Localized Pustular Psoriasis Research Articles

Acrodermatitis Continua of Hallopeau: A Diagnostic Challenge.

Acrodermatitis Continua of Hallopeau: A Diagnostic Challenge.

Generalized pustular psoriasis with nail psoriasis in children: a case report

Background: Pustular psoriasis in children is one of the clinical variants of psoriasis. It is classified into generalized pustular psoriasis (GPP) and localized pustular psoriasis. The aetiology of psoriasis in children has not been known but is believed to be multifactorial. Nail psoriasis rarely occurs in children who suffer from skin psoriasis, with an incidence lower than that reported in adults. The diagnosis is generally made from the clinical and histological examination. The choice of therapy depends on the severity of the disease. Until now, there have been no specific guidelines for the management of psoriasis in children.
 Case report: A-15-years-old Balinese girl presenting with erythema, confluent scaly plaques over the trunk and extremities with pustules localized on the lower extremity. She had a history of fever before the lesions appear. Right third digital nails examination showed subungual hyperkeratosis and onycholysis. Positive auspitz sign and karsvlek phenomena were found. Biopsy result suitable for psoriasis. The patient got improvement after treated with methotrexate tablet orally and desoximetasone cream topically within four weeks without any side effect.
 Conclusion: Combination therapy with methotrexate tablet and desoximetasone cream give an effective result. However, the safety and side effects of methotrexate in children still need further monitoring.

Localized pustular psoriasis in a child treated successfully with topical dapsone gel

<p class="abstract">Psoriasis is a chronic inflammatory, immune mediated dermatosis in children and adults. About one third of cases affected with psoriasis have their onset in first and second decade of life. Of paediatric population, about 0.5-2% is affected, infants are rarely affected. Overall plaque psoriasis is most common type followed by guttate and pustular psoriasis. Treating severe forms of psoriasis such as pustular psoriasis and erythrodermic psoriasis can pose difficulties, especially in paediatric population. Hence paediatric psoriasis needs to be managed effectively, however effective therapy also poses the risk of producing adverse effects, more so in paediatric age group. We report a case of localized pustular psoriasis, with an antineutrophil agent which is much safer and may target directly the pathophysiology of pustular psoriasis.</p>

Similarity and difference between palmoplantar pustulosis and pustular psoriasis.

Palmoplantar pustulosis is a chronic inflammatory disorder characterized by sterile pustules predominantly involving the palms and soles. Palmoplantar pustulosis has many similar aspects to psoriasis, either plaque and pustular type, namely familial occurrence between palmoplantar pustulosis and psoriasis, the appearance of the Köbner phenomenon, joint involvement, and nail involvement. Pustular psoriasis is classified into generalized and localized types, and there are a number of papers regarding palmoplantar pustulosis as an acral variant of localized pustular psoriasis. Many Japanese dermatologists consider palmoplantar pustulosis to be a distinct entity from pustular psoriasis, and the coexistence of palmoplantar pustulosis and psoriasis is rare. However, outside Japan, palmoplantar pustulosis is often considered to be palmoplantar psoriasis or palmoplantar pustular psoriasis, and extra-palmoplantar lesions are also considered to be psoriasis. The purpose of the current review is to compare the similarities and differences between palmoplantar pustulosis and generalized/localized pustular psoriasis. Japanese patients with palmoplantar pustulosis have a close relationship with focal infection, and the associated bone-joint manifestation exclusively involves the anterior chest wall. Furthermore, pediatric occurrence of palmoplantar pustulosis is extremely rare, and difference of genetic background between palmoplantar pustulosis and psoriasis has also been reported. Treatment of focal infection often results in dramatic effects on both cutaneous lesions and joint pain of palmoplantar pustulosis. Those findings suggest that palmoplantar pustulosis should be separately considered from either palmoplantar psoriasis or palmoplantar pustular psoriasis. The clinicopathological features and therapeutic approach of both diseases are discussed.

Lack of association between mutation in IL36RN and palmoplantar pustular psoriasis in Chinese patients

BackgroundPalmoplantar pustulosis is considered to be a localized pustular psoriasis confined to the palms and soles. Mutation of the IL36RN gene, encoding interleukin-36 receptor antagonist (IL-36Ra), is associated with generalized pustular psoriasis, but IL36RN mutations in Chinese palmoplantar pustulosis patients have not previously been investigated. ObjectiveThe aim of this study was to evaluate the mutation of IL36RN in Chinese patients with palmoplantar pustulosis. MethodsFifty-one Han Chinese patients with palmoplantar pustulosis were recruited. All exons and exon-intron boundary sequences of IL36RN were amplified in polymerase chain reactions, and Sanger sequencing of the amplicons was performed. ResultsAmong the 51 palmoplantar pustulosis patients, four different single-base substitutions were identified in nine patients. The mutations were c.140A>G/p.Asn47Ser in five patients, c.258G>A/p.Met86IIe in two patients, and c.115+6T>C and c.169G>A/p.Val57IIe in one patient each. All mutations were heterozygous. Comparison with the human genome database and reported literature suggested that these variants may not be pathogenic mutations causing palmoplantar pustulosis. Furthermore, there was no difference in disease severity, onset age, or disease duration between patients with these heterozygous IL36RN variants and those without (p>0.1). Study limitationLack of the further evaluation of IL36Ra protein in palmoplantar pustulosis lesions. ConclusionsThe four variants of IL36RN identified did not appear to be associated with the specific phenotypes of palmoplantar pustulosis.

Interleukin (IL)-8 and IL-36γ but not IL-36Ra are related to acrosyringia in pustule formation associated with palmoplantar pustulosis.

Palmoplantar pustulosis (PPP) is a refractory, nonbacterial impetigo confined to the palms and soles. Its pathogenesis is still obscure, but it may be associated with the large eccrine sweat glands and pores of palmoplantar skin. PPP is considered to be a localized pustular psoriasis. Interleukin (IL)-8, IL-36γ and IL-36Ra play important roles in the pathogenesis of pustular psoriasis, but their role in PPP is unclear. To evaluate IL-8, IL-36γ and IL-36Ra expression in PPP, and their relationship with acrosyringia and pustule formation. mRNA expression was quantified in skin samples from patients with PPP (n = 7), patients with psoriasis vulgaris (PSV; n = 8) and healthy controls (HCs) (n = 6) by reverse-transcription-real-time PCR. Protein expression was characterized by immunohistochemistry (PPP, n = 17; PSV, n = 14; HCs, n = 12). Sweat ducts, including acrosyringia, were stained for epithelial membrane antigen (EMA). IL-8 mRNA and protein were markedly increased in PPP lesions compared with PSV lesions or HC skin. IL-36γ mRNA and protein were significantly more abundant in PPP lesions than in HC skin. IL-36Ra mRNA was significantly overexpressed in PPP lesions compared with HC skin, but there was no difference in IL-36Ra protein between PPP, PSV and HCs. IL-8 was abundantly expressed by neutrophils in PPP pustules, while IL36Ra was localized in the keratinocytes of PPP, PSV and HC skin. IL-36γ and EMA were colocalized in cells surrounding PPP pustules, and IL-36γ was also expressed in sweat duct cells in the dermis. IL-8, IL-36γ and IL-36Ra are overexpressed in PPP lesions. IL-8, IL-36γ and acrosyringia, rather than IL-36Ra, are associated with pustule formation in PPP.

Could Psoriatic Arthritis Be Easily Diagnosed from Current Suspicious Physical Findings in the Dermatology Clinic?

BackgroundThe prevalence and clinical characteristics of psoriatic arthritis (PsA) in patients with psoriasis are not well described in Asian populations, including Koreans.ObjectiveThe purpose of this study was to investigate the prevalence of PsA by using the classification of psoriatic arthritis (CASPAR) criteria on the basis of physical examination only, as well as its correlation with psoriasis severity and other medical conditions including nail psoriasis.MethodsA single-center, cross-sectional observational cohort study was conducted, and the included patients were evaluated for PsA according to the CASPAR criteria. The psoriasis area severity index (PASI) and the nail psoriasis severity index (NAPSI) were calculated.ResultsThe prevalence of PsA in patients with psoriasis in Korea was 13.5%. When performing logistic regression, hyperlipidemia and localized pustular psoriasis were found to be significant predictors of PsA. The PASI score was significantly higher in PsA patients than in those with psoriasis alone (p=0.014). Psoriatic nail involvement was found in 85.5% of the study population, and all PsA patients had nail psoriasis. The mean NAPSI score was higher in patients with PsA; however, the difference was not statistically significant.ConclusionThere was a close relation between psoriasis severity and PsA, although nail psoriasis severity was not related to PsA status. Dermatologists can diagnose PsA from current physical findings by using the CASPAR criteria. To validate the CASPAR criteria for PsA diagnosis, the definition of nail psoriasis clinical types and severity in the CASPAR criteria should be reviewed again.

Identification of nail features associated with psoriasis severity.

There are no detailed studies of the prevalence of nail psoriasis and clinical characteristics of psoriatic nail involvement, including nail features associated with disease severity. Therefore, we designed a study to investigate the prevalence and characteristics of psoriatic nail involvement in patients with psoriasis and determine the relationship between psoriatic nail features and severity of nail psoriasis and cutaneous psoriasis. The Nail Psoriasis Severity Index (NAPSI) was used for evaluation of the severity of nail lesions. The presence of nail fold psoriasis (NFP) was also assessed. The severity of psoriasis was evaluated by calculating the Psoriasis Area and Severity Index (PASI). As a result, the prevalence of nail psoriasis was 85.5%. Pitting was the most common clinical feature (55.6%). The severity of nail psoriasis was not affected by medical parameters, although patients with localized pustular psoriasis tended to have more severe nail psoriasis than did those with chronic plaque psoriasis. When comparing the mean NAPSI and the mean PASI according to nail lesions, we found that subungual hyperkeratosis (SH) and NFP were significantly associated with the severity of both nail psoriasis and cutaneous psoriasis. Psoriatic nail changes were most common in the first digit. Conclusively, the majority of patients with psoriasis had psoriatic nail involvement, and Koebner's response seems to be closely related to the induction of nail psoriasis. To limit progression of the disease, psoriatic patients with SH or NFP should be examined thoroughly because those clinical features reflect the levels of severity of both nail and cutaneous psoriasis.

Caveolin-1 expression in different types of psoriatic lesions: analysis of 66 cases.

Background:Caveolin-1 is a key structural and functional protein. Caveolin-1 is known to modulate multiple signal-transducing pathways involved in cell differentiation and proliferation. Psoriasis is viewed as a multifactorial pathology characterized by keratinocyte hyperproliferation and abnormal cell maturation.Objectives:To examine the expression of caveolin-1 in skin biopsies from normal subjects, patients, and subjects with the three respective isoforms of psoriasis (psoriasis vulgaris, localized pustular psoriasis, and erythrodermic psoriasis). The expression level of caveolin-1 was compared among psoriasis vulgaris, localized pustular psoriasis, erythrodermic psoriasis, and normal subjects.Materials and Methods:Using immunohistochemical methods, caveolin-1 protein expression was assayed in four groups. An analysis was conducted on skin samples obtained from 22 normal subjects and 28 patients with psoriasis vulgaris, 22 patients with localized pustular psoriasis, and 16 patients with erythrodermic psoriasis. The statistical analysis of the scoring criteria reflecting the level of Caveolin-1 immunostaining between different groups was determined using the Mann–Whitney U-test.Results:In the normal skin, intense and consistent caveolin-1 staining was present in 22 cases. The Caveolin-1 protein was significantly reduced and showed very weak or absent staining within the tissues of psoriasis vulgaris, localized pustular psoriasis, and erythrodermic psoriasis (respective P < 0.001). Caveolin-1 protein expression in psoriasis vulgaris was higher than that in localized pustular psoriasis and erythrodermic psoriasis (respective P < 0.05). Caveolin-1 protein expression was no different in localized pustular psoriasis and erythrodermic psoriasis (P > 0.05).Conclusion:The finding of this study was consistent with a downregulation of Caveolin-1, which might serve as an etiological factor in the development of psoriasis vulgaris, localized pustular psoriasis, and erythrodermic psoriasis. Further mechanistic investigations are required to prove that Caveolin-1 protein has the potential and may be a novel target for therapy of psoriasis vulgaris, localized pustular psoriasis, and erythrodermic psoriasis.

Analysis of psoriasis patients registered with the Japanese Society for Psoriasis Research from 2002-2008

A survey of psoriasis patients from 1982-2001 has been reported by the Japanese Society for Psoriasis Research. The aim of this study is to analyze psoriasis patients in Japan registered from 2002-2008. A total of 11 631 cases were registered from 152 dermatological institutions in Japan. Males (7738 cases, 66.5%) were predominant over females (3893 cases, 33.5%). The clinical types of psoriasis were psoriasis vulgaris (88.5%), guttate psoriasis (3.9%), psoriasis arthropathica (3.3%), generalized pustular psoriasis (1.3%), psoriatic erythroderma (1.2%), localized pustular psoriasis (0.9%) and infantile psoriasis (0.1%). Topical corticosteroids (85.4%) and vitamin D(3) (59.7%) products were the main previous topical agents. Previous systemic treatments included etretinate (8.8%), cyclosporin (8.3%) and methotrexate (2.0%). Use of topical vitamin D(3) and systemic cyclosporin therapies has been increasing during the past 7 years. Topical psoralen and ultraviolet A therapy (PUVA) (7.6%) was the predominant phototherapy followed by UV-B (7.3%) and systemic PUVA (4.7%). Use of UV-B phototherapy has been increasing during the past 5 years. The survey of Japanese psoriasis patients during 2002-2008 disclosed that psoriasis arthropathica is more prevalent (1%) than that of the previous survey during 1982-2001. Use of topical vitamin D(3) and systemic cyclosporin has been increasing during the past 7 years.

Analysis of psoriatic patients registered in Asahikawa Medical College Hospital from 1983 to 2007

Psoriasis is a chronic inflammatory skin disease, which has been increasing during the last 50 years in Japan. The aim of the present study is to analyze psoriatic patients registered from 1983-2007 in Asahikawa Medical College Hospital, which is located in the northern part of Japan. A total of 607 cases were registered at the first inspection in the Department of Dermatology, Asahikawa Medical College. Men (403 cases, 66.4%) were predominant over women (204 cases, 33.6%). The clinical types of psoriasis were psoriasis vulgaris (91.5%), guttate psoriasis (4.2%), psoriasis arthropathica (2.8%), psoriatic erythroderma (0.6%), generalized pustular psoriasis (0.6%), localized pustular psoriasis (0.15%) and infantile psoriasis (0.15%). Topical corticosteroids (78.1%) and vitamin D3 (18.1%) products were the main previous topical agents. Previous systemic treatments included etretinate (7.7%), cyclosporine (1.5%) and methotrexate (0.3%). Use of topical vitamin D3 and cyclosporine therapies have been gradually increasing during the past 25 years. Regarding the previous phototherapy, topical psoralen and ultraviolet A therapy (PUVA) (4.9%) was predominant over ultraviolet B (0.9%), and systemic PUVA (0.7%). Use of ultraviolet B phototherapy has been increasing during the past 5 years. The results are essentially similar to those of a survey of psoriasis in Japan from 1982-2001. Although the incidence of psoriasis might be higher in Hokkaido Prefecture, there is essentially no variation in the disease profile of psoriatic patients.

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

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

Flare-up of pustular psoriasis with fluoxetine: Possibility of a serotoninergic influence?

In this article, we report two cases of pustular psoriasis flaring up after fluoxetine administration. A 21-year-old male patient with localized pustular psoriasis became erythrodermic following commencement of fluoxetine. Even though the lesions were unresponsive to cyclosporine A (Cyc A) treatment, dramatic resolution was observed with discontinuation of fluoxetine. A 44-year-old female patient with pustular psoriasis who was on Cyc A and acitretin therapy was given fluoxetine for her psychiatric symptoms. In the following 5 days, her lesions flared. Owing to previous experience, fluoxetin was stopped. Her lesions improved dramatically in the following 3 days. Exacerbation of psoriasis with antidepressant therapy has been rarely described. An extensive review of the literature revealed four such cases, all of which were seen after the use of selective serotonin reuptake inhibitors (SSRI). A serotoninergic influence in the etiopathogenesis of psoriasis may be possible together with a pharmacogenetic difference in the drug metabolism of these patients. Considering the two patients we presented and the patients previously reported in the literature, aggravation of pustular psoriasis by SSRI should be borne in mind.

Undiagnosed acrodermatitis continua leading to amputations of the distal phalanxes

AbstractAcrodermatitis continua is a localized pustular psoriasis which usually affects the extremities. We report the case of a 79‐year‐old patient presenting with osteolysis of the distal phalanxes of the hands. Some pustules were noted on the finger pulps. A skin biopsy led to the diagnosis of acrodermatitis continua.

A survey of psoriasis patients in Japan from 1982 to 2001

Background: The Japanese Society for Psoriasis Research has conducted an annual survey of psoriasis patients in Japan from 1982 to 2001. Objective: To perform the epidemiological study about a survey of psoriasis patients conducted in Japan for twenty years. Methods: A sample of 28628 cases was collected from 148 dermatology centers throughout Japan. The reports from each center were analyzed. Results: Males (65.8%) were predominant over females (34.2%) in number. The vast majority of cases (86.0%) had plaque-form of psoriasis vulgaris, and 812 cases (2.8%) showed guttate psoriasis. Psoriatic erythroderma (0.8%), generalized pustular psoriasis (0.9%), and localized pustular psoriasis (0.5%) were rare. Three hundred of the patients (1.0%) manifested psoriatic arthritis. Local corticosteroids (67.8%) were the most used modalities, whereas local vitamin D 3 preparations (2.4%) were rarely used. For phototherapeutical treatments, topical (12.1%) and systemic (7.5%) PUVA were predominant over UVB therapy (0.5%). In systemic treatments, drugs from the herbal medicine was the first (14.2%), followed by etretinate (7.6%), nonsteroidal anti-inflammatory drugs (4.4%), oral corticosteroids (4.1%), methotrexate (2.8%), cyclosporine (1.6%), and anti-cancer drugs (1.4%). Conclusion: This survey was the first epidemiological study throughout Japan.

Levels of proelafin peptides in the sera of the patients with generalized pustular psoriasis and pustulosis palmoplantaris

The levels of proelafin peptides in the sera of patients with pustulosis palmoplantaris, a unique type of localized pustular psoriasis, and generalized pustular psoriasis were determined by competitive enzyme-linked immunosorbent assays using antibodies against synthetic proelafin polypeptides corresponding to the elastase inhibitor (elafin) and transglutaminase substrate domains. The sera of patients with pustulosis palmoplantaris (9 cases) exhibited a normal range of proelafin peptide levels. The sera of patients with generalized pustular psoriasis (3 cases) showed high titres of proelafin peptide. There were no large differences in the titres between the 2 antibodies. The antibodies for 2 different domains of proelafin showed a similar immunoreactivity for the non-pustular region of the epidermis in all pustulosis palmoplantaris tested. The results indicate that serum proelafin peptides in pustular psoriasis may depend on the extent of the involved area, and that proelafin peptide level in pustulosis palmoplantaris remains normal despite enhanced local expression in the lesional skin. Since the skin lesions of patients with pustulosis palmoplantaris are limited to the palms and soles, enhanced expression of proelafin in the lesional skin may not lead to elevation of proelafin peptides in the serum.

The profile and outcome of pustular psoriasis in Singapore: a report of 28 cases.

Pustular psoriasis is a rare form of psoriasis that can be divided into generalized and localized forms. The aim of this study is to describe the patient profile and outcome of pustular psoriasis seen at a tertiary referral skin center in a tropical country. The records of all patients with pustular psoriasis during the 4 years from 1989 to 1993 were reviewed. Diagnostic criteria for selection included at least one episode of either generalized or localized macroscopic noninfective pustulation. There were 28 patients with pustular psoriasis, with an age range of 4-77 years. Nineteen patients had generalized pustular psoriasis: Von Zumbusch (seven), annular form (two), juvenile form (six), pustular psoriasis of pregnancy (one), and the localized form of generalized pustular psoriasis (three). Nine patients had localized pustular psoriasis: palmoplantar pustulosis (five) and acrodermatitis continua (four). Patients with the acute Von Zumbusch pattern had recalcitrant disease with multiple flares and significant morbidity and mortality. Patients with the annular form had a subacute onset and a chronic course. In patients with the juvenile form of generalized pustular psoriasis, two patterns could be recognized: Zumbusch form (four) and annular form (two). Despite significant morbidity, each of our young patients had a relatively benign course with no deaths and an excellent response to etretinate therapy. Our nine patients with localized pustular psoriasis all had a chronic course: the average duration of disease was 6 years for patients with palmoplantar pustulosis and 12 years for patients with acrodermatitis continua. The pattern of pustular psoriasis seen in Singapore is similar to that reported in the Western literature. Using these categories we can provide guidelines for treatment and prognosis.

Up-Regulation of Elafin/SKALP Gene Expression in Psoriatic Epidermis

The expression of mRNA for elafin/SKALP, an inhibitor of leukocyte elastase and proteinase 3, in human normal and psoriatic epidermis was examined by in situ hybridization. In normal epidermis, elafin/SKALP mRNA was detected in the granular layer, but not in the spinous or basal layers. In fully developed psoriatic lesions, elafin/SKALP mRNA was found in the suprabasal layers of the ridges, and in the upper two thirds of the stratum malpighii at the elongated rete ridges. Intense staining was noted near the subcorneal microabscess in psoriasis vulgaris and under the subcorneal pustule in localized pustular psoriasis. In the marginal psoriatic epidermis, elafin/SKALP mRNA was expressed from the middle or upper spinous layer to the subcorneal layer, and the cells expressing elafin/SKALP mRNA increased especially under the parakeratotic corneal layer intermingled with pyknotic nuclei of neutrophils. These findings suggest that the induction of elafin/SKALP gene expression is related closely to the infiltration of neutrophils into the epidermis in psoriasis and plays an important role in protecting the skin components against the tissue damage caused by the infiltrated leukocytes.

Pityriasis rubra pilaris: a new proposed classification.

Clinical features of pityriasis rubra pilaris (PRP) were classified into four types based on a study of 168 cases. All patients, except one case of type IV, and 12 cases of type II, have salmon-coloured or erythematous thick plaques on the palms and soles, which extended beyond the dorsopalmar and plantar junctions. Twenty-one children and 11 adults presenting with such plaques were classified as type I PRP. Fifty-nine children and 27 adults presenting with circumscribed scaly erythematous patches on the elbows and knees were classified as type II. Twenty children and 16 adults with similar patches involving large areas of the trunk which were not generalized were classified as type III. Four children and 10 adults with exfoliative erythroderma associated with diffuse follicular plugging were classified as type IV. The diagnosis was confirmed by histopathology which showed follicular plugging and focal parakeratosis. However, this was not seen in type I. This new clinical classification facilitates recognition of this type of PRP despite the absence of follicular plugging. Two children, initially classified as type I, showed gradual progression to type II on follow-up, with two adults progressing to type III, and 1 adult to type IV. Similarly, two adults classified as type II, progressed to type III, and 1 adult into type IV. Associated findings were an adult of type II who developed localized pustular psoriasis and another adult who developed ovarian adenocarcinoma.

Treatment of Palmoplantar Psoriasis With Topical Methoxsalen Plus Long-Wave Ultraviolet Light

Fourteen patients with chronic plaque-type psoriasis involving the palms and soles and 14 patients with palmoplantar vesiculopustular dermatosis, including three cases of localized pustular psoriasis, were treated with topical application of methoxsalen, followed by exposure to long-wave ultraviolet energy (topical PUVA). Approximately two thirds of the patients with palmoplantar plaque-type psoriasis and half of those with vesiculopustular dermatosis responded with considerable improvement, as evidenced by flattening of plaques, decreased scaling and erythema, and decreased vesicle and pustule formation after 15 to 40 treatments. Complete clearing of the treated areas was achieved in five patients with palmoplantar psoriasis, three of whom had the pustular variety. Most patients required continued maintenance therapy with topical PUVA. The condition of the patients with palmoplantar vesiculopustular dermatosis, especially those with severe involvement, was more labile and more difficult to control with PUVA therapy than in those with chronic scaling plaques.