Primary cutaneous amyloidosis (PCA) is a chronic pruritic skin disease. The apple-green birefringence of Congo red-stained amyloid under a polarized light microscope (CR-PLM) remains the gold standard in the diagnosis of PCA. However, there are some limitations to this approach. In this study, eighty-two paraffin-embedded biopsy skin samples were collected from patients with a clinical diagnosis of PCA. The sections were respectively stained with hematoxylin–eosin (HE), crystal violet (CV), and Congo red (CR) and observed under a light microscope. CR-stained sections were also observed under a polarized light microscope (CR-PLM) or an ultraviolet (UV)-emitted fluorescence microscope (CR-UFM). Further, 35 cases clinically diagnosed with psoriasis, lichen planus, and prurigo nodularis were selected as the negative control group. The positive rate of amyloid protein detected by CR-UFM (81.71%) was significantly higher than that detected by CR-PLM (70.73%, p = 0.004), CR staining (56.10%, p < 0.001), CV staining (30.49%, p < 0.001), or HE staining (28.05%, p < 0.001). In the control group, 34 (97.14%) cases were negative for amyloid deposits in CR staining, CR-PLM, and CR-UFM sections. The relative number of positive dermal papillae observed by CR-UFM (0.35 ± 0.27) was much more than that observed by CR-PLM (0.15 ± 0.17, p<0.001), CR staining (0.12 ± 0.16, p < 0.001), CV staining (0.07 ± 0.12, p < 0.001), or HE staining (0.05 ± 0.12, p < 0.001). The intensity of fluorescence by CR-UFM was significantly greater than that of the appl-green birefringence by CR-PLM (p < 0.001). Moreover, the amyloid was easily distinguished from the surrounding tissues using the CR-UFM method. In conclusion, the CR-UFM method was superior to CR-PLM, CR staining, CV staining, and HE staining in diagnosing PCA.
The treatment of inflammatory skin diseases is entering a new era. The advent of biologics and small-molecule agents has facilitated the therapy of some of the previously difficult-to-treat skin conditions. However, there are still several pruritic skin conditions that many dermatologists find difficult to manage. Understanding the precise clinical features and actual pathological mechanisms of these diseases is essential for the development and appropriate use of new therapies. This special issue of the Journal of Dermatology focuses on three common pruritic skin diseases.
Eczema is the most common and well-known pruritic disease in dermatology. However, how many dermatologists fully understand the exact nature of eczema and its characteristics? In this special issue, Tokura et al. discuss the nature of eczema. They explain the etymology and classic concept of eczema, including the ‘eczema triangle’. They also discuss the pathological mechanisms of eczema based on the current understanding of cutaneous immunology.
While we have recently gained powerful therapeutic tools for treating chronic inflammatory skin conditions, such as atopic dermatitis and psoriasis, erythroderma in the elderly remains difficult to treat. In general, the diagnosis of erythroderma is relatively straightforward. Nevertheless, despite careful examinations, it is often difficult to determine the actual cause of the disease. Yamamoto explains the concept, clinical characteristics, and pathogenesis of erythroderma in the elderly, and the difference between elderly erythroderma and elderly atopic dermatitis.
Research into itching has lagged far behind that into pain, and, for a long time, antihistamines have dominated the treatment of itching associated with skin diseases. However, recent advances in the study of itch have provided us with a wealth of knowledge about nonhistaminergic itch. In this issue, Hashimoto et al. describe the latest findings on the pathophysiology of itch. They also explain the mechanisms of generalized and localized pruritus without rash and how we can manage these conditions.
The review articles in this issue will undoubtfully help dermatologists to better understand, diagnose, and manage these common pruritic skin conditions.
Itch, also known as pruritus, is one of the most prevalent symptoms observed in dermatological practices. Itch frequently arises from primary pruritic dermatoses, although it may also manifest in the absence of a primary pruritic skin rash. The latter itchy condition is referred to as “cutaneous pruritus” in the Japanese guidelines published in 2020. Cutaneous pruritus can be classified into two categories based on its distribution: localized cutaneous pruritus and generalized cutaneous pruritus. Localized cutaneous pruritus is indicative of a neuropathic cause, whereas generalized cutaneous pruritus suggests underlying systemic disease(s), drug-induced itch, psychogenic itch (also known as functional itch disorder), or chronic pruritus of unknown origin (CPUO). Systemic diseases associated with cutaneous pruritus include disorders of iron metabolism, chronic kidney disease, chronic liver disease (especially cholestasis), endocrine/metabolic diseases, hematological disorders, and malignant solid tumors. CPUO is a term used to describe chronic itch that is often generalized and for which no underlying cause can be identified despite a comprehensive and careful diagnostic workup. A variety of treatment approaches are available for cutaneous pruritus, including device-based physical therapies (such as phototherapy) and medications that act on the itch-perception processing pathway from the skin, peripheral sensory nerves, the spinal cord, to the brain. This review presents an overview of the current knowledge regarding cutaneous pruritus, from its underlying pathophysiologic mechanisms to the diagnostic procedures and treatment approaches that are currently available.
Hereditary palmoplantar keratoderma (hPPK) comprises a clinical and heterogeneous group of skin disorders characterized by hyperkeratosis of the palms and soles. Variants of SERPINA12 have been implicated in autosomal recessive diffuse hPPK, which shares similarities with Nagashima-type PPK due to biallelic variants in SERPINB7. To date, seven SERPINA12 variants have been found in 11 patients with biallelic SERPINA12 variants worldwide. Herein, we described six new cases of hPPK caused by biallelic SERPINA12 variants from southwestern China. Our study showed commonly extensive distribution of skin lesions and various comorbidities in patients with SERPINA12-related hPPK. Moreover, we revealed the variant c.635-7A>G was a founder variant in patients with SERPINA12-related hPPK in southwestern China. Our work is helpful to improve the knowledge of clinical and genetic characteristics of SERPINA12-related hPPK.
Kodamaea ohmeri infection is a relatively rare condition, primarily affecting immunocompromised individuals or those with a history of invasive procedures. The diagnosis of this infection is challenging because of its diverse and complex atypical clinical presentations. In this study, we describe a case of cutaneous infection with Kodamaea ohmeri presenting as erythematous and scaly lesions on both armpits and groin with itching in an 82-year-old man, mimicking intertrigo, and review the relevant literature. The diagnosis was confirmed through fungal microscopy, fungal culture, and mass spectrometry. Antifungal therapy proved to be effective in managing the infection. This case suggests that K. ohmeri, similar to Candida, is a potential pathogen in secondary infection associated with intertrigo. The findings highlight that early identification and diagnosis are crucial for managing K. ohmeri infections effectively.
Deucravacitinib, an oral, selective, allosteric tyrosine kinase 2 inhibitor, is approved in Japan for adult patients with plaque, generalized pustular, or erythrodermic psoriasis. POETYK PSO-4 (NCT03924427), an open-label, single-arm, phase 3 trial, showed that deucravacitinib was effective and well tolerated in Japanese patients with plaque (n = 63), generalized pustular (n = 3), or erythrodermic (n = 8) psoriasis. Additional end points in POETYK PSO-4 included change measured by the patient-reported outcome measures Psoriasis Symptoms and Signs Diary and Dermatology Life Quality Index. Mean changes from baseline in score on each measure and the response rate for achieving Dermatology Life Quality Index scores of 0 or 1 were assessed in each patient group over 52 weeks. All assessments were reported as observed, without imputation, in the as-treated population. Each group reported week 16 score improvements from baseline for both patient-reported outcome measures that were maintained or numerically improved at week 52. At week 52, approximately two-thirds of patients in each group achieved a Dermatology Life Quality Index score of 0 or 1, indicating no impact of disease on quality of life. These results demonstrate improvements in psoriasis symptoms and signs and in quality of life with deucravacitinib treatment in Japanese patients with plaque, generalized pustular, or erythrodermic psoriasis. The small number of patients with generalized pustular or erythrodermic psoriasis limits the generalized interpretability of the findings in these groups.
We encountered two cases of onychomycosis caused by Aspergillus (A.) subramanianii and A. sclerotiorum. These species belong to the Aspergillus section Circumdati, which is reportedly common in a wide range of habitats. The members of section Circumdati rarely cause onychomycosis. We report the second and fifth cases of A. subramanianii and A. sclerotiorum in the world. Positive findings in dermatophyte antigen kits helped us to diagnose these infections. These isolates were sensitive to ravuconazole, with a minimum inhibitory concentration of 0.5 μg/mL. They were successfully treated with oral fosravuconazole. We discuss 14 cases of onychomycosis caused by Aspergillus section Circumdati.
Secukinumab is the first human monoclonal antibody that targets human interleukin-17A. This open-label, multicenter, uncontrolled, single-arm, prospective observational surveillance evaluated the long-term safety and effectiveness of secukinumab in patients with psoriasis vulgaris and psoriatic arthritis (PsA) in Japan. Of 997 patients whose surveillance forms were collected, 976 were included in the safety analysis and 729 in the effectiveness analysis. Prior to the start of secukinumab treatment, biologics were used in 42.52% of patients for the treatment of conditions including psoriasis. The mean ± standard deviation (SD) duration of secukinumab administration was 288.1 ± 106.51 days and the median (range) was 344.0 (1–365) days. The most commonly used dose per administration was 300 mg in 96.21% (939 patients) and the mean ± SD total number of administrations was 13.6 ± 3.87. Adverse events (AEs), AEs suspected to be related to secukinumab, AEs that led to secukinumab treatment discontinuation, serious AEs, and deaths were reported in 36.17%, 18.85%, 8.09%, 5.84%, and 1.13%, respectively. The proportion of patients with an Investigator's Global Assessment score improvement to 0/1 increased over time from the start of secukinumab treatment to week 24 and remained stable thereafter. The Psoriasis Area and Severity Index 75 response rates and the proportions of patients with a Dermatology Life Quality Index score of 0/1 increased from baseline and were maintained up to week 52. This surveillance did not show any new safety concerns of secukinumab treatment. The effectiveness of secukinumab treatment was observed in patients with psoriasis vulgaris and PsA.
A 58-year-old woman sought care for painful skin papules and blisters on her right lower extremity, ongoing for a month. Initially diagnosed with herpes zoster, she received no relief from famciclovir or acyclovir treatments. The lesions spread to her thighs, manifesting as itchy, tingling spots. With a history of poorly differentiated endometrial cancer for 11 years, she had undergone radical surgery and multiple chemotherapy regimens. Systemic examination revealed left cervical lymph node enlargement. Skin inspection showed swelling and redness with bumps on her right lower leg and left thigh base (referenced in Figure 1a,b). A thigh biopsy showed tumor cells arrayed among dermal collagen in various sizes with intense chromatin and clear nuclear division (referenced in Figure 1c,d). The dermal collagen was markedly sclerotic with few inflammatory cells. Immunohistochemistry was positive for P53, EMA, and PAX8, and D2-40 staining indicated lymphatic infiltration by tumor cells. She was diagnosed with cutaneous metastasis from poorly differentiated endometrial adenocarcinoma.
Cutaneous metastasis from carcinoma is a rare event in the spread of cancer, seen in 0.7%–9% of metastatic cases. It is even rarer in endometrial cancer, with an incidence of less than 0.8%.1 Zosteriform metastasis in skin cancer is a rare phenomenon linked to malignancies such as lung, breast, and gastric cancers, and melanoma. It typically manifests in a band-like pattern, often associated with pain, indicating disease progression and correlating with the primary tumor's location.2
In this case, the patient was diagnosed with endometrial cancer. When skin metastasis was subsequently discovered, the patient had already entered the late stage of malignant tumor treatment, with a poor prognosis. Therefore, when patients with pre-existing endometrial cancer suddenly present with new skin lesions, such as shingles, they need to be alert to the possibility of skin metastasis.
None declared.
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