Treatment-Resistant Immunoproliferative Small Intestinal Disease (IPSID) Leading to Lymphoma.

Immunoproliferative small intestinal disease (IPSID) is a distinct variant of mucosa-associated lymphoid tissue (MALT) lymphoma, often linked to chronic Campylobacter jejuni infection. Characterized as an extra-nodal marginal zone B-cell lymphoma, IPSID predominantly affects the proximal small intestine. It features lymphoplasmacytic infiltration and deposition of monotypic α-heavy chains in the lamina propria, leading to blunted intestinal villi, malabsorption, and protein-losing enteropathy. IPSID's clinical spectrum ranges from lymphoid infiltration to malignant diffuse large B-cell lymphoma. Similar to MALT lymphoma, early-stage IPSID can be resolved with antibiotic therapy. This case study documents a 50-year-old Nigerian woman presenting with recurrent watery diarrhea, abdominal pain, and weight loss, unresponsive to antibiotics. A 50-year-old female immigrant from Nigeria presented with recurrent watery diarrhea, abdominal pain, and significant weight loss, all refractory to antibiotic treatment. Initial diagnostic investigations revealed a positive Campylobacter stool antigen, mesenteric lymphadenopathy on CT and gallium scans, and diffuse mucosal lymphoplasmacytic infiltration with villi flattening on small bowel biopsies. An octreotide scan identified a reactive mesenteric lymph node, confirmed by surgical biopsy as reactive lymphadenitis. The patient was diagnosed with IPSID and commenced antibiotic therapy, which initially resolved her symptoms. However, she experienced frequent recurrences requiring multiple hospitalizations and repeated courses of intravenous antibiotics. Eventually, the disease progressed to lymphoma, necessitating chemotherapy initiation. This case underscores the diagnostic complexities of IPSID, particularly in distinguishing it from other causes of mesenteric lymphadenopathy. It also highlights the challenges in preventing disease progression from a benign to a malignant state despite appropriate antibiotic treatment. Given IPSID's prevalence in endemic regions, it should be considered in differential diagnoses for similar presentations. Continuous monitoring is crucial to evaluate therapeutic response and mitigate the risk of progression to lymphoma. IPSID presents a significant diagnostic and therapeutic challenge. This case exemplifies the necessity for heightened clinical awareness, especially in patients from endemic regions, and the importance of rigorous monitoring to prevent malignant transformation. Further research is warranted to elucidate the mechanisms behind IPSID progression in certain patients despite repeated antibiotic interventions.

Kaposi's sarcoma: epidemiologic aspects, the immune reconstitution inflammatory syndrome, and more along the Silk Road of cognition.

Kaposi's sarcoma remains enignmatic with many clinical and epidemiological patterns. We review them and describe the groove sign, an important association worthy of recognition. We also stress Kaposi's sarcoma (KS) without coexistent human immunodefiency virus infection, with recent data from China describing an extraordinarily high classical KS prevalence rate among Uygurs and Kazaks in the Xinjiang Uygur Autonomous Region in northwestern China, presumably derived from elderly men residing there. The possible travel of HHV-8 along the ancient silk road from Italy to the Xinjiang Uyghur region remains intriguing. If only one in 10,000 HHV-8-infected patients develops classical KS worldwide, then triggers for its overrepresentation in this population within China are of particular concern. The KS-related immune reconstitution inflammatory syndrome is also emphasized.

Levamisole-induced and COVID-19-induced retiform purpura: two overlapping, emerging clinical syndromes.

Levamisole exposure in cocaine users is a well-recognized cause of retiform purpura, a distinctive net-like maculopapular patch. Prolonged exposure to levamisole can lead to a serious systemic syndrome known as levamisole-induced vasculitis, most commonly involving the kidneys and lungs. More recently, retiform purpura has been observed in patients with the novel coronavirus disease of 2019 (COVID-19). Due to their overlapping dermatologic and systemic manifestations, levamisole-induced and COVID-19-induced retiform purpura may mimic one another in clinical presentation. The possibility that patients may present with one or both syndromes creates a diagnostic challenge. This review of levamisole-induced and COVID-19-induced retiform purpura highlights their corresponding and distinctive features. Additionally, we propose a unique staging system for levamisole-induced retiform purpura that may be valid for future classification of COVID-19-induced retiform purpura.

Malignant syphilis: a new and revised definition.

Malignant syphilis is a rare, severe variant of secondary syphilis that primarily affects immunocompromised patients. Historically, the clinical presentation included a prodrome of constitutional symptoms followed by the development of characteristic skin lesions. The definition of malignant syphilis has not been updated since it was first described as only a cutaneous manifestation. In recent years, syphilis has reemerged as a major public health issue, particularly in men who have sex with men (MSM) and patients with HIV. With increasing rates of syphilis in the HIV-positive population, the systemic manifestations of malignant syphilis have become more apparent. We propose a revised definition and elucidate why the term malignant syphilis should be expanded to include systemic manifestations, stressing musculoskeletal, central nervous system, ocular, ear, cardiovascular, rectal, liver, lung, and renal involvement. This updated definition is dramatically expanded to reflect its systemic manifestations. Recognition of these manifestations may prevent devastating long-term effects.

Leprosy: what is new.

Leprosy, also known as Hansen's disease, is an age-old chronic granulomatous infection characterized by prominent cutaneous and neurologic findings. Long known to be caused by Mycobacterium leprae, a new etiologic species was identified and linked in 2008, Mycobacterium lepromatosis. The BCG vaccine with highly variable efficacy may soon be replaced by the first leprosy-specific subunit vaccine LepVax, which has recently moved forward in human trials. Recent evidence supporting theories of zoonotic transmission from armadillos and the less-discussed Eurasian red squirrels has emerged. Knowledge on genetic polymorphisms that may increase leprosy susceptibility, such as the newly uncovered mitochondrial ribosomal protein S5 (MRPS5) polymorphism in the Chinese population, has provided a fresh perspective and direction. Further, we will delineate the latest information on leprosy, including the possible effects of leprosy coinfection with COVID-19, HIV, and HTLV-1, and the shift to newer leprosy therapies and treatment regimens.

Public health issues with Candida auris in COVID-19 patients.

The ongoing pandemic of coronavirus disease 2019 (COVID-19) has overwhelmed a number of medical facilities as well as a few entire health-care systems. A novel issue co-incident with the expeditious deployment of specialty care units for COVID-19 inpatients is the worldwide epidemic of Candida auris infections. Since its first identification and classification in Japan in 2009, it has spread globally. This threat was predicted as C. auris has a high mortality rate, cryptic fomite spread, frequent misidentification since conventional methods do not detect it, and multidrug-resistance. Since the April 2020 warning at the start of the COVID-19 pandemic in the United States, C. auris has been delineated as an increasingly consequential source of significant nosocomial infections, emphasizing the added hazard of C. auris to COVID-19 inpatients, particularly those in intensive care units.

Recognition and treatment of devastating vasculopathic systemic disorders: Coronavirus disease 2019 and rickettsioses.

Cutaneous involvement can be an important sign of both COVID-19 and rickettsioses. Rickettsial infections may be first evident as an exanthem with eschars as a key finding. In contrast, eschars and necrotic lesions can be seen in critically ill COVID-19 patients. Both illnesses share a similar mechanism of infecting endothelial cells resulting in vasculopathy. Rickettsia parkeri and Rickettsia 364D are both characterized by eschars unlike Rickettsia rickettsii. Other eschar causing rickettsioses such as Rickettsia conorii, Rickettsia africae, and Orientia tsutsugamushi are commonly diagnosed in people from or having traveled through endemic areas. While there is no consensus on treatment for COVID-19, rickettsioses are treatable. Due to possibly serious consequences of delayed treatment, doxycycline should be administered given an eschar-presenting patient's travel history and sufficient suspicion of vector exposure. The proliferation of COVID-19 cases has rendered it critical to differentiate between the two, both of which may have overlapping vasculopathic cutaneous findings. We review these diseases, emphasizing the importance of cutaneous involvement, while also discussing possible therapeutic interventions.

Pandemics throughout the centuries.

Pandemics have ravished the globe periodically, often associated with war, at times commencing as fever and rash, beginning in recorded history in the crowded walled city of Athens during the Peloponnesian War as described in great detail by the Athenian historian and military general Thucydides in 430 BCE. As the world now faces the first major pandemic of the 21st century, we focus on the "plague" commencing in Athens in 430 BCE and the 2 pandemics of the more recent century, which killed more than one million, the Spanish flu of 1918 and the Asian flu of 1957. The latter linked with successful vaccine development thanks to the heroic efforts of microbiologist Maurice Hilleman. We now look back and then forward to the viral infection coronavirus disease 2019 now devastating the world.

Neurosyphilis and the Jarisch-Herxheimer reaction: A therapy concern with HIV disease.

Jarisch-Herxheimer reaction (JHR) should be anticipated in treating neurosyphilis with coexistent human immunodeficiency virus (HIV) encephalitis. In that context we have devised a staging classification for JHR. In addition, an illustrative case is provided to emphasize the need to consider the diagnosis of neurosyphilis in HIV patients, and if delineated, to be prepared for a severe JHR.

Puffy Foot Syndrome: An Important Often Overlooked Clinical Entity.

The puffy foot syndrome, a novel clinical entity, describes the complication of secondary lymphedema with chronic progression in the feet, a finding that has often been overlooked in the non-tropical setting. While previously well described in the upper extremities, this complication has not been fully explored in the lower extremities. However, given increasing rates of diabetes mellitus, obesity, and a myriad of other possible etiologies in the United States, it is important to understand this entity and its non-viral, non-parasitic causes in non-tropical regions. This review delineates common illustrative properties of this syndrome observed in clinical practice as well as long-term complications, including Ruocco's immunocompromised cutaneous district, that are often overlooked. Furthermore, a novel method of staging is suggested for this condition, reflective of increasing risk of complication, infection, and malignancy. We also highlight the increased need for improved detection and recognition of this condition to avoid possibly deleterious outcomes.

The Botfly, A Tropical Menace: A Distinctive Myiasis Caused by Dermatobia hominis.

Dermatobia hominis, also known as the human botfly, is native to tropical and subtropical Central and South America and seen in travelers from endemic to temperate regions including the United States and Europe. Cutaneous infestation botfly myiasis involves the development of D. hominis larvae in the skin and is common in tropical locations. The distinct appearance of a cutaneous D. hominis infestation facilitates early diagnosis and intervention where cases are common. However, the identification of D. hominis in temperate regions may prove challenging due to its rarity. D. hominis may be misdiagnosed as folliculitis, an epidermal cyst, or an embedded foreign object with secondary impetigo. One should have a heightened suspicion in someone returning from a vacation in an endemic area, such as Belize. Here we describe the presentation, differential diagnosis, and treatment and encourage enhanced preventative measures among tourists when visiting tropical and subtropical regions. Additionally, we propose a novel classification system for assessing the various stages of infestation and suggest that patients reporting travel to Latin America and experiencing pain disproportionate to an insect bite should lead physicians to consider myiasis caused by D. hominis.

Aggregatibacter actinomycetemcomitans Causing Empyema Necessitans and Pyomyositis in an Immunocompetent Patient.

Empyema necessitans is a relatively rare clinical entity in which the empyema extends through the parietal pleura into the adjacent soft tissue and musculature of the chest wall. It usually occurs due to inadequate treatment of a primary lung infection. Aggregatibacter (formerly Actinobacillus) actinomycetemcomitans is a facultative anaerobic gram-negative coccobacillus that is part of the normal oral flora. Infections due to this organism usually result from aspiration in conjunction with dental disease or trauma to the oral mucosa resulting in pneumonia or empyema. It often coinfects with Actinomyces and is known to cause empyema necessitans. Cases of monomicrobial empyema necessitans due to Aggregatibacter actinomycetemcomitans in adults have rarely been reported with four such publications found on review of the literature. We present a patient with severe periodontitis who developed empyema necessitans due to Aggregatibacter actinomycetemcomitans likely from aspiration complicated by pyomyositis of the right triceps brachii and a left posterior thigh abscess.

Crohn's disease or histoplasmosis? A case of severe disseminated histoplasmosis mimicking Crohn's disease and literature review.

Disseminated histoplasmosis (DH) often mimics other diseases, leading to misdiagnosis and delays in treatment. We present a patient who developed DH after treatment with immunosuppressants for an initial diagnosis of inflammatory bowel disease (IBD). Upon diagnosing her with DH, liposomal amphotericin B was started, and she eventually recovered after a prolonged hospitalization. Intrabdominal histoplasmosis has many similarities with IBD. Treatment with immunosuppressants in undiagnosed histoplasmosis can lead to dissemination with potentially catastrophic results.

Cutaneous manifestations of a 21st century worldwide fungal epidemic possibly complicating the COVID-19 pandemic to jointly menace mankind.

In view of the new viral COVID-19 pandemic, the fungal Candida auris epidemic still in progress worldwide highlights non-Candida albicans candidal infections. We describe an immunocompetent woman with a cutaneous manifestation of Candida parasilopsis fungemia, a prominent eschar, which proved to be the nidus for the candidemia. We stress the value of selectively removing eschars. C. parasilopsis and C. auris are increasingly important causes of sepsis and wound infections. We emphasize that commercially available biochemical-based tests may misidentify C. auris as C. parapsilosis, and stress the added danger of C. auris to critically ill-hospitalized COVID-19 patients. Any health care facility with evidence of infection or colonization with C. auris requires very close monitoring, since this fungus is a nosocomial threat comparable to SARS-CoV-2 in its mortality and fomite adhesiveness! Both organisms have the potential to be transmitted as nosocomial pathogens; health care workers need to follow strict CDC guidelines. During this COVID-19 pandemic, every health care facility should closely monitor for the possible deadly combination of the SARS-CoV-2 and C. auris. The identification of C. auris necessitates use of sophisticated technology not readily available to make this essential diagnosis since C. auris is multi-drug resistant and isolation precautions would become paramount.

Cervicofacial actinomycosis.

Cervicofacial actinomycosis is an uncommon, chronic, suppurative, and granulomatous bacterial infection. It is often of dental origin and tends to mimic other dental infections, granulomatous disorders, and cancers. The initial diagnostic workup, predicated upon imaging and tissue biopsies, is frequently nonspecific. A definitive diagnosis is usually rendered only after surgical excision and histologic examination of the cervicofacial mass. We propose a classification of three stages: localized infection without sinus involvement, localized infection with sinus involvement, and disseminated infection, to facilitate recognition, diagnosis, and early aggressive treatment. Untreated infection may be life-threatening. Therapy may require long-term antibiotics; however, many cases may also necessitate complete surgical excision.

Chloronychia: The Goldman-Fox Syndrome - Implications for Patients and Healthcare Workers.

Nail coloration has many causes and may reflect systemic disease. White nails (leukonychia) are common; rubronychia is rare, whereas green (chloronychia) is occasionally evident. Chloronychia, the Fox-Goldman syndrome, is caused by infection of an often damaged nail plate by Pseudomonas aeruginosa. P. aeruginosa is an opportunistic pathogen known for localized and systemic infections. It can spread cryptically in a variety of ways, whether from an infected nail to a wound either autologously or to a patient as a surgical site infection, and many represent a threat to elderly, neonatal, or immunocompromised patients who are at increased risk of disseminated pseudomonas infection. We will review the Goldman-Fox syndrome as an occupational disorder of homemakers, nurses, plumbers, and others often with wet hands. At a time when hand washing is being stressed, especially in healthcare settings, examination of nails should be emphasized too, recalling the possibility of surgical site infection even with a properly washed and gloved medical care provider. Pseudomonas may be a community-acquired infection or a hospital or medical care setting-acquired one, a difference with therapeutic implications. Since healthcare workers represent a threat of nosocomial infections, possible guidelines are suggested.