Diagnostic and management challenges in primary cutaneous anaplastic large cell lymphoma with necrosis,inflammation,and surgical intervention:A case report

BACKGROUND Primary cutaneous anaplastic large cell lymphoma(PC-ALCL)poses significant diagnostic difficulties due to its similarity in the appearance of skin lesions with chronic inflammatory disorders and other dermatological conditions.This study aims to investigate these challenges by conducting a comprehensive analysis of a case presenting with PC-ALCL,emphasizing the necessity of accurate differentiation for appropriate management.CASE SUMMARY An 89-year-old female patient with diabetes and hypertension presented with arm and abdominal ulcerated mass lesions.Diagnostic procedures included skin biopsies,histopathological assessments,and immunohistochemistry,complemented by advanced imaging techniques to confirm the diagnosis.The patient’s lesions were determined as PC-ALCL,characterized by necrosis,chronic inflammation,and a distinct immunophenotypic profile,including CD30,CD3,CD4,and EBER,CD56,MUM-1,Ki 67-positive in>80%of tumor cells,CD10,but negative for anaplastic lymphoma kinase,CD5,CD20,PAX-5,Bcl-2,Bcl-6,CD8,and CD15.Recurrence was not reported at the 6-month follow-up.CONCLUSION Accurate PC-ALCL differentiation from similar conditions is crucial for effective management and requires a multidisciplinary approach.

Ultrasound-guided peripheral nerve blocks for anterior cutaneous nerve entrapment syndrome after robot-assisted gastrectomy: A case report

BACKGROUND Anterior cutaneous nerve entrapment syndrome(ACNES)is a condition mani-festing with pain caused by strangulation of the anterior cutaneous branch of the lower intercostal nerves.This case report aims to provide new insight into the selection of peripheral nerve blocks for the ACNES treatment.CASE SUMMARY A 66-year-old woman manifested ACNES after a robot-assisted distal gastrec-tomy.An ultrasound-guided rectal sheath block was effective for pain triggered by the port scar.However,the sudden severe pain,which radiated laterally from the previous site,remained.A transversus abdominis plane block was performed for the remaining pain and effectively relieved it.CONCLUSION In this case,the trocar port was inserted between the rectus and transverse abdominis muscles.The intercostal nerves might have been entrapped on both sides of the rectus and transversus abdominis muscles.Hence,rectus sheath and transverse abdominis plane blocks were required to achieve complete pain relief.To the best of our knowledge,this is the first report on use of a combination of rectus sheath and transverse abdominis plane blocks for pain relief in ACNES.

Clinical Study on the Effectiveness of Xian Fang Huo Ming Yin for Treating Cutaneous Infections and Promoting Wound Healing in Patients with Perianal Abscess

Objective:To explore the effect of the Xian Fang Huo Ming Yin(XFHM)for treating cutaneous infections and promoting wound healing in patients with perianal abscesses.Methods:Sixty-one patients with perianal abscesses who were admitted to our hospital(Xinghua City People’s Hospital)from May 2022 to May 2023 were selected and randomly divided into two groups,a control group(30 cases)and a study group(31 cases).Both groups received surgical treatment.The control group received conventional treatment and warm water fumigation,sitz bath,and surgical dressing change after surgery,while the research group received XFHM based on the control group.XFHM was taken orally and replaced with warm water for fumigation and sitz bathing.Both groups received treatment for 4 weeks but discontinued sitz bathing after 2 weeks.Various clinical indicators between the two groups were compared.Results:The total clinical effective rate and wound recovery rate of the study group were higher than that of the control group.There were differences in the wound pain scores,surrounding tissue edema,and wound secretions at different time points.Both groups experienced wound pain.The scores of wound pain,surrounding tissue edema,and wound secretions of the study group were lower than those of the control group,7 and 14 days after surgery.The serum interleukin 6(IL-6),tumor necrosis factor-alpha(TNF-α)levels,and pH values of the study group were lower than those of the control group 10 days after surgery(P<0.05).Conclusion:The application of XFHM for treating cutaneous infections and promoting wound healing in patients with perianal abscesses improved the treatment outcome,alleviated clinical symptoms,and promoted healing.

Endometriosis beyond the Pelvis: A Case Series of Cutaneous Endometriosis and Literature Review

Introduction: Cutaneous endometriosis is an uncommon but well-known skin disorder that represents about 0.5% to 1% of all endometriosis. The objective of this case series is to report clinical presentation, diagnosis, and management of various forms of cutaneous endometriosis. Material and Methods: It was an observational, retrospective and descriptive review of cases presenting with cutaneous endometriosis among Cameroonian women managed at the gynaecological outpatient department of Yaounde Gynaeco-Obstetric and Pediatric Hospital. All the following parameters were analysed: age, parity, previous pelvic surgery, presenting symptoms and duration, associated symptoms, localizations, imaging, size of the lesion, other localization of endometriosis, management and histopathological results. Results: we reported 4 cases of cutaneous endometriosis, with 3 umbilical endometriosis and 1 abdominal scar endometriosis. Patient age ranged from 28 to 39 years with an average of 33 years. All patients described infertility (two primary and two secondary) and two had a history of abdominal surgery. All patients presented local cyclical signs such as pain, swelling, color change and bleeding. The duration of symptoms varied from 2 to 3 years and the size of lesions ranged from 2 to 3.5 cm for umbilical lesions and was 9 cm for abdominal scar endometriosis. In all cases, no imaging was required for the diagnosis, which was suspected on the basis of patient’s history and the cyclical nature of local signs, followed by wide surgical excision and confirmation on histopathology. Conclusion: Cutaneaous endometriosis is a rare benign condition. Umbilical endometriosis seems to be the main cutaneous localization and can be described as primary or secondary. Even if its diagnosis must be confirmed by histopathology, it should be considered in patient with cutaneous cyclic signs such as pain, swelling or bleeding with or without history of abdominal surgery.

Antibody-platinum(IV)prodrugs conjugates for targeted treatment of cutaneous squamous cell carcinoma

Antibody-drug conjugates(ADCs)are a new type of targeting antibodies that conjugate with highly toxic anticancer drugs via chemical linkers to exert high specificity and efficient killing of tumor cells,thereby attracting considerable attention in precise oncology therapy.Cetuximab(Cet)is a typical antibody that offers the benefits of good targeting and safety for individuals with advanced and inoperable cutaneous squamous cell carcinoma(cSCC);however,its anti-tumor activity is limited to a single use.Cisplatin(CisPt)shows good curative effects;however,its adverse effects and non-tumor-targeting ability are major drawbacks.In this study,we designed and developed a new ADC based on a new cytotoxic platinum(IV)prodrug(C8Pt(IV))and Cet.The so-called antibody-platinum(IV)prodrugs conjugates,named Cet-C8Pt(IV),showed excellent tumor targeting in cSCC.Specifically,it accurately delivered C8Pt(IV)into tumor cells to exert the combined anti-tumor effect of Cet and CisPt.Herein,metabolomic analysis showed that Cet-C8Pt(IV)promoted cellular apoptosis and increased DNA damage in cSCC cells by affecting the vitamin B6 metabolic pathway in tumor cells,thereby further enhancing the tumor-killing ability and providing a new strategy for clinical cancer treatment using antibody-platinum(IV)prodrugs conjugates.

Volumetric measurement techniques for assessment of cutaneous neurofibromas:A review

Cutaneous neurofibroma(cNF)is a prevalent clinical manifestation of neurofibromatosis type 1,significantly affecting the well-being and quality of life of the affected individuals.The adoption of reliable and reproducible volumetric measurement techniques is essential for precisely evaluating tumor burden and plays a critical role in the development of effective treatments for cNF.This study focuses on widely used volumetric measurement techniques,including vernier calipers,ultrasound,computed tomography,magnetic resonance imaging,and three-dimensional scanning imaging.It outlines the merits and drawbacks of each technique in assessing the cNF load,providing an overview of their current applications and ongoing research advancements in this domain.

A Case Report on Cutaneous Larva Migrans (CLM)

Cutaneous larva migrans (CLM) is a zoonotic hookworm infection of dogs and cats commonly found in low-income countries in the tropical and subtropical regions and travellers to these regions. It is caused by invasion of the skin by the parasitic larvae of hookworms which, after entry, move under the skin causing an inflammatory reaction resulting in a single or multiple tracks. Patients commonly present with a progressive, itchy, erythematous serpiginous skin rash, affecting the feet, although it can affect any other parts of the body. The diagnosis is mainly clinical, based on history and physical examination. Treatment of this condition can effectively be achieved with either albendazole or ivermectin. We present a case of a 7-year-old boy who was diagnosed with CLM after presenting to the hospital with a history of a progressive itchy rash with tracks on the left foot. Although CLM is a self-limiting disease, it causes a lot of suffering and, therefore, it is of public concern. There is a need to increase awareness of this disease among health workers, and to implement and promote preventive measures against the disease since the cause is known.

Forecasting the number of zoonotic cutaneous leishmaniasis cases in south of Fars province, Iran using seasonal ARIMA time series method

Objective: To predict the trend of cutaneous leishmaniasis and assess the relationship between the disease trend and weather variables in south of Fars province using Seasonal Autoregressive Integrated Moving Average(SARIMA) model,Methods: The trend of cutaneous leishmaniasis was predicted using Mini tab software and SARIMA model,Besides,information about the disease and weather conditions was collected monthly based on time series design during January 2010 to March 2016,Moreover,various SARIMA models were assessed and the best one was selected,Then,the model's fitness was evaluated based on normality of the residuals' distribution,correspondence between the fitted and real amounts,and calculation of Akaike Information Criteria(AIC) and Bayesian Information Criteria(BIC),Results: The study results indicated that SARIMA model(4,1,4)(0,1,0)(12) in general and SARIMA model(4,1,4)(0,1,1)(12) in below and above 15 years age groups could appropriately predict the disease trend in the study area,Moreover,temperature with a three-month delay(lag3) increased the disease trend,rainfall with a four-month delay(lag4) decreased the disease trend,and rainfall with a nine-month delay(lag9) increased the disease trend,Conclusions: Based on the results,leishmaniasis follows a descending trend in the study area in case drought condition continues,SARIMA models can suitably measure the disease trend,and the disease follows a seasonal trend.

Gastrointestinal manifestations of systemic mastocytosis

Systemic mastocytosis (SM) is a rare disease with abnormal proliferation and infiltration of mast cells in the skin, bone marrow, and viscera including the mucosal surfaces of the digestive tract. Gastrointestinal (GI) symptoms occur in 14%-85% of patients with systemic mastocytosis. The GI symptoms may be as frequent as the better known pruritis, urticaria pigmentosa, and flushing. In fact most recent studies show that the GI symptoms are especially important clinically due to the severity and chronicity of the effects that they produce. GI symptoms may include abdominal pain, diarrhea, nausea, vomiting, and bloating. A case of predominantly GI systemic mastocytosis with unique endoscopic images and pathologic confirmation is herein presented, as well as a current review of the GI manifestations of this disease including endoscopic appearances. Issues such as treatment and prognosis will not be discussed for the purposes of this paper.

Topical halometasone cream combined with fire needle pretreatment for treatment of primary cutaneous amyloidosis:Two case reports

BACKGROUND Primary cutaneous amyloidosis(PCA)is a chronic metabolic skin disease that has a detrimental impact on physical and mental health.It appears as mossy papules and severe itching,which is long-term and recurrent.Traditional treatments are unsatisfactory,especially for refractory cases.Fire needle therapy,which is widely used in China,has shown good clinical efficacy,as well as advantages concerning safety and cost.Clinical reports about fire needle treatment of this disease are few at present.CASE SUMMARY We report two older men who had developed maculopapules with itchiness on the trunk and arms for more than 10-15 years.Due to the dermatopathological findings,PCA was our primary consideration.They received topical halometasone cream and pretreatment with fire needle for 8-16 wk.Both patients showed significant improvement of lesions.Neither patient had recurrence with a minimum of 2 years of follow-up.CONCLUSION Topical halometasone cream and pretreatment with fire needle could be a fast,safe,and economic treatment for PCA.

Disseminated cutaneous leishmaniasis due to Leishmania(Leishmania) amazonensis in human immunodeficiency virus(HIV)-infected patients: A report of two cases

Rationale: Co-infection of human immunodeficiency virus(HIV) and Leishmania spp. has impact on clinical and therapeutic outcomes of leishmaniases. Most studies do not present the identification of Leishmania species causing American tegumentary leishmaniasis in co-infections. In the Americas, Leishmania(L.) Viannia(V.) braziliensis and L.(V.) guyanensis have been identified. Patient concerns: In this study, two cases of American tegumentary leishmaniasis in patients infected with HIV are described. Patients presented several lesions with rapid dissemination and mucosal involvement. Diagnosis: Disseminated cutaneous leishmaniasis caused by L. amazonensis was identified by molecular test. Interventions: The patients were treated with conventional therapies for HIV infection and American tegumentary leishmaniasis. Outcomes: In co-infection, the clinical manifestations are atypical and the treatment response can be impaired. Lessons: These cases show that HIV infection impacts L. amazonensis infection and point to the relevance of identifying Leishmania species, which can lead to a better patient management.

Ultrasound-guided rectus sheath block for anterior cutaneous nerve entrapment syndrome after laparoscopic surgery:A case report

BACKGROUND Anterior cutaneous nerve entrapment syndrome is defined as abdominal pain due to entrapped intercostal nerves.This is the first report of a patient successfully treated for anterior cutaneous nerve entrapment syndrome after laparoscopic surgery with an ultrasound-guided rectus sheath block.The rectus sheath block physically lysed adhesions and relieved pain from anterior cutaneous nerve entrapment syndrome.CASE SUMMARY The patient is a 44-year-old man who presented with severe left upper abdominal pain at an operative scar one month after laparoscopic ulcer repair.Diagnosis and treatment were performed using an ultrasound-guided rectus sheath block with 0.1%lidocaine 20 mL.The pain was relieved after the block.The diagnosis was anterior cutaneous nerve entrapment syndrome.Rectus sheath block may be effective for patients with anterior cutaneous nerve entrapment syndrome.CONCLUSION Ultrasound-guided rectus sheath block is a promising treatment modality for patients with postoperative anterior cutaneous nerve entrapment syndrome due to adhesions.

An unusual case of aggressive systemic mastocytosis mimicking hepatic cirrhosis

Hepatic involvement in aggressive systemic mastocytosis(ASM) is relatively common, and the main clinical features of this disease include hepatomegaly, portal hypertension, ascites, and fibrosis. Cirrhosis is a rare ASM symptom. We report an ASM case that initially mimicked cirrhosis based on clinical and radiographic analyses. The portal tract was expanded by mononuclear inflammatory cells, and an increase in collagen amount was observed in routine histological sections of the biopsied liver. A diagnosis of systemic mastocytosis(SM) was made after ancillary tests for mast cells using bone marrow aspirates. Extensive involvement of the liver and gastrointestinal tract was observed. Clinicians and pathologists need to consider ASM as a diagnosis or differential diagnosis in a clinical case of cirrhosis with unknown etiology. The diagnosis can be confirmed or disregarded by immunohistochemical staining and molecular analysis.

Systemic mastocytosis: A rare cause of non-cirrhotic portal hypertension

Mastocytosis is a clonal neoplastic disorder of the mast cells(MC) that can be limited to the skin(cutaneous mastocytosis) or involve one or more extracutaneous organs(systemic mastocytosis). The clinical manifestations of mastocytosis are heterogeneous ranging from indolent disease with a long-term survival to a highly aggressive neoplasm with survival of about 6 mo. Although liver involvement in aggressive systemic mastocytosis(ASM) is relatively common, the development of portal hypertension with or without cirrhosis is rare. We report a case of ASM without skin involvement in a 72-year-old caucasian male who presented with non-cirrhotic portal hypertension based on clinical, analytical, imagiological and endoscopic findings. Given the hematological picture, the correct diagnosis was established based on ancillary tests for MC using bone marrow aspirates and biopsy. Extensive involvement of the liver and gastrointestinal tract was histologically documented. The disease progressed rapidly and severe pancytopenia and recurrent upper gastrointestinal bleeding became the dominant problem. This case illustrates the challenge in establishing a diagnosis of ASM especially when the clinical picture is atypical and without skin involvement. Gastroenterologists should consider infiltrative disease, particularly systemic mastocytosis, as a differential diagnosis in a clinical case of portal hypertension of unknown etiology.

The Inhibitory Effects of Tetracycline on Mastocytosis

Mastocytosis is a rare disease that causes its victims pains in the body, anaphylactic shocks and in some cases death. There is currently no cure for this disease but there are some medications taken to manage the symptoms of this disease. The cause of mastocytosis is the overabundance of mast cells in the blood stream, body tissues, and bone marrow. These mast cells are well meaning hematopoietic cells which are members of the body’s response to an allergy. It has been established that tetracyclines possess anti-bacterial properties, but recent development has also shown that it also possesses anti-inflammatory properties as well as apoptotic properties which can be applied in the treatment of mastocytosis. This paper focuses on the use of the principle of “Creative synthesis” to formulate a possible treatment to mastocytosis using tetracycline and polyphenols as well as chemically modified tetracyclines (CMTs). In addition, investigations will be carried out to proffer a possible combination which will help in inhibiting the effects of mastocytosis. Based on the synthesis, an economic analysis will be carried out to show the economic potential of this proffered treatment.

T-CELL RECEPTOR GENE REARRANGEMENT ANALYSIS IN THE PRIMARY CUTANEOUS T-CELL LYMPHOMA

Object: The present paper is to evaluate the significance of T cell receptor (TCR) gene rearrange ments in primary cutaneous T cell lymphomas (PCTCL) as detected by analysis of Southern Blot (SBA) and polymerase chain reaction (PCR). Patients and Methods: Skin specimens and peripheral blood samples were taken from 44 patients with PCTCL, including 30 patients with mycosis fungoides (MF), 2 patients with Sezary's syndrome (SS), and 12 patients with PCTCL other than MF and SS (PNCTCL). 11 patients with a presumptive diagnosis of MF, 23 patients with lymphoproliferative dermatoses including lymphomatoid papulosis (LyP) and 8 patients with benign cutaneous lymphoid infiltrates were simultaneously studied by the amplification of junctional V (variable) J (joining) sequences of the rearranged TCRγ genes by PCR(TCRγPCR) and the analysis of TCRb chain genes by SBA(TCRβSBA) for detection of clonal gene rearrangements (GR). One lymph node specimen of a case with MF IIA was also detected by TCRγ PCR and TCRβSBA. Results: In MF, GR were detected by TCRγPCR and TCRβSBAb in 83.3 85.7% and 66.7% 71.4% of skin specimens of cases IIA IIB and in 57.1% 70.0% and 14.3% 10.0% of those of cases IA IB, respectively. GR were seen in 66.7% 71.4% and 33.3% 43.0.% of blood samples of cases IIA IIB, and 42.9% 40.0% and 0 10.0% of those of cases IA IB, respectively. GR was confirmed by TCRγ PCR and TCRβSBA in one lymph node showing dermato pathic lymphadenopathy of a case with MF IIA. In 11 patients of clinically suspected MF, GR were present in skin specimens of 5 cases (45.4%) and in blood samples of 3 cases ( 27.3% ) by TCRγ PCR. In PNCTCL, GR were found in 9 skin specimens (90.0%) from 10 patients detected by TCRγ PCR and in 6 skin specimens (75.0%) from 8 patients detected by TCRβSBA. GR were also seen in 6 blood samples (72.8%) from 11 patients detected by TCRγ PCR, and in 7 blood samples (70.0%) from 10 patients by TCRβSBA. In SS and LyP, GR were detected by TCRγ PCR and TCRβSBA in each of the two skin specimens of two cases with LyP and in each of the two blood samples of two cases with SS. GR were seen in one skin specimen of one case with SS and one blood sample of one case with LyP detected by TCRγPCR. Conclusions: This study demonstrated that TCRγ PCR is a rapid, more sensitive tool than TCRβSBA, can be used in the analysis of T cell clonality in skin, lymph node and blood samples of patients with PCTCL and indicated that this method forms a useful supplement to other methods for diagnosis of early and suspected MF, confirmation of PNCTCL and determination of extracutaneous involvement of lymph node and blood.

Peripheral Nerve Blockade in a Patient with Mastocytosis

Mastocytosis is a rare group of disorders with chronic and episodic mast cell release of mediators which can have systemic and cutaneous manifestations. Triggers of anaphylaxis include commonly used medications for anesthesia, analgesia, and muscle relaxation. There is little in the literature regarding local anesthesia in emergent surgery for patients with mastocytosis. This case details the use of a peripheral nerve blockade for multiple surgeries and pain control in a patient with biopsy-proven mastocytosis.

Recalcitrant Cutaneous Mastocytosis Treated With Genetically Informed Targeted Therapy:A Case Report

Introduction:Mastocytosis,a clonal proliferation of mast cells commonly involving the skin and bone marrow,has a varied clinical presentation ranging from cutaneous lesions to systemic disease.Cutaneous mastocytosis is managed symptomatically,but systemic mastocytosis is treated with targeted therapy against the mutated receptor tyrosine kinase c-KIT,the pathogenic driver of mastocytosis.However,there are no guidelines for the treatment of cutaneous mastocytosis refractory to symptomatic management.We herein report a method to select genetically informed therapy for symptomatic and recalcitrant cutaneous mastocytosis.Case presentation:We performed a mutational analysis of dermal mast cells after enrichment by laser capture in a 23-year-old woman with recalcitrant cutaneous mastocytosis.The analysis revealed a aspartic acid to valine substitution at codon 816(D816V)mutation in the protein c-KIT.Based on these results,we initiated treatment with the multi-kinase/KIT inhibitor midostaurin,a treatment effective against the D816V c-KIT mutation.After 3 months of treatment,the patient exhibited a reduction in the number and size of cutaneous lesions and reported resolution of pruritus and decreased severity of other mast cell-related symptoms.Discussion:The treatment of mastocytosis relies heavily on whether the disease is limited to the skin or systemic.However,there are no guidelines for cutaneous mastocytosis that does not respond to symptomatic management.In the present report describing a patient with recalcitrant cutaneous mastocytosis,we describe a strategy in which skin mutational analysis is used to guide the selection of targeted therapy.Conclusion:Performing mast cell mutational analyses in the skin provides a means to select targeted therapy for symptomatic and refractory cutaneous mastocytosis.

Evaluation of the Effects of Corticosteroids on Histamine Release by ex Vivo Cutaneous Microdialysis

The purpose of the study was to evaluate the effects of corticosteroids on histamine release and to compare their potency with the MacKenzie classification based on their vasoconstrictor effects. Thanks to ex Vivo cutaneous microdialysis, we studied histamine-induced release over a period of time on excised abdominal skin from women. Eight corticosteroids were topically applied with occlusive dressing onto the skin, above probes, before anti-IgE injection. Histamine levels were assessed by an EIA method. In order to compare the different corticosteroids, AUC was calculated allowing an estimation of the amount of released histamine for 60 min of ex vivo cutaneous microdialysis. Diflucortolone 0.1% and micronized betamethasone dipropionate 0.05% are considered as corticosteroids with high potency in MacKenzie classification. Betamethasone dipropionate associated with propylene glycol 0.05%, belongs to a stronger class in Mackenzie classification. Our results showed that the decrease in histamine release was more important with difluocortolone than with both of these corticosteroids. Therefore there was no correlation between the vasoconstrictor potency of topical corticosteroids and their ability to inhibit histamine release.

Clinical challenge for gastroenterologists–Gastrointestinal manifestations of systemic mastocytosis:A comprehensive review

Mastocytosis is a rare and heterogeneous disease characterized by various clinical and biological features that affect different prognoses and treatments.The disease is usually divided into 2 principal categories:cutaneous and systemic disease(SM).Clinical features can be related to mast cell(MC)mediator release or pathological MC infiltration.SM is a disease often hard to identify,and the diagnosis is based on clinical,biological,histological,and molecular criteria with different specialists involved in the patient’s clinical work-up.Among all manifestations of the disease,gastrointestinal(GI)symptoms are common,being present in 14%-85% of patients,and can significantly impair the quality of life.Here we review the data regarding GI involvement in SM,in terms of clinical presentations,histological and endoscopic features,the pathogenesis of GI symptoms,and their treatment.