severe episodes. Patients rarely require hospitalization.
Classically, itching, burning, and prickling sensations of the palms and soles precede the eruption of vesicles.
Thereafter, small (1- to 2-mm) vesicles form, most commonly on the lateral sides of the fingers. Inpompholyx, the central areas of the palms and soles may or may not be involved.
Large vesicles can develop on the palms and soles and may coalesce to form confluent bullae.
The lesions last for 2-3 weeks, after which spontaneous resolution generally occurs. Occasionally,large bullae may need to be aspirated. This phase is followed by desquamation.
Chronic forms typically recur, and episodes are more frequent during the spring and summer than inthe fall and winter.
The chronic hyperkeratotic variety results in severe itching accompanied by thickening and fissuringof the palm. This effect may decrease the mobility of the affected hand.
PhysicalClinical signs depend on the stage of vesicular palmoplantar eczema. An absence of erythema isoften an important clinical feature in the acute and chronic forms.
Pompholyx of the palms. Acute episodes are characterized by a sudden onset of small, clear vesicles or bullae that are said tobe sagolike or tapiocalike in appearance (see the image above).
Vesicles and/or bullae are accompanied by severe, occasionally painful pruritus.
Small vesicles may enlarge or become more confluent and present as large bullae (especially onthe palms and soles).
Vesicles and bullae subsequently dry out and resolve, usually without rupturing.
In most individuals, desquamation occurs 2-3 weeks after the onset of vesicles and bullae.
In some patients, a milder recurrence follows the initial severe episode. Secondary infections, such as impetigo, cellulitis, or lymphangitis, are possible in patients with
recurrent hand eczema.
Secondary nail changes (eg, dystrophic nails, irregular transverse ridging, pitting, thickening,discoloration) can also occur.
Subacute vesicular eczema tends to have a chronic relapsing course with more vesiculation and moreerythema in the acute phases than in later phases.
Residual erythema or some dryness or scaling occurs in the less-active phases.
Fissures are common and painful sequelae. A form of microvesicular palmar eczema also occurs in association with dry nummular (discoid)eczema.
When they occur on the hands, id reactions typically involve the lateral sides of the fingers. Thesereactions often resolve when the primary infection is treated
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PhysicalClinical signs depend on the stage of vesicular palmoplantar eczema. An absence of erythema isoften an important clinical feature in the acute and chronic forms.
Pompholyx of the palms. Acute episodes are characterized by a sudden onset of small, clear vesicles or bullae that are said to
be sagolike or tapiocalike in appearance (see the image above).
Vesicles and/or bullae are accompanied by severe, occasionally painful pruritus.
Small vesicles may enlarge or become more confluent and present as large bullae (especially onthe palms and soles).
Vesicles and bullae subsequently dry out and resolve, usually without rupturing.
In most individuals, desquamation occurs 2-3 weeks after the onset of vesicles and bullae.
In some patients, a milder recurrence follows the initial severe episode.
Secondary infections, such as impetigo, cellulitis, or lymphangitis, are possible in patients withrecurrent hand eczema.
Secondary nail changes (eg, dystrophic nails, irregular transverse ridging, pitting, thickening,discoloration) can also occur.
Subacute vesicular eczema tends to have a chronic relapsing course with more vesiculation and more
erythema in the acute phases than in later phases.
Residual erythema or some dryness or scaling occurs in the less-active phases.
Fissures are common and painful sequelae. A form of microvesicular palmar eczema also occurs in association with dry nummular (discoid)eczema.
When they occur on the hands, id reactions typically involve the lateral sides of the fingers. Thesereactions often resolve when the primary infection is treated
CausesThe etiology of hand eczema is unknown, but most observers suggest that intrinsic changes in theskin are responsible for vesicular palmoplantar eczema. A study of an autosomal dominant form of pompholyx found a genetic linkage on chromosome 18.[3] Whether other forms have a similar geneticlinkage is not clear. However, several exogenous factors have been implicated in the causation or worsening of vesicular palmoplantar eczema.[4]
Coexisting atopy is common in patients with palmoplantar eczema. A recent study found a strongassociation between pompholyx and atopic status.[2]However, this is by no means the only causalrelationship because many patients have no history of atopy.
Emotional stress may also trigger episodes.
Seasonal changes seem to be directly related to relapses, as episodes are most common in thespring and summer months. Warm weather has been known to initiate episodes, with several casesreporting photo-induced pompholyx.
o Although dysfunction of the sweat glands is no longer accepted as the cause of dyshidroticeczema, increased sweating seems to exacerbate the condition and many patients with palmar hyperhidrosis also have coexisting dyshidrotic eczema.
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o Photosensitivity to ultraviolet A (UVA) has been reported as an etiologic factor in a small subset of patients with eczema.[5, 6] Therefore, worsening of the disease in summer months may be due tothe increase in exposure to sunlight. Conversely, UVA therapy is a widely accepted form of treatment for palmoplantar eczema.[7]
Sensitivity to certain metals, particularly nickel and cobalt, has been linked to vesicular palmoplantar eczema.
Exogenous factors causing allergic contact pompholyx include balsams and cosmetic and hygieneproducts.[8]
Drugs responsible for inducing episodes include oral contraceptive pills and aspirin. Palmoplantar eczema occurring after intravenous immunoglobulin (IVIG) therapy is reported.[9] One case reportdescribes occurrence in the pediatric population after IVIG administration for Kawasakisyndrome.[10] A review of eczematous reactions linked with IVIG therapy cited pompholyx asoccurring in 62.5% of the cases reported to have an eczematous reaction associated with IVIG.[7]
Fungal infections, particularly tinea pedis, are most commonly implicated in id reactions. Bacterialinfections play a role in both causation and in secondarily infecting lesions.
Cigarette smoking may reduce the efficacy of topical therapy with psoralen and UVA (PUVA) andhas been itself, linked to pompholyx.
HIV infection has been associated with pompholyx, with response to antiretroviral therapy;conversely, one case report describes of 2 HIV-positive patients who developed severe dyshidrotic
eczema after starting antiretroviral treatment, thought to be due to an immune reconstitutioninflammatory syndrome.[11, 12]
Differential Diagnoses Contact Dermatitis, Allergic
Contact Dermatitis, Irritant
Lichen Planus
Pityriasis Rubra Pilaris
Psoriasis, Pustular
Syphilis
Laboratory StudiesThe diagnosis of palmoplantar eczema is essentially a clinical one, and laboratory tests are notroutinely performed. However, laboratory studies may be helpful in excluding other disorders.
Elevated serum immunoglobulin E levels or positive results on prick tests may suggest an atopictendency.
Skin scrapings can be used to exclude the presence of a fungus.
Skin swabs may exclude bacterial infection.
Perform KOH staining of skin scrapings to rule out fungal infection, especially in hyperkeratotic formsof the disease.
Swab and culture suspected lesions to exclude secondary bacterial infection.
ProceduresPerform patch tests to exclude contact dermatitis or a systemic reaction to contact allergen.
Perform biopsy to distinguish eczema from psoriasis or some forms of palmoplantar hyperkeratoses
Histologic FindingsHistologic features vary according to the stage of the evolution vesicular palmoplantar eczema.Usually, evidence suggests intracellular edema or spongiosis, lymphocytic infiltration of the epidermis,and intraepidermal vesicles or bullae in acutely affected persons. In chronically affected persons,spongiosis is present and often associated with epithelial proliferation and/or hyperkeratosis or psoriasiform epidermal hyperplasia. Dermis is often edematous, with a mixed perivascular
inflammatory cell infiltrate.
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Medical CareSeveral modalities of therapy are available for the treatment and control of vesicular palmoplantar eczema. Therapy should be chosen according to the type and severity of the condition. Whenever possible, eliminate known triggers. If pruritus is a problem, antihistamines (eg. hydroxyzine) canrelieve some symptoms.
Preventative measures
Regular use of hand emollients and avoidance of frequent contact with irritants are important meansto prevent flare-ups of vesicular palmoplantar eczema. Contact allergy has been noted in one study tobe responsible for 67.5% of pompholyx eczema, and all patients should be considered for patchtesting to identify relevant allergens.[8]
Topical therapy
Topical therapy for vesicular palmoplantar eczema includes high-potency glucocorticoids, Burowsolution (aluminum acetate 1% or potassium permanganate solution [1:8000 dilution]), tacrolimus,and/or psoralen plus UVA (PUVA).
Topical high-potency glucocorticoids, such as betamethasone dipropionate and clobetasolpropionate, are first-line therapies. Application of these medications under plastic and vinylocclusion enhances their efficacy. However, this method may predispose the patient to secondarybacterial or fungal infection and to both local and systemic adverse effects of corticosteroids.Therefore, it should be used only intermittently and should never be used in the presence of coexisting infection.
Patients with mild vesicular palmoplantar eczema may be controlled with the use of less potentcorticosteroids such as betamethasone valerate, triamcinolone, or mometasone.
Acute, severe episodes of pompholyx benefit from rest, and bland applications with wet soaks andcompresses and with drying agents such as Burow solution. Occasionally, large blisters may needto be aspirated.
Newer agents, such as topical tacrolimus and pimecrolimus, have been shown to be as effective asmometasone furoate in the treatment of chronic relapsing eczema of the hands.[14] These topical
immunomodulators may be used as steroid-sparing agents to treat resistant palmar eczema, withminimal systemic absorption or systemic effect.[15] Use of other agents should be considered whenplantar eczema is being treated because this therapy is less effective on the soles of the feet thanon the hands. The use of occlusion with these agents has also been shown to increase their efficacy.
A small open-label study demonstrated efficacy of topical vitamin D-3 derivatives (ie, calcipotriol,maxacalcitol) for the control of hyperkeratotic palmoplantar eczema.[16]
Systemic therapy
Systemic therapy includes steroids, immunosuppressive agents (eg, azathioprine,[17] cyclosporine),retinoids (eg, acitretin, alitretinoin), and PUVA.
Consider the use of systemic glucocorticoids or intralesional steroids in acute episodes of vesicular
palmoplantar eczema when local therapy fails. These agents are not helpful for long-term treatmentbecause of a potential for severe adverse effects.
Cyclosporine, mycophenolate mofetil, and methotrexate either alone or in combination with steroidsmay be used for severe, recalcitrant cases of vesicular palmoplantar eczema.[18, 19] These therapieshave also been tried as steroid-sparing agents in chronic relapsing eczema.
For hyperkeratotic eczema, consider the use of aromatic retinoids, such as acitretin, which helpcontrol hyperkeratosis. These agents are best used in relatively low doses because of adverseeffects. Therapy may need to be continued indefinitely in cases of hyperkeratotic eczema and isoften accompanied by topical occlusive therapy, with combined or alternating steroids andkeratolytics (5-20% salicylic acid) or tar preparations.
Increasingly, the retinoid alitretinoin has been shown to be a favorable option for severe handeczema. One randomized, double-blind, placebo-controlled multicenter trial examining severechronic hand eczema found alitretinoin superior to placebo, with 48% of patients achieving full or
almost full resolution of signs and symptoms.[20] A 2012 observational study noted alitretinoinimproved vesicular eczema in 47.9% of patients.[21]
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The use of etanercept in a case study achieved a 4-month remission of vesicular palmoplantar eczema, which was followed by relapse.[22]
Phototherapy has been shown to be effective in dyshidrotic eczema, in particular PUVA. However,the use of psoralen has been associated with carcinogenic risk of the skin. Although conventionallyused with psoralen for its photosensitizing effects, UVA-1 alone has also shown success in treatingpalmoplantar eczema, with the advantage that it does not require psoralen.[23, 24] PUVA can be
administered orally or topically. In a study comparing the effectiveness of the 2 modalities,dyshidrotic eczema responded well to both oral and topical (bath) treatment, while hyperkeratoticeczema cleared significantly better with oral therapy than with topical (bath) PUVA.[25] NarrowbandUVB therapy has been shown to be equally efficacious as PUVA therapy and can be used as analternative to PUVA, with fewer adverse effects.[26]
Other therapies
Other treatment options[27] f or vesicular palmoplantar eczema that have been reported includetreatment with intradermal injections of botulinum toxin A, x-ray therapy, disulfiram for nickel-induceddisease, and, in patients with obstructive sleep apnea, continuous positive airway pressure (CPAP).
Botulinum toxin A is a potent neurotoxin that blocks the autonomic cholinergic fibers.[28, 29] It has beenshown to improve symptoms of itching and vesicular formation in a controlled left-right hand
comparison study with 8 subjects.
[30]
This therapy may be used alone or in combination with topicalsteroids. However, the mechanism of action in reducing the severity of palmoplantar eczema isdisputed. Some proposed mechanisms are a disruption of the afferent nerve supply of the skin, whichmay reduce sweating, because sweat is known to exacerbate the condition. Another mechanism isthe possible effect of the toxin on afferent nerve fibers. Blockade of the inflammatory process andinhibition of neuropeptides such as substance P via toxic effects may explain the reduction of pruritusin treated patients.
The inflammatory cells functioning in eczema are highly radiosensitive, and, therefore, x-rayirradiation has been used in some patients with resistant chronic eczema of the hand when other treatments have not been successful.
Grenz rays and superficial radiotherapy were popular treatments for chronic severe hand eczema inthe 1980s; however, they have currently decreased in popularity, mostly because of a lack of
availability than because of the risk for potential carcinogenesis. Superficial radiation therapyappears to have a higher success rate than grenz ray therapy because of its deeper penetration intothe skin.[31]
One study revealed excellent results with the use of superficial radiation for palmoplantar eczema,while others have not.[32, 33] However, the potential risk of irradiation for a benign non –life-threateningdisease must be recognized, and care must be taken not to exceed the maximum safe cumulativelifetime dose by using dosimetry. External-beam megavoltage radiation therapy was reportedlysuccessful in treatment of this condition in one patient.
Disulfiram may be administered as a nickel-chelating agent in patients with known nickel sensitivity,but this should not be used in thiuram-sensitive patients.
One case report describes a patient with obstructive sleep apnea and dyshidrotic palmar eczemawhose dermatitis resolved after being placed on a CPAP machine. The authors speculated the
resolution of the eczema may reflect the effects of increased tissue oxygenation and decreasedcirculating inflammatory factors associated with better sleep quality.[34]
Id reactions tend to resolve with treatment of the primary infection. Consider systemic antibiotics if secondary infection is suspected, and culture suspicious lesions.
onsultationsRefer patients to a dermatologist because vesicular palmoplantar eczema is likely to be a lifelongdisease (albeit intermittent in some patients).
DietMaintaining a low-cobalt diet has been suggested to decrease the number of dyshidrotic eczemaflares.[35]
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Patients with established nickel sensitivity may benefit from nickel-free diets. Medication SummaryThe goals of pharmacotherapy for vesicular palmoplantar eczema are to reduce morbidity and toprevent complications.
The dyshidrotic eczema severity index (DASI), a standardized severity scale for palmoplantar eczema, has made it easier to compare the efficacy of various therapies in controlled clinical trial
Class Summary
These agents have anti-inflammatory properties and cause profound and varied metabolic effects.They modify the immune response of the body to diverse stimuli.
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Betamethasone topical (Diprolene, Luxiq)
For inflammatory dermatoses responsive to steroids. Decreases inflammation by suppressing
migration of polymorphonuclear leukocytes and reversing capillary permeability. Affects production of lymphokines and has inhibitory effect on Langerhans cells.
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Clobetasol (Temovate, Olux-E, Temovate E, Cormax)
Class I superpotent topical steroid; suppresses mitosis and increases synthesis of proteins thatdecrease inflammation and cause vasoconstriction.
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Prednisone
Immunosuppressant to treat autoimmune disorders; may decrease inflammation by reversingincreased capillary permeability and suppressing PMN activity. Stabilizes lysosomal membranes andsuppresses lymphocytes and antibody production.
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