Journal of Clinical and Diagnostic Research, ISSN - 0973 - 709X

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On Sep 2018

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On Sep 2018

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"Journal of Clinical and Diagnostic Research is at present a well-known Indian originated scientific journal which started with a humble beginning. I have been associated with this journal since many years. I appreciate the Editor, Dr. Hemant Jain, for his constant effort in bringing up this journal to the present status right from the scratch. The journal is multidisciplinary. It encourages in publishing the scientific articles from postgraduates and also the beginners who start their career. At the same time the journal also caters for the high quality articles from specialty and super-specialty researchers. Hence it provides a platform for the scientist and researchers to publish. The other aspect of it is, the readers get the information regarding the most recent developments in science which can be used for teaching, research, treating patients and to some extent take preventive measures against certain diseases. The journal is contributing immensely to the society at national and international level."

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On Aug 2018

Dr. Arundhathi. S
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MBBS, MD (Pathology),
Sanjay Gandhi institute of trauma and orthopedics,
On Aug 2018

Dr. Mamta Gupta,
"It gives me great pleasure to be associated with JCDR, since last 2-3 years. Since then I have authored, co-authored and reviewed about 25 articles in JCDR. I thank JCDR for giving me an opportunity to improve my own skills as an author and a reviewer.
It 's a multispecialty journal, publishing high quality articles. It gives a platform to the authors to publish their research work which can be available for everyone across the globe to read. The best thing about JCDR is that the full articles of all medical specialties are available as pdf/html for reading free of cost or without institutional subscription, which is not there for other journals. For those who have problem in writing manuscript or do statistical work, JCDR comes for their rescue.
The journal has a monthly publication and the articles are published quite fast. In time compared to other journals. The on-line first publication is also a great advantage and facility to review one's own articles before going to print. The response to any query and permission if required, is quite fast; this is quite commendable. I have a very good experience about seeking quick permission for quoting a photograph (Fig.) from a JCDR article for my chapter authored in an E book. I never thought it would be so easy. No hassles.
Reviewing articles is no less a pain staking process and requires in depth perception, knowledge about the topic for review. It requires time and concentration, yet I enjoy doing it. The JCDR website especially for the reviewers is quite user friendly. My suggestions for improving the journal is, more strict review process, so that only high quality articles are published. I find a a good number of articles in Obst. Gynae, hence, a new journal for this specialty titled JCDR-OG can be started. May be a bimonthly or quarterly publication to begin with. Only selected articles should find a place in it.
An yearly reward for the best article authored can also incentivize the authors. Though the process of finding the best article will be not be very easy. I do not know how reviewing process can be improved. If an article is being reviewed by two reviewers, then opinion of one can be communicated to the other or the final opinion of the editor can be communicated to the reviewer if requested for. This will help one’s reviewing skills.
My best wishes to Dr. Hemant Jain and all the editorial staff of JCDR for their untiring efforts to bring out this journal. I strongly recommend medical fraternity to publish their valuable research work in this esteemed journal, JCDR".

Dr. Mamta Gupta
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Aug 2018

Dr. Rajendra Kumar Ghritlaharey

"I wish to thank Dr. Hemant Jain, Editor-in-Chief Journal of Clinical and Diagnostic Research (JCDR), for asking me to write up few words.
Writing is the representation of language in a textual medium i e; into the words and sentences on paper. Quality medical manuscript writing in particular, demands not only a high-quality research, but also requires accurate and concise communication of findings and conclusions, with adherence to particular journal guidelines. In medical field whether working in teaching, private, or in corporate institution, everyone wants to excel in his / her own field and get recognised by making manuscripts publication.

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In the era of fast growing newer technologies, and in computer and internet friendly environment the manuscripts preparation, submission, review, revision, etc and all can be done and checked with a click from all corer of the world, at any time. Of course there is always a scope for improvement in every field and none is perfect. To progress, one needs to identify the areas of one's weakness and to strengthen them.
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Thanking you
With sincere regards
Dr. Rajendra Kumar Ghritlaharey, M.S., M. Ch., FAIS
Associate Professor,
Department of Paediatric Surgery, Gandhi Medical College & Associated
Kamla Nehru & Hamidia Hospitals Bhopal, Madhya Pradesh 462 001 (India)
On May 11,2011

Dr. Shankar P.R.

"On looking back through my Gmail archives after being requested by the journal to write a short editorial about my experiences of publishing with the Journal of Clinical and Diagnostic Research (JCDR), I came across an e-mail from Dr. Hemant Jain, Editor, in March 2007, which introduced the new electronic journal. The main features of the journal which were outlined in the e-mail were extensive author support, cash rewards, the peer review process, and other salient features of the journal.
Over a span of over four years, we (I and my colleagues) have published around 25 articles in the journal. In this editorial, I plan to briefly discuss my experiences of publishing with JCDR and the strengths of the journal and to finally address the areas for improvement.
My experiences of publishing with JCDR: Overall, my experiences of publishing withJCDR have been positive. The best point about the journal is that it responds to queries from the author. This may seem to be simple and not too much to ask for, but unfortunately, many journals in the subcontinent and from many developing countries do not respond or they respond with a long delay to the queries from the authors 1. The reasons could be many, including lack of optimal secretarial and other support. Another problem with many journals is the slowness of the review process. Editorial processing and peer review can take anywhere between a year to two years with some journals. Also, some journals do not keep the contributors informed about the progress of the review process. Due to the long review process, the articles can lose their relevance and topicality. A major benefit with JCDR is the timeliness and promptness of its response. In Dr Jain's e-mail which was sent to me in 2007, before the introduction of the Pre-publishing system, he had stated that he had received my submission and that he would get back to me within seven days and he did!
Most of the manuscripts are published within 3 to 4 months of their submission if they are found to be suitable after the review process. JCDR is published bimonthly and the accepted articles were usually published in the next issue. Recently, due to the increased volume of the submissions, the review process has become slower and it ?? Section can take from 4 to 6 months for the articles to be reviewed. The journal has an extensive author support system and it has recently introduced a paid expedited review process. The journal also mentions the average time for processing the manuscript under different submission systems - regular submission and expedited review.
Strengths of the journal: The journal has an online first facility in which the accepted manuscripts may be published on the website before being included in a regular issue of the journal. This cuts down the time between their acceptance and the publication. The journal is indexed in many databases, though not in PubMed. The editorial board should now take steps to index the journal in PubMed. The journal has a system of notifying readers through e-mail when a new issue is released. Also, the articles are available in both the HTML and the PDF formats. I especially like the new and colorful page format of the journal. Also, the access statistics of the articles are available. The prepublication and the manuscript tracking system are also helpful for the authors.
Areas for improvement: In certain cases, I felt that the peer review process of the manuscripts was not up to international standards and that it should be strengthened. Also, the number of manuscripts in an issue is high and it may be difficult for readers to go through all of them. The journal can consider tightening of the peer review process and increasing the quality standards for the acceptance of the manuscripts. I faced occasional problems with the online manuscript submission (Pre-publishing) system, which have to be addressed.
Overall, the publishing process with JCDR has been smooth, quick and relatively hassle free and I can recommend other authors to consider the journal as an outlet for their work."

Dr. P. Ravi Shankar
KIST Medical College, P.O. Box 14142, Kathmandu, Nepal.
On April 2011

Dear team JCDR, I would like to thank you for the very professional and polite service provided by everyone at JCDR. While i have been in the field of writing and editing for sometime, this has been my first attempt in publishing a scientific paper.Thank you for hand-holding me through the process.

Dr. Anuradha
On Jan 2020

Important Notice

Case Series
Year : 2023 | Month : September | Volume : 17 | Issue : 9 | Page : WR01 - WR05 Full Version

A Case Series of Amicrobial Pustulosis of Folds: An Eye-opener for the Diagnosis of Autoimmune Disorders

Published: September 1, 2023 | DOI:
Rajkumar Kannan, Samuel Jeyaraj Daniel, Parimalam Kumar, Akshaya Pradeep Nagalakshmi

1. Professor, Department of Dermatology, Madras Medical College, Chennai, Tamil Nadu, India. 2. Associate Professor, Department of Dermatology, Madras Medical College, Chennai, Tamil Nadu, India. 3. Professor, Department of Dermatology, Madras Medical College, Chennai, Tamil Nadu, India. 4. Resident, Department of Dermatology, Madras Medical College, Chennai, Tamil Nadu, India.

Correspondence Address :
Dr. Akshaya Pradeep Nagalakshmi,
Postgraduate Student, Department of Dermatology, RGGGH and Madras Medical College, Parktown, P.H. Road, Chennai-600003, Tamil Nadu, India.


Amicrobial Pustulosis of Folds (APF) is a rare, chronic, relapsing cutaneous disease seen exclusively in younger women with a history of autoimmune disease, most commonly Systemic Lupus Erythematosus (SLE), or who simply have circulating autoantibodies. This case series highlights the occurrence of an unusual manifestation of APF as a marker of autoimmune disorders and emphasises the importance of double-stranded Deoxyribose Nucleic Acid (dsDNA) positivity in such cases. A total of five female patients with APF were included in this case series. All five cases presented with pustular lesions lasting 10-12 weeks, involving conchal bowls, eyes, perineal, and perianal regions, either as an initial presentation or as a flare-up of pre-existing autoimmune disease. All patients underwent screening for autoimmune disorders, which revealed dsDNA positivity and met the diagnostic criteria for APF. Therefore, a diagnosis of APF with SLE was made. All patients showed significant improvement with oral steroids. Although SLE is a complex multisystem disorder where patients may not always present with malar rash, photosensitivity, arthritis, and arthralgia, APF could serve as an eye-opener for diagnosing underlying autoimmune diseases.


Neutrophilic dermatosis, Pustular dermatosis, Sterile pustules, Systemic lupus erythematosus

Amicrobial pustulosis of the folds is a rare neutrophilic dermatosis seen predominantly in young adult female patients with underlying autoimmune or rheumatic disease. APF is characterised by recurrent crops of pustules primarily in the skin folds and periorificial regions that eventually coalesce into plaques (1). It is characterised by the sudden onset of chronic, relapsing small follicular and non follicular pustules on an erythematous base, coalescing to form erosive plaques in patients with underlying autoimmunity. Associated with a wide variety of dermatoses, this entity was first reported in 1991 by Antille C et al., (2). In 2008, Marzano AV et al., outlined diagnostic criteria for APF that included major and minor criteria. The major criteria include pustulosis affecting one or more of the major folds or one or more minor folds in the anogenital area, histopathology showing intraepidermal spongiform pustules and a predominantly neutrophilic infiltrate in the dermis, negative culture of an intact pustule. The minor criteria include association with one or more autoimmune disorders, Antinuclear Antibody (ANA) titres of 1/160 or more, presence of autoantibodies such as Extractable Nuclear Antigens (ENA), anti-DNA, antismooth muscle, antimitochondrial, antigastric parietal cell, or endomysial antibodies (3). Clinically, these patients experience constitutional symptoms such as fever, arthritis, arthralgia, myalgia, sudden appearance of pustules, along with signs of flare-up of mucocutaneous disease manifestations like bullous SLE, acute palmar telangiectasia. The present case series entails five cases of APF associated with underlying autoimmune disorders, which can be an eye-opener for diagnosing such diseases.

Case Report

Case 1

A 31-year-old female came to the Dermatology OPD with a chief complaint of fluid-filled vesicles and pustules over the genital region, gluteal cleft, and axilla. She also had a painful erosion over the lip for the past four months, along with intermittent gingival bleeding and discharge from the left ear and left eye for the past three months. There was no history of drug intake, topical application of any drugs, or any medical co-morbidities. No family history of similar complaints was noted. The patient had previously been evaluated at another institution three months ago and was diagnosed with mucosal pemphigoid, acute mucopurulent conjunctivitis, and Chronic Suppurative Otitis Media (CSOM). She was treated with antibiotics and other supportive measures for two weeks, but without any relief. Since the lesions recurred, the patient sought further evaluation at the Outpatient Department (OPD).

On examination, the patient had open pustules that were forming oozing erosions in both the groin area. Redness and crusted plaques were observed on the lower left eyelid, and oozing from both conchal bowls was noted. Oral mucosal erosions, nasal erosions, and lip erosions with crusting were also observed (Table/Fig 1)a-e. A culture swab taken from the groin area tested positive for Staphylococcus aureus. Lab investigations revealed pancytopenia, elevated Erythrocyte Sedimentation Rate (ESR) and C-Reactive Protein (CRP), low C3 and C4 levels, normal renal and liver function tests, normal 24-hour urine protein, and spot Polymerase Chain Reaction (PCR). Antinuclear Antibodies (ANA) (1:100 titre) and dsDNA showed positivity.

Since the eczematous lesions resembled late-onset acrodermatitis enteropathica in adults, the patient’s serum zinc levels were measured, which were found to be low-49 μg/dL [Normal range: 52-286 μg/dL]. A skin biopsy was performed from the pustules in the inguinal region, which showed psoriasiform acanthosis and spongiotic neutrophil collections in the papillary dermis, leading to a diagnosis of APF. Based on the major and minor criteria, including pustules in the inguinal folds and conchal bowls, intraepidermal spongiotic pustules, and positive ANA and dsDNA, a final diagnosis of cutaneous lupus erythematosus with amicrobial pustulosis of the folds was made.

The patient was treated with Mycophenolate mofetil 500 mg BD and Dexamethasone 8 mg OD for a period of 4-6 weeks until the last pustule of APF resolved. Then, the treatment was slowly tapered to Prednisolone 5 mg every two weeks. Currently, the patient is in remission with Prednisolone 20 mg OD and Mycophenolate mofetil 500 mg BD as a steroid-sparing agent. The patient has been under follow-up for the past six months and has shown no new lesions. During the follow-up period, the patient’s lab parameters remained within normal range.

Case 2

A 31-year-old female presented with chief complaints of persistent lip erosions, photosensitivity, fever, and arthralgia lasting for four months. There was no significant medical or family history. Dermatological examination revealed a malar rash and coalesced pustules with discharge from both conchal bowls (Table/Fig 2)a-c. Swabs taken from unopened pustules from the concha were negative for any organisms. Lab investigations showed pancytopenia and elevated inflammatory markers (ESR, CRP). The ANA level was 6.6, and dsDNA was positive. Renal function tests revealed high urea creatinine, and C3 and C4 levels were below normal. Skin biopsy showed vacuolar degeneration in the epidermis with inflammatory infiltrates in the papillary dermis, consistent with the diagnosis of lupus erythematosus. Additionally, renal biopsy revealed Class II Mesangioproliferative glomerulonephritis.

Based on the presence of bacteriologically sterile pustules involving minor skin folds, and positive ANA, dsDNA, and associated SLE, a final diagnosis of SLE with lupus nephritis and APF was made. The patient was treated with Inj. Dexamethasone 8 mg OD and Tablet Mycophenolate mofetil 500 mg BD for five weeks. Subsequently, the patient was maintained on Tablet Prednisolone 30 mg OD and Tablet Mycophenolate mofetil 500 mg BD for eight months. All mucocutaneous lesions and pustules of APF resolved after eight months.

Case 3

A 35-year-old female, known to have SLE with Class V Lupus nephritis, presented to the Dermatology OPD with swelling of the face and extremities, as well as pus along both her popliteal folds and oral mucosal erosions that developed within 24-48 hours. The patient had been prescribed tablet prednisolone 20 mg per day but had stopped taking it for one month. Examination revealed anasarca, facial edema with redness, and pustules that had coalesced into sheets on both her lower limbs, extending from the popliteal fossa to the ankle. Oral mucosal erosions were seen in her buccal mucosa and palate (Table/Fig 3)a,b. A swab taken from a discrete pustule was negative for organisms. Blood investigations showed a positive ANA with a titre of 1:160, positive dsDNA, and elevated ESR and CRP. A diagnosis of amicrobial pustulosis of folds was made.

During this disease flare-up, the patient was treated with Inj. Dexamethasone 8 mg OD and Tablet Mycophenolate mofetil 500 mg BD for 4-6 weeks. Over the course of the eight-month follow-up period, the pustules resolved and the disease activity gradually decreased.

Case 4

A 24-year-old female presented with chief complaints of fever, arthralgia, facial puffiness, tenderness, and swelling in the right breast, as well as tense fluid-filled blisters over the trunk for a duration of four months. She also reported a history of tiny pustules in both her inguinal folds for two months. The patient had been previously treated as a case of Rheumatoid arthritis with Hydroxychloroquine, Sulfasalazine, and Ivabradine for the past one year. Two months ago, she had undergone incision and drainage for right breast swelling.

On examination, tiny pustules were observed in both inguinal folds, along with vesicles on the abdomen and erosions on the palate (Table/Fig 4)a-d. Cultures from the inguinal pustules showed no bacterial growth. Further screening for autoimmune disorders revealed a speckled pattern of ANA, positive dsDNA, and chest X-ray findings consistent with lupus pneumonitis. Histopathology revealed subcorneal spongiotic pustules and intraepidermal neutrophil-rich infiltrates, suggestive of APFs. Given that the patient met two major and two minor criteria, a diagnosis of SLE with APF and Lupus pneumonitis was made.

Treatment was initiated with Inj. Dexamethasone 8 mg for four weeks, which was gradually tapered to oral prednisolone 20 mg per day. During a six-month follow-up, the patient’s condition resolved, and no new lesions were observed.

Case 5

A 41-year-old female presented to the OPD with complaints of lip erosions, painful oral ulcers, redness of the eyes, vaginal erosions, and pustular lesions in her groin, gluteal cleft, and both axilla for a duration of four months. There was no significant medical or family history, and the patient denied any history of drug intake.

On examination, multiple pustules were noted in her inguinal folds, axilla, and gluteal folds. The oral mucosa showed erosions on the lip, buccal mucosa, and palatal ulcers with an erythematous floor. Conjunctival congestion was observed in both eyes (Table/Fig 5)a-d. Swabs and cultures from the axillary pustules showed no organisms. The patient’s ANA and dsDNA were negative. Biopsy of the pustules in the groin revealed a subcorneal pustule containing neutrophils and red blood cells, along with capillary dilation and collections of neutrophils and mononuclear cells in the papillary dermis. These findings were suggestive of amicrobial pustulosis of the folds. Direct immunofluorescence of perilesional skin was negative for immunoreactants. Biopsy of the buccal mucosa revealed a lesion consistent with mucosal pemphigus vulgaris. Therefore, a final diagnosis of mucosal pemphigus vulgaris and APF was made. This documented a new case of autoimmune disorder association between amicrobial pustulosis of the folds and mucosal pemphigus vulgaris, which was the first of its kind.

Treatment was initiated with Inj. Dexamethasone 8 mg once daily and tablet Cyclophosphamide 100 mg once daily as a steroid-sparing agent for five weeks. The patient achieved remission and was maintained on tablet Prednisolone 20 mg OD and tablet Cyclophosphamide 100 mg OD for the past four months. There have been no new pustules, and the mucosal erosions have healed at the four-month follow-up (Table/Fig 6) shows the profiles of all the patients included in the case series.


The condition known as APFs is also referred to as ‘pustular dermatosis,’ ‘follicular impetigo,’ ‘pyodermatitis vegetans,’ and ‘amicrobial pustulosis associated with autoimmune diseases’ (APF). It is a neutrophilic dermatosis caused by autoimmune dysregulation.

To date, a total of 78 cases of APF have been reported in the literature, with a female preponderance of nearly 90% (4),(5),(6),(7),(8). This case series also reports five cases of APF, all of whom were females.

Similar to the findings reported by Wang MZ et al., all the cases in this series presented with involvement of the major and minor folds, as well as the genital area, with erythematous plaques, erosions, and coalesced pustules in the conchal bowls and popliteal fossa (9). As mentioned by Márquez Balbás G et al., cultures for bacteria and fungi collected from unopened pustules before antibiotic treatment were either negative or positive for Staphylococcus aureus (10). Histopathology showing subcorneal pustules with neutrophil-rich dermal infiltrates was a common finding in most of the cases.

In previously reported cases, associations were found with conditions such as Lupus erythematosus, Psoriasis, Autoimmune thyroiditis, Sjogren’s syndrome, Crohn’s disease (11), and Rheumatoid arthritis (12). In the present case series, an association with Lupus erythematosus was established in four of the cases, and one case was associated with mucosal pemphigus, which is the first of its kind.

Although the exact pathogenesis of amicrobial pustulosis of the folds is unclear, possible explanations for its occurrence could be high levels of neutrophils, including low-density granulocytes seen in SLE and other autoimmune disorders. These abnormal neutrophils have an increased capacity to synthesise Neutrophil Extracellular Traps (NETs), and the aberrant and/or excessive formation of NETs seems to play a role in the development and perpetuation of autoimmune disorders (13). The affected skin and kidneys are infiltrated with the netting neutrophils. Additionally, the antimicrobial components exposed by NETosis can serve as an immunostimulatory signal that facilitates the recognition of self dsDNA. Activated neutrophils in amicrobial pustulosis of the folds influence damaged organs like the kidneys to increase the uptake of dsDNA. This mechanism ultimately results in organ damage in patients with SLE and other autoimmune disorders.

It has been noted that zinc restriction can lead to reduced autoantibodies, anti-dsDNA titres, and reduced lymphoproliferation (14),(15). Therefore, zinc supplements are not recommended for patients with SLE. Additionally, matrix metalloproteinase, a zinc-containing proteinase that plays a role in remodelling the extracellular matrix, has been reported to be related to SLE activity and Lupus nephritis due to its high levels (16),(17).

In young females with a prior history of Connective Tissue Disorder (CTD) or individuals with new symptom onset and pustules in the intertriginous areas, skin folds, and conchal bowls, APF should be considered as a differential diagnosis. In the present cases, the clinical and morphological picture raised a high degree of suspicion, which was supported by investigations such as high ANA, ESR, CRP, positive dsDNA, neutrophil-rich cellular infiltrate in histopathology, and negative culture for microorganisms. All of these findings were compatible with the criteria for APF (Table/Fig 7) shows the approach to amicrobial pustulosis of the folds. The most effective treatment for managing APF is still systemic corticosteroids. Systemic antibiotics are not effective in treating this disease unless there is secondary impetiginization of the lesions.

New therapeutic alternatives are emerging, such as anakinra and anti-TNF-alpha drugs (18). Doxycycline has been tried for its anti-inflammatory action. Other drugs that have been tried include cimetidine and ascorbic acid (19). A good clinical response to hydroxychloroquine (400 mg/d) in combination with prednisone (0.5 mg/kg/d) has also been described in a patient who failed to tolerate treatment with oral sulfone (2). Other treatments include zinc (20), cyclosporine, methotrexate, levamisole, and colchicine (21), although the results are variable.

Given its rarity, a high degree of suspicion is necessary to diagnose amicrobial pustulosis of the folds. This can lead to the identification and prompt treatment of the underlying autoimmune condition.


The presence of neutrophilic leukocytosis is a clue that patients may have amicrobial pustulosis of the folds. Cutaneous lupus erythematosus with histopathological features of a neutrophil-rich inflammatory infiltrate in the perivascular and perifollicular areas is a strong predictor of cutaneous lupus erythematosus progressing to SLE, thus acting as a prognostic indicator. APF can serve as a marker of disease activity or organ involvement, and dsDNA is the most common antigen among ENA associated with APF. Due to the frequent association of APF with lupus erythematosus and other immunologic disorders, all cases of APF need to be closely monitored with the aim of making an early diagnosis of any autoimmune disorder at the time of presentation or in the future.


The authors are extremely thankful and express their sincere gratitude to Dr. A Ramesh, Professor, Department of Dermatology, for assisting with the histopathological diagnosis of a patient with APF.


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DOI and Others

DOI: 10.7860/JCDR/2023/61466.18386

Date of Submission: Nov 14, 2022
Date of Peer Review: Feb 04, 2023
Date of Acceptance: Jun 05, 2023
Date of Publishing: Sep 01, 2023

• Financial or Other Competing Interests: None
• Was informed consent obtained from the subjects involved in the study? Yes
• For any images presented appropriate consent has been obtained from the subjects. Yes

• Plagiarism X-checker: Nov 29, 2023
• Manual Googling: May 13, 2023
• iThenticate Software: Jun 03, 2023 (9%)

ETYMOLOGY: Author Origin


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