Pustulosis palmaris et plantaris: Difference between revisions

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{{Infobox medical condition (new)
{{Short description|A chronic skin condition affecting the palms and soles}}
| name            = Pustulosis palmaris et plantaris
| synonyms        ='''Pustulosis of palms and soles,'''<ref name="Bolognia">{{cite book |author=Rapini, Ronald P. |author2=Bolognia, Jean L. |author3=Jorizzo, Joseph L. |title=Dermatology: 2-Volume Set |publisher=Mosby |location=St. Louis |year=2007 |pages= |isbn=978-1-4160-2999-1 |oclc= |doi= |accessdate=}}</ref> '''Palmoplantar pustulosis''', '''Persistent palmoplantar pustulosis''', '''Pustular psoriasis of the [[Harold Wordsworth Barber|Barber]] type''', and '''Pustular psoriasis of the extremities'''
| image          = Pustular Psoriasis.jpg
| caption        = Pustulosis palmaris et plantaris
| pronounce      =
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| onset          =
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| types          =
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| risks          =
| diagnosis      =
| differential    = SAPHO syndrome
| prevention      =
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== '''Alternate names''' ==
Palmoplantar pustulosis; LPP; PPP; Localized pustular psoriasis


== '''Definition''' ==
'''Pustulosis palmaris et plantaris''' is a chronic inflammatory skin condition characterized by the presence of pustules on the palms of the hands and the soles of the feet. It is considered a variant of [[psoriasis]] and is sometimes referred to as palmoplantar pustulosis.
Pustulosis palmaris et plantaris (PPP) is a rare and chronic inflammatory condition that primarily affects the palms of the hands and soles of the feet.
The classification of PPP is controversial. Some have proposed that PPP is a variant of [[psoriasis]], while others believe it to be a separate disease.


== '''Cause''' ==
==Clinical Presentation==
* The cause of pustulosis palmaris et plantaris (PPP) is not fully understood, though many theories have been proposed. Some have suggested PPP may be a disorder of the sweat glands.
Pustulosis palmaris et plantaris primarily affects the [[palms]] and [[soles]], presenting with sterile pustules that can coalesce into larger areas of pus-filled blisters. These pustules are often accompanied by erythema (redness) and scaling. The condition can be painful and may significantly impact a patient's quality of life due to discomfort and the potential for secondary [[infection]].
* Environmental factors are believed to play a part as well.
* For instance, there is a strong association between smoking and PPP.
* Other environmental factors that may contribute to PPP include [[stress]], infections (especially [[tonsillitis]] and [[sinusitis]]), and certain medications. Some have also suggested an association with allergies to certain metals.
* Although most cases occur in people with no family history of the condition, more than one family member can be affected.
* This suggests that genetic factors may contribute to the development of the condition in some cases.


== '''Signs and symptoms''' ==
==Pathophysiology==
* Signs and symptoms of the condition generally develop during adulthood and include crops of [[pustule]]s on one or both hands and/or feet that erupt repeatedly over time.  
The exact cause of pustulosis palmaris et plantaris is not fully understood, but it is believed to be an autoimmune disorder. The condition is associated with an abnormal immune response that leads to the accumulation of neutrophils in the epidermis, resulting in pustule formation. Genetic factors, environmental triggers, and lifestyle factors such as smoking have been implicated in the development and exacerbation of the disease.
* Affected individuals may also experience itching ([[pruritus]]), pain, or a burning sensation.
* Though the area of involvement is often limited, PPP can have a significant effect on quality of life and can interfere with walking or other daily activities.


== '''Diagnosis''' ==
==Diagnosis==
A diagnosis of pustulosis palmaris et plantaris (PPP) was made by histopathological examination that reveals [[hyperkeratosis]] with [[orthoheratosis]], [[acanthosis]], [[spongiosis]] and [[Papillomatosis of breasts|papillomatosis]].  
Diagnosis of pustulosis palmaris et plantaris is primarily clinical, based on the characteristic appearance of the pustules on the palms and soles. A [[skin biopsy]] may be performed to rule out other conditions and to confirm the diagnosis by demonstrating the presence of neutrophils in the epidermis.
A subgranular [[pustule]], filled with polymorph nuclear neutrophils, was also observed within the [[epidermis]]. <ref>Uzun, G., Karabacak, E., Mutluoglu, M., & Aydin, E. (2013). Pustulosis palmaris et plantaris. BMJ case reports, 2013, bcr2013009400. https://doi.org/10.1136/bcr-2013-009400</ref>.


== '''Treatment''' ==
==Treatment==
* Although there is no cure for pustulosis palmaris et plantaris, various measures may be useful to manage symptoms.  
Treatment of pustulosis palmaris et plantaris can be challenging and often requires a combination of therapies. Topical treatments such as corticosteroids and vitamin D analogs are commonly used to reduce inflammation and promote healing. In more severe cases, systemic treatments such as [[methotrexate]], [[cyclosporine]], or biologic agents may be necessary. Phototherapy is another option that can be effective for some patients.
* General suggestions include smoking cessation and [[Emollient|emollients]] (non-cosmetic moisturizers) to soften dry skin and prevent cracking.  
* [[Topical corticosteroids]] (steroids), an oral [[retinoid]] such as [[acitretin]], and [[photochemotherapy]] (ultraviolet radiation treatment) are often first-line treatments.
* Other methods that may be utilized when initial treatment options fail include a combination of oral retinoid and photochemotherapy or immunosuppressive therapy with medications such as [[cyclosporine]].


== '''References''' ==
==Prognosis==
<references />
The prognosis for pustulosis palmaris et plantaris varies. While some patients may experience periods of remission, others may have chronic, persistent symptoms. The condition can be difficult to manage, and ongoing treatment is often required to control symptoms and prevent flare-ups.


==Related Pages==
* [[Psoriasis]]
* [[Autoimmune disease]]
* [[Dermatology]]
* [[Skin biopsy]]


{{Papulosquamous disorders}}
[[Category:Dermatology]]
{{Authority control}}
[[Category:Autoimmune diseases]]
[[Category:Psoriasis]]
[[Category:Skin conditions]]
[[Category:Recalcitrant palmoplantar eruptions]]
 
{{Cutaneous-condition-stub}}
<gallery>
File:Pustular_Psoriasis.jpg
</gallery>

Revision as of 19:16, 22 March 2025

A chronic skin condition affecting the palms and soles


Pustulosis palmaris et plantaris is a chronic inflammatory skin condition characterized by the presence of pustules on the palms of the hands and the soles of the feet. It is considered a variant of psoriasis and is sometimes referred to as palmoplantar pustulosis.

Clinical Presentation

Pustulosis palmaris et plantaris primarily affects the palms and soles, presenting with sterile pustules that can coalesce into larger areas of pus-filled blisters. These pustules are often accompanied by erythema (redness) and scaling. The condition can be painful and may significantly impact a patient's quality of life due to discomfort and the potential for secondary infection.

Pathophysiology

The exact cause of pustulosis palmaris et plantaris is not fully understood, but it is believed to be an autoimmune disorder. The condition is associated with an abnormal immune response that leads to the accumulation of neutrophils in the epidermis, resulting in pustule formation. Genetic factors, environmental triggers, and lifestyle factors such as smoking have been implicated in the development and exacerbation of the disease.

Diagnosis

Diagnosis of pustulosis palmaris et plantaris is primarily clinical, based on the characteristic appearance of the pustules on the palms and soles. A skin biopsy may be performed to rule out other conditions and to confirm the diagnosis by demonstrating the presence of neutrophils in the epidermis.

Treatment

Treatment of pustulosis palmaris et plantaris can be challenging and often requires a combination of therapies. Topical treatments such as corticosteroids and vitamin D analogs are commonly used to reduce inflammation and promote healing. In more severe cases, systemic treatments such as methotrexate, cyclosporine, or biologic agents may be necessary. Phototherapy is another option that can be effective for some patients.

Prognosis

The prognosis for pustulosis palmaris et plantaris varies. While some patients may experience periods of remission, others may have chronic, persistent symptoms. The condition can be difficult to manage, and ongoing treatment is often required to control symptoms and prevent flare-ups.

Related Pages