This classification takes into consideration both clinical aspects and response to treatment, and allows a prognosis of the various types of pustular psoriasis. Owing to its mild course and good treatment results, EACP represents a special form of psoriasis pustulosa generalisata. In our group of patients, systemic glucocorticosteroid therapy has proved deleterious, whereas oral photochemotherapy and etretinate have been found to be highly effective modes of therapy with only mild side effects and have been able to induce even permanent remission in EACP. This site needs JavaScript to work properly. Please enable it to take advantage of the complete set of features! Clipboard, Search History, and several other advanced features are temporarily unavailable.

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Psoriasis vulgaris is a chronic inflammatory disease that classically affects skin and joints and is associated with numerous comorbidities. There are several clinical subtypes of psoriasis including the uncommon pustular variants, which are subdivided into generalized and localized forms. Generalized forms of pustular psoriasis include acute generalized pustular psoriasis, pustular psoriasis of pregnancy, and infantile and juvenile pustular psoriasis. Localized forms include acrodermatitis continua of Hallopeau and palmoplantar pustular psoriasis.

These subtypes vary in their presentations, but all have similar histopathologic characteristics. The immunopathogenesis of each entity remains to be fully elucidated and some debate exists as to whether these inflammatory pustular dermatoses should be classified as entities distinct from psoriasis vulgaris.

Due to the rarity of these conditions and the questionable link to the common, plaque-type psoriasis, numerous therapies have shown variable results and most entities remain difficult to treat. With increasing knowledge of the pathogenesis of these variants of pustular psoriasis, the development and use of biologic and other immunomodulatory therapies holds promise for the future of successfully treating pustular variants of psoriasis. Psoriasis vulgaris is a chronic inflammatory condition associated with significant morbidity and mortality.

Psoriasis can also involve the nails and scalp or progress to erythroderma. Less common clinical presentations include guttate, inverse, or pustular psoriasis. Within the classification of pustular psoriasis, the disease is further subdivided into generalized pustular psoriasis GPP and localized pustular psoriasis. Localized pustular psoriasis includes palmoplantar psoriasis and acrodermatitis continua of Hallopeau ACH. All variants of pustular psoriasis have similar presentations in that they involve an eruption of superficial pustules in a given distribution, typically with an erythematous base or studded on a background of erythema.

As such, histopathologic examination of all variants reveals parakeratosis, extensive mononuclear and neutrophilic inflammatory infiltrate in the epidermis, and epidermal edema and hyperplasia. Acute GPP, also known as von Zumbusch type, is a dermatologic disease for which classification is somewhat debated. However, recent research indicates that acute GPP and plaque psoriasis are genetically distinct conditions without a common pathophysiologic mechanism.

Acute GPP is associated with significant morbidity and in some cases, mortality, especially in the absence of appropriate treatment.

The pustules often expand and coalesce, forming lakes of pus. Notes: Innumerable pustules studded on a background of erythematous plaques on the chest, abdomen, and upper arms. Some areas of pustules have coalesced into large plaques of crust on the lower abdomen. The mean age at onset of acute GPP is The use and subsequent withdrawal of systemic corticosteroids is an important cause of an eruption of acute GPP. Acute withdrawal of systemic corticosteroids can lead to an acute inflammatory process acting as a trigger for acute GPP.

Upper respiratory tract infections, and other infections, can also lead to an acute activation of neutrophils, which may act as a trigger for the onset of acute GPP. GPP may also be strongly associated with certain predisposing genetic factors. ILR can be located on a number of cells found in the skin, including dendritic cells, keratinocytes, and monocytes. Activation of ILR within these cells leads to activation of inflammation within the skin and development of the clinical features of inflammatory skin conditions, specifically GPP.

GPP and its important genetic association with ILRA deficiency has also been shown to exhibit a gene dosage effect. Another study regarding the various possible genetic mutations associated with GPP was conducted in nine children from six families who presented with neonatal sterile osteomyelitis, periostitis, and generalized pustulosis. This study discovered the condition present in these children was linked to a genetic mutation involving IL1RN , which encodes the IL-1 receptor antagonist.

While the treatment of chronic plaque psoriasis has become relatively effective in the era of biologic therapies, treatment of pustular forms of psoriasis remains an area lacking in research, in part due to the rarity of the disease.

Acute GPP has proven to be, like many psoriasis variants, a difficult disease to treat. Guidelines published in by the Medical Board of the National Psoriasis Foundation recommend acitretin, cyclosporine, or methotrexate to be the first-line therapies for acute GPP Table 1.

One study described the efficacy in nine of ten patients with acute GPP who experienced results within 24 hours to 7 days of the first treatment. The recommended second-line therapies include adalimumab, etanercept, psoralen plus ultraviolet-A PUVA phototherapy, topical corticosteroids, topical calcipotriene, and topical tacrolimus, or combination therapy for recalcitrant disease which includes a first-line oral systemic agent plus a biologic agent such as adalimumab or etanercept.

If necessary, however, they may be used in circumstances in which other therapeutic options are not available. Despite reports of successful treatment of acute GPP with various biologic agents, the literature supporting the treatment of acute GPP is weak. However, the classification of this dermatitis as a disease distinct from pustular psoriasis is still debated, and in various studies, it continues to be discussed as impetigo herpetiformis.

Pustular psoriasis of pregnancy is a rare autoimmune inflammatory dermatosis observed most commonly in the second half to third trimester of pregnancy. Some publications, however, report the occurrence as early as the first month of pregnancy.

Patients typically present with an eruption of numerous sterile pustules overlying annular or polycyclic erythematous patches, with lesions usually beginning within skin folds and spreading radially.

The presence of pustular psoriasis of pregnancy has been historically associated with poor neonatal outcomes including placental insufficiency, stillbirth, fetal abnormalities, and early neonatal death, with a correlation between severity and duration of the disease and poor neonatal prognosis.

The pathophysiology of pustular psoriasis of pregnancy is even more elusive than in other forms of pustular psoriasis, which is likely due to the low incidence of the disease. Literature on the theoretical pathogenesis of pustular psoriasis of pregnancy typically discusses possible triggers associated with development of the disease. Potential triggers include increasing levels of progesterone during the last trimester of pregnancy, hypocalcemia, and lower levels of elafin, an epidermal skin-derived antileukoproteinase, which may all contribute to the production of epidermal pustules.

Another important consideration in defining the theoretical pathogenesis of pustular psoriasis of pregnancy involves specific situations in which patients suffer from an acute eruption or worsening of GPP during pregnancy. Pregnancy has been noted as a precipitating factor for the new development or acute flare of GPP. Patients should be assessed by their obstetrician for potential early induction of labor as the first and best therapeutic option for this disease. In terms of therapeutic options, systemic corticosteroids are widely accepted to be the most commonly used first-line treatment for pustular psoriasis of pregnancy Table 2.

Note: Induction of labor for delivery of the infant should be performed if possible. As mentioned, delivery of the fetus often results in rapid resolution of the dermatitis, which should be considered in cases of severe disease despite optimal medical management. While psoriasis is a common disease in children and adolescents, pustular psoriasis in patients younger than 18 years is exceedingly rare.

The etiology and pathogenesis of juvenile pustular psoriasis are unknown. The rarity of pustular psoriasis in children has also led to a lack of evidence-based treatment literature. First-line therapy for this disease is similar to that of pustular psoriasis in adults. Acitretin, cyclosporine, etanercept, or methotrexate can each be used safely as first-line therapeutic options in children with pustular psoriasis Table 3.

Palmoplantar pustular psoriasis PPP presents clinically as sterile pustules with erythema, hyperkeratosis, and scaling on the palms and soles Figure 2.

Notes: A, B Multiple pustules studded on the palmar surface of the hands with a few areas of erythematous desquamative changes. C, D Innumerable pustules and small areas of hyperpigmentation in areas of prior pustules noted on the plantar feet with desquamation and mild erythema.

Multiple precipitating factors have been found to predispose patients to the induction of palmoplantar psoriasis, including smoking, metal sensitivities, infections, trauma, stress, and medications. Smoking is widely known as one of the most important precipitating factors for the development of PPP. Patients with PPP are thought to have an abnormal response to nicotine, resulting in inflammation with smoking.

Sensitivities to metals have also been cited as precipitating factors in PPP. Infections are another well-recognized precipitating factor for PPP. The most commonly cited infection resulting in an eruption or exacerbation of PPP is tonsillitis. The Koebner phenomenon can also be associated with new lesions in PPP. Stressful psychological conditions also impact the onset and worsening of PPP. Furthermore, a seasonal influence has also been described for the onset of PPP with the onset and exacerbation worsened by humid and hot conditions.

In support of the theory that PPP is a clinical variant of psoriasis, authors have discussed the morphological, clinical, and histological similarities between PPP and psoriasis. Brunasso et al 83 supported the same relationship with a retrospective study comparing two groups of patients, one with PPP and the other with psoriasis, and found close similarities between the groups in age of onset, family history of psoriasis, duration of the disease, comorbidities, and smoking status.

Eriksson et al 9 discussed similarities regarding the histology of the two diseases, specifically regarding neutrophil dysfunction and specific serum studies including eosinophilic cationic protein elevations and other chemokinetic effects in PPP compared to psoriasis vulgaris. Authors in support of the theory that PPP is a distinct entity from psoriasis vulgaris argue several different points.

The pathophysiology of PPP remains as obscure as its classification. It is widely accepted, however, that the eccrine sweat gland is significant to its pathogenesis. Specifically, the acrosyringium the most intraepidermal portion of the eccrine sweat gland consisting of the terminal spiral duct serves as the primary site of inflammation and pustule formation, as supported by evidence of specific chemicals unique to eccrine sweat glands found in the cells lining the vesicles of PPP patients.

Langerhans cells can be found in increased numbers in both lesional and nonlesional skin of PPP patients, supporting an antigen-driven process for inflammation in the disease. Palmoplantar forms of psoriasis are notoriously recalcitrant to therapy. Like other pustular psoriasis forms, high-quality data regarding the treatment options and efficacies are lacking for PPP. First-line topical therapy involves corticosteroids under occlusion Table 4. Second-line topical therapy includes calcipotriene, PUVA, photodynamic therapy, and tacrolimus.

Second-line systemic therapy involves the use of cyclosporine and biologic agents. The use of biologic agents in the treatment of PPP is becoming more pervasive, given the recalcitrant nature of the disease. Cases of PPP successfully treated with efalizumab, an anti-leukocyte function-associated antigen-1 monoclonal antibody, have also been reported.

ACH is another localized form of pustular psoriasis which is historically described using a number of various aliases including acrodermatitis perstans, dermatitis repens, acropustulosis, and pustular acrodermatitis. ACH is a chronic form of pustular psoriasis that characteristically involves the digits and most commonly, the fingertips and nails. Note: Erythematous plaques, hyperkeratosis, and pustules confined to the distal tips of all digits with nail involvement.

Those precipitating factors most closely associated with ACH include localized trauma to the distal portion of a digit and localized infections. Treatment of ACH is primarily based on data from case reports. ACH is notoriously recalcitrant to many therapies. Second-line topical treatments include calcipotriol, tacrolimus, and fluorouracil, or a combination of these medications.

Biologic agents have more recently become the mainstay of treatment in recalcitrant forms of ACH. Ustekinumab was successfully used to treat a patient with ACH.

Evidence-based treatment strategies for ACH are lacking due to the difficulty in diagnosis of ACH and the lack of knowledge regarding the pathophysiology and etiology of the disease.

Future management of ACH requires larger controlled studies and further studies on the pathophysiology of the disease, which will likely guide the development of new and effective medications for treatment. There are several clinical subtypes of psoriasis including the uncommon pustular variants, which include acute GPP, pustular psoriasis of pregnancy, infantile and juvenile pustular psoriasis, ACH, and PPP.

These variants have similar histopathologic characteristics. However, the immunopathogenesis of each entity remains to be fully elucidated. As such, numerous therapies have shown variable results and most entities remain difficult to treat.


What Does Pustular Psoriasis Look Like?

Psoriasis vulgaris is a chronic inflammatory disease that classically affects skin and joints and is associated with numerous comorbidities. There are several clinical subtypes of psoriasis including the uncommon pustular variants, which are subdivided into generalized and localized forms. Generalized forms of pustular psoriasis include acute generalized pustular psoriasis, pustular psoriasis of pregnancy, and infantile and juvenile pustular psoriasis. Localized forms include acrodermatitis continua of Hallopeau and palmoplantar pustular psoriasis.


Psoriasis pustulosa palmaris et plantaris

Psoriasis is a skin condition that causes red, scaly skin patches. You can get psoriasis at any age, but the average age of adults who get it is 15—35 years old. Psoriasis is not contagious, and it can actually appear in different forms. One of these forms is pustular psoriasis, which produces white, noninfectious pus-filled blisters pustules. Pustular psoriasis can happen in conjunction with other forms of psoriasis, such as plaque psoriasis.


Pustular Psoriasis

Jump to navigation. Pustular [PUHS-choo-lar] psoriasis is characterized by white pustules blisters of noninfectious pus surrounded by red skin. The pus consists of white blood cells and is not an infection, nor is it contagious. Adults are the primary targets for pustular psoriasis and may be limited to certain areas of the body, like the the hands and feet. Generalized pustular psoriasis may also cover most of the body.

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