The Journal of dermatology最新文献

Amicrobial pustulosis of the folds (APF) is a rare, chronic-relapsing neutrophilic dermatosis characterized by sterile pustules affecting major and minor skin folds. It predominantly affects women and is frequently associated with autoimmune diseases such as systemic lupus erythematosus, inflammatory bowel disease, and autoimmune thyroiditis. Due to its rarity, standardized treatment guidelines are lacking, and management remains challenging. Systemic corticosteroids, dapsone, colchicine, methotrexate, and biologics have been employed with variable outcomes, but long-term control is often difficult to achieve. Apremilast, an oral phosphodiesterase-4 inhibitor with anti-inflammatory properties, has demonstrated efficacy in various neutrophilic dermatoses. We report two women with treatment-refractory APF who achieved clinical remission within 2 months of initiating apremilast, with sustained disease control at 6 months and successful corticosteroid tapering. A literature review of 78 APF cases confirmed a strong female predominance (93.6%) and frequent association with autoimmune conditions (91%). Systemic corticosteroids were the most frequently employed treatment but often failed to provide sustained disease control without the addition of other systemic agents. These cases represent the first reports of apremilast use in APF and suggest its potential as a safe and effective steroid-sparing option in patients with refractory disease. Further studies are needed to validate its role in this setting.

The stratum corneum, as the outermost layer of the skin, functions as a critical barrier that maintains cutaneous hydration and systemic homeostasis. Among its structural lipids, ceramides constitute the most abundant and diverse component. These molecules are essential for the formation of lamellar structures that secure barrier integrity. Increasing evidence has established that abnormalities in stratum corneum ceramides are not merely epiphenomena but fundamental contributors to the pathophysiology of atopic dermatitis (AD). In this review, we provide an overview of the structure, biosynthesis, and diversity of ceramides within the stratum corneum, followed by a discussion of their pivotal role in skin barrier function. We highlight recent insights into how ceramide abnormalities manifest in AD, including reduced total content, altered class distribution, and a shift toward shorter-chain fatty acids. Such alterations are associated with increased transepidermal water loss and impaired hydration. Mechanistic studies further reveal that type 2 cytokines, particularly IL-4 and IL-13, directly disrupt lipid metabolism by inhibiting enzymes, thereby establishing a vicious cycle of inflammation and barrier dysfunction. Beyond pathophysiology, advances in lipidomics and tape-stripping techniques now enable noninvasive assessment of stratum corneum ceramides. These analyses have revealed their utility as biomarkers of disease activity, therapeutic response, and relapse risk. Collectively, ceramides of the stratum corneum provide a unique window into the biology of AD. Their accessibility, mechanistic relevance, and prognostic potential underscore their importance not only for understanding disease pathogenesis but also for advancing personalized management and the concept of disease modification in AD.

Prurigo nodularis (PN) is a chronic inflammatory skin disorder associated with various systemic disorders. However, its potential link to increased malignancy risk remains unclear. We conducted a retrospective cohort study using the US Collaborative Network in the TriNetX database, encompassing data from January 1, 2016 to January 1, 2022. Adults diagnosed with PN (n = 10 941) were matched 1:1 with controls without PN (n = 10 941) based on demographics, comorbidities, and medication use. The primary outcome was the hazard ratio (HR) for malignancy occurring between 3 months and 5 years after the index date. The HRs and 95% confidence intervals (CIs) were calculated using Cox proportional hazards models. Patients with PN exhibited a significantly increased risk of developing malignancies compared with the controls (HR 2.10; 95% CI 1.81-2.43). Notably, higher risks were observed for cutaneous squamous cell carcinoma (HR 4.24; 95% CI 2.69-6.69), basal cell carcinoma (HR 2.49; 95% CI 1.68-3.69), hematopoietic cancers (HR 1.97; 95% CI 1.26-3.06), gastrointestinal cancers (HR 1.87; 95% CI 1.24-2.81), respiratory system cancers (HR 1.86; 95% CI 1.23-2.82), and female genital cancers (HR 2.77; 95% CI 1.29-5.95). In conclusion, PN is associated with a significantly elevated risk of malignancy, particularly cutaneous cancers. These preliminary findings underscore the necessity for heightened clinical vigilance. Further prospective studies are needed to confirm this association, elucidate the underlying mechanisms, and evaluate the potential benefits of routine cancer screening in this high-risk population.

We reported a 15-year-old boy with extensive linear porokeratosis confirmed by clinical, histopathologic, and immunohistochemical assessment. Ixekizumab treatment lead to a near-complete clearance of lesions by 3 months, while nail dystrophy showed minimal response. The presence of IL-17A-positive infiltrates supported the immunologic basis for therapeutic efficacy. These findings suggest that ixekizumab is a promising targeted therapy for refractory linear porokeratosis.

Systemic amyloidosis is a multisystem disorder that requires histological confirmation of amyloid deposition in at least one organ for a definitive diagnosis. While biopsies of organs such as the myocardium provide high diagnostic accuracy, they are highly invasive and technically demanding. Thus, skin biopsies are frequently performed as a less invasive alternative. However, when skin biopsies fail to detect amyloid depositions, more invasive procedures-such as gastrointestinal or myocardial biopsies-are often required. Despite the clinical importance of improving diagnostic yield, few studies have systematically evaluated optimal skin biopsy techniques. Then, we conducted a retrospective observational study of 100 patients who underwent skin biopsies for suspected systemic amyloidosis at Kobe University Hospital between April 2014 and November 2024. In this study, two skin biopsy methods were analyzed: Punch biopsies from multiple random sites (1-5 sites) using Dermapunch ("punch (multiple-punch) biopsy"), and spindle-shaped biopsy of long axis approximately 10 mm or more ("incision (spindle-shaped) biopsy"). As a result, among 69 cases diagnosed as systemic amyloidosis based on amyloid detection in any organ, including the skin, 13 of 28 punch biopsies (46.4%) were positive, and 21 of 41 incision biopsies (51.2%) were positive. The difference in sensitivity was not statistically significant (p = 0.81), but incision biopsies showed a numerically higher sensitivity. Furthermore, fatty tissue was the most common amyloid deposition site, with a mean depth of 5.1 mm. In two cases, depositions were found at a depth of approximately 12 mm. In this study, no significant difference was observed in the diagnostic yield between the two biopsy methods. However, because amyloid depositions may occur deep within subcutaneous fat and incision biopsy enables deliberate and consistent sampling of this layer, it potentially improves diagnostic accuracy. We, therefore, recommend incision biopsy as the preferred method for diagnosing systemic amyloidosis.

Hyperhidrosis decreases an individual's quality of life (QOL). The Hyperhidrosis Quality of Life Index (HidroQoL) measures the impact of hyperhidrosis on QOL and has established reliability and validity. However, a Japanese version does not exist. Hence, this study aimed to develop the Japanese version of the HidroQoL (HidroQoL-J) and verify its reliability and validity. The first survey included 528 participants (272 males, 256 females, mean age ± standard deviation 41.89 ± 15.24 years) who met the criteria for hyperhidrosis and scored ≥ 2 on the Hyperhidrosis Disease Severity Scale (HDSS). The second survey was conducted for reliability and included 210 participants (105 males, 105 females, mean age ± standard deviation 43.56 ± 14.60 years). The main survey items were (1) HidroQoL-J, (2) Dermatology Life Quality Index (DLQI), (3) Skindex-16, and (4) Anxiety Scale Specific to Hyperhidrosis Symptoms (ASSHS). Confirmatory factor analysis revealed the HidroQoL-J had a two-factor structure: a "daily life activities domain" with six items and a "psychosocial life domain" with 12 items, as in the original English version of the instrument. Cronbach's alphas (α) for the HidroQoL-J were 0.93, 0.85, and 0.91 for overall, daily life activities, and psychosocial life, respectively. Test-retest reliability was r = 0.70, 0.67, and 0.69 for overall, daily life activities, and psychosocial life (all p < 0.001), respectively. Furthermore, the intraclass correlation coefficients were 0.70, 0.67, and 0.69, respectively. Moderate positive correlations were observed between the overall HidroQoL-J score and the DLQI (r = 0.56) and Skindex-16 (r = 0.43) scores (all p < 0.001). There was also a moderate positive correlation between the overall score of the HidroQoL-J and HDSS (r = 0.42) and a weak positive correlation with ASSHS (r = 0.39) (all p < 0.001). Therefore, the HidroQoL-J exhibited sufficient reliability and validity to measure the impact of hyperhidrosis symptoms on QOL.