VESICULAR
PALMOPLANTAR ECZEMA
Key points
|
· An acute and/or chronic
dermatitis clinically characterized by small to large blisters on the palms
and soles and histopathology by spongiotic vesicles. ·
Considered an endogenous dermatitis, but can be exacerbated by
exogenous factors, most notably irritant and allergic contact dermatitis. In
addition, atopy may in some cases predispose to the development of vesicular
palmoplantar eczema. · Can be divided into four
categories: (1) pompholyx, (2) chronic vesiculobullous hand dermatitis, (3)
hyperkeratotic hand dermatitis, and (4) id reactions. · Does not respond well to
treatment. |
Pompholyx
Salient features
·
Firm,
pruritic vesicles of the palms, soles, and lateral and medial aspects of the
fingers and toes
·
Association
with atopic dermatitis and contact dermatitis (allergic and irritant)
·
No
disturbance of sweat gland function
Introduction
Pompholyx is an idiopathic,
common, chronic, relapsing palmoplantar eczematous dermatosis characterized by
firm, pruritic vesicles and bullae. Pompholyx is not an independent disease entity because it is often a
manifestation of other types of eczema, especially atopic dermatitis and
irritant or allergic contact dermatitis.
Pompholyx is a special vesicular type of hand and foot
dermatitis. Acute, chronic or recurrent dermatosis of the fingers, palms, and
soles.
Pompholyx is a form of eczema of the palms and soles in
which edema fluid accumulates (spongiosis) within the epidermis to form visible
vesicles or bullae. As a result of the thick tear-proof horny layer in these
sites, the blisters tend to become relatively large before they burst.
Pompholyx probably accounts for about 5–20% of all cases of hand eczema. When
pompholyx occurs on the palms, it may be called ‘cheiropompholyx’, and when on
the soles, ‘podopompholyx’. The term ‘dyshidrotic eczema’ as an alternative to
pompholyx hand eczema has been dropped because no causal relationship with the
sweat glands or sweating has been demonstrated.
Pathogenesis
Although
the formation of vesicles is not linked to sweat gland dysfunction or trapping
of sweat within the epidermis, hyperhidrosis can be an aggravating factor in
some patients. Notably, treatment of hyperhidrosis with onabotulinum toxin A
may ameliorate pompholyx. Pompholyx is
frequently an expression of atopic dermatitis, particularly as a late-stage
manifestation. In these patients, its intensity is usually low to moderate with
a protracted and relapsing course, and it is accompanied by only a few other
signs of atopic dermatitis. Less often, pompholyx is a
manifestation of acute or subacute allergic contact dermatitis. Flares can also
result from exposure to known irritants.
Occasionally, administration of
IVIg is followed by acute episodes of pompholyx. The
role of ingestants, in particular nickel and cobalt, has been debated, but
improvement with a low-nickel diet (in nickel-sensitive patients with a
positive oral provocation test) has been observed. Lastly, flares can also
follow periods of emotional stress as well as exposure to hot climates and
rarely sunlight.
Clinical Features
Pompholyx
It is characterized by explosive outbreak (sudden onset) of symmetric, firm, deep-seated vesicles of the palms, the lateral
and medial aspects of the fingers, and less often the soles and toes.
Discomfort and itching usually precede the development of the vesicles. The
size of the vesicles may vary from pinhead-sized to several centimeters. Intact
large bullae can be drained, but should not be unroofed. However, large bullae
may rupture spontaneously, leaving oozing or dried up erosions. When smaller
lesions are clustered, they have been likened to tapioca pudding. While the
vesicles initially contain clear fluid, they have a tendency for purulent super
infection. This acute phase resolves via desquamation of characteristically
thick scales. Later
fissures and lichenification may occur. Individual outbreaks are usually self-limited over 2-3
weeks. Recurrent
attacks are the rule. Attacks are most common among
adolescents and young adults and seem to be more common in the spring and
summer months.
Histologically,
spongiotic dermatitis is seen with the formation of micro- and macrovesicles
within the epidermis. There is no association with sweat glands.
Keratolysis exfoliativa (recurrent focal palmar
peeling)
It is a
chronic, asymptomatic, and noninflammatory peeling of the on the sides of the fingers and on the palms or on the feet,
which displays no blisters and most commonly seen during the summer months. It is thought to occur more
frequently in people with hyperhidrosis in these areas. Scaling usually starts
from one to two fine points and expands outward to form small to larger annular collarettes of white scale. The condition is usually
self-limited and requiring only emollients.
The condition is sometimes a mild form of pompholyx. Some patients subsequently
develop true pompholyx.
Chronic Vesiculobullous
Dermatitis
Chronic vesiculobullous hand dermatitis is more common than pompholyx
and more difficult to manage because of its relapsing course. The clinical
presentation includes small 1- to 2-mm vesicles filled with clear fluid
localizing to the lateral aspects of the fingers, palms, and soles as in
pompholyx. As the condition becomes more chronic, the clinical appearance may
evolve and subsequently appear more fissured and hyperkeratotic. A clear
history of vesicles or exacerbations characterized by blistering may help to
narrow the classification of a given presentation of hand dermatitis.
Hyperkeratotic Hand Dermatitis
Patients with hyperkeratotic hand dermatitis are usually middle-aged to
elderly male and generally present with chronic hyperkeratotic scaly, pruritic
plaques, sometimes with fissures on the central palm and are often very
refractory to treatment. Plantar involvement is present in a minority of cases.
ID Reaction (Auto
sensitization dermatitis)
Auto sensitization dermatitis refers to a phenomenon in which an acute
dermatitis develops at cutaneous sites distant from an inflammatory focus, and
where the secondary acute dermatitis is not explained by the inciting cause of
the primary inflammation. The phenomenon results from the release of cytokines in
the primary dermatitis, as a result of sensitization. These cytokines circulate
in the blood and heighten the sensitivity of the distant skin areas. The
diagnosis of auto sensitization dermatitis is often post hoc; i.e. the distant
eruption disappears when the primary dermatitis is controlled.
Disseminated eczema appears later
than the primary lesions. Typically, 1 to 2 weeks after an acute inflammation,
there is sudden development of an extremely pruritic, and symmetric, scattered,
erythematous eruption with macules, papules, and vesicles develop. Predilection
for analogous body sites (e.g. extensor aspects of the lower and upper
extremities, palms and soles).
The eruption involves the
forearms, thighs, legs, trunk, face, hands (lateral aspects of the fingers and
the palms), neck, and feet in descending order of frequency. During the
evolution of the dermatitis, its morphology may change in a manner consistent
with the chronicity (i.e., vesicles to scale).
For example, a
patient with venous stasis dermatitis on the lower legs may develop pruritic,
symmetric, scattered, erythematous, maculopapular, or papulovesicular lesions
on the trunk, forearms, thighs, or legs. Similarly, auto sensitization may
occur as an "id reaction in inflammatory tinea pedis and manifests as a
dyshidrosiform, vesicular eruption on the feet and hands and papulovesicular
eczematoid lesions on the trunk.
These persist and
spread until the basic underlying primary dermatitis is controlled.
Treatment of the underlying disease
results in resolution.
Diagnosis
The diagnosis of vesiculobullous hand dermatitis is usually made on the
basis of clinical presentation, history, and sometimes histology.
Differential
diagnosis
In most cases of psoriasis on the
hands, however, the silvery nature of the scale, involvement of the knuckles,
sharply demarcated ‘scalloped’ edges to the erythema along the borders of the
hands and fingers, and the relative absence of pruritus are helpful pointers. A
family history of psoriasis and the presence of nail pits in the absence of
nail fold lesions are also suggestive.
Tinea manuum can be missed,
particularly when it is extensive or secondarily infected. Unilateral scaling
of the palm should always suggest a possible Trichophyton infection.
Pompholyx can resemble palmoplantar pustulosis.
Clinically, the two conditions are distinguished by the presence of vesicles in
the former and sterile pustules that resolve with characteristic brown marks in
the latter. Repeated attacks of pompholyx may produce hyperkeratotic lesions
that mimic psoriasis vulgaris.
Atopic hand dermatitis is
associated with a number of factors: hand dermatitis before age 15 years,
persistent eczema on the body, dry or itchy skin in adult life, and widespread
atopic dermatitis in childhood. The backs of the hands, particularly the
fingers, are affected with erythema, vesiculation, crusting, excoriation, and
scale.
It
must be emphasized that the whole skin should be examined in any case of hand
eczema in which the diagnosis is in doubt. There may, for example, be evidence of
nickel allergy or tinea pedis, or small patches of psoriasis of which the
patient is unaware.
Disease
course and prognosis
Unless a responsible allergen can be
identified and removed, the prognosis of hand eczema for an individual is
uncertain. Even if a relevant allergen is identified, it may be difficult to
avoid the offending allergens such as nickel and fragrance. Atopic hand eczema
probably has the worst prognosis of all types of hand eczema. In general,
eczema on the dorsa of the hands clears more readily, and is less likely to
recur than palmar eczema.
Pompholyx has an unpredictable course. Following an acute attack of pompholyx, about one‐third of patients experience no
further episodes, one‐third
suffer from recurrent episodes and in the remainder the condition develops into
a chronic, possibly hyperkeratotic phase. Those forms of hand eczema that are
due to the effects of irritants carry a particularly poor prognosis unless
these irritants can be completely removed. The condition seems to flare when a susceptible
person is under stress that is greater than can be handled with serenity.
Patients who have suffered from
severe hand eczema will often remain vulnerable to mild irritants for several
months after the eczema has apparently cleared. Interdigital dermatitis has
been shown to be a potential precursor to more severe hand dermatitis in
hairdressers. Recognition of this sign by the patient may allow early
intervention to prevent progression of the disease.
Investigations
A
circumscribed and asymmetrical area of scaling and vesiculation of the palm or
sole should suggest the possibility of dermatophytosis, and scrapings should be
examined for fungus. If the erythema is limited to one or two interdigital
clefts, or is asymmetrical, or involves the dorsal skin to any extent, the
possibility of a contact dermatitis must be considered and investigated by
patch testing. If there are immediate symptoms on wearing latex gloves, then
type 1 latex hypersensitivity should be excluded with latex prick testing.
Chronic
hand eczema
Topical
treatment
Management of chronic hand eczema,
defined as persisting for at least 6 weeks, and involves the avoidance of
irritants, frequent application of emollients and use of topical corticosteroids
when indicated.
Avoidance of irritants is
particularly difficult for patients with hand eczema because they are so
ubiquitous. Patient education is of paramount importance and this can be
reinforced by printed advice sheets. Gloves usually provide the best protection
against irritants. Rubber gloves generally give good protection for housework.
In patients with a rubber allergy, polyvinyl chloride household gloves should
be worn instead, but some allergens, such as acrylates and epoxy resins, can
penetrate vinyl or rubber gloves. Gloves that develop holes should be discarded
immediately and, if sweating makes the condition worse, it may be helpful to
wear cotton gloves beneath the protective ones.
Barrier creams are used in an
attempt to prevent hand eczema of occupational origin.
Emollients should be applied
frequently, and containers should be left at convenient locations at home and
at work so that they are readily available. In general, choice of emollient is
directed by the patient to ensure maximal compliance. Soap substitutes should
be used in place of soap for all hand washing. Patients should be warned that
some topical preparations that can contain irritants such as alcohol or
propylene glycol should be avoided.
Topical corticosteroids are required
for all but the mildest cases of hand eczema. For severe hand eczema, potent or
very potent topical corticosteroids may be needed. Painful fissures of the
fingertips are a particular therapeutic problem and these can be treated with
corticosteroid‐impregnated
adhesive tape, which provides both physical protection and local delivery of
topical corticosteroid.
In difficult, unresponsive cases the
use of a topical corticosteroid under occlusion may be considered. The steroid
is applied at bedtime, and polythene gloves, sealed at the wrist with sticky
tape, are worn overnight. This can be an effective treatment, but it greatly
increases the risk of atrophy and secondary bacterial infection, and should be
discontinued as soon as the eczema shows satisfactory improvement. After
improvement with daily corticosteroid use, the intermittent use of a potent
corticosteroid cream can be used safely to prevent relapse.
If hand eczema does not respond to
topical corticosteroid therapy, the possibility of tinea, exposure to irritants
or allergens, or contact sensitization to medicament bases, preservatives or
the corticosteroid itself should be considered, with patch testing if
necessary.
Topical calcineurin inhibitors
provide a further option for treating hand eczema. Topical tacrolimus is shown to be
as effective as momethasone furoate 0.1% ointment in patients with vesicular
pompholyx of the palms. After 2 weeks of treatment, there is significant
improvement. The response to tacrolimus is better
on the palms than soles.
Hyperkeratotic palmar eczema is
notoriously difficult to manage. Topical retinoids and calcipotriene, both of which act to regulate epidermal cell
maturation, have been shown to improve this category of hand dermatitis. Tar pastes have
been used for chronic unresponsive cases and salicylic acid ointment is also
sometimes helpful for hyperkeratosis and persistent scaling. Intradermal
injection of triamcinolone (10 mg/mL) into recalcitrant, localized patches of
hand eczema may also be beneficial.
Phototherapy
Oral
psoralen and UVA (PUVA) phototherapy and UVB therapy have been used to treat
chronic hand eczema and evidence suggests that oral PUVA may be more effective
than UVB. Topical hand PUVA soaks are frequently used in clinical practice for
hand eczema but they are less effective for hyperkeratotic hand eczema.
Systemic Therapy
At present, the retinoid
alitretinoin (9‐cis‐retinoic acid) is the only licensed
systemic therapy for chronic hand eczema who has failed potent topical
corticosteroid therapy. It interacts with both retinoid X and retinoic acid
receptors. After 24 weeks of alitretinoin 30 mg daily, 48% of patients were
clear or almost clear of their hand eczema. Mucocutaneous adverse effects are
less common than for other systemic retinoids and the commonest issue reported
was headache, which occurred in one‐fifth of patients.
For recurrent pompholyx and
chronic vesicular dermatitis, oral prednisone may be required and is often effective if
treatment is initiated early, at the onset of the itching prodrome. However,
because of significant side effects, systemic glucocorticoids are typically
inappropriate for long-term management.
Cyclosporine is used at dose of 3 mg/kg/day with a potent topical
corticosteroid in the
treatment of chronic vesicular dermatitis. Although patients showed improvement
with treatment, relapses occurred shortly after discontinuation of
cyclosporine.
Mycophenolate mofetil has been used in the treatment
of chronic vesicular dermatitis at dosing levels of 2–3 g/day (in divided
doses). It has been anecdotally shown to improve chronic vesicular dermatitis
that has been otherwise recalcitrant to corticosteroids, and phototherapy.
However, it has also been anecdotally shown to induce biopsy-proven dyshidrotic
eczema.
Azathioprine
50 mg daily with a very potent topical corticosteroid gives possible
additive benefit from low‐dose
azathioprine.
Methotrexate In chronic vesicular eczema, it has been reported
to partially or completely clears lesions at low doses ranging from 12.5 to
22.5 mg/week. However, its wide spectrum of potential side effects remains a
limiting factor to its use in this particular skin disease.
Acute hand eczema
For hand eczema that presents acutely, it is important to
eliminate any precipitant, for example a contact allergen. Emollients should be applied copiously. For
exudates lesions, dilute potassium permanganate soaks are helpful. Large bullae
may be aspirated using a sterile syringe. In
severe cases, a short course of prednisone is given starting with 70 mg and
tapering by 10 or 5 mg over 7 or 14 days.
Systemic antibiotics will be required if secondary bacterial
infection develops.
Therapeutic ladder
First line
·
Hand care advice
·
Irritant and allergen avoidance
·
Emollients
·
Soap substitute
Second line
·
Potent or very potent topical
corticosteroids
Third line
·
Alitretinoin/PUVA/azathioprine/ciclosporin/methotrexate
