Acantholytic Anaplastic Extramammary Paget's Disease: A Case Report and Review of the Literature

Extramammary Paget's disease (EMPD) is an uncommon intraepithelial neoplasm that most commonly arises on the vulva and perianal region. Very few cases of EMPD revealing a histological Bowenoid appearance have been reported. This study describes scrotal EMPD presenting with histological features of Bowen's disease in a 79-year-old man. He presented with a 5-year history of a pruritic erythematous plaque and a verrucous papule on the scrotum. The verrucous papule histopathologically showed Bowenoid features, and the erythematous plaque demonstrated acantholytic EMPD. Immunohistochemical findings revealed strong expression for carcinoembryonic antigen, Cam 5.2, epithelial membrane antigen, cytokeratin (CK) 7, and pancytokeratin (AE1/AE3) in both areas, but negative CK20 staining, supporting the overall diagnosis of primary acantholytic anaplastic EMPD. This is the first reported case of acantholytic anaplastic EMPD in the Korean literature

Eccrine Porocarcinoma Presenting with Unusual Clinical Manifestations: A Case Report and Review of the Literature

Eccrine porocarcinoma is a rare neoplasm that originates from the intraepidermal ductal portion of the eccrine sweat gland, and it usually occurs on the pre-existing lesion of benign eccine poroma. Its occurrence is more common in females and elderly persons. We present a case of a 44-year-old man who suffered from eccrine porocarcinoma, which developed on the right scrotum and pelvic area with metastases to the lung, adrenal gland, esophagus, ureter, and distant lymph nodes. Here we report on a unique case of eccrine porocarcinoma that developed primarily on the scrotum, which is an uncommon site, and showed rapid metastasis to the internal organs

Ceramides and Cell Signaling Molecules in Psoriatic Epidermis: Reduced Levels of Ceramides, PKC-α, and JNK

Ceramides are the main lipids in the stratum corneum and are generated during cellular stress and apoptosis by de novo synthesis or by the action of sphingomyelinase. In addition, they are lipid second messengers produced by sphingolipid metabolism and trigger important cell responses, including protein kinase C-alpha (PKC-α) activation and the stimulation of signal transduction pathways with apoptosis and stress-activated protein kinases (SAPK), such as c-jun N-terminal kinase (JNK). Thus, ceramides have anti-proliferative and apoptotic effects. This study measured the changes in the levels of epidermal ceramides and ceramide-related apoptotic signaling molecules in psoriasis patients. Samples from lesional and non-lesional epidermis were obtained from psoriasis patients. Total ceramides were fractionated using thin-layer chromatography, and the levels of PKC-α and JNK expression were measured using Western blot analysis with specific antibodies. The ceramide level was reduced significantly, and this was associated with the downregulation of apoptotic signaling molecules, such as PKC-α and JNK, in the lesional epidermis of psoriasis patients. These results suggest that the decreased level of ceramides downregulates the apoptotic pathway, leading to epidermal proliferation in psoriasis

An Inverse Relationship Between Ceramide Synthesis and Clinical Severity in Patients with Psoriasis

Ceramides play major roles in maintaining the epidermal barrier. It has been sus-pected that the depletion of ceramides, associated with disrupted barrier function in the epidermis, leads to the clinical manifestation of dryness and inflammation seen in patients with psoriasis. The aim of the present study was to determine the relation-ship between the level of ceramide synthesis in the epidermis and the clinical severity in patients with psoriasis. Samples from lesional and unlesional epidermis obtained from psoriasis patients were incubated with [14C]serine, an initiator of ceramide syn-thesis. otal ceramide was fractionated using high performance thin layer chromato-graphy, and the radioactivity was measured. The clinical severity of psoriasis was graded according to the psoriasis area and severity index scoring system. The level of ceramide synthesis in the lesional epidermis of patients was significantly lower than that in the unlesional epidermis and bore a negative correlation with the clinical severity of psoriasis. The present results suggest that the decreased level of ceramide synthesis in the epidermis contributes to the clinical severity of psoriasis

Assessment of Disease Severity and Quality of Life in Patients with Atopic Dermatitis from South Korea

BACKGROUND: Data illustrating the impact of atopic dermatitis (AD) on lives of adults with AD in South Korea are limited. OBJECTIVE: To assess the AD disease severity and its impact on quality of life (QoL) in patients with AD from South Korea. METHODS: Patients with AD utilizing the specialist dermatology services of major hospitals in South Korea were assessed for disease severity using Eczema Area and Severity Index (EASI) score, for QoL using Dermatology Life Quality Index (DLQI) (for QoL), and for comorbidities and treatment experience via retrospective review of 12-month medical records. Clinical and sociodemographic characteristics were also measured. RESULTS: Of the 1,163 patients, 695 (59.8%) were men (mean age [years]±standard deviation: 31.6±12.1). Overall, 52.9% (n=615) patients had moderate-to-severe disease (EASI\u3e7). The QoL of 72.3% (n=840) patients was affected moderately-to-severely (DLQI score: 6~30). Systemic immunosuppressants were used ≥1 over past 12 months in 51.9% (n=603) patients, and the most commonly used were cyclosporines (45.7%, n=531) and systemic corticosteroids (40.5%, n=471). Approximately, 10.8% (n=126) patients consulted or received treatment for AD-related eye problem. Of these, 40% (n=50) patients reported poor, very poor, or completely blind status; approximately, 16.7% patients (n=192) reported having depression or anxiety; and 35.5% (n=410) reported suicidal ideation or suicidal attempt. CONCLUSION: A large proportion of patients had moderate-to-severe AD, a compromised QoL, and ocular or mental health comorbidities, indicating a high disease burden despite systemic treatment. These findings highlight the importance of a holistic approach for the evaluation and treatment of patients with AD

Serial Hydrolysis for the Simultaneous Analysis of Catecholamines and Steroids in the Urine of Patients with Alopecia Areata

Catecholamines and steroids are well-known neurotransmitters and hormones that rapidly change the excitability of neurons. Alopecia areata is a disease for which the exact cause is unknown, but it is considered to be associated with stress, and so the simultaneous analysis of catecholamines and steroids is required for the diagnosis of alopecia areata. Thus, we herein report the simultaneous analysis of catecholamines and steroids bearing different functional groups for the first time, during which it was necessary to carry out a serial hydrolysis procedure. Following hydrolysis of the urine samples to produce the free forms from the urinary conjugates, ethyl acetate extractions were carried out, and chemical derivatization was performed using dansyl chloride to increase the sensitivity of the liquid chromatography–tandem mass spectrometry method. The matrix effects and recoveries of this analytical method were validated, giving values of 85.4–122.9% and 88.8–123.0%, respectively. In addition, the method accuracy and precision were assessed, giving values of 0.4–21.5% and 2.0–21.6% for the intra-day and inter-day precisions, respectively. This validated method was then applied to identify differences between patients with and without alopecia areata, wherein the metanephrine content was found to be significantly higher in the alopecia areata patient group. This quantitative profiling method can also be applied to steroid-dependent diseases, as well as catecholamine-related diseases