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A 52-year-old woman presented to a primary care clinic with a 3-week history of rash on her feet that had spread to her hands in the previous week. She described the rash as painful, burning, and itching with no drainage. She denied any recent illness, fever, chills, medication changes, or environmental exposures. Home treatments included Epsom salt baths and lotion with no improvement.
Past medical history included hypertension. She was a smoker with a 30-pack-year history and drank alcohol on a daily basis. Her medications included losartan and atorvastatin.
On examination, multiple papular and scabbed lesions were present with mild scaling. Additional review of systems and physical exam were benign. A KOH prep showed hyphae. The patient was diagnosed with tinea pedis and prescribed fluconazole (150-mg tablet once per week for 2 weeks).
Two weeks later, after completing the antifungal therapy, the patient returned with pain limiting her ability to bear weight or grasp objects. Clinical examination showed well-demarcated erythematous scaly and hyperkeratotic plaques with scattered papular and pustular lesions on bilateral palmar and medial aspects of plantar surfaces (see Figures 1 and 2). A repeat KOH was not completed. The patient was diagnosed with palmoplantar pustulosis (PPP).
DISCUSSION
PPP is a chronic, relapsing, inflammatory skin condition that results in painful lesions on the palms and the soles.1,2 There is debate as to whether PPP is a variant of psoriasis or a separate condition; depending on physical manifestations, one can be diagnosed with palmoplantar plaque psoriasis, PPP, or a combination of the two.3,4
The exact cause of PPP is unknown; however, increased levels of inflammatory cytokines interleukin (IL)-17 and IL-22 may be involved in the pathogenesis of the disease.5 Additional genetic and environmental factors, most significantly smoking, play an important role in its development.2,6
Clinical presentation
Inflammation associated with PPP typically manifests in the classic features of pustules that coalesce and resolve over several days, resulting in brown macules, hyperkeratosis, fissures, and debilitating pain.4,7 Some patients may have co-occurring onycholysis resulting from nail dystrophy and destruction or plaque psoriasis elsewhere on their body.8 PPP often persists for years with periods of exacerbation and remission, and it significantly affects the patient’s ability to perform activities of daily living without pain.8,9 It is exceedingly rare and most commonly affects middle-aged women with a smoking history or current smoking status.7
Continue to: Laboratory diagnosis
Laboratory diagnosis
The diagnosis of PPP is based on clinical presentation and physical exam. Laboratory testing, such as KOH prep, may assist in ruling out dermatophyte infection; a complete blood count may assist in eliminating a bacterial infection as the cause. Skin biopsy is not necessary unless diagnosis is uncertain or prolonged treatment has not produced a response.
Differential diagnosis
The differential diagnosis of PPP includes skin conditions that involve the palms and/or the soles and may have fungal, allergic, or bacterial origins.
Fungal. Tinea manuum (palms) and tinea pedis (soles) result from dermatophyte infection and manifest with erythema and/or scaling and pruritis. A positive KOH examination can confirm diagnosis. On examination, fungal infections are commonly unilateral and asymmetric.8 Treatment with an antifungal agent should result in resolution of symptoms.
Allergic. Contact with an allergen can result in skin erythema, pruritis, and pain at the exposed area. Contact dermatitis can result from an inflammatory response to an allergen or irritant, and it is often localized and well demarcated. This is an acute condition that resolves over time with antihistamines and avoidance of irritants.
Dyshidrotic eczema results in small, pruritic blisters on the palms and the soles and can be recurrent and related to seasonal allergen exposure. Diagnosis is made from history and physical exam. Treatment often consists of emollients and occasionally topical steroids, depending on the severity.
Continue to: Bacterial
Bacterial. A primary bacterial cause of bilateral skin lesions on the palms and the soles is uncommon. However, any open skin lesion can result in secondary bacterial infection. The pustules of PPP are often sterile and do not require bacterial culture; however, additional symptoms of fever, purulence, warmth, and worsening of symptoms may prompt further evaluation for a bacterial origin or complication.
Management
Due to limited quality data on treatment recommendations, the treatment options for PPP vary greatly. Most studies recommend topical versus systemic therapy for initial management.1-2,8,10-11 Firstline therapy often consists of topical corticosteroids and occlusive dressings, followed by oral retinoids (acitretin, alitretinoin) or photochemotherapy.1,8 Third-line therapy can include immunosuppressants (ciclosporin, methotrexate) or biologics (secukinumab).1,12 Recent data have shown positive results with vitamin D3 analogs (maxacalcitol, betamethasone butyrate propionate) as monotherapy or in combination with corticosteroids.10-11 Duration of therapy ranges from 4 to 8 weeks throughout the literature, depending on severity; however, many patients see improvement in the first few weeks.
Conservative measures to maintain remission include smoking cessation, skin emollients, and avoidance of irritants. It is important to educate patients about the chronicity of the disease and early treatment to prevent secondary infection or significant impact on quality of life.
OUTCOME FOR THE CASE PATIENT
The patient was prescribed triamcinolone acetonide (0.5% ointment applied bid), to be used until symptoms improved. After 1 week of treatment, she confirmed (verbally) that symptoms had resolved. She declined a follow-up visit or referral to dermatology.
CONCLUSION
Although PPP is fairly uncommon, it is important for clinicians to consider this diagnosis in patients presenting with localized rash on their palms and soles. This debilitating condition greatly affects a patient’s quality of life and, although it is chronic in nature, available treatments described in the literature have shown success in both acute resolution and ongoing remission of the disease.
1. Sevrain M, Richard M-A, 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.
2. Olazagasti JM, Ma JE, Wetter DA. Clinical features, etiological factors, associated disorders, and treatment of palmoplantar pustulosis: the Mayo Clinic experience, 1996-2013. Mayo Clin Proc. 2017;92(9):1351-1358.
3. Bissonnette R, Suárez-Fariñas M, Li X, et al. Based on molecular profiling of gene expression, palmoplantar pustulosis and palmoplantar pustular psoriasis are highly related diseases that appear to be distinct from psoriasis vulgaris. PLoS One. 2016;11(5):1-11.
4. Raposo I, Torres T. Palmoplantar psoriasis and palmoplantar pustulosis: current treatment and future prospects. Am J Clin Dermatol. 2016;17(4):349-358.
5. Bissonnette R, Fuentes-Duculan J, Mashiko S, et al. Palmoplantar pustular psoriasis (PPPP) is characterized by activation of the IL-17A pathway. J Dermatol Sci. 2017;85(1):20-26.
6. Misiak-Galazka M, Wolska H, Rudnicka L. What do we know about palmoplantar pustulosis? J Eur Acad Dermatol Venereol. 2017;31(1):38-44.
7. Brunasso AMG, Puntoni M, Aberer W, et al. Clinical and epidemiological comparison of patients affected by palmoplantar plaque psoriasis and palmoplantar pustulosis: a case series study. Br J Dermatol. 2013;168(6):1243-1251.
8. Engin B, As¸kın Ö, Tüzün Y. Palmoplantar psoriasis. Clin Dermatol. 2017; 35(1):19-27.
9. Chung J, Callas Duffin K, Takeshita J, et al. Palmoplantar psoriasis is associated with greater impairment of health-related quality of life compared to moderate-to-severe plaque psoriasis. J Am Acad Dermatol. 2014;71(4):623-632.
10. Umezawa Y, Nakagawa H, Tamaki K. Phase III clinical study of maxacalcitol ointment in patients with palmoplantar pustulosis: a randomized, double-blind, placebo-controlled trial. J Dermatol. 2016;43(3):288-293.
11. Muro M, Kawakami H, Matsumoto Y, et al. Topical combination therapy with vitamin D3 and corticosteroid ointment for palmoplantar pustulosis: a prospective, randomized, left-right comparison study. J Dermatolog Treat. 2016;27(1):51-53.
12. Gottlieb A, Sullivan J, van Doorn M, et al. Secukinumab shows significant efficacy in palmoplantar psoriasis: results from GESTURE, a randomized controlled trial. J Am Acad Dermatol. 2017;76(1):70-80.
A 52-year-old woman presented to a primary care clinic with a 3-week history of rash on her feet that had spread to her hands in the previous week. She described the rash as painful, burning, and itching with no drainage. She denied any recent illness, fever, chills, medication changes, or environmental exposures. Home treatments included Epsom salt baths and lotion with no improvement.
Past medical history included hypertension. She was a smoker with a 30-pack-year history and drank alcohol on a daily basis. Her medications included losartan and atorvastatin.
On examination, multiple papular and scabbed lesions were present with mild scaling. Additional review of systems and physical exam were benign. A KOH prep showed hyphae. The patient was diagnosed with tinea pedis and prescribed fluconazole (150-mg tablet once per week for 2 weeks).
Two weeks later, after completing the antifungal therapy, the patient returned with pain limiting her ability to bear weight or grasp objects. Clinical examination showed well-demarcated erythematous scaly and hyperkeratotic plaques with scattered papular and pustular lesions on bilateral palmar and medial aspects of plantar surfaces (see Figures 1 and 2). A repeat KOH was not completed. The patient was diagnosed with palmoplantar pustulosis (PPP).
DISCUSSION
PPP is a chronic, relapsing, inflammatory skin condition that results in painful lesions on the palms and the soles.1,2 There is debate as to whether PPP is a variant of psoriasis or a separate condition; depending on physical manifestations, one can be diagnosed with palmoplantar plaque psoriasis, PPP, or a combination of the two.3,4
The exact cause of PPP is unknown; however, increased levels of inflammatory cytokines interleukin (IL)-17 and IL-22 may be involved in the pathogenesis of the disease.5 Additional genetic and environmental factors, most significantly smoking, play an important role in its development.2,6
Clinical presentation
Inflammation associated with PPP typically manifests in the classic features of pustules that coalesce and resolve over several days, resulting in brown macules, hyperkeratosis, fissures, and debilitating pain.4,7 Some patients may have co-occurring onycholysis resulting from nail dystrophy and destruction or plaque psoriasis elsewhere on their body.8 PPP often persists for years with periods of exacerbation and remission, and it significantly affects the patient’s ability to perform activities of daily living without pain.8,9 It is exceedingly rare and most commonly affects middle-aged women with a smoking history or current smoking status.7
Continue to: Laboratory diagnosis
Laboratory diagnosis
The diagnosis of PPP is based on clinical presentation and physical exam. Laboratory testing, such as KOH prep, may assist in ruling out dermatophyte infection; a complete blood count may assist in eliminating a bacterial infection as the cause. Skin biopsy is not necessary unless diagnosis is uncertain or prolonged treatment has not produced a response.
Differential diagnosis
The differential diagnosis of PPP includes skin conditions that involve the palms and/or the soles and may have fungal, allergic, or bacterial origins.
Fungal. Tinea manuum (palms) and tinea pedis (soles) result from dermatophyte infection and manifest with erythema and/or scaling and pruritis. A positive KOH examination can confirm diagnosis. On examination, fungal infections are commonly unilateral and asymmetric.8 Treatment with an antifungal agent should result in resolution of symptoms.
Allergic. Contact with an allergen can result in skin erythema, pruritis, and pain at the exposed area. Contact dermatitis can result from an inflammatory response to an allergen or irritant, and it is often localized and well demarcated. This is an acute condition that resolves over time with antihistamines and avoidance of irritants.
Dyshidrotic eczema results in small, pruritic blisters on the palms and the soles and can be recurrent and related to seasonal allergen exposure. Diagnosis is made from history and physical exam. Treatment often consists of emollients and occasionally topical steroids, depending on the severity.
Continue to: Bacterial
Bacterial. A primary bacterial cause of bilateral skin lesions on the palms and the soles is uncommon. However, any open skin lesion can result in secondary bacterial infection. The pustules of PPP are often sterile and do not require bacterial culture; however, additional symptoms of fever, purulence, warmth, and worsening of symptoms may prompt further evaluation for a bacterial origin or complication.
Management
Due to limited quality data on treatment recommendations, the treatment options for PPP vary greatly. Most studies recommend topical versus systemic therapy for initial management.1-2,8,10-11 Firstline therapy often consists of topical corticosteroids and occlusive dressings, followed by oral retinoids (acitretin, alitretinoin) or photochemotherapy.1,8 Third-line therapy can include immunosuppressants (ciclosporin, methotrexate) or biologics (secukinumab).1,12 Recent data have shown positive results with vitamin D3 analogs (maxacalcitol, betamethasone butyrate propionate) as monotherapy or in combination with corticosteroids.10-11 Duration of therapy ranges from 4 to 8 weeks throughout the literature, depending on severity; however, many patients see improvement in the first few weeks.
Conservative measures to maintain remission include smoking cessation, skin emollients, and avoidance of irritants. It is important to educate patients about the chronicity of the disease and early treatment to prevent secondary infection or significant impact on quality of life.
OUTCOME FOR THE CASE PATIENT
The patient was prescribed triamcinolone acetonide (0.5% ointment applied bid), to be used until symptoms improved. After 1 week of treatment, she confirmed (verbally) that symptoms had resolved. She declined a follow-up visit or referral to dermatology.
CONCLUSION
Although PPP is fairly uncommon, it is important for clinicians to consider this diagnosis in patients presenting with localized rash on their palms and soles. This debilitating condition greatly affects a patient’s quality of life and, although it is chronic in nature, available treatments described in the literature have shown success in both acute resolution and ongoing remission of the disease.
A 52-year-old woman presented to a primary care clinic with a 3-week history of rash on her feet that had spread to her hands in the previous week. She described the rash as painful, burning, and itching with no drainage. She denied any recent illness, fever, chills, medication changes, or environmental exposures. Home treatments included Epsom salt baths and lotion with no improvement.
Past medical history included hypertension. She was a smoker with a 30-pack-year history and drank alcohol on a daily basis. Her medications included losartan and atorvastatin.
On examination, multiple papular and scabbed lesions were present with mild scaling. Additional review of systems and physical exam were benign. A KOH prep showed hyphae. The patient was diagnosed with tinea pedis and prescribed fluconazole (150-mg tablet once per week for 2 weeks).
Two weeks later, after completing the antifungal therapy, the patient returned with pain limiting her ability to bear weight or grasp objects. Clinical examination showed well-demarcated erythematous scaly and hyperkeratotic plaques with scattered papular and pustular lesions on bilateral palmar and medial aspects of plantar surfaces (see Figures 1 and 2). A repeat KOH was not completed. The patient was diagnosed with palmoplantar pustulosis (PPP).
DISCUSSION
PPP is a chronic, relapsing, inflammatory skin condition that results in painful lesions on the palms and the soles.1,2 There is debate as to whether PPP is a variant of psoriasis or a separate condition; depending on physical manifestations, one can be diagnosed with palmoplantar plaque psoriasis, PPP, or a combination of the two.3,4
The exact cause of PPP is unknown; however, increased levels of inflammatory cytokines interleukin (IL)-17 and IL-22 may be involved in the pathogenesis of the disease.5 Additional genetic and environmental factors, most significantly smoking, play an important role in its development.2,6
Clinical presentation
Inflammation associated with PPP typically manifests in the classic features of pustules that coalesce and resolve over several days, resulting in brown macules, hyperkeratosis, fissures, and debilitating pain.4,7 Some patients may have co-occurring onycholysis resulting from nail dystrophy and destruction or plaque psoriasis elsewhere on their body.8 PPP often persists for years with periods of exacerbation and remission, and it significantly affects the patient’s ability to perform activities of daily living without pain.8,9 It is exceedingly rare and most commonly affects middle-aged women with a smoking history or current smoking status.7
Continue to: Laboratory diagnosis
Laboratory diagnosis
The diagnosis of PPP is based on clinical presentation and physical exam. Laboratory testing, such as KOH prep, may assist in ruling out dermatophyte infection; a complete blood count may assist in eliminating a bacterial infection as the cause. Skin biopsy is not necessary unless diagnosis is uncertain or prolonged treatment has not produced a response.
Differential diagnosis
The differential diagnosis of PPP includes skin conditions that involve the palms and/or the soles and may have fungal, allergic, or bacterial origins.
Fungal. Tinea manuum (palms) and tinea pedis (soles) result from dermatophyte infection and manifest with erythema and/or scaling and pruritis. A positive KOH examination can confirm diagnosis. On examination, fungal infections are commonly unilateral and asymmetric.8 Treatment with an antifungal agent should result in resolution of symptoms.
Allergic. Contact with an allergen can result in skin erythema, pruritis, and pain at the exposed area. Contact dermatitis can result from an inflammatory response to an allergen or irritant, and it is often localized and well demarcated. This is an acute condition that resolves over time with antihistamines and avoidance of irritants.
Dyshidrotic eczema results in small, pruritic blisters on the palms and the soles and can be recurrent and related to seasonal allergen exposure. Diagnosis is made from history and physical exam. Treatment often consists of emollients and occasionally topical steroids, depending on the severity.
Continue to: Bacterial
Bacterial. A primary bacterial cause of bilateral skin lesions on the palms and the soles is uncommon. However, any open skin lesion can result in secondary bacterial infection. The pustules of PPP are often sterile and do not require bacterial culture; however, additional symptoms of fever, purulence, warmth, and worsening of symptoms may prompt further evaluation for a bacterial origin or complication.
Management
Due to limited quality data on treatment recommendations, the treatment options for PPP vary greatly. Most studies recommend topical versus systemic therapy for initial management.1-2,8,10-11 Firstline therapy often consists of topical corticosteroids and occlusive dressings, followed by oral retinoids (acitretin, alitretinoin) or photochemotherapy.1,8 Third-line therapy can include immunosuppressants (ciclosporin, methotrexate) or biologics (secukinumab).1,12 Recent data have shown positive results with vitamin D3 analogs (maxacalcitol, betamethasone butyrate propionate) as monotherapy or in combination with corticosteroids.10-11 Duration of therapy ranges from 4 to 8 weeks throughout the literature, depending on severity; however, many patients see improvement in the first few weeks.
Conservative measures to maintain remission include smoking cessation, skin emollients, and avoidance of irritants. It is important to educate patients about the chronicity of the disease and early treatment to prevent secondary infection or significant impact on quality of life.
OUTCOME FOR THE CASE PATIENT
The patient was prescribed triamcinolone acetonide (0.5% ointment applied bid), to be used until symptoms improved. After 1 week of treatment, she confirmed (verbally) that symptoms had resolved. She declined a follow-up visit or referral to dermatology.
CONCLUSION
Although PPP is fairly uncommon, it is important for clinicians to consider this diagnosis in patients presenting with localized rash on their palms and soles. This debilitating condition greatly affects a patient’s quality of life and, although it is chronic in nature, available treatments described in the literature have shown success in both acute resolution and ongoing remission of the disease.
1. Sevrain M, Richard M-A, 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.
2. Olazagasti JM, Ma JE, Wetter DA. Clinical features, etiological factors, associated disorders, and treatment of palmoplantar pustulosis: the Mayo Clinic experience, 1996-2013. Mayo Clin Proc. 2017;92(9):1351-1358.
3. Bissonnette R, Suárez-Fariñas M, Li X, et al. Based on molecular profiling of gene expression, palmoplantar pustulosis and palmoplantar pustular psoriasis are highly related diseases that appear to be distinct from psoriasis vulgaris. PLoS One. 2016;11(5):1-11.
4. Raposo I, Torres T. Palmoplantar psoriasis and palmoplantar pustulosis: current treatment and future prospects. Am J Clin Dermatol. 2016;17(4):349-358.
5. Bissonnette R, Fuentes-Duculan J, Mashiko S, et al. Palmoplantar pustular psoriasis (PPPP) is characterized by activation of the IL-17A pathway. J Dermatol Sci. 2017;85(1):20-26.
6. Misiak-Galazka M, Wolska H, Rudnicka L. What do we know about palmoplantar pustulosis? J Eur Acad Dermatol Venereol. 2017;31(1):38-44.
7. Brunasso AMG, Puntoni M, Aberer W, et al. Clinical and epidemiological comparison of patients affected by palmoplantar plaque psoriasis and palmoplantar pustulosis: a case series study. Br J Dermatol. 2013;168(6):1243-1251.
8. Engin B, As¸kın Ö, Tüzün Y. Palmoplantar psoriasis. Clin Dermatol. 2017; 35(1):19-27.
9. Chung J, Callas Duffin K, Takeshita J, et al. Palmoplantar psoriasis is associated with greater impairment of health-related quality of life compared to moderate-to-severe plaque psoriasis. J Am Acad Dermatol. 2014;71(4):623-632.
10. Umezawa Y, Nakagawa H, Tamaki K. Phase III clinical study of maxacalcitol ointment in patients with palmoplantar pustulosis: a randomized, double-blind, placebo-controlled trial. J Dermatol. 2016;43(3):288-293.
11. Muro M, Kawakami H, Matsumoto Y, et al. Topical combination therapy with vitamin D3 and corticosteroid ointment for palmoplantar pustulosis: a prospective, randomized, left-right comparison study. J Dermatolog Treat. 2016;27(1):51-53.
12. Gottlieb A, Sullivan J, van Doorn M, et al. Secukinumab shows significant efficacy in palmoplantar psoriasis: results from GESTURE, a randomized controlled trial. J Am Acad Dermatol. 2017;76(1):70-80.
1. Sevrain M, Richard M-A, 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.
2. Olazagasti JM, Ma JE, Wetter DA. Clinical features, etiological factors, associated disorders, and treatment of palmoplantar pustulosis: the Mayo Clinic experience, 1996-2013. Mayo Clin Proc. 2017;92(9):1351-1358.
3. Bissonnette R, Suárez-Fariñas M, Li X, et al. Based on molecular profiling of gene expression, palmoplantar pustulosis and palmoplantar pustular psoriasis are highly related diseases that appear to be distinct from psoriasis vulgaris. PLoS One. 2016;11(5):1-11.
4. Raposo I, Torres T. Palmoplantar psoriasis and palmoplantar pustulosis: current treatment and future prospects. Am J Clin Dermatol. 2016;17(4):349-358.
5. Bissonnette R, Fuentes-Duculan J, Mashiko S, et al. Palmoplantar pustular psoriasis (PPPP) is characterized by activation of the IL-17A pathway. J Dermatol Sci. 2017;85(1):20-26.
6. Misiak-Galazka M, Wolska H, Rudnicka L. What do we know about palmoplantar pustulosis? J Eur Acad Dermatol Venereol. 2017;31(1):38-44.
7. Brunasso AMG, Puntoni M, Aberer W, et al. Clinical and epidemiological comparison of patients affected by palmoplantar plaque psoriasis and palmoplantar pustulosis: a case series study. Br J Dermatol. 2013;168(6):1243-1251.
8. Engin B, As¸kın Ö, Tüzün Y. Palmoplantar psoriasis. Clin Dermatol. 2017; 35(1):19-27.
9. Chung J, Callas Duffin K, Takeshita J, et al. Palmoplantar psoriasis is associated with greater impairment of health-related quality of life compared to moderate-to-severe plaque psoriasis. J Am Acad Dermatol. 2014;71(4):623-632.
10. Umezawa Y, Nakagawa H, Tamaki K. Phase III clinical study of maxacalcitol ointment in patients with palmoplantar pustulosis: a randomized, double-blind, placebo-controlled trial. J Dermatol. 2016;43(3):288-293.
11. Muro M, Kawakami H, Matsumoto Y, et al. Topical combination therapy with vitamin D3 and corticosteroid ointment for palmoplantar pustulosis: a prospective, randomized, left-right comparison study. J Dermatolog Treat. 2016;27(1):51-53.
12. Gottlieb A, Sullivan J, van Doorn M, et al. Secukinumab shows significant efficacy in palmoplantar psoriasis: results from GESTURE, a randomized controlled trial. J Am Acad Dermatol. 2017;76(1):70-80.
