A Case Report of Mycosis Fungoides Presenting With Blister Formation.

Mycosis fungoides (MF) is the most common primary cutaneous T-cell lymphoma with a usually indolent course. Early detection is crucial for effective intervention. We present a case of a 40-year-old male with MF exhibiting blistering as a rare precursor symptom. Despite initial treatment for eczema, the condition worsened over 10 months, leading to erythema, edema, and enlarged lymph nodes. Laboratory and imaging findings confirmed the diagnosis of MF. The patient responded partially to cyclophosphamide/doxorubicin/prednisone in combination with brentuximab vedotin (A-CHP) therapy. This case highlights the significance of recognizing blistering as a prodromal symptom for early detection and management of MF.

Purpuric Type Drug Eruption Caused by Azithromycin: A Case Report and Literature Review.

Azithromycin, an azolide antibiotic with structural and functional similarities to macrolides, possesses distinct features such as its effects persisting for seven days, an extended half-life by administering it once daily for three days, and strong antimicrobial activity. Notably, vomiting and diarrhea are recognized as the primary adverse events related to azithromycin. In this particular case, we present a unique case describing a purpuric-type drug eruption associated with azithromycin, which represents an uncommon cutaneous manifestation. A 64-year-old female developed a purpuric eruption on her trunk and lower extremities seven days after receiving daily intravenous azithromycin for upper bronchitis. A previous occurrence of punctate purpuric eruption following azithromycin administration was documented in her medical history. The diagnosis of azithromycin-induced skin eruption was confirmed based on the clinical progression and the recurrence of the eruption upon re-administration of the drug. In response to this diagnosis, the patient underwent treatment involving the discontinuation of azithromycin and the application of topical betamethasone butyrate propionate ointment. Remarkably, her eruption significantly improved within two weeks, although residual pigmentation persisted post-treatment. Additionally, we offer a comprehensive review of the literature, examining cases of drug eruptions related to azithromycin.

Clinical Characteristics of Cryopyrin-Associated Periodic Syndrome and Long-Term Real-World Efficacy and Tolerability of Canakinumab in Japan: Results of a Nationwide Survey.

OBJECTIVE: We assess the clinical characteristics of patients with cryopyrin-associated periodic syndrome (CAPS) in Japan and evaluate the real-world efficacy and safety of interleukin-1 (IL-1) inhibitors, primarily canakinumab. METHODS: Clinical information was collected retrospectively, and serum concentrations of canakinumab and cytokines were analyzed. RESULTS: A total of 101 patients were included, with 86 and 15 carrying heterozygous germline and somatic mosaic mutations, respectively. We identified 39 mutation types, and the common CAPS-associated symptoms corresponded with those in previous reports. Six patients (5.9% of all patients) died, with four of the deaths caused by CAPS-associated symptoms. Notably, 73.7% of patients (100%, 79.6%, and 44.4% of familial cold autoinflammatory syndrome, Muckle-Wells syndrome, and chronic infantile neurological cutaneous articular syndrome/neonatal onset multisystem inflammatory disease, respectively) achieved complete remission with canakinumab, and early therapeutic intervention was associated with better auditory outcomes. In some patients, canakinumab treatment stabilized the progression of epiphysial overgrowth and improved height gain, visual acuity, and renal function. However, 23.7% of patients did not achieve inflammatory remission with crucial deterioration of organ damage, with two dying while receiving high-dose canakinumab treatment. Serological analysis of canakinumab and cytokine concentrations revealed that the poor response was not related to canakinumab shortage. Four inflammatory nonremitters developed inflammatory bowel disease (IBD)-unclassified during canakinumab treatment. Dual biologic therapy with canakinumab and anti-tumor necrosis factor-α agents was effective for IBD- and CAPS-associated symptoms not resolved by canakinumab monotherapy. CONCLUSION: This study provides one of the largest epidemiologic data sets for CAPS. Although early initiation of anti-IL-1 treatment with canakinumab is beneficial for improving disease prognosis, some patients do not achieve remission despite a high serum concentration of canakinumab. Moreover, IBD may develop in CAPS after canakinumab treatment.

A Remitting Seronegative Symmetrical Synovitis With Pitting Edema (RS3PE) Syndrome Patient With High-Grade Serous Ovarian Cancer: A Possible Pathogenesis of Tumor-Derived Vascular Endothelial Growth Factor.

A 65-year-old female was previously diagnosed with remitting seronegative symmetrical synovitis with pitting edema (RS3PE) syndrome by internal doctors in our hospital nine years ago. Computed tomography revealed the presence of multiple disseminated peritoneal nodules with a large tumor mass. Histological analysis of the tumor and peritoneal nodules confirmed the diagnosis of high-grade serous ovarian cancer. The serum vascular endothelial growth factor (VEGF) level was highly elevated (1,223.9 pg/mL) (normal range: <38.3 pg/mL). One month after the first administration of docetaxel and cyclophosphamide chemotherapy, her peripheral edema decreased with a parallel reduction of serum VEGF (675.2 pg/mL). These findings suggest the correlation of VEGF with both RS3PE and ovarian cancer in this case.

Fixed Drug Eruption Caused by Garenoxacin: A Case Report and Literature Review.

A new quinolone antibiotic called garenoxacin was developed in Japan. Garenoxacin is known to produce cutaneous adverse effects, particularly fixed drug eruption in Japan, despite several reports of cutaneous adverse events in English-language literature. However, English-language literature has not yet reported that fixed drug eruption is a common clinical manifestation of garenoxacin-induced drug eruption. In this article, we present a case of multiple fixed drug eruptions and review the literature on case reports of drug eruptions caused by garenoxacin.

Congenital Langerhans Cell Histiocytosis With the Skin and Lung Involvement: A Case and Literature Review.

Langerhans cell histiocytosis (LCH) is a clonal proliferative disease of immature Langerhans cells that expand in various organs, leading to organ and tissue dysfunction. Although LCH is most commonly seen in children under the age of three, a small number of cases of congenital LCH have been described. With a review of the literature on congenital LCH with lung and skin lesions, we present a case of congenital LCH with involvement of skin and lung, which was effectively treated with chemotherapy without recurrence for 3 years during the observational period. In addition, we summarized previously published case studies of congenital LCH with skin and lung involvement.

Cardiac Arrest Due to Kounis Syndrome Following Cephazolin Administration During Surgery Under Local Anesthesia.

The Kounis syndrome is described as the co-occurrence of allergic responses brought on by mast cell activation and acute coronary syndromes. We present a case of Kounis syndrome leading to cardiac arrest following the cephazolin sodium administration during the surgical resection of basal cell carcinoma. An 87-year-old woman was diagnosed with basal cell cancer. She received surgical excision of the tumor while anesthetized with lidocaine hydrochloride and 1% epinephrine. This patient began to itch around five minutes after cefazolin (CEZ) administration and eventually experienced cardiac arrest following diffuse rashes that spread throughout her body and edema in her eyelids. In line with the response, the electrocardiogram (ECG) also showed an elevated ST segment in V1-6, leading to possibly the diagnosis of Kounis syndrome. We also review the literature on Kounis syndrome following CEZ administration.

Co-occurrence of Malignant Melanoma and Squamous Cell Carcinoma on Burn Scars: A Case Report.

Tumors arising from burn scars are not rare but sometimes cause the rare co-existence of different tumors. However, detailed information on this topic remains largely unknown. We present a case of the co-occurrence of malignant melanoma and squamous cell carcinoma in a patient with a history of burn scars. A 73-year-old man presented with an erythematous plaque on his left lower leg that gradually turned into a tumor with ulceration. He also presented with scaly tumors at other sites within the same burn scar lesion. He had a history of burns on the left leg at the age of 20 years. After surgical resection of the tumors, histological analysis revealed that the posterior aspect of the largest tumor was malignant melanoma, and the remaining two tumors were squamous cell carcinomas, indicating the co-existence of different types of malignant skin cancers. Based on a literature review of previously published case reports, this is the first report to highlight the importance of complete skin grafts in reducing this risk.

Three Cases of Occupational Allergic Contact Dermatitis Where Causative Agents Were Identified Using Patch Test Panel S.

Identifying the causative substances of occupational contact dermatitis is challenging because of several chemicals and materials in the workplace that can cause contact dermatitis. We experienced three cases of intractable eczema identified as work-related contact dermatitis by Patch Test Panel S, which helped identify the possible substances. We experienced three cases of occupational allergic contact dermatitis, and their causative agents were identified by Patch Test Panel S. Although there are some limitations, Patch Test Panel S might be useful to determine the substrates to cause allergic contact dermatitis in occupational scenarios.

The Efficacy of Platinum Chemotherapy in a Japanese Malignant Melanoma Patient With a BRCA2 Mutation Identified by Gene Panel Testing.

A BRCA2 mutation increases the chance of developing cancer and has been linked to several diseases, including hereditary breast, ovarian, pancreatic, and prostate cancers. We present a case of advanced malignant melanoma treated with platinum-containing chemotherapy and demonstrate a momentarily favorable clinical outcome as determined by a Next Generation Sequencer (NGS) gene panel testing. A 54-year-old female with BRAF wild-type of anal primary melanoma received adjuvant immunotherapy with nivolumab following surgical resection. Novel distant lung metastasis was identified four months after the adjuvant therapy. Multi-gene panel testing figured out another potential treatment strategy using a sample from a distant metastatic tumor and identified a BRCA2 mutation in the tumor. Based on the sensitivity to platinum agents in BRCA2 mutation-positive tumors, DAC-Tam therapy (Dacarbazine, Nimustine, Cisplatin, and Tamoxifen) was administrated and showed tumor size reduction. After five rounds of DAC-Tam treatment, the metastatic lesion decreased from 17 mm to 5 mm. The parent was treated with platinum and Dacarbazine alone because of deteriorated renal function and grade 3 myelosuppression. In addition, the tumor showed resistance to the platinum plus Dacarbazine chemotherapy. Her chemotherapy-induced renal failure and bone marrow suppression did not improve well. Additionally, she felt significant weakness due to poor dietary intake and did not want to receive additional chemotherapy. To relieve her symptoms, she and her family desired the best supporting care and moved her to another hospital. The patient died 12 months after submitting the gene panel.

S100 Proteins in the Pathogenesis of Psoriasis and Atopic Dermatitis.

The skin, the outermost layer of the human body, is exposed to various external stimuli that cause inflammatory skin reactions. These external stimulants trigger external epithelial cell damage and the release of intracellular substances. Following cellular damage or death, intracellular molecules are released that enhance tissue inflammation. As an important substance released from damaged cells, the S100 protein is a low-molecular-weight acidic protein with two calcium-binding sites and EF-hand motif domains. S100 proteins are widely present in systemic organs and interact with other proteins. Recent studies revealed the involvement of S100 in cutaneous inflammatory disorders, psoriasis, and atopic dermatitis. This review provides detailed information on the interactions among various S100 proteins in inflammatory diseases.

Ixekizumab-induced urticarial drug eruption.

Biological agents targeting inflammatory skin diseases have dramatically overcome many of the limitations of older oral therapeutic options. Among the various biological agents, ixekizumab is a humanised monoclonal antibody that blocks the biological activity of IL-17A, which exhibited high efficacy against psoriasis. Although there are a limited number of cutaneous adverse reactions, biologic-induced type I allergic reactions are rare. Herein, we report a case of ixekizumab-induced urticaria.

The Development of Systemic Inflammatory Diseases in Hidradenitis Suppurativa.

It is understood that the skin is a peripheral lymphoid tissue that defends against external environmental stimuli. Continuous activation from these factors, on the other hand, promotes persistent inflammation at the local location and, occasionally, tissue damage. Hidradenitis suppurativa (HS) is a typical inflammatory skin disease and becomes a source of numerous inflammatory cytokines due to the chronic intractable repeated inflamed tissues. Because inflammatory cells and cytokines circulate throughout the body from the inflamed organ, it has been hypothesized that HS-mediated skin inflammation impacts the systemic functioning of numerous organs. Recent updates to clinical and experimental investigations revealed that HS has a significant connection with systemic inflammatory disorders. We provide the details and comprehensive molecular mechanisms associated with systemic inflammatory illnesses due to HS.

The incidence of Jarisch-Herxheimer reactions and associated risk factors in pregnant women and nonpregnant women: A retrospective chart review at a university hospital in Japan.

AIMS: Jarisch-Herxheimer reactions (JHR) is a transient adverse event that occurs during initial antimicrobial treatment for syphilis patients, and is known to develop uterine contractions and fetal distress in pregnant women complicated with syphilis. The aim of this study is to identify risk factors for JHR in patients with syphilis, and to clarify whether pregnancy status is a risk factor for JHR, and to describe the characteristics of pregnant women who develop JHR. METHODS: This was a retrospective chart review in a singleton university hospital in Japan. We collected data of syphilis patients who were diagnosed and treated at department of obstetrics and gynecology, dermatology between January 2010 and May 2022. There were no validated diagnostic criteria for JHR, we defined JHR as one or more of the following in addition to raised body temperature (≧38.0°C) within 24 h of initial antibiotic treatment: headache, chills, myalgias, tachycardia (≧110 bpm), new rash. RESULTS: There were 30 syphilis patients. Of whom nine (30%) were pregnant women and all their neonates were not diagnosed with congenital syphilis. Five patients (17%) developed JHR at the time of initial treatment (JHR group, n = 5). There was no difference between JHR group and non-JHR group (n = 25) in pregnancy status. Secondary syphilis was an only significant risk factor for JHR. Two pregnant women with JHR were both treated for secondary syphilis in the third trimester of pregnancy. CONCLUSION: Pregnancy status was not a risk factor for JHR in syphilis patients. Further research is needed.

Survival rates of systemic interventions for psoriasis in the Western Japan Psoriasis Registry: A multicenter retrospective study.

Psoriasis affects approximately 0.3% of the Japanese population. Recently, various effective systemic drugs have become available, and the continuation of a given treatment has become critical because of the chronic nature of psoriasis. Factors affecting drug survival (the time until treatment discontinuation) in psoriasis treatment include efficacy, safety, ease of use, and patient preference. In the present study, the authors retrospectively surveyed a multifacility patient registry to determine the real-world evidence of the survival rate of systemic interventions for psoriasis treatment. Patients with psoriasis who visited 20 facilities in the Western Japan area between January 2019 and May 2020 and gave written consent were registered as study participants, and their medical history of systemic interventions for psoriasis (starting from 2010) was retrospectively collected and analyzed. The drugs investigated were adalimumab, infliximab, ustekinumab, secukinumab, ixekizumab, brodalumab, guselkumab, risankizumab, cyclosporine, and apremilast. When drugs were discontinued, the reasons were also recorded. A total of 1003 patients with psoriasis including 268 with psoriatic arthritis (PsA) were enrolled. In biologics, more recently released drugs such as interleukin 17 inhibitors showed a numerically higher survival rate in the overall (post-2010) analysis. However, in the subset of patients who began treatment after 2017, the difference in the survival rate among the drugs was smaller. The reasons for discontinuing drugs varied, but a loss of efficacy against dermatological or joint symptoms were relatively frequently seen with some biologics and cyclosporine. The stratification of drug survival rates based on patient characteristics such as bio-naive or experienced, normal weight or obese, and with or without PsA, revealed that bio-experienced, obese, and PsA groups had poorer survival rates for most drugs. No notable safety issues were identified in this study. Overall, the present study revealed that the biologics show differences in their tendency to develop a loss of efficacy, and the factors that negatively impact the survival rate of biologics include the previous use of biologics, obesity, and PsA.

Identification of Multiple Bowen's Disease Skin Lesions by Careful Physical Examination in a Patient With Fanconi Anemia.

Because Fanconi anemia is a hereditary bone marrow failure disease caused by DNA repair dysfunction, malignant skin tumors have been recognized in patients with Fanconi anemia. Herein, we report a 32-year-old male with Fanconi anemia presenting multiple Bowen's disease skin lesions. He first recognized skin eruption in his scrotum, which was diagnosed with Bowen's disease by dermoscopy examination and histological analysis. Due to the elevated risk of skin cancers in Fanconi anemia, we conducted additional meticulous examinations using dermoscopy on the entire body's skin, revealing another skin tumor on his back. A skin biopsy confirmed the diagnosis of another site of Bowen's disease. Therefore, additional thorough examinations using dermoscopy might aid in identifying multiple skin tumors in high-risk cases of skin malignancies, such as Fanconi anemia.

STING expression is an independent prognostic factor in patients with mycosis fungoides.

Mycosis fungoides is recognized as an indolent cutaneous malignant T-cell lymphoma. In contrast, there are few therapeutic options for advanced forms of mycosis fungoides. Since immunotherapy is desirable as an alternative therapeutic option, identifying candidate molecules is an important goal for clinicians. Although tumor-derived negative immunomodulatory molecules, such as PD-1/PD-L1, have been identified in various malignancies, the useful positive immunological drivers of mycosis fungoides are largely unknown. We found that the stimulator of interferon (IFN) genes (STING) was highly upregulated in early-stage mycosis fungoides. Immunohistochemical examination revealed different STING staining patterns in patients with mycosis fungoides. Although there were no significant differences in clinical factors' characteristics, STING expression was associated with the survival of patients with mycosis fungoides. The survival rate was significantly poor in patients with low STING-expressing mycosis fungoides. Univariate and multivariate analyses revealed that low STING expression was associated with an increased hazard ratio. Our results indicate that STING expression independently influences the prognosis of mycosis fungoides.

[A Case of a Subcutaneous Foreign Body Discovered by Coincidence During a Head MRI Imaging Examination].

An 80-year-old male was admitted to the department of neurology for intensive examination and treatment of peri-optic nerve inflammation. Magnetic resonance imaging examination could not be conducted because a magnetic resonance imaging examination at a previous clinic revealed a subcutaneous foreign body on his head, possibly a piece of metal. He was referred to our department for the removal of this foreign body. There was no traumatic scar in the skin and we could not identify this subcutaneous foreign body by physical examination and superficial echography, but radioscopy could find this subcutaneous material and we could remove this foreign body under the guidance of the radioscopy.

Vasospasm-induced ST-segment elevation myocardial infarction in a premenopausal woman with endothelial dysfunction.

ST-segment elevation myocardial infarction (STEMI) can be caused by coronary artery vasospasm (VSA) due to endothelial dysfunction. However, the clinical role of endothelial function tests in VSA-induced STEMI is not fully understood. We present the case of a 43-year-old woman with atypical chest pain and no coronary risk factors. STEMI caused by VSA was diagnosed. Flow-mediated vasodilatation (FMD) and EndPAT tests were performed; the FMD and reactive hyperaemia index were 3.8% and 1.23, respectively. Endothelial dysfunction is the putative cause of STEMI. FMD and EndPAT tests might be useful for predicting adverse outcomes in young premenopausal women with VSA.