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Author: Camila K Janniger, MD, Clinical Professor, Dermatology and Chief, Pediatric Dermatology, Clinical Associate Professor, Pediatrics, University Medicine and Dentistry of New Jersey, New Jersey Medical School

Camila K Janniger is a member of the following medical societies: American Academy of Dermatology

Coauthor(s): Julie K Keck, MD, Assistant Professor of Clinical Pediatrics, Neurodevelopmental Pediatrician, Department of Developmental Pediatrics, Riley Hospital for Children; James D Korb, MD, Program Director, Department of Pediatrics, Children's Hospital of Orange County

Editors: Kevin P Connelly, DO, Clinical Assistant Professor, Department of Pediatrics, Division of General Pediatrics and Emergency Care, Virginia Commonwealth University; Medical Director, Paws for Health Pet Visitation Program; Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine.com, Inc; Robert A Schwartz, MD, MPH, Professor and Head of Dermatology, Professor of Medicine, Professor of Pediatrics, Professor of Pathology, Professor of Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School; Merrily P M Poth, MD, Professor, Department of Pediatrics and Neuroscience, Uniformed Services University of the Health Sciences; William D James, MD, Paul R Gross Professor of Dermatology, University of Pennsylvania School of Medicine; Vice-Chair, Program Director, Department of Dermatology, University of Pennsylvania Health System

Author and Editor Disclosure

Synonyms and related keywords: dyshidrotic eczema, pompholyx, dyshidrosis, cheiropompholyx, chiropompholyx, dyshidria, palmoplantar hyperhidrosis, dermatitis, pruritic vesicular eruption

Background

Dyshidrotic eczema is a type of eczema (dermatitis) of unknown cause that is characterized by a pruritic vesicular eruption on the fingers, palms, and soles. The condition affects teenagers and adults and may be acute, recurrent, or chronic. A more appropriate term for this vesicular eruption is pompholyx, which means bubble. The clinical course of pompholyx can range from self-limited to chronic, severe, or debilitating. The condition's unresponsiveness to treatment can be frustrating for the patient and physician.

Pathophysiology

The etiology of pompholyx is unknown. The condition was inaccurately described in 1873 as dyshidrosis because of the clinical symptom of sweaty palms. The term dyshidrosis indicates a sweating abnormality, although histologic examination shows no evidence of eccrine glandular involvement. Histologically, the vesicles are intraepidermal and spongiotic with little to no inflammatory changes. The more appropriate term for this vesicular eruption is pompholyx, which means bubble. While strong reasons to use the term pompholyx have been noted, dyshidrotic eczema remains a commonly used term. A tiny percentage of individuals with the disorder note flares after ingesting metal salts, specifically chromium, cobalt, and nickel. Diets that eliminate these metal salts may rarely have some clinical benefit.

A genetic component to the development of pompholyx may be involved in some patients. Pompholyx has been described in few large families, so no gene or locus had been identified1. A genome-wide search in a large Chinese family identified a locus at chromosome 18q22.1-18q22.3, with a maximum 2-point logarithm of the odds (LOD) score of 3.61 at marker D18S1131 (theta = 0.00). Haplotype analyses showed the gene to be located within 12.07 cM region between markers D18S465 and D18S1362, which corresponds to 8.0 Mb.

Frequency

United States

Pompholyx accounts for 5% of all cases of eczema of the hand.

Mortality/Morbidity

Pompholyx has no associated mortality, although some severe cases can become debilitating.

Race

No racial predilection is reported.

Sex

The female-to-male ratio is 2:1.

Age

Peak incidence occurs in patients aged 20-40 years, although the disorder also occurs in teenagers and older patients.



History

Patients first describe several hours of itching or burning sensations in their hands, feet, or both before the eruption develops. Tiny vesicles erupt first along lateral aspects of the fingers and then on the palms or soles. Palms and soles may be red and wet with perspiration. The vesicles usually persist for 3-4 weeks. Vesicle outbreaks may occur in waves. A photo-induced form of hand dermatitis resembling pompholyx has been described2.

Physical

Physical examination performed early in the course of the flare reveals small (ie, 1-2 mm), clear, deep-seated vesicles without erythema erupting on the lateral aspects of fingers, the central palm, and plantar surfaces. The vesicles have been described as resembling tapioca pudding. Eruptions are usually bilateral and symmetric. Patients treated later in the course of this disorder may have unroofed vesicles with inflamed bases, possibly accompanied by peeling or rings of scale or lichenification. Transverse furrows can develop on the nail when eruptions occur in the periungual area, nail matrix, or both.

Causes

Although the etiology of pompholyx remains undefined, suspected risk factors include stress, exposure to metal salts, allergic contact dermatitis, and female sex. In a recent article, Iannaccone et al (1999) cite exposure to intravenous immunoglobulin G (IVIG) as a possible risk factor3.



Other Problems to be Considered

Id reaction (ie, autoeczematization)
Pustular psoriasis
Primary fungal infection
Recurrent focal palmar peeling (previously termed keratolysis exfoliativa)
Dyshidrosiform bullous pemphigoid



Histologic Findings

The vesicles in patients with pompholyx are intraepidermal and spongiotic with little or no inflammatory changes.



Medical Care

Typical first-line treatment includes high-strength topical steroids and cold compresses. Short courses of oral steroids are the second line of treatment for acute flares, and other immunosuppressants have also been tried. Corticosteroids are cornerstones of topical therapy, although calcineurin inhibitors may also be effective4. Variable effects have been reported using oral administration of psoralen and subsequent exposure to long-wavelength ultraviolet light (PUVA) therapy. Topical photochemotherapy with 8-methoxypsoralen is probably as effective as systemic photochemotherapy or high-dose ultra violet type A-1 irradiation. For recalcitrant cases, corticosteroids are combined with immunosuppressants. A new evolving treatment seems to be the intradermal injection of botulinum toxin.

  • Topical khellin and natural sunlight therapy have been suggested for patients with recalcitrant palmoplantar pompholyx5.

  • Identification of the causes of stress and use of stress management techniques as adjuncts may be helpful in some patients.

Diet

Pompholyx requires no dietary restrictions, although some patients have reported improvement by avoiding foods rich in heavy metal salts.

Activity

Pompholyx may restrict activity; some refractory cases become debilitating. Some cases are precipitated by an environmental contact, which could also influence activities.



Pompholyx treatment can be quite challenging because of the severe inflammatory process or because of frequent recurrences. Pharmacologic treatment begins with high-strength topical corticosteroids. In recalcitrant cases, systemic corticosteroids are the next line of treatment. Two recent case reports also note some success with other immunosuppressants (eg, methotrexate, mycophenolate mofetil).

The long-term efficacy of occlusive therapy with pimecrolimus (Elidel), a topical calcineurin inhibitor, has been reported in patients with severe dyshidrosiform hand and foot eczema6. However, the authors recommend caution in the extended use of calcineurin inhibitors.

In March 2005, the US Food and Drug Administration (FDA) issued a public health advisory to inform healthcare professionals and patients about a potential cancer risk from use of pimecrolimus. This concern is based on information from animal studies, case reports in a small number of patients, and knowledge of how drugs in this class work. Human studies of 10 years or longer may be needed to determine if pimecrolimus administration is linked to cancer. In the meantime, this risk is uncertain, and the FDA advises pimecrolimus should only be used in patients in whom other prescription treatments have failed or cannot be tolerated. This information reflects the FDA’s preliminary analysis of data concerning this drug.

Drug Category: Corticosteroids

Topical corticosteroids are the first line of therapy. Steroid potency choice is based on the patient's response to treatment; however, the higher-strength steroids are usually necessary for disease control.

Drug NameClobetasol propionate (Temovate)
DescriptionA high-potency corticosteroid with anti-inflammatory, antipruritic, and vasoconstrictive properties.
Adult DoseApply to affected areas bid
Pediatric Dose<12 years: Not recommended
>12 years: Administer as in adults
ContraindicationsDocumented hypersensitivity; viral or fungal skin infections
InteractionsNone reported
PregnancyC - Safety for use during pregnancy has not been established.
PrecautionsMay suppress adrenal function in prolonged therapy

Drug NamePrednisone (Deltasone, Meticorten)
DescriptionA glucocorticoid readily absorbed from GI tract. Used as second-line pharmacologic treatment of pompholyx. It is a potent anti-inflammatory agent that has salt-retaining properties and varied metabolic effects. Can modify immune response.
Adult Dose5-60 mg PO qd
Pediatric Dose0.5-2 mg/kg/d PO qd or divided bid/qid
ContraindicationsDocumented hypersensitivity; viral infection; peptic ulcer disease; hepatic dysfunction; connective tissue infections; fungal or tubercular skin infections; GI bleeding
InteractionsCoadministration with estrogens may decrease prednisone clearance; concurrent use with digoxin may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics
PregnancyB - Usually safe but benefits must outweigh the risks.
PrecautionsAbrupt discontinuation of glucocorticoids may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections may occur with glucocorticoid use

Drug Category: Topical calcineurin inhibitor

These agents are topical immune suppressants that block early T-cell activation, degranulation of mast cells, and multiple cytokines.

Drug NamePimecrolimus (Elidel cream)
DescriptionFirst nonsteroid cream approved in the United States for mild-to-moderate atopic dermatitis. Derived from ascomycin, a natural substance produced by the fungus Streptomyces hygroscopicus var. ascomyceticus. Selectively inhibits production and release of inflammatory cytokines from activated T cells by binding to cytosolic immunophilin receptor macrophilin-12. The resulting complex inhibits phosphatase calcineurin, thus blocking T-cell activation and cytokine release. Cutaneous atrophy was not observed in clinical trials, a potential advantage over topical corticosteroids. Indicated only after other treatment options have failed.
Adult DoseApply topically to affected areas bid
Short-term and intermittent use only
Pediatric Dose<2 years: Not established
>2 years: Administer as in adults
Short-term and intermittent use only
ContraindicationsDocumented hypersensitivity
InteractionsNone reported
PregnancyC - Safety for use during pregnancy has not been established
PrecautionsPotential exacerbation of existing infection at site of application; may cause burning and irritation; caution with conditions that suppress the immune system (eg, AIDS, cancer); possible risk of lymph node or skin cancer based on animal studies and a small number of patients; may increase risk of viral infections; other adverse effects include headache, sore throat, flulike symptoms, fever, and cough



Further Inpatient Care

  • Inpatient care is unnecessary.

Further Outpatient Care

  • Further outpatient care includes physician follow-up for treatment options.

Deterrence/Prevention

  • Decrease stress and avoid ingesting metal salts.

Complications

  • Complications include poor response to treatment, resulting in continued rash, pruritus, and possible superinfection.

Prognosis

  • The prognosis for patients with pompholyx varies. Some individuals recover completely; some experience chronic unremitting pompholyx.

Patient Education

  • Inform individuals with this disorder about the difficulty of achieving successful treatment.
  • For excellent patient education resources, visit eMedicine's Skin, Hair, and Nails Center. Also, see eMedicine's patient education article Eczema.



Medical/Legal Pitfalls

  • Failure to educate patients on the recurrent nature of pompholyx



  1. Chen JJ, Liang YH, Zhou FS, et al. The gene for a rare autosomal dominant form of pompholyx maps to chromosome 18q22.1-18q22.3. J Invest Dermatol. Feb 2006;126(2):300-4. [Medline].
  2. Man I, Ibbotson SH, Ferguson J. Photoinduced pompholyx: a report of 5 cases. J Am Acad Dermatol. Jan 2004;50(1):55-60. [Medline].
  3. Iannaccone S, Sferrazza B, Quattrini A, Smirne S, Ferini-Strambi L. Pompholyx (vesicular eczema) after i.v. immunoglobulin therapy for neurologic disease. Neurology. Sep 22 1999;53(5):1154-5. [Medline].
  4. Wollina U, Abdel Naser MB. Pharmacotherapy of pompholyx. Expert Opin Pharmacother. Jul 2004;5(7):1517-22. [Medline].
  5. Capella GL. Topical khellin and natural sunlight in the outpatient treatment of recalcitrant palmoplantar pompholyx: report of an open pilot study. Dermatology. 2005;211(4):381-3. [Medline].
  6. Schurmeyer-Horst F, Luger TA, Bohm M. Long-term efficacy of occlusive therapy with topical pimecrolimus in severe dyshidrosiform hand and foot eczema. Dermatology. 2007;214(1):99-100. [Medline].
  7. Colebunders R, Zolfo M, Lynen L. Severe dyshidrosis in two patients with HIV infection shortly after starting highly active antiretroviral treatment. Dermatol Online J. 2005;11(2):31. [Medline].
  8. Egan CA, Rallis TM, Meadows KP, Krueger GG. Low-dose oral methotrexate treatment for recalcitrant palmoplantar pompholyx. J Am Acad Dermatol. Apr 1999;40(4):612-4. [Medline].
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  14. Petering H, Breuer C, Herbst R, Kapp A, Werfel T. Comparison of localized high-dose UVA1 irradiation versus topical cream psoralen-UVA for treatment of chronic vesicular dyshidrotic eczema. J Am Acad Dermatol. Jan 2004;50(1):68-72. [Medline].
  15. Pickenacker A, Luger TA, Schwarz T. Dyshidrotic eczema treated with mycophenolate mofetil. Arch Dermatol. Mar 1998;134(3):378-9. [Medline].
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Dyshidrotic Eczema excerpt

Article Last Updated: May 22, 2007