Solar urticaria: clinical characteristics, treatment effectiveness, long-term prognosis, and QOL status in 29 patients.

Introduction: Solar urticaria (SU), a relatively rare skin inflammatory and photosensitivity disease, is often resistant to standard urticaria treatment. Quality of life (QOL) among SU patients has not been extensively explored. This study was performed to clarify the clinical features and effectiveness of therapies (e.g., hardening therapy) for SU and to determine QOL among SU patients. Methods: The authors examined the characteristics, treatments, and QOL statuses of 29 Japanese SU patients using medical records and a questionnaire approach. Results: Among 29 patients, H1 antihistamine therapy (H1) was effective in 22 (75.8%) patients. H2 antihistamine therapy (H2) was effective in three of seven (42.9%) patients. Ultraviolet radiation A (UVA) hardening therapy was effective in eight of nine (88.9%) patients. Visible light (VL) hardening therapy was ineffective in three of three patients. In one patient who underwent both UVA and VL hardening therapy, only UVA hardening therapy was effective. In the questionnaire, 18 patients (90%) reported some improvement compared with disease onset (four had complete remission, six had completed treatment although mild symptoms persisted, and eight were receiving treatment with moderate symptoms), whereas two patients reported exacerbation. Patients in complete remission had a mean disease duration of 4 years, whereas patients not in remission had a mean disease duration of 8.8 years. The mean Dermatology Life Quality Index (DLQI) score for the current status was 7.4. There was a correlation between DLQI and symptom/treatment status. However, neither DLQI and action spectra nor DLQI and treatments exhibited significant differences. Discussion: The questionnaire revealed current QOL status and long-term prognosis in SU patients. Compared with disease onset, most patients showed improvement when assessed for this study. Both H1 and H2 should be attempted for all SU patients. UVA hardening therapy may be an option for SU patients with an action spectrum that includes UVA.

Cholinergic Urticaria: Subtype Classification and Clinical Approach.

Cholinergic urticaria (CholU) is a subtype of chronic inducible urticaria with a chief complaint of itching and/or stinging, painful papular wheals that develop simultaneously with sweating. This review specifically focuses on several subtypes of CholU and specifically investigates the relationship between CholU and anhidrosis. We review recent publications and update the evidence around CholU, including the epidemiology, clinical features, diagnostic approaches, physiopathology, subtype classification, and therapeutic approaches. Multiple mechanisms contribute in a complex manner to the development of CholU, including histamine, sweat allergy, cholinergic-related substances, poral occlusion, and hypohidrosis/anhidrosis. A new schematic of the currently known pathological conditions has been created. Specific methods for diagnosing CholU, a provocation test, and evaluation methods for disease severity/activity and disease burden of CholU are summarized. The characteristics of the diseases that should be differentiated from CholU and examination methods are also summarized. The primary finding of this review is that CholU should be categorized based on the etiology and clinical characteristics of each subtype to properly manage and treat the disease. This categorization leads to improvement of therapeutic resistance status of this disease. In particular, a sweating abnormality should be given more attention when examining patients with CholU. Because CholU is not a homogeneous disease, its subtype classification is important for selection of the most suitable therapeutic method. Further elucidation of the pathophysiology of each subtype is expected.

IgE receptor responsiveness of basophils in chronic inducible urticaria.

Introduction: Chronic inducible urticaria (CIndU) is a subgroup of chronic urticaria induced by a specific stimulus. We evaluated basophil characteristics in patients with CIndU and compared with those in patients with chronic spontaneous urticaria (CSU) and healthy controls (HCs). Methods: Blood was collected from patients, and a basophil activation test (BAT) was performed. Basophil responsiveness and surface marker expression in patients with CIndU were compared with those in patients with CSU and HCs. For some patients with CIndU, blood was collected before and after wheals were induced. In these cases, we compared the responsiveness of basophils before and after the appearance of wheals. Result: HCs (n=23) and patients with CIndU (n=24) or CSU (n=38) were enrolled in the study. The degree of basophil activation at steady state in patients with CIndU was higher than in HCs. Basophil responsiveness via high-affinity IgE receptor (FcϵRI) stimulation with anti-IgE or anti-FcϵRI antibody in patients with CIndU was equivalent to that in HCs, and higher than that in patients with CSU. No abnormalities in IgE and FcϵRI expressions on the surface of basophils in patients with CIndU were observed. When we induced wheals in some patients with CIndU and performed a BAT before and after the appearance of wheals, no significant changes were found. Conclusion: Peripheral blood basophils in CIndU were slightly activated at steady state, but no abnormalities in basophil responsiveness. In future, a higher number of cases should be enrolled to confirm the role of basophils and refine therapeutic targets for CIndU.

Activated steady status and distinctive FcεRI-mediated responsiveness in basophils of atopic dermatitis.

BACKGROUND: Although basophils are considered to play an important role for maintenance of type 2 inflammation in atopic dermatitis (AD), studies on basophils in AD patients are limited. Some studies have reported the activation status, including CD203c and CD63, of peripheral blood basophils in AD patients. METHODS: We examined the features of circulating basophils in AD patients, assessed cell surface marker expressions and total serum IgE, and compared basophil responsiveness to stimulation between AD patients and healthy controls (HCs). In addition, the correlations among AD severity, laboratory factors, and features of basophils were examined. Blood samples from 38 AD patients and 21 HCs were analyzed. Basophil response markers CD203c and CD63, and expression of surface-bound IgE and FcεRI on basophils were measured. CD203c and CD63 expressions induced by stimulation with anti-IgE and anti-FcεRI antibodies were measured. Clinical/laboratory factors including total serum IgE were examined for correlations with these basophil parameters. RESULTS: Baseline CD203c and CD63 expression on basophils were significantly higher in AD patients compared with HCs. The CD203c/CD63 response ratio to anti-FcεRI stimulation was higher than that to anti-IgE stimulation in AD patients, but not HCs. FcεRI expression on basophils was higher in AD patients than in HCs, although surface-bound IgE on basophils was equivalent. Total serum IgE had negative correlations with surface-bound IgE and CD63 responsiveness to anti-IgE stimulation. CONCLUSIONS: Basophils were spontaneously activated under steady-state conditions in AD patients and responsiveness to anti-IgE stimulation was lower than in HCs. Despite high serum IgE and high basophil FcεRI expression, surface-bound IgE on basophils remained relatively low. Basophils might be suppressed or exhausted regarding FcεRI signaling via IgE in severe AD.

Improved FcεRI-Mediated CD203c Basophil Responsiveness Reflects Rapid Responses to Omalizumab in Chronic Spontaneous Urticaria.

BACKGROUND: Omalizumab is effective in patients with chronic spontaneous urticaria (CSU) although its mechanism of action is poorly understood. Several studies reported that decreased high-affinity IgE receptor (FcεRI)-mediated histamine release and/or responsiveness was characteristic of basophils in patients with CSU. However, few studies have focused on the relationship between changes in basophil responsiveness via FcεRI after omalizumab treatment and the therapeutic effect in patients with CSU. OBJECTIVE: To assess basophil responsiveness via FcεRI stimulation, as well as FcεRI expression and IgE binding on blood basophils from patients with CSU before and after omalizumab treatment and its possible association with the clinical response. METHODS: We analyzed 34 patients with CSU treated with omalizumab who were categorized as fast responders (FRs) (n = 20) and non or slow responders (N/SRs) (n = 14). CD203c expression induced by FcεRI stimulation, and IgE and FcεRI expressions on blood basophils from patients with CSU before and after omalizumab treatment were analyzed. Basophil responsiveness via FcεRI stimulation was observed in vitro using basophils pretreated with omalizumab. RESULTS: FRs had increased CD203c responsiveness after treatment with omalizumab compared with N/SRs. This improvement of basophil responsiveness via FcεRI stimulation in FRs was not observed in peripheral blood basophils preincubated with omalizumab in vitro, suggesting that omalizumab does not directly affect circulating pre-existing abnormal basophils. CONCLUSION: Increased basophil responsiveness via FcεRI after omalizumab treatment is associated with the therapeutic effect and mechanism of action of omalizumab.

[Kinematic aspects of the step movement in Parkinson's disease].

In an attempt to understand the fundamental mechanism for movement disturbances in Parkinson's disease (PD) patients, we investigated their straightforward or right diagonally (laterally) steps and step over a stick. In all tasks, in PD patients, the movement latency was significantly longer and the movement duration was significantly shorter than those of the controls. In the lateral step task, the maximum velocity of PD subjects was significantly faster than that of the controls, while the reaching time to maximum velocity was longer. Moreover, in the task to step over a stick, the reaching time to maximum velocity was significantly longer than that of the controls. In the velocity profiles, in PD patients, the deceleration after the maximum acceleration was rapider than that of the controls. In addition, the PD patients' jerk was significantly larger than that of the controls. These results suggest that PD patients have deficits in motor programming and motor control which may be due to dysfunction of the cortico-basal ganglia loop.