Annular Pustular Psoriasis Research Articles

Epidemiological survey of patients with pustular psoriasis in the Japanese Society for Psoriasis Research from 2017 to 2020.

The Japanese Society for Psoriasis Research (JSPR) has been conducting annual epidemiological surveys of patients with pustular psoriasis in Japan since 2017. This study aimed to conduct a recent epidemiological analysis of patients with pustular psoriasis who were enrolled in the JSPR from 2017 to 2020. A total of 291 patients from 131 medical institutions were enrolled, of which 47.4% (138 cases) were males and 52.6% (153 cases) were females. The mean ± standard deviation (SD) age of the patients was 57.4 ± 20.3 years (males, 61.2 ± 17.3 years; females, 54.1 ± 22.1 years). The mean ± SD age of the patients at disease onset was 48.5 ± 22.5 years (males, 50.8 ± 20.6 years; females, 46.4 ± 24.0 years). The types of pustular psoriasis observed included the von Zumbusch type (59.8%), annular pustular psoriasis (8.2%), impetigo herpetiformis (6.5%), and acrodermatitis continua of Hallopeau (4.8%), of which, the majority of the patients with impetigo herpetiformis were female. Among the patients, 58.4% were treated with oral medications and 44.0% were treated with biologics. The most common oral medication prescribed was etretinate (52.4%), followed by corticosteroids (24.7%) and cyclosporin (22.9%). The most common biologics used were IL-17 inhibitors (ixekizumab [28.1%] and secukinumab [22.7%]), followed by tumor necrosis factor (TNF) inhibitors (infliximab [15.6%]) and IL-23 inhibitors (guselkumab [14.8%] and risankizumab [10.2%]). This survey thus provides new and significant information regarding the recent perspective of pustular psoriasis, such as patient characteristics and treatment trends, in Japan.

Clinical Course and Characteristics of Generalized Pustular Psoriasis

Generalized pustular psoriasis (GPP) is a rare, potentially life-threatening disease characterized by episodes of widespread sterile macroscopic pustules, with or without systemic inflammation and/or plaque psoriasis. Multiple GPP subtypes have been described, from acute GPP of von Zumbusch to milder, annular pustular psoriasis. Generalized pustular psoriasis mainly affects adults, with a female preponderance, but juvenile GPP also occurs. Flares are a hallmark of GPP and may occur de novo or be provoked by triggers, including withdrawal of systemic corticosteroids, infections, stress, pregnancy, and menstruation. Severity of flares varies widely between patients, and between flares in an individual patient. Significant flares are often accompanied by systemic symptoms, notably fever, general malaise, and extracutaneous manifestations such as arthritis, uveitis, and neutrophilic cholangitis. Common laboratory abnormalities include neutrophilia, elevated C-reactive protein levels, hypocalcemia, and abnormal liver function tests. The clinical course of GPP is highly variable; it can be a relapsing disease with recurrent flares and no pustulation between flares or a persistent disease with perpetual mild pustulation punctuated with flares of greater severity. Patients may have multiple flares per year or a flare every few years. Most flares last 2–5 weeks and approximately 50% require hospitalization. Life-threatening complications include sepsis and renal, hepatic, respiratory, and heart failure. Reported mortality rates are 2–16%.

Clinical characteristics of Japanese pustular psoriasis: A multicenter observational study.

Generalized pustular psoriasis (GPP) is a rare and severe subtype of psoriasis. Because of its rarity, GPP studies with a large sample size have been scarce. We studied the characteristics of GPP and pustular psoriasis using data from the West Japan Psoriasis Registry that had been registered until the end of December 2020. The dataset included 104 patients with pustular psoriasis and 1290 patients with other subtypes of psoriasis. Multivariate analysis revealed a significantly greater number of female patients, a significantly lower mean body mass index, and a significantly lower ratio of habitual drinkers in pustular psoriasis, compared to other subtypes of psoriasis. Of the 104 patients, 102 had GPP, including 88 von Zumbusch, 10 juvenile-onset, and four annular pustular psoriasis. Although the male:female ratio of GPP with psoriasis vulgaris (GPP+PsV) (47/20) was similar to that of psoriasis in Japan, the GPP without PsV (GPP-PsV) group highlighted a female predominance (13/22). The mean age at GPP onset was 45.3years, and the mean interval from PsV onset to GPP onset was 12.5years. Four of nine patients with GPP had an IL36RN gene mutation. Infection, medicine, and pregnancy were the precipitating factors for GPP. A family history of psoriasis was present in eight (7.8%) patients with GPP. Twenty-four patients with GPP had psoriatic arthritis. Biologics were used in 76.5% of patients with GPP, followed by etretinate (37.3%), cyclosporine (24.5%), methotrexate (13.7%), apremilast (8.8%), and granulocyte and monocyte adsorption apheresis (6.9%). Etretinate was used in 17 (51.5%) of 33 patients with GPP with less than 10-year history. Thus, etretinate remains a good treatment option for GPP even in the era of biologics. Hypertension was the most commonly identified comorbidity, followed by diabetes. We believe that the characteristics revealed in this study can further contribute to effective GPP management.

Generalized annular pustular psoriasis (Milian-Katchoura type) induced by systemic steroids.

The aim of my clinical image is to recall this rare and benign form of pustular psoriasis and to insist on the role of the triggers of pustular psoriasis, mainly the drugs.

Generalized pustular psoriasis: a review and update on treatment.

Generalized pustular psoriasis (GPP) is a subtype of pustular psoriasis characterized by painful and occasionally disfiguring cutaneous manifestations with sepsis-like systemic symptoms. Affecting any age and race, GPP can occur with other forms of psoriasis or by itself. Stimuli for flares include medications, infections and environmental triggers. The interleukin family and caspase recruitment domain family have been implicated in its pathogenesis. Other forms of pustular psoriasis include impetigo herpetiformis, palmoplantar pustular psoriasis, annular pustular psoriasis and acrodermatitis continua of Hallopeau. Treatment is not well established, but includes the use of retinoids, methotrexate, cyclosporine, corticosteroids, TNF-alpha inhibitors, topical therapy and phototherapy. The use of TNF-alpha inhibitors may result in the formation of antidrug antibodies and should be administered with methotrexate.

Clinicopathologic retrospective analysis of annular pustular psoriasis

Annular pustular psoriasis (APP) is a rare form of pustular psoriasis with a chronic relapsing course and a good prognosis. The clinical picture is characterized by erythematous lesions, usually polycyclic, with the presence of small, sterile pustules on the circumference of the lesions and fine peeling. We present two cases of APP with diagnostic problems: a 65-year-old woman that suffered from intermittent APP with remission and exacerbation for many years, and an 83-year-old man with lesions that developed after atenolol treatment. In both cases, the patients were thought to have drug provocation, and therefore acute generalized exanthematous pustulosis (AGEP) was diagnosed. Only a thorough analysis of the course of the disease and histopathological examination allowed correct diagnosis. The clinical picture of APP is similar to AGEP, generalized pustular psoriasis (GPP), also known as von Zumbusch pustular psoriasis, and subcorneal pustular disease, and it requires accurate differential diagnosis.

Annular pustular psoriasis responding to itraconazole masquerading dermatophytosis both clinically and therapeutically

Annular pustular psoriasis responding to itraconazole masquerading dermatophytosis both clinically and therapeutically

Subcorneal Pustular Dermatosis: A Review of 30 Years of Progress.

Subcorneal pustular dermatosis (SPD), also known as Sneddon-Wilkinson disease, is a rare, benign yet relapsing pustular dermatosis. Its incidence and prevalence have not been well studied. It characteristically presents as hypopyon pustules on the trunk and intertriginous areas of the body. SPD is similar to two other disease entities. Both SPD-type immunoglobulin (Ig)-A pemphigus and annular pustular psoriasis clinically and histologically present similarly to SPD. Immunologic studies separate SPD-type IgA pemphigus from SPD and pustular psoriasis. However, there is still an unclear designation as to whether SPD is its own entity distinct from pustular psoriasis, as the once thought characteristic histologic picture of psoriasis does not hold true for pustular psoriasis. SPD has been reported to occur in association with several neoplastic, immunologic, and inflammatory conditions. Dapsone remains the first-line treatment for SPD, although dapsone-resistant cases have been increasingly reported. Other therapies have been used singly or as adjunctive therapy with success, such as corticosteroids, immunosuppressive agents, tumor necrosis factor inhibitors, and ultraviolet light therapy. This article provides a review of the last 30years of available literature, with a focus on successful treatment options and a suggestion for reappraisal of the classification of SPD.

Annular pustular psoriasis associated with colon cancer

Annular pustular psoriasis (APP), also known as circinate and annular pustular psoriasis, is a rare form of pustular psoriasis characterized by erythematous, annular or polycyclic lesions, eruptions of small pustules and fine desquamation without systemic inflammation [1]. APP runs a benign course, in contrast to generalized pustular psoriasis, which is a severe form of pustular psoriasis with systemic inflammation [2]. We herein describe a patient with APP associated with colon cancer.A 71-year-old [...]

Nonpustular annular psoriasis: a variant of annular pustular psoriasis but without clinical or histopathological evidence of pustule formation

Nonpustular annular psoriasis: a variant of annular pustular psoriasis but without clinical or histopathological evidence of pustule formation

A Rare Case of Annular Pustular Psoriasis Associated with Pemphigus Foliaceus

Dear Editor: A 56-year-old Japanese woman suffered from multiple, scaly erythemas on the trunk for approximately 30 years. She had been previously diagnosed with psoriasis vulgaris in the clinic due to the clinical appearance and histological findings. She was also previously diagnosed with pemphigus foliaceus (PF) at our hospital 10 years ago, based on the clinical appearance and histological findings of a subcorneal blister, as well as on the direct immunofluorescence findings of superficial epidermal intercellular immunoglobulin (Ig) G deposition. During her clinical course, serum anti- desmoglein (Dsg)-1 IgG antibody levels were elevated to 99 index points in 2010, whereas anti-Dsg-3 antibody levels remained at <5 index points. Recently, she was treated for PF by administration of oral betamethasone (0.5 mg/d) and cyclosporine (100 mg/d). She was admitted to our hospital because the annular erythemas with pustular margins on her trunk were exacerbated and accompanied by high fever (Fig. 1). No lesions resembling PF were seen. Laboratory findings were as follows (abnormal values are underlined): white blood cell count, 21.3×103/µl (neutrophils: 72%); C-reactive protein: 16.4 mg/L. Anti-Dsg1 and anti-Dsg3 antibodies were within the normal range. Because of the clinical appearance and the laboratory and histological findings of neutrophilic pustules in the upper spinous layer (Fig. 2), the patient was diagnosed with annular pustular psoriasis (APP). In addition to systemic betamethasone and cyclosporine, she was treated with systemic etretinate (40 mg/d) and topical steroid ointment. Although the patient's high fever resolved rapidly, the annular scaly erythemas gradually spread centrifugally. After cyclosporine was increased to 200 mg/d, the erythemas disappeared. No relapse was seen after the doses of etretinate and cyclosporine were decreased, and she was discharged 15 days after admission. No flare-ups have been observed after a 1.5-year treatment with systemic betamethasone (0.5 mg/d) and tapering of etretinate (20 mg/d) and cyclosporine (100 mg/d). Fig. 1 Clinical appearance of scaly, annular pustular erythemas on the patient's abdomen at the time of admission. The 10 cm-diameter erythemas were scaly and annular with pustular margins. Bacteriological culture of the contents of the pustules was not performed; ... Fig. 2 Histological findings of scaly erythemas with pustules on the abdomen. H&E staining revealed acanthosis, spongiosis, and neutrophilic pustules in the upper spinous layer (×100). Generalized pustular psoriasis (GPP) is a form of psoriasis that is characterized by an eruption of sterile pustules. APP is a rare form of pustular psoriasis, characterized by erythematous, annular, or polycyclic lesions, eruptions of small sterile pustules, and fine desquamation1. It differs from GPP by its more subacute and limited clinical course2. Pemphigus is an autoimmune, bullous skin disease, divided into subtypes including pemphigus vulgaris, PF, and IgA pemphigus. It was previously reported that APP might be associated with IgA pemphigus3. A case of PF with prominent neutrophilic pustules has also been reported4. IgG autoantibody-induced interleukin (IL)-8 expression in keratinocytes was reported to cause neutrophil infiltration into the lesional skin of a patient with a pemphigus variant5. Additionally, systemic steroid therapy can transform psoriasis vulgaris into GPP. This patient had a history of persistent psoriasis vulgaris and annular erythemas with pustules that appeared with a high fever and she never relapsed; therefore, we diagnosed her with APP, not GPP. Her disorder transformed from psoriasis vulgaris associated with PF, which was likely induced by elevated IL-8 levels in PF-related lesions and systemic steroid therapy for PF. In the future, we will analyze epidermal and serum IL-8 levels in similar cases.

Clinical profile, morbidity, and outcome of adult‐onset generalized pustular psoriasis: analysis of 102 cases seen in a tertiary hospital in Johor, Malaysia

Generalized pustular psoriasis (GPP) is a severe but rare variant of psoriasis. Our objective is to review the clinical profile, comorbidities, and outcome of patients with GPP. A retrospective note review of all patients with adult-onset GPP. A total of 102 patients with adult-onset GPP were diagnosed between 1989 and November 2011, with a female to male ratio of 2:1. The mean age at onset of GPP was 40.9years (range: 21-81years). Acute GPP was the most common variant seen (95 cases), followed by four localized variants of GPP and three with annular pustular psoriasis. Fever and painful skin were present in 89% of patients, arthritis in 34.7%, and leukocytosis in 78.4%. Common triggers were systemic steroids (45 cases), pregnancy (17 cases), and upper respiratory tract infections (16 cases). A positive family history of psoriasis and GPP was present in 29% and 11%, respectively. Comorbidities included obesity (42.9%), hypertension (25.7%), hyperlipidemia (25.7%), and diabetes mellitus (23.7%). The mean duration of admission and pustular flare for acute GPP was 10.3days (range: 3-44days) and 16days (range: 7-60days), respectively. Fifty-four patients responded to systemic retinoid, 21 to methotrexate, eight to cyclosporine, and one to adalimumab, but recurrences were common. Our study confirms the poor response of GPP to currently available anti-psoriatic agents, with frequent flare-ups. There is a need for a more effective targeted therapy for this condition.

Etanercept in the Treatment of Generalized Annular Pustular Psoriasis

1. Some authors consider a separate variant of generalized psoriasis, well described by Lapiere, as recurrent circinate erythematous psoriasis. It presents with erythematous, annular or polycyclic lesions, and an eruption of small sterile pustules and fine desquamation. The patches extend from the center and resolve within some weeks, leaving scales and changes in pigmentation and pigmentary changes. Frequent relapses are described in the bordering areas 2,3 . In this study, we report the case of a woman affected by generalized annular pustular (Lapiere) psoriasis. This patient had previously been treated with conventional therapeutics, and demonstrated a significant improvement after treatment with etanercept. This 70-year-old Caucasian woman, described in our report, has a 35-year history of psoriasis. Physical examination revealed the presence of small erythematous papules, centered by a pustule, a few millimeters in diameter (Fig. 1). Histological examination showed Kogoj-Lapiere spongiform multilocular typical pustules (an epidermal pustule formed by infiltration of neutrophils into necrotic areas of the epidermis, where the cell walls form a swampy network), features compatible with the clinical diagnosis of Lapiere psoriasis. The patient had previously been treated with other topical and systemic drugs (colchicine, acitretin, ciclosporin, methotrexate) and ultraviolet B narrow band phototherapy, with partial and temporary benefits, side effects, and frequent relapses. Differential diagnosis of our case included other generalized pustular psoriasis: acute generalized exanthematous pustolosis (AGEP) was perhaps the most important differential diagnosis. AGEP, which occurs as an acute, spontaneously healling reaction to drugs (usually antibiotics), was excluded on the basis of the absence of vasculitis associated with spongiform pustules and based on the presence of psoriatic anamnesis. Lapiere psoriasis can be differentiated from pustular lesions caused by prolonged application of topical steroids or tar ointments on the periphery of pre-existent psoriatic plaques. Unlike the Von Zumbusch generalized form, the general state of health is not compromised. The patient was treated with 50 mg of etanercept twice weekly subcutaneously for three months. There was an

Juvenile localized annular pustular psoriasis developing severe exacerbation after topical indomethacin application

Juvenile localized annular pustular psoriasis developing severe exacerbation after topical indomethacin application

Elevated serum parathyroid hormone levels in a patient with annular pustular psoriasis

Elevated serum parathyroid hormone levels in a patient with annular pustular psoriasis

Primary Annular Plaque‐Type Psoriasis

We described two adolescent girls with untreated, consistently annular, plaque-type psoriasis without pustules, a presentation that is to our knowledge, not previously described. No typical confluent plaque-type lesions were present. The plaques in our patients resembled other entities such as tinea corporis and erythema annulare centrifugum, given the erythematous, scaling borders and central clearing. Biopsy specimens from our patients showed features characteristic of psoriasis vulgaris. Both patients responded to combination therapy with calcipotriene and a mid-potency steroid. We conclude that primary annular plaque-type psoriasis shares features of both typical plaque-type and annular pustular psoriasis, suggesting that these entities represent a spectrum of psoriatic disease.

Pustular psoriasis in childhood

Recurrent annular pustular psoriasis over the trunk of an 11-year-old boy followed infantile psoriasis in the napkin area. Etretinate therapy induced a partial remission. The difficulty in distinguishing between annular pustular psoriasis and subcorneal pustular dermatosis of Sneddon and Wilkinson is discussed.

Annular pustular psoriasis induced by UV radiation from tanning salon use

UV radiation is usually beneficial for psoriasis. Over-exposure, however, rarely leads to generalized pustular psoriasis of von Zumbusch (GPPZ). GPPZ is characterized by repetitive waves of pustules, fiery red erythema, and marked systemic toxicity. Annular pustular psoriasis (APP) differs from GPPZ by its more subacute and limited course and lack of toxicity. 1 A patient was recently encountered in whom APP developed after a burn in a tanning salon, a finding not previously reported

Annular Pustular Psoriasis

Generalized pustular psoriasis is a rare form of psoriasis characterized by the following four clinical patterns: Zumbusch, annular, exanthematic, and localized. 1 In contrast to the Zumbusch variety, the annular type is less severe and benign. It consists of gyrate, annular lesions with an erythematous, scaly, pustular margin. A history of psoriasis vulgaris is usually absent. Subcorneal pustular dermatosis (SPD) is frequently confused with annular pustular psoriasis (APP). 1,2 We report a case of APP in a 3-year-old girl who had a previous diagnosis of SPD. Report of a Case A 3-year-old girl was referred to Children's Medical Center, Dayton, Ohio, in January 1979, with an exacerbation of a pustular eruption that began at 3 weeks of age. A skin biopsy done in January 1977 was read as "psoriasiform dermatitis." A second biopsy specimen in October 1977 showed a subcorneal pustule. A diagnosis of SPD was made and dapsone therapy,

Erythema Annulare-like Pustular Psoriasis

Psoriasis is a disease that at times can take on unusual clinical forms. This is the report of a patient with a rare variant of annular pustular psoriasis in which the lesions have the configuration of erythema annulare centrifugum and resemble subcorneal pustular dermatosis. This rare variant is characterized by rapid evolution, mild toxicity, and rapid response to therapy. Classical lesions of psoriasis can be lacking, and the diagnosis may rest on the clinical course and histologic findings.