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Journal of Investigative Medicine High... 2024Subcutaneous panniculitis-like T-cell lymphoma (SPTLP), a unique variant of primary cutaneous T-cell lymphomas, clinically mimics subcutaneous panniculitis. It is... (Review)
Review
Subcutaneous panniculitis-like T-cell lymphoma (SPTLP), a unique variant of primary cutaneous T-cell lymphomas, clinically mimics subcutaneous panniculitis. It is typified by the development of multiple plaques or subcutaneous erythematous nodules, predominantly on the extremities and trunk. Epidemiological findings reveal a greater incidence in females than males, affecting a wide demographic, including pediatric and adult cohorts, with a median onset age of around 30 years. Diagnosis of SPTLP is complex, hinging on skin biopsy analyses and the identification of T-cell lineage-specific immunohistochemical markers. Treatment modalities for SPTLP are varied; while corticosteroids may be beneficial initially for many patients, a substantial number require chemotherapy, especially in cases of poor response or relapse. Generally, SPTLP progresses slowly, yet approximately 20% of cases advance to hemophagocytic lymphohistiocytosis (HLH), often correlating with a negative prognosis. We report a case of a young male patient presenting with prolonged fever, multiple skin lesions accompanied by HLH, a poor clinical course, and eventual death, diagnosed postmortem with SPTLP. In addition, we also present a literature review of the current evidence of some updates related to SPTLP.
Topics: Humans; Male; Biopsy; Diagnosis, Differential; Fatal Outcome; Lymphohistiocytosis, Hemophagocytic; Lymphoma, T-Cell; Lymphoma, T-Cell, Cutaneous; Panniculitis; Skin; Skin Neoplasms; Young Adult
PubMed: 38742532
DOI: 10.1177/23247096241253337 -
Frontiers in Medicine 2024Retroperitoneal fibrosis, a condition of uncertain origin, is rarely linked to 8% of malignant cases, including breast, lung, gastrointestinal, genitourinary, thyroid,...
BACKGROUND
Retroperitoneal fibrosis, a condition of uncertain origin, is rarely linked to 8% of malignant cases, including breast, lung, gastrointestinal, genitourinary, thyroid, and carcinoid. The mechanism leading to peritoneal fibrosis induced by tumors is not well understood, possibly encompassing direct infiltration of neoplastic cells or the initiation of inflammatory responses prompted by cytokines released by tumor cells. We report a case of breast cancer with renal metastasis and retroperitoneal fibrosis detected using F-FDG PET/CT, providing help for clinical diagnosis and treatment.
CASE REPORT
A 49-year-old woman was referred to the hospital with elevated creatinine and oliguria for over a month. Abdominal computer tomography (CT) and magnetic resonance imaging (MRI) showed a retroperitoneal fibrosis-induced acute kidney injury (AKI) was suspected. However, a percutaneous biopsy of the kidney lesion confirmed metastasis from breast cancer. The physical examination revealed inverted nipples and an orange peel appearance on the skin of both breasts. Ultrasonography revealed bilateral hyperplasia (BIRADS 4a) of the mammary glands and bilateral neck and axillary lymphadenopathy. Subsequently, F-deoxyglucose positron emission tomography/computer tomography (F-FDG PET/CT) detected abnormally high uptake (SUVmax) in the bilateral mammary glands and axillary lymph nodes, suggesting bilateral breast cancer. Furthermore, abnormal F-FDG uptake was detected in the kidney, suggesting renal metastasis. In addition, abnormal F-FDG uptake was observed in the vertebrae, accompanied by an elevation in inhomogeneous bone mineral density, raising suspicion of bone metastases. However, the possibility of myelodysplasia cannot be dismissed, and further investigations will be conducted during close follow-ups. There was significant F-FDG uptake in the retroperitoneal position indicating a potential association between retroperitoneal fibrosis and breast cancer. The final pathological diagnosis of the breast tissue confirmed bilateral invasive ductal carcinoma. The patient had been treated with 11 cycles of albumin-bound (nab)-paclitaxel (0.3 mg) and had no significant adverse reaction.
CONCLUSION
In this case, neither the bilateral breast cancer nor the kidney metastatic lesion showed typical nodules or masses, so breast ultrasound, abdominal CT, and MRI did not suggest malignant lesions. PET/CT played an important role in detecting occult metastases and primary lesions, thereby contributing to more accurate staging, monitoring treatment responses, and prediction of prognosis in breast cancer.
PubMed: 38741768
DOI: 10.3389/fmed.2024.1353822 -
Clinical, Cosmetic and Investigational... 2024Sporotrichosis is a subacute or chronic infectious disease caused by sporothrix. It is mainly caused by Sporothrix inoculation after accidental skin injury during human...
Sporotrichosis is a subacute or chronic infectious disease caused by sporothrix. It is mainly caused by Sporothrix inoculation after accidental skin injury during human labor. The clinical manifestations of sporotrichosis are variable, ranging from nodules, plaques, ulcers, verrucous lesions, and subcutaneous masses. Some reports indicate that sporotrichosis can mimic psoriasis-like lesions. We herein report a case of sporotrichosis mimicking rosacea lesions. In addition, the patient had a history of nasal trauma, and we believe that the patient was inoculated with Sporothrix after the nasal skin was damaged during labor activities. The patient was given itraconazole 200 mg orally daily for 3 months, which effectively resolved the rash.
PubMed: 38737947
DOI: 10.2147/CCID.S461558 -
Journal of Inflammation Research 2024To explore the clinical and imaging features of rare site Kimura's disease (KD).
PURPOSE
To explore the clinical and imaging features of rare site Kimura's disease (KD).
METHODS
Retrospective analysis was conducted on the clinical manifestations, laboratory examinations, and imaging features of five patients with rare site KD. All imaging data, including the location, quantity, size, uniformity, boundary, and enhanced appearance of the lesion were evaluated by two independent radiologists.
RESULTS
Of the five patients, four were asymptomatic, and one experienced localized skin itching. Four cases involved subcutaneous nodules in the upper arm, while one was in the inguinal region. The main manifestations were single (three cases) or multiple (two cases) subcutaneous nodules/masses, with three patients accompanied by local lymph node enlargement. Four patients exhibited elevated eosinophil counts in their peripheral blood. Four patients had lesions with vascular flow voids; in three of these, the lesions also showed prominent enhancement. Notably, the lesion in a 5-year-old did not show vascular flow voids but displayed significant enhancement. Additionally, two patients showed edema around the lesions.
CONCLUSION
The presence of solitary or multiple subcutaneous nodules/masses in the upper arm or inguinal area, accompanied by lymph node enlargement, elevated eosinophils in the peripheral blood, and the observation of internal vascular within the lesion, can aid in the diagnosis of KD occurring in uncommon anatomical locations.
PubMed: 38737107
DOI: 10.2147/JIR.S459978 -
Journal of Cutaneous and Aesthetic... 2024Acne keloidalis nuchae is a chronic inflammatory condition affecting the occipital area or nape of the neck characterized by fibrotic papules, firm pustules, and nodules...
Treatment of Acne Keloidalis Nuchae by Simply Combining Two Conventionally Available Modalities: Ablation with Carbon Dioxide Laser and Intralesional Triamcinolone Acetonide.
Acne keloidalis nuchae is a chronic inflammatory condition affecting the occipital area or nape of the neck characterized by fibrotic papules, firm pustules, and nodules that coalesce into keloid like mass or plaques. It was first described by Kaposi in 1969. It is also known as lichen keloidalis nuchae, dermatitis papillaris capillitia, and folliculitis nuchae scleroticans. If not treated in the early stages of the disease it tends to be refractory to conventional methods like topical, intralesional steroids with antibiotics or retinoids. Surgical interventions remain the only choice in such cases. Various surgical modalities including surgical excision with primary or secondary closure, split-thickness skin grafts, and laser-assisted treatments have been tried. Most surgical methods have longer downtime with a higher chance of relapse. We studied the results of a combined treatment option carbon dioxide laser ablation followed by intralesional triamcinolone injection in the same sitting. This outpatient procedure has shown good results with no relapse and less downtime.
PubMed: 38736851
DOI: 10.4103/JCAS.JCAS_112_23 -
Photodiagnosis and Photodynamic Therapy Jun 2024Nodulocystic acne is a severe form of acne, which is commonly treated with oral isotretinoin, hormones, or antibiotics. However, drug therapy often has some side effects...
BACKGROUND
Nodulocystic acne is a severe form of acne, which is commonly treated with oral isotretinoin, hormones, or antibiotics. However, drug therapy often has some side effects and poor compliance. Fire needle combined with 5-aminolevulinic acid photodynamic therapy (ALA-PDT) is a simple, effective, short-term treatment with few adverse reactions, which is expected to be an effective physiotherapy for nodulocystic acne. Moreover, the combination with isotretinoin can reduce the dosage of the drug, thereby reducing the side effects of isotretinoin.
OBJECTIVES
To evaluate the safety and efficacy of fire-needle pretreated ALA-PDT combined with low-dose isotretinoin in the treatment of severe refractory nodulocystic acne.
METHODS
This study reported 10 patients with refractory nodulocystic acne who received combined treatment. During the treatment period, all patients received a low dose of oral isotretinoin capsules daily. The acne lesions were pretreated with fire needle before ALA-PDT treatment. The number of acne lesions, including papules, pustules, and nodular cysts, was documented at weeks 0, 2, 4, 8, and 12 to assess the therapeutic efficacy. Concurrently, adverse reactions such as pain, pruritus, and pigmentation changes were recorded and evaluated throughout the treatment course.
RESULTS
After combined treatment, all patients achieved good therapeutic effects, with an overall effective rate of 90 % at week 12. After treatment, skin lesions such as nodules, and cysts subsided significantly. The combination therapy has no serious adverse effects and has a favorable safety profile.
CONCLUSION
Fire needle pretreatment ALA-PDT combined with low-dose isotretinoin is effective and safe in the treatment of severe refractory nodular cystic acne, which is worthy of clinical promotion and research.
Topics: Humans; Isotretinoin; Aminolevulinic Acid; Photochemotherapy; Acne Vulgaris; Photosensitizing Agents; Male; Female; Young Adult; Adult; Adolescent; Combined Modality Therapy; Dermatologic Agents; Dose-Response Relationship, Drug
PubMed: 38735352
DOI: 10.1016/j.pdpdt.2024.104215 -
Journal of Clinical Medicine May 2024Nodular or keloidal scleroderma is a rare condition with unclear cause and sporadic mentions in the medical literature. It was first recognized in the 19th century, yet... (Review)
Review
Nodular or keloidal scleroderma is a rare condition with unclear cause and sporadic mentions in the medical literature. It was first recognized in the 19th century, yet its classification is still debated due to the limited number of reported cases. This rare variant of scleroderma is associated with either progressive systemic sclerosis or localized morphea. Clinically, it presents with asymptomatic nodules or plaques, resembling spontaneous keloid formation, often found on the trunk and proximal extremities. Recent literature reviews show a predominance of women with a mean age of 44 years. Diagnosis relies on clinical and histopathological findings, which usually show overlapping features of both scleroderma and true keloids, secondarily to an excessive fibrosing reaction attributed to collagen formation. We present an unusual case of a 70-year-old female patient who displayed the coexistence of two distinct subtypes of morphea (nodular/keloidal and linear), and exclusive skin involvement, which contrasts with the typical presentation of nodular/keloidal scleroderma, often associated with organ-specific disease. However, recent publications have diverged from previous ones regarding systemic sclerosis, with no systemic involvement reported between 2018 and 2024, which we evaluated in our descriptive literature review. With less than 50 cases reported in total, our case underlines the importance of recognizing this rare disease, ensuring appropriate evaluation, treatment, and follow-up.
PubMed: 38731191
DOI: 10.3390/jcm13092662 -
Cureus Apr 2024Nocardial mycetoma is a neglected tropical disease reported worldwide, especially in tropical and subtropical regions. It is ubiquitous in nature and is a soil-borne,...
Nocardial mycetoma is a neglected tropical disease reported worldwide, especially in tropical and subtropical regions. It is ubiquitous in nature and is a soil-borne, gram-positive, filamentous, aerobic bacteria with acute angle branching. Traumatic inoculation in endemic areas is the primary mode of infection of this debilitating disease. The clinical triad of tumefaction, draining sinus, and pus discharge with granules is very much characteristic and specific for clinching the diagnosis of mycetoma. However, the painless nature of the primary skin lesion often makes the patient present late to the clinician, often in the advanced stages of the disease. Here, we present a very intriguing case report of a young female patient who presented with a single neck nodule but was later diagnosed as a case of nocardial mycetoma. Timely diagnosis and initiation of therapy proved to be a boon for the patient with almost complete recovery within a few weeks in the form of healed skin lesions and insignificant scarring.
PubMed: 38716027
DOI: 10.7759/cureus.57753 -
Neurology. Genetics Apr 2024Tuberous sclerosis complex (TSC) is a genetic disorder caused by a or gene variation characterized by widespread hamartomas in organs such as the skin, brain, heart,...
OBJECTIVES
Tuberous sclerosis complex (TSC) is a genetic disorder caused by a or gene variation characterized by widespread hamartomas in organs such as the skin, brain, heart, lungs, liver, and kidneys.
METHODS
We report a case of a patient with TSC who presented with broad clinical manifestations, including epilepsy.
RESULTS
An 18-year-old man was diagnosed with recurrent drug-resistant epilepsy. Neuroimaging revealed bilateral cortical and subcortical tubers with multiple calcified subependymal nodules. His skin involvement and psychomotor retardation raised the suspicion of TSC. Genetic testing confirmed the diagnosis, and a combined treatment including mTOR inhibitors was initiated.
DISCUSSION
TSC, although considered rare, needs to be considered when evaluating patients with broad clinical manifestations. Our report has significant implications for understanding the impact of genotype on the prognosis of TSC and the selection of treatment strategies for TSC-related refractory epilepsy.
PubMed: 38715652
DOI: 10.1212/NXG.0000000000200127 -
Frontiers in Immunology 2024Patients with the multibacillary form of leprosy can develop reactional episodes of acute inflammation, known as erythema nodosum leprosum (ENL), which are characterized...
INTRODUCTION
Patients with the multibacillary form of leprosy can develop reactional episodes of acute inflammation, known as erythema nodosum leprosum (ENL), which are characterized by the appearance of painful cutaneous nodules and systemic symptoms. Neutrophils have been recognized to play a role in the pathogenesis of ENL, and recent global transcriptomic analysis revealed neutrophil-related processes as a signature of ENL skin lesions.
METHODS
In this study, we expanded this analysis to the blood compartment, comparing whole blood transcriptomics of patients with non-reactional lepromatous leprosy at diagnosis (LL, n=7) and patients with ENL before administration of anti-reactional treatment (ENL, n=15). Furthermore, a follow-up study was performed with patients experiencing an ENL episode at the time of diagnosis and after 7 days of thalidomide treatment (THAL, n=10). Validation in an independent cohort (ENL=8; LL=7) was performed by RT-qPCR.
RESULTS
An enrichment of neutrophil activation and degranulation-related genes was observed in the ENL group, with the gene for the neutrophil activation marker being the most enriched gene of ENL episode when compared to its expression in the LL group. A more pro-inflammatory transcriptome was also observed, with increased expression of genes related to innate immunity. Validation in an independent cohort indicated that expression could discriminate ENL from LL. Supernatants of blood cells stimulated with sonicate showed higher levels of CD177 compared to the level of untreated cells, indicating that the leprosy bacillus can activate neutrophils expressing CD177. Of note, suggestive higher CD177 protein levels were found in the sera of patients with severe/moderate ENL episodes when compared with patients with mild episodes and LL patients, highlighting CD177 as a potential systemic marker of ENL severity that deserves future confirmation. Furthermore, a follow-up study was performed with patients at the time of ENL diagnosis and after 7 days of thalidomide treatment (THAL, n=10). Enrichment of neutrophil pathways was sustained in the transcriptomic profile of patients undergoing treatment; however, important immune targets that might be relevant to the effect of thalidomide at a systemic level, particularly and , were revealed.
DISCUSSION
In conclusion, our study reinforces the key role played by neutrophils in ENL pathogenesis and shed lights on potential diagnostic candidates and novel therapeutic targets that could benefit patients with leprosy.
Topics: Humans; Erythema Nodosum; Neutrophil Activation; Leprosy, Lepromatous; Adult; Male; Neutrophils; Female; Transcriptome; Middle Aged; Gene Expression Profiling; GPI-Linked Proteins; Thalidomide; Receptors, Cell Surface; Leprostatic Agents; Young Adult; Biomarkers; Isoantigens
PubMed: 38715615
DOI: 10.3389/fimmu.2024.1366125