Clinical efficacy and safety of secukinumab in the treatment of 13 cases of refractory localized pustular psoriasis / 中华皮肤科杂志

Objective:To evaluate clinical efficacy and safety of secukinumab in the treatment of patients with localized pustular psoriasis irresponsive or intolerant to conventional treatment.Methods:Clinical data were collected from 13 patients with refractory localized pustular psoriasis, who received secukinumab treatment in Department of Dermatology, Xijing Hospital from December 2019 to April 2022. Efficacy was evaluated by comparing palmoplantar pustulosis (PPP) area and severity index (PPPASI) score and physician global assessment (PGA) score in PPP patients before and after the treatment, and by comparing clinical global impression (CGI) score in patients with acrodermatitis continua of Hallopeau (ACH). Adverse events were recorded during the treatment.Results:Among the 13 patients with refractory localized pustular psoriasis, 6 were diagnosed with PPP, 3 with ACH, and 4 with PPP complicated by ACH. There were 3 males and 10 females, and their age was 33.2 ± 14.6 years. After 12-week treatment, the PPPASI score decreased from 13.88 ± 3.62 points at baseline to 6.81 ± 2.31 points in 10 patients presenting with PPP lesions, 4 achieved 75% improvement in the PPPASI score (PPPASI75), and 5 achieved PGA0/1; at the same time, 6 of the 7 patients presenting with ACH lesions achieved moderate or marked improvement in the CGI score, and 4 of the 6 patients achieved marked improvement. Two patients with PPP discontinued the treatment after 3- and 5-week treatment respectively due to poor response, and 1 patient with ACH achieved mild improvement in the CGI score after 12-week treatment. No severe adverse events were reported during the treatment. However, inflammatory follicular papules occurred in 1 patient, and eczematoid lesions occurred in another 1 patient, which both regressed after symptomatic treatment.Conclusion:Secukinumab was effective and safe in the treatment of refractory localized pustular psoriasis, and may serve as a new treatment option for refractory PPP and ACH.

Interleukin-17 and interleukin-23 inhibitors in the treatment of pustular psoriasis / 中华皮肤科杂志

Pustular psoriasis is a serious life-threatening disease, and patients usually show poor response to traditional treatments. In recent years, interleukin-17 and interleukin-23 inhibitors have shown favorable efficacy in the treatment of psoriasis. This review summarizes the latest progress in interleukin-17 and interleukin-23 inhibitors for the treatment of pustular psoriasis.

A Case of Palmoplantar Pustulosis Successfully Treated with Kampo Medicine Switched Between Fundamental Treatment and Local and Symptomatic Treatment / 日本東洋医学雑誌

We experienced a case of palmoplantar pustulosis in which the patient's dermatological symptoms im­proved after treatment with a combination of bofutsushosan and keishibukuryogan. The patient was a 42-year-old woman. She had been prescribed biotin and a steroid ointment, but her condition did not improve. At her first visit to our Kampo clinic, we observed impetigo, cracks, and scaling on both palms and plantar surfaces. We determined that the patient was of the poisoned organ (zodoku) and stagnant blood (oketsu) constitutions,as outlined in the Ikkando school of medical thought. We prescribed bofutsushosan and keishibukuryogan as fundamental treatment and then in a few days, the patient's dermatological symptoms resolved. The patient later developed hyperthyroidism, which caused her symptoms to worsen again, we therefore switched to shofusan and eppikajutsuto as local and symptomatic treatment, and then she once again improved. Soon after, we returned to keishibukuryogan and bofutsusho for her to maintain remission. Since poisoned organ and stagnant blood constitutions contribute greatly to the Kampo pathology of palmoplantar pustulosis, we believe bofutsushosan and keishibukuryogan are effective formulations for the treatment of this condition. As far as we searched, there were no papers that selected bofutsushosan or blood stasis agents as the main cure from the viewpoint of the poisoned organ constitution and the stagnant blood constitution. Therefore, this case was considered to be a valuable case.

A unique leukoencephalopathy accompanied by palmoplantar pustulosis with identical pathological feature of helper T cell accumulation

@#Palmoplantar pustulosis is a chronic inflammatory skin disease involving the palms and soles where mild accumulation of helper T cells and neutrophils in the dermis histologically are observed. Leukoencephalopathy is a brain disease affecting white matter but is rarely accompanied by skin lesion. Here we report a unique case of leukoencephalopathy accompanied by palmoplantar pustulosis with identical pathological feature of helper T cell accumulation in both the brain and skin, suggesting a possible link in the pathogenesis.

A Case of SAPHO Syndrome Associated with Lytic Bone Lesions Resembling Metastases / 대한피부과학회지

SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome includes a variety of inflammatory bone disorders associated with dermatologic pathology. A 57-year-old female presented with pustulosis on both hands that had persisted for several months. She also had lower back pain without trauma history. On physical examination, tenderness on her lower back and left anterior chest wall pain were found, and claudication was observed. Radiological studies including computed tomography (CT), magnetic resonance imaging (MRI), and positron emission tomography (PET)-CT showed endplate lytic changes in her spine, a focal hypermetabolic lesion in a left rib, and costochondral junction. These findings raised doubt on the presence of metastatic bone lesions, and there was no indication for primary cancer after a complete medical checkup. Palmoplantar pustulosis was well controlled by treatment with acitretin. The osteitis associated with SAPHO syndrome usually presents as osteosclerosis, while reports on osteolytic lesions are rare. We report herein a rare case of SAPHO syndrome associated with bone lesions resembling bone metastasis.

Helpful Clinical Features for Differential Diagnosis of Palmoplantar Pustulosis and Pompholyx / 대한피부과학회지

BACKGROUND: Palmoplantar pustulosis (PPP) and pompholyx are both chronic vesiculopustular conditions of the palms and soles. Because both share similar clinical and histological features, it is difficult even for dermatologists to differentiate between these two diseases. OBJECTIVE: To analyze clinical features that can be helpful in differentiating the two diseases. METHODS: The clinical history of 133 patients with vesicles or pustules on the palms and/or soles was evaluated. Patients were divided into a PPP group and a pompholyx group. RESULTS: There was no sex or age predilection in either group and no significant difference in overall localization and symmetry of lesions. Bilaterality was found in 75.0% of cases of PPP and 82.0% of cases of pompholyx. More lesions were found on the tip of the finger/toe, and the side of the toe in the pompholyx group than in the PPP group. However, there was no difference between two groups in lesions on the sides of fingers. More cases of PPP involved right distal interphalangeal (DIP) and bilateral proximal interphalangeal (PIP) joint areas compared with pompholyx. There were more smokers in the PPP group. Among several comorbidities, only atopic dermatitis and nummular eczema were associated with pompholyx. CONCLUSION: Several characteristics of patients and clinical findings may serve as useful “clues” to differentiate between and determine treatment for PPP and pompholyx.

Palmoplantar pustulosis: causative factors and efficacy of total glucosides of paeony / 中华皮肤科杂志

Objective To analyze causative factors for and therapeutic effect of total glucosides of paeony (TGP) on palmoplantar pustulosis (PPP).Methods Ninety patients with PPP were recruited in this study.A questionnaire survey was carried out to collect clinical data from these patients,and a patch test to identify contact allergens for these patients.All the patients were randomly divided into two groups:observation group treated with TGP capsule 0.6 g thrice a day,and control group treated with tripterygium glycosides tablets 20 mg thrice a day.Both groups topically applied mometasone furoate cream once a day.The efficacy and side effects were evaluated 4 and 8 weeks after initiation of treatment.Results Of the 90 patients,the ratio of male to female was 1 ∶ 1.64,smokers amounted to 42.22％,and 23 (26.67％) reported a history of infection before the onset of PPP.As the patch test showed,potassium dichromate and nickel sulfate were the most common contact allergens in these patients.The response rate was significantly higher in the observation group than in the control group after 4 weeks of treatment (64.44％ vs.40.00％,x2 =5.388,P ＜ 0.05),but similar between the two groups after 8 weeks of treatment (88.89％ vs.80.00％,x2 =1.353,P ＞ 0.05).No significant difference was observed in the occurrence of adverse reactions between the two groups (17.78％ vs.11.11％,x2 =0.809,P ＞ 0.05).Conclusions Smoking may play a certain role in the development of PPP,and infection seems to be a primary inducing factor.TGP combined with mometasone furoate cream is effective for the treatment of PPP.

Association between Palmoplantar Pustulosis and Cigarette Smoking in Korean / 대한피부과학회지

BACKGROUND: Palmoplantar pustulosis (PPP), a skin disease, is characterized by chronically recurring sterile pustules on the palms and the soles. Although the etiology of PPP is unknown, it is interesting to note high prevalence of tobacco use in these patients. It would seem that there may be a relationship between PPP, autoimmune disease and alterations of thyroid function. However, this fact was never verified among Korean. OBJECTIVE: This study aims to explore smoking as a risk or exacerbation factor for PPP in Korean patients with PPP, and confirm the relationship between PPP and other factors, including thyroid disease. METHODS: We studied a total of 109 patients (M:F=38:71) with ages ranging from 20 to 70 years, diagnosed with PPP. A control group was matched according to gender and age: 109 patients with other dermatologic problem. Patients were interviewed about tobacco use, and thyroid disease, and autoimmune diseases. The data were analyzed to determine whether there was any association between PPP and various factors. RESULTS: The proportion of PPP patients using tobacco was much higher than that of the control group (p<0.05). Smoking was more strongly associated with PPP among women. Although there was no association between the quantity of cigarettes smoked daily and the severity of PPP, there was a dose-dependent correlation between the pack*year and the severity of PPP (p<0.05). Furthermore, 14 patients had been diagnosed with thyroid disease. In addition, all of them were smokers. CONCLUSION: There was significant association between PPP and smoking. However, the impact of giving it up in the clinical course of the disease remains to be established. In addition, it would seem that there may be a relationship between PPP and alteration of thyroid function.

A Case of Palmoplantar Pustulosis Followed by Joint Pain Successfully Treated with Saikokeishito / 日本東洋医学雑誌

Orthopedic symptoms are not rare in palmoplantar pustulosis, but standardized treatments have not yet been established.Here we report a case of palmoplantar pustulosis followed by joint pain that was successfully treated with the Kampo formulation saikokeishito. The patient was a 44-year-old Japanese man. He was suffering from pustules on the soles of his feet due to palmoplantar pustulosis, and joint pain of the sternoclavicular area, hip and lumbo-sacral area, which had not been controlled with diclofenac sodium suppositories. We prescribed saikokeishito for 1 month, and he felt less joint pain and experienced less pustulosis. After that, he suffered from an upper respiratory tract infection, and he again developed plantar pustulosis. We added kikyo (<I>Platycodon grandiflorum</I> root) to treat the sore throat, and his symptoms almost disappeared following this treatment. No author has reported a patient with palmoplantar pustulosis accompanied by joint pain who was treated with saikokeishito. The results experienced in this case suggest that saikokeishito could be a useful formulation for the treatment of palmoplantar pustulosis accompanied by joint pain.

SAPHO syndrome:clinical analysis of 22 cases / 中华皮肤科杂志

Objective To investigate the clinical features of SAPHO syndrome.Methods Clinical data of 22 cases of SAPHO syndrome were analysed.Results There were 7 males and 15 females among the 22 patients.The average age at onset of cutaneous and osteoarticular lesions was 45 years and 44 years, respectively.Of the 22 patients,21 had palmoplantar pustulosis and 1 had acne fulminans.Anterior chest wall (ACW) was involved in 19 patients,peripheral joints in 4 patients and sacroiliac joints in 2 patients.Osteoarticular manifestations occurred prior to the onset of skin lesions in 10 cases.after that in 9 cases,and simultaneously in 3 cases.The mean interval between the onset of cntaneous and osteoarticular lesions was 2.7 years and the longest interval was 20 years.Conclusions Middle-aged females predominate in patients with SAPHO syndrome seen in dermatological clinics.Palmoplantar pustulosis and ACW involvement are the most common clinical manifestations of SAPHO syndrome.

A Case of Palmoplantar Pustulosis Successfully Treated with Combined Formulation of Hainosankyuto and Maobushisaishinto / 日本東洋医学雑誌

We successfully treated a patient with palmoplantar pustulosis, with a combined formulation of hainosankyuto and maobushisaishinto. The patient was a 64-year-old female, who had frequently occurring pustulae of about 2 mm in size on her palms and soles, from about May 2000. These pustulae became a crust over time, and the skin peeled off and healed. However, pustulae would persist again every two weeks. We diagnosed this as palmoplantar pustulosis and administered hainosankyuto, but with limited results, whereupon we added maobushisaishinto. As a result of this combined hainosankyuto and maobushisaishinto formulation, our patient with palmoplantar pustulosis recovered significantly after 4 weeks.

A Case of SAPHO Syndrome in a Palmoplantar Pustulosis Patient / 대한피부과학회지

SAPHO syndrome is characterized by sternocostoclavicular osteoarthritis and hyperostosis in the anterior chest wall, and skin changes such as palmoplantar pustulosis and acne. Despite the higher frequency of psoriasis in this syndrome, its inclusion in the psoriatic arthropathy spectrum is not yet clearly established to date. According to a familial series of the SAPHO syndrome, both sacroiliac and sternoclavicular joints could be involved. We report a case of SAPHO Syndrome in a palmoplantar pustulosis patient who had on associated osteoarticular manifestation. A 47-year-old woman had been treated for palmoplantar pustulosis for 15 months. Pain and tenderness then developed abruptly on the chest and neck, and multiple erythematous papules and pustules broke our over the whole body. Laboratory tests were negative for serum RA factor and ANA, and positive for HLA-B27. An X-ray showed a hyperostosis, osteolytic and osteosclerotic lesions in the costo-sterno-clavicular junction. 99mTc isotope scan showed the typical "Bull's head sign" in the anterior chest wall due to increased uptake in the manubrium and both sternoclavicular joints.

A Clinical Study of the Osteo-arthropathy in Palmoplantar Pustulosis / 대한피부과학회지

BACKGROUND: Palmoplantar pustulosis(PPP) is often associated with osteo-arthropathy, especially sternocostoclavicular hyperostosis(SCCH). The relation between PPP and SCCH or other types of seronegative arthropathy remains unclear. OBJECTIVE: The purpose of this study was to investigate the distribution and the frequency of osteo-arthropathy in patients with PPP. METHODS: Twenty fiv patients with PPP were included in this study, and checked for clinical symptoms and signs, labiratory findings, and radiologic findings including plain X-ray, 99mTc-MDP bone scan, and computed tomography. RESULTS: The incidenc of osteo-arthropathy in PPP was 64%. The prevalent age at onset of osteo arthropathy was the forth decade in males, and the sixth decade in females. The frequent, sites of osteo arthralgia were sternocostoclavicular areas, spines, shoulders, knees, and sacroiliac joints, and the frequent sites of increased radioisotope uptake were similar to t,he distribution of sites of osteoarthropathy. 99mTc-MDP bone scan was more sensitive in detecting osteo-arthropathy than plain X ray and commputed tomography. In laboratory findings, we observed an increase in the erythrocyte sedimentation rate, leukocyte counts, alkaine phosphatase, and the positivity of C-reactive pr otein. CONCLUSIONS: The frequent involvement of the anterior chest, wall, knees, spines, and sacroiliac joints was noted in patients with PPP. 99mTc MDP bone scan may be the most helpful in making a diagnosis of osteo-arthropathy accompanied with PPP.