The term pustular psoriasis is used for a heterogeneous group of diseases that share pustular skin characteristics. [1]
Contents
| Pustular Psoriasis | |
|---|---|
 | |
| Severe pustular psoriasis. | |
| Types | localized pustular psoriasis, generalized pustular psoriasis |
The term pustular psoriasis is used for a heterogeneous group of diseases that share pustular skin characteristics. [1]
| Pustular Psoriasis | |
|---|---|
| | |
| Severe pustular psoriasis. | |
| Types | localized pustular psoriasis, generalized pustular psoriasis |
Characteristics may vary according to the subtype of pustular psoriasis. For example, it can be localized, commonly to the hands and feet (localized pustular psoriasis), or generalized with widespread patches appearing randomly on any part of the body (generalized pustular psoriasis). [2] [3] However, all forms of pustular psoriasis share in common the presence of red and tender blotchy skin covered with pustules. [1]
Pustular psoriasis can be localized, commonly to the hands and feet (palmoplantar pustulosis), or generalized with widespread patches occurring randomly on any part of the body. Acrodermatitis continua is a form of localized psoriasis limited to the fingers and toes that may spread to the hands and feet. [4] Pustulosis palmaris et plantaris is another form of localized pustular psoriasis similar to acrodermatitis continua with pustules erupting from red, tender, scaly skin found on the palms of the hands and the soles of the feet. [1]
Pustular psoriasis is classified into two major forms: localized and generalized pustular psoriasis. [1] Within these two categories there are several variants:
| Classification of Localized and Generalized Pustular Psoriasis |
|---|
Localized pustular psoriasis
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Generalized pustular psoriasis
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Main article: Generalized pustular psoriasis
Generalized pustular psoriasis (GPP) is also known as (von Zumbusch) acute generalized pustular psoriasis in acute cases, and as impetigo herpetiformis during pregnancy. [3] [5] GPP is a rare and severe form of psoriasis that may require hospitalization. This form of psoriasis is characterized by an acute onset of numerous pustules on top of tender red skin. This skin eruption is often accompanied by a fever, muscle aches, nausea, and an elevated white blood cell count. [1]
A rare form of GPP, annular pustular psoriasis (APP), is the most common type seen during childhood. [6] APP tends to occur in women more frequently than in men, and is usually less severe than other forms of generalized pustular psoriasis such as impetigo herpetiformis. [6] This form of psoriasis is characterized by ring-shaped plaques with pustules around the edges and yellow crusting. [6] APP most often affects the torso, neck, arms, and legs. [6]
Psoriasis is a long-lasting, noncontagious autoimmune disease characterized by patches of abnormal skin. These areas are red, pink, or purple, dry, itchy, and scaly. Psoriasis varies in severity from small localized patches to complete body coverage. Injury to the skin can trigger psoriatic skin changes at that spot, which is known as the Koebner phenomenon.
Pyoderma gangrenosum is a rare, inflammatory skin disease where painful pustules or nodules become ulcers that progressively grow. Pyoderma gangrenosum is not infectious.
Pustulosis is highly inflammatory skin condition resulting in large fluid-filled blister-like areas - pustules. Pustulosis typically occurs on the palms of the hands and/or the soles of the feet. The skin of these areas peels and flakes (exfoliates). This condition—also referred to as "palmo-plantar pustulosis"—is a feature of pustular psoriasis.
Interleukin 36 receptor antagonist (IL-36RA) is a member of the interleukin-36 family of cytokines. It was previously named Interleukin-1 family member 5 (IL1F5).
Neonatal acne, also known as acne neonatorum, is a type of acne that develops in newborns, typically before 6 weeks of life. It presents with open and closed comedones on the cheeks, chin and forehead.
Impetigo herpetiformis is a form of severe pustular psoriasis occurring in pregnancy which may occur during any trimester.
Erosive pustular dermatitis of the scalp presents with pustules, erosions, and crusts on the scalp of primarily older Caucasian females, and on biopsy, has a lymphoplasmacytic infiltrate with or without foreign body giant cells and pilosebaceous atrophy.
Psoriatic erythroderma represents a form of psoriasis that affects all body sites, including the face, hands, feet, nails, trunk, and extremities. This specific form of psoriasis affects 3 percent of persons diagnosed with psoriasis. First-line treatments for psoriatic erythroderma include immunosuppressive medications such as methotrexate, acitretin, or ciclosporin.
Generalized pustular psoriasis (GPP) is an extremely rare type of psoriasis that can present in a variety of forms. Unlike the most general and common forms of psoriasis, GPP usually covers the entire body and with pus-filled blisters rather than plaques. GPP can present at any age, but is rarer in young children. It can appear with or without previous psoriasis conditions or history, and can reoccur in periodic episodes.
Localized pustular psoriasis presents as two distinct conditions that must be considered separate from generalized psoriasis, and without systemic symptoms, these two distinct varieties being pustulosis palmaris et plantaris and acrodermatitis continua.
Pustulosis palmaris et plantaris is a chronic recurrent pustular dermatosis localized on the palms and soles only, characterized histologically by intraepidermal pustules filled with neutrophils. It can occur as part of the SAPHO syndrome.
Acute generalized exanthematous pustulosis (AGEP) is a rare skin reaction that in 90% of cases is related to medication.
Linear IgA bullous dermatosis is a rare immune-mediated blistering skin disease frequently associated with medication exposure, especially vancomycin, with men and women being equally affected. It was first described by Tadeusz Chorzelski in 1979 and may be divided into two types:
Stasis papillomatosis is a disease characterized by chronic congestion of the extremities, with blood circulation interrupted in a specific area of the body. A consequence of this congestion and inflammation is long-term lymphatic obstruction. It is also typically characterized by the appearance of numerous papules. Injuries can range from small to large plates composed of brown or pink, smooth or hyperkeratotic papules. The most typical areas where injuries occur are the back of the feet, the toes, the legs, and the area around a venous ulcer formed in the extremities, although the latter is the rarest of all. These injuries include pachydermia, lymphedema, lymphomastic verrucosis and elephantosis verrucosa. The disease can be either localized or generalized; the localized form makes up 78% of cases. Treatment includes surgical and pharmaceutical intervention; indications for partial removal include advanced fibrotic lymphedema and elephantiasis. Despite the existence of these treatments, chronic venous edema, which is a derivation of stasis papillomatosis, is only partially reversible. The skin is also affected and its partial removal may mean that the skin and the subcutaneous tissue are excised. A side effect of the procedure is the destruction of existing cutaneous lymphatic vessels. It also risks papillomatosis, skin necrosis and edema exacerbation.
Interleukin 36, or IL-36, is a group of cytokines in the IL-1 family with pro-inflammatory effects. The role of IL-36 in inflammatory diseases is under investigation.
Von Zumbusch (acute) generalized pustular psoriasis is the most severe form of generalized pustular psoriasis, and can be associated with life-threatening complications.
Spesolimab, sold under the brand name Spevigo, is a monoclonal antibody used for the treatment of generalized pustular psoriasis (GPP). It is an interleukin-36 receptor (IL-36R) antagonist. It is given via injection into a vein.
Joel M. Gelfand is an American dermatologist and epidemiologist at the University of Pennsylvania in Philadelphia, Pennsylvania. He currently serves as the James J. Leyden Professor in Clinical Investigation, the Vice Chair of Clinical Research, the director of the Psoriasis and Phototherapy Treatment Center, and the medical director of the Clinical Studies Unit in the Department of Dermatology at the Perelman School of Medicine at the University of Pennsylvania. He studies systemic comorbidities of psoriasis and much of his research has centered on the connection between cardiovascular disease and psoriasis.
Neonatal pustular eruptions are a group of disorders characterized by various forms of pustulosis seen in the first four weeks of life.