BASICS
DESCRIPTION
A variant of psoriasis that characteristically affects the skin of the palms and soles. It is chronic and inflammatory in nature, with hyperkeratotic, pustular, or mixed morphologies. A therapeutically challenging condition that can produce significant functional disability and is associated with substantial impairment in quality of life (1,2). ‚
EPIDEMIOLOGY
- Palmoplantar psoriasis
- Age: no specific age range
- Gender specificity is unclear. Men and women almost equally affected in two studies (3,4), whereas male predominance is suggested by another study (5). The disease may be more common among farmers, manual laborers, and housewives.
- Palmoplantar pustulosis (possibly related condition)
- Age: onset 20 to 60 years
- Predominant sex: female > male (8:2)
Incidence
Incidence has not been determined. ‚
Prevalence
Psoriasis affects 2 " “5% of the population, with the palmoplantar variant comprising 3 " “4% of all cases of psoriasis (5). ‚
ETIOLOGY AND PATHOPHYSIOLOGY
Antigenic triggers activate dendritic cells and T cells; IL-20 is produced locally, which accelerates keratinocyte proliferation; IL-23 is released from lymph nodes, recruiting TH1 and TH17 to the lesions; T cells produce numerous cytokines, including TNF-α, IL-17, and IL-22, which stimulate keratinocytes to proliferate and produce proinflammatory antimicrobial peptides and cytokines; neutrophils are recruited to the epidermis and activate dermal fibroblasts (6). ‚
Genetics
- Psoriasis associated with human leukocyte antigen (HLA) Cw6
- Psoriasis is related to the psoriasis-susceptibility [PSORS1] locus on chromosome 6p21, but relation of this gene to palmoplantar psoriasis remains unclear.
- No association between palmoplantar pustulosis and the PSORS1 locus found in one study (7).
- Variations of the CARD14 gene and genes in the IL-19 subfamily on chromosome 1q31 to 1q32 may be linked to palmoplantar pustulosis and plaque psoriasis (8,9).
RISK FACTORS
- Smoking
- Irritants
- Friction
- Manual or repetitive trauma
- Koebner phenomenon
- Anti " “tumor necrosis factor-alpha (TNF-α) agents have been shown to paradoxically induce palmoplantar eruptions (1).
- Palmoplantar pustulosis: linked to thyroid disease, smoking, and arthritis of the anterior thorax (10)
COMMONLY ASSOCIATED CONDITIONS
- Palmoplantar pustulosis (PPP) (1,10)
- Possibly related to dermatosis characterized by small, sterile pustules
- It remains controversial if PPP is a type of palmoplantar psoriasis or a distinct entity.
- The International Society of Psoriasis deemed PPP as an independent entity in 2007.
- Psoriasis vulgaris is present on other parts of the body in cases of palmoplantar psoriasis (10).
DIAGNOSIS
HISTORY
Symptoms may include (4)[B]: ‚
- Itching
- Pain
- Fissuring
- Persistence of flares
- Spontaneous remissions
- Exacerbation by seasonal changes, household work, detergents
- Psoriatic nail involvement
- Joint pain and swelling
PHYSICAL EXAM
- Palmoplantar psoriasis (1,3)
- Thick hyperkeratotic plaques, sterile pustules, or a mixture of morphologies
- Hyperkeratotic plaques are the most common subtype.
- Symmetrically distributed lesions are common.
- Erythema, fissuring, and scaling are common.
- Location
- Sites other than the hands and feet are commonly involved.
- 33% of patients had eruptions on <10% of their BSA (body surface area).
- Nail: commonly involved (60% in one study) (4)[B]. Findings include coarse pitting, subungual hyperkeratosis, and longitudinal ridging.
- Joints: less commonly involved; findings include swelling and pain
- Palmoplantar pustulosis (10)
- Begins as a unilateral eruption of pin-sized sterile yellow pustules
- Hyperkeratosis with erythema, scaling, and fissuring
- Often becomes symmetric
- Location: thenar, hypothenar, and central portion of palms and soles
- Resolves with residual brown pigmentation
DIFFERENTIAL DIAGNOSIS
- Nonpsoriasiform dermatoses
- Eczema
- Contact dermatitis
- Pityriasis rubra pilaris
- Acquired palmoplantar keratoderma
- Tinea pedis
- Tinea manuum
- Acrodermatitis of continua of Hallopeau: painful pustular periungual and subungual lesions with an inflammatory base that is chronic and recurrent
DIAGNOSTIC TESTS & INTERPRETATION
Clinical diagnosis based on history and physical exam. C-reactive protein (CRP) and uric acid level may be elevated in pustular variant. Fungal studies performed to rule out dermatophytosis. ‚
Initial Tests (lab, imaging)
- PPPASI: Palmoplantar Pustulosis Psoriasis Area and Severity Index
- PASI: Psoriasis Area and Severity Index
- Palmar-Plantar Quality-of-Life Index: a statistically unverified assessment tool used in studies to quantify disease severity and quality of life (1,2).
Test Interpretation
- Histopathologic examination of psoriatic lesions: parakeratosis; decrease/loss of granular layer of the epidermis; psoriasiform epidermal hyperplasia; Munro microabascesses (neutrophils in stratum corneum)
- Common in palmoplantar psoriasis: foci of parakeratosis vertically oriented alternating with orthohyperkeratosis
TREATMENT
- Limited data on treatment: Patients have been excluded from clinical trials of psoriasis because <10% of the BSA is affected (1).
- No standardized treatment exists for patients with palmoplantar psoriasis (10)[A].
MEDICATION
- Most patients require systemic agents given the recalcitrant nature of palmoplantar psoriasis.
- One study reports that 27.4% of patients showed improvement with topical agents, whereas the remaining patients needed systemic treatment (11)[A].
First Line
- Topical treatments (11)[A],(12)[B]
- Potent to superpotent corticosteroids: applied twice daily with gradual reduction in frequency, for up to 6 to 8 weeks; with/without occlusion with hydrocolloid or hydrogel dressings. Betamethasone valerate 0.12% foam and clobetasol propionate 0.05% foam are easy to apply with minimal residue after application.
- Coal tar: a low-cost treatment often combined with salicylic acid/corticosteroids under occlusion; often not tolerable to patients due to messy application
- Salicylic acid: a keratolytic agent often used in combination with topical steroids; cream and ointment preparations in varying concentrations used
- Calcipotriene: a vitamin D analogue that is often alternated with potent topical corticosteroids; should not be used in combination with salicylic acid, which deactivates the molecule
- Combinations of topical medications are more efficacious than a single agent: superpotent steroids plus calcipotriene/retinoid
- Systemic medications
- Acitretin: 10 to 50 mg/day with a maximal effect seen between 3 and 6 months after treatment in nonchildbearing women (11)[A]
Second Line
- Light therapy (13)[B]
- Psoralen + ultraviolet-A (PUVA)
- Oral PUVA is more efficacious than bath PUVA in the first 4 weeks of treatment but has greater side effects.
- Frequent, bi-/tri-weekly, PUVA soaks are preferred to oral PUVA to avoid systemic side effects.
- Local PUVA is more efficacious than local narrow-band ultraviolet-B (NB-UVB) (14)[B].
- Monochromatic excimer light (MEL) (308 nm) is efficacious and well tolerated in a study with 25 weekly MEL treatments (15)[A].
- Systemic medications
- Methotrexate 7.5 to 20 mg/week PO or IM over 3 to 6 weeks (11)[A]
- Indicated for disabling palmoplantar psoriasis after failure of topicals and phototherapy
- One study showed that methotrexate is superior to acitretin (11)[A].
- Another study demonstrated that eight different topical regimens combined with systemic methotrexate resulted in early and improved quality of life; the addition of NB-UVB improved efficacy (14)[B]
- Cyclosporine: used in immunocompetent patients with severe recalcitrant palmoplantar psoriasis; start at 2.5 to 5.0 mg/kg/day for a maximum of 1 year; dose should be decreased by 0.5 to 1.0 mg/kg if hypertension or abnormal renal function test results seen
- Biologics: reserved for patients who fail or cannot complete treatment with topicals or systemic medications
- Etanercept: TNF-α inhibitor; statistically significant reduction in PPPASI with 50 mg twice weekly dosing for 24 weeks of therapy (16)[A]
- Infliximab: TNF-α inhibitor; Dosed at 5 mg/kg at weeks 0, 2, 6, and then every 8 weeks in one study; 50% reduction in mean surface area of palms and soles (17)[A]
- Adalimumab: TNF-α inhibitor; continuous treatment of 40 mg every 2 weeks for a total of 3 months demonstrated improved quality of life (18)[A].
- Ustekinumab: IL-12 and IL-23 inhibitor; One study recommended dosing at 90 mg every 3 months in patients weighing >100 kg (12)[A].
ADDITIONAL THERAPIES
- Palmoplantar pustulosis
- Ideal treatment remains unclear per Cochrane review (10)[A].
- Case studies and reports of potential treatments
- Systemic retinoids and oral PUVA (13)[C]
- Photodynamic therapy: 20% 5-aminolevulinic acid + 630 ‚ ± 50 nm diode that emits light (12)[C]
- Alitretinoin 30 mg daily for 12 weeks demonstrated significant improvement in clinical symptoms of recalcitrant cases (19)[B].
- Anakinra 100 mg SC daily showed partial clinical response in two refractory cases (20)[B].
- Reported improvement with smoking cessation, tonsillectomy, ileojejunal bypass, and shunt surgery (1)
ONGOING CARE
FOLLOW-UP RECOMMENDATIONS
It is difficult to monitor disease progression and response to treatment given the paucity of standardized evaluation tools specific for palmoplantar psoriasis. ‚
PATIENT EDUCATION
- National Psoriasis Foundation: http://www.psoriasis.org/about-psoriasis/types/pustular; 800-723-9166
- American Academy of Dermatology: www.aad.org/skin-conditions/dermatology-a-to-z/psoriasis; 866-503-7546
PROGNOSIS
- Significant morbidity and limitations in daily activities with lesions of the palms and soles, particularly when fissuring occurs
- Patients have greater functional impairments and disease-related pain compared with those with psoriasis alone.
REFERENCES
11 Farley ‚ E, Masrour ‚ S, McKey ‚ J, et al. Palmoplantar psoriasis: a phenotypical and clinical review with introduction of a new quality-of-life assessment tool. J Am Acad Dermatol. 2009;60(6):1024 " “1031.22 Chung ‚ J, Callis Duffin ‚ K, Takeshita ‚ J, et al. Palmoplantar psoriasis is associated with greater impairment of health-related quality of life compared with moderate to severe plaque psoriasis. J Am Acad Dermatol. 2014;71(4):623 " “632.33 Kumar ‚ B, Saraswat ‚ A, Kaur ‚ I. Palmoplantar lesions in psoriasis: a study of 3065 patients. Acta Derm Venereol. 2002;82(3):192 " “195.44 Chopra ‚ A, Maninder, Gill ‚ SS. Hyperkeratosis of palms and soles: clinical study. Indian J Dermatol Venereol Leprol. 1997;63(2):85 " “88.55 Khandpur ‚ S, Singhal ‚ V, Sharma ‚ VK. Palmoplantar involvement in psoriasis: a clinical study. Indian J Dermatol Venereol Leprol. 2011;77(5):625.66 Coimbra ‚ S, Figueiredo ‚ A, Castro ‚ E, et al. The roles of cells and cytokines in the pathogenesis of psoriasis. Int J Dermatol. 2012;51(4):389 " “395.77 Asumalahti ‚ K, Ameen ‚ M, Suomela ‚ S, et al. Genetic analysis of PSORS1 distinguishes guttate psoriasis and palmoplantar pustulosis. J Invest Dermatol. 2003;120(4):627 " “632.88 Kingo ‚ K, M ƒ ¶ssner ‚ R, K ƒ ¶ks ‚ S, et al. Association analysis of IL19, IL20 and IL24 genes in palmoplantar pustulosis. Br J Dermatol. 2007;156(4):646 " “652.99 M ƒ ¶ssner ‚ R, Frambach ‚ Y, Wilsmann-Theis ‚ D, et al. Palmoplantar pustular psoriasis is associated with missense variants in CARD14, but not with loss-of-function mutations in IL36RN in European patients. J Invest Dermatol. 2015;135(10):2538 " “2541.1010 Marsland ‚ AM, Chalmers ‚ RJ, Hollis ‚ S, et al. Interventions for chronic palmoplantar pustulosis. Cochrane Database Syst Rev. 2006;(1):CD001433.1111 Janagond ‚ AB, Kanwar ‚ AJ, Handa ‚ S. Efficacy and safety of systemic methotrexate vs. acitretin in psoriasis patients with significant palmoplantar involvement: a prospective, randomized study. J Eur Acad Dermatol Venereol. 2013;27(3):e384 " “e389.1212 Au ‚ SC, Goldminz ‚ AM, Kim ‚ N, et al. Investigator-initiated, open-label trial of ustekinumab for the treatment of moderate-to-severe palmoplantar psoriasis. J Dermatolog Treat. 2013;24(3):179 " “187.1313 Carrascosa ‚ JM, Plana ‚ A, Ferr ƒ ¡ndiz ‚ C. Effectiveness and safety of psoralen-UVA (PUVA) topical therapy in palmoplantar psoriasis: a report on 48 patients [in English, Spanish]. Actas Dermosifiliogr. 2013;104(5):418 " “425.1414 Gupta ‚ SK, Singh ‚ KK, Lalit ‚ M. Comparative therapeutic evaluation of different topicals and narrow band ultraviolet B therapy combined with systemic methotrexate in the treatment of palmoplantar psoriasis. Indian J Dermatol. 2011;56(2):165 " “170.1515 Sevrain ‚ M, Richard ‚ MA, Barnetche ‚ T, et al. Treatment for palmoplantar pustular psoriasis: systematic literature review, evidence-based recommendations and expert opinion. J Eur Acad Dermatol Venereol. 2014;28(Suppl 5):13 " “16.1616 Bissonnette ‚ R, Poulin ‚ Y, Bolduc ‚ C, et al. Etanercept in the treatment of palmoplantar pustulosis. J Drugs Dermatol. 2008;7(10):940 " “946.1717 Bissonnette ‚ R, Poulin ‚ Y, Guenther ‚ L, et al. Treatment of palmoplantar psoriasis with infliximab: a randomized, double-blind placebo-controlled study. J Eur Acad Dermatol Venereol. 2011;25(12):1402 " “1408.1818 Richetta ‚ AG, Mattozzi ‚ C, Giancristoforo ‚ S, et al. Safety and efficacy of adalimumab in the treatment of moderate to severe palmo-plantar psoriasis: an open label study. Clin Ter. 2012;163(2):e61 " “e66.1919 Irla ‚ N, Navarini ‚ AA, Yawalkar ‚ N. Alitretinoin abrogates innate inflammation in palmoplantar pustular psoriasis. Br J Dermatol. 2012;167(5):1170 " “1174.2020 Tauber ‚ M, Viguier ‚ M, Alimova ‚ E, et al. Partial clinical response to anakinra in severe palmoplantar pustular psoriasis. Br J Dermatol. 2014;171(3): 646 " “649.
ADDITIONAL READING
Spuls ‚ PI, Hadi ‚ S, Rivera ‚ L, et al. Retrospective analysis of the treatment of psoriasis of the palms and soles. J Dermatolog Treat. 2003;14(Suppl 2):21 " “25. ‚
SEE ALSO
Psoriasis ‚
CODES
ICD10
- L40.3 Pustulosis palmaris et plantaris
- L40.8 Other psoriasis
ICD9
696.1 Other psoriasis ‚
SNOMED
- Pustular psoriasis of the palms AND/OR soles
- Psoriasis palmaris (disorder)
- Psoriasis plantaris (disorder)
- Hypertrophic palmoplantar psoriasis (disorder)
CLINICAL PEARLS
- Palmoplantar psoriasis is a chronic and difficult-to-treat type of psoriasis with significant morbidity.
- Topical treatments often fail, and systemic therapy is often needed.
- There is limited data on the treatment of palmoplantar psoriasis and no standardized treatment regimen.
- It remains controversial if palmoplantar pustulosis is associated with palmoplantar psoriasis or is a separate entity.