Pseudoxanthoma elasticum veiled as vasculitis: shedding light on an uncommon disorder and an in-depth review of the literature.

Pseudoxanthoma Elasticum (PXE) is a rare genetic disorder caused by an autosomal recessive mutation in the ABCC6 gene. It manifests with distinctive clinical symptoms impacting the skin, eyes, and cardiovascular system, along with an elevated risk of cardiovascular diseases. We present a case of a 34-year-old male patient who was initially referred to the rheumatology clinic for evaluation due to suspected large vessel vasculitis. The patient's primary complaint was severe hemifacial pain radiating to the neck and upper limb. Radiological imaging studies unveiled substantial vascular narrowing and collateral vessel formation, prompting further investigation to exclude systemic vasculitis. Intriguingly, the patient also exhibited cutaneous manifestations, which were later confirmed via skin biopsy as consistent with PXE. An ophthalmological examination further revealed the presence of the classic PXE findings of angioid streaks. Given the rarity of PXE and its multifaceted clinical presentation, it can be particularly challenging to diagnose and manage. As such, cases like the one presented here may necessitate a referral to a rheumatologist for evaluation of potential systemic involvement. To provide a comprehensive perspective on PXE, we conducted a systematic review of case reports published in the past decade in English, collected from PubMed, Scopus, and the Directory of Open Access databases. The analysis of these cases will be discussed to shed light on the diversity of PXE's clinical features and the diagnostic and management dilemmas it poses and to facilitate ongoing exploration and research into this intricate condition, ultimately leading to improved care for individuals affected by PXE.

Diplopia and Vision Loss Associated With Presumed Systemic Lymphohistiocytic Disease: A Case Report.

Systemic lymphohistiocytic infiltration is a rare peculiar condition that can raise the possibility of more specific entities such as autoimmune disease, drug interaction, viral or mycobacterial infection, or malignancy. A hyper-inflammatory state can ensue leading to multi-organ failure. We report the case of a 42-year-old Jordanian male with a past history of moderate Covid-19 infection presenting with binocular diplopia and acute loss of vision in the left eye. Ophthalmic evaluation revealed limitation of extraocular motility in all directions of gaze in the left eye and a visual acuity of 6/30 with a sluggish pupil. Orbital imaging revealed a 10 mm mass at the orbital apex suspicious of malignant metastasis. A positron emission tomography CT scan showed significant pleural thickening and was highly suggestive of metastatic mesothelioma seeding to the orbit, liver, and bone. A CT guided biopsy of the right lung was negative for malignancy but had features of lymphohistiocytic pleuritis. The patient dramatically succumbed to respiratory and renal failure. Systemic lymphohistiocytic infiltration is an aggressive benign inflammatory process that may masquerade as malignancy and raise the possibility of past viral infections, autoimmune diseases, or cancer. A high index of suspicion and a multidisciplinary approach is warranted. In this particular devastating instance, a diagnostic dilemma presented to the eye clinic with diplopia, loss of vision, and an orbital mass, culminating in rapid onset respiratory and renal failure and death.

Immunohistochemical Expression Patterns of CD45RO, p105/p50, JAK3, TOX, and IL-17 in Early-Stage Mycosis Fungoides.

The morphologic changes in early-stage mycosis fungoides (MF) might overlap with benign inflammatory dermatitis (BID). Previous studies have described altered expression patterns of several proteins in MF, but their diagnostic significance is uncertain. This study aims at examining the frequency of expression of CD45RO, NFkB-p105/p50, JAK3, TOX, and IL-17 proteins by immunohistochemistry. The cohorts included 21 patients of early-stage MF and 19 with benign BID as a control group. CD45RO was positive in all patients of MF and BID. NFkB-p105/p50 showed normal cytoplasmic staining, indicating an inactive status in all patients of both groups. JAK3 was positive in 3 (14%) MF and in 17 (89%) BID patients (p = 0.003). TOX was expressed in 19 (90%) and 13 (68%) patients of MF and BID, respectively (p = 0.120). IL-17 was detected in 13 (62%) MF and in 7 (37%) BID patients (p = 0.056). Co-expression of TOX and IL-17 was seen in 11 (52%) MF patients but in only 3 (16%) BID patients, which was statistically significant (p = 0.021). We conclude that a double expression of TOX and IL-17 may support the diagnosis of MF in the right clinicopathologic setting, while none of the immunohistochemical stains alone provided a significant discrimination between MF and BID.

Subtotal hysterectomy reviewed: a stable or aperture for stump cervical malignancy. A referral hospital experience.

Introduction: To review the malignant potential of the stump after subtotal abdominal hysterectomy. Material and methods: Thirty-three patients with stump malignancy were diagnosed and treated between January 2018 and January 2022. All patients primarily underwent subtotal hysterectomy (STH) outside our hospital due to different indications, most of which seemed non-convincing. Upon presentation, they were evaluated properly and offered the best management plan. Results: The presenting symptoms were abnormal histopathology report in 8 patients (24.24%), abnormal bleeding in 7 patients (21.21%), and postcoital bleeding and abnormal Pap smear in 6 patients (18.18%). The primary site of malignancy was endometrial in 17 patients (51.51%), on top of fibroid in 6 patients (18.18%), and cervical in 5 patients (15.15%). Eighteen patients (54.54%) underwent proper surgery, 9 patients (27.277%) were referred for chemoradiation, and 6 patients (18.18%) were candidates for palliative therapy. Conclusions: Stump cancer cases show a worse stage silhouette compared with cancer cases in intact uteruses. The high prevalence of cervical stump problems should be taken into account before a change in surgical approach from total to STH is deemed possible. Further prospective studies with prolonged follow-up periods are needed to evaluate the risks and benefits of retaining the cervix at hysterectomy. Subtotal hysterectomy is easier, does not require distinct skills that lead to experience and follow-up, and must be limited to the narrowest limits of practice, provided that the woman knows that there are no health benefits to keeping the cervix in place.

Attitude towards HPV Vaccination and the Intention to Get Vaccinated among Female University Students in Health Schools in Jordan.

Cervical cancer is a leading cause of morbidity and mortality in women worldwide. The availability of prophylactic vaccines for high-risk types of human papillomavirus (HPV) infection represents an important advancement in the prevention of cervical cancer. In Jordan, the availability of the HPV vaccination is restricted to individuals who are willing to pay. The aim of the current study was to evaluate the willingness and attitude of female university students in health schools/faculties in Jordan to get HPV vaccination and their knowledge about the virus. A self-administered online questionnaire was distributed in October 2021, which comprised 27 items to evaluate HPV knowledge, history of HPV vaccination, intentions to get the HPV vaccine, and the reason(s) behind vaccine refusal for those who rejected vaccination. The study sample comprised 836 participants: medical students (39.7%), pharmacy students (26.0%), dental students (21.2%), and nursing students (13.2%). Only 524 participants had heard of HPV prior to the study (62.7%), of which 48.7% knew about the availability of HPV vaccines. The lowest level of HPV knowledge was observed among nursing students. Only 19/524 students reported a history of HPV vaccination (3.6%). The overall willingness to receive HPV vaccination if provided freely was 75.0%, while only 16.0% were willing to pay for the vaccine. The most common reason for HPV vaccine rejection was the perceived low risk to get HPV infection. Significantly higher intentions to get HPV vaccination were found among older participants and medical students. The embrace of vaccine conspiracy beliefs was associated with a significantly less willingness to get the HPV vaccination (p < 0.001). Dependence on the internet/social media as the source of HPV knowledge was associated with a significantly lower intention to get HPV vaccination (p = 0.002). The coverage of the HPV vaccination among female university students in health schools in Jordan appeared extremely low; however, three-fourths of the students who had heard of HPV were willing to receive the HPV vaccination if provided freely. Complacency appeared as a major factor for HPV vaccine rejection. Increasing the levels of knowledge and awareness of HPV infection and its association with cervical cancer through reliable sources is recommended. This can be helpful for the individual benefit of the students besides the potentially positive role they can play in community education. Countering vaccine conspiracy beliefs with proper education and awareness programs can be helpful to appraise the role of HPV vaccines in cancer prevention.

The Girl With the Bleeding Earlobe Mass.

ABSTRACT: Pilomatrixomas, also known as epithelioma calcificans, are benign tumors of hair follicle matrix cells that are often mistaken for other lesions, especially cutaneous abscesses. We report an illustrative case in which a teenage girl developed a red, swollen earlobe that required multiple care visits and interventions until definitive diagnosis and treatment were provided. Although the lesion was initially treated as an abscess, it continued to progress in size and discomfort. The correct diagnosis was established after imaging and complete excision with pathologic examination. Ultimately, our patient was subjected to avoidable procedures that carried the risk of potentially negative cosmetic sequelae before the proper intervention. Although abscesses are common, it is important for clinicians to avoid incision and drainage of lesions, unless the diagnosis is certain.

Positive MITF and NKI/C3 Expression in Cellular Neurothekeoma and Dermatofibroma.

BACKGROUND: Cellular neurothekeoma (CNT) is a benign mesenchymal tumor with uncertain cellular differentiation. Studies have found evidence of myofibroblastic differentiation and possible relation to dermatofibromas (DFs). As microphthalmia transcription factor (MITF) and NKI/C3 stains are routinely positive in CNT, we compared expression patterns of both markers in CNT and DF to assess their relationship. MATERIALS AND METHODS: We assessed cases of CNT (n=25) and DFs (n=35) for histopathologic characteristics and MITF and NKI/C3 expression. Immunostaining results were classified as negative, focally positive (<50%), and diffusely positive (>50%). At least 1 additional melanocytic marker was assessed in each case of CNT. RESULTS: Both DFs and CNTs showed a female predilection and a wide age range. Immunostaining in CNTs for MITF was positive in the vast majority (focal 68%, diffuse 24%), as was NKI/C3 (focal 72%, diffuse 24%). All DFs were MITF positive (diffuse 74%, focal 26%), and most DFs were NKI/C3 positive (focal 57%, diffuse 3%). CONCLUSION: CNT and DF share demographic, histopathologic, and immunohistochemical features, including shared expression of MITF and NKI/C3, especially cellular DF.

Sclerosing Dermatofibrosarcoma Protuberans Shows Significant Overlap With Sclerotic Fibroma in Both Routine and Immunohistochemical Analysis: A Potential Diagnostic Pitfall.

Dermatofibrosarcoma protuberans (DFSP) is an uncommon, low-to-intermediate grade sarcoma with several histologic variants, including pigmented (Bednar tumor), sclerosing, myxoid, atrophic, and DFSP with fibrosarcomatous changes. Two patterns of sclerosis in DFSP can be observed, a sclerotic fibroma-like pattern and a morphea/lichen sclerosus-like pattern. Partial biopsies of sclerosing DFSPs with the sclerotic fibroma pattern can be misdiagnosed as sclerotic fibroma or other benign sclerosing tumors (eg, perineurioma, dermatofibroma). DFSPs from our tissue archives were screened for tumors with a sclerosing pattern, and then studied with epithelial membrane antigen (EMA), CD34, and elastic tissue staining to investigate whether such stains can differentiate sclerosing DFSP from sclerotic fibroma. Ten cases of sclerotic fibroma were similarly studied. Two of the 27 DFSPs were predominantly sclerosing and 5 additional DFSPs had a mixed histopathologic pattern including a sclerosing component. Immunohistochemically, all DFSPs with sclerosing (predominant or mixed) pattern were positive for CD34, and 5/7 were at least focally positive for EMA. Elastic tissue staining was reduced or absent in the sclerotic areas. All cases of sclerotic fibroma were either positive or focally positive for CD34, whereas EMA was focally positive in 5/10. Elastic tissue staining ranged from reduced to totally absent in the sclerotic fibromas. In conclusion, the similar histopathologic and immunophenotypic characteristics in sclerotic fibroma and sclerosing DFSP found in this analysis highlight the importance of obtaining clinical information and potentially additional excision for partial biopsies showing a sclerotic fibroma-like pattern.

Multiple calcifying pseudoneoplasms of the neuraxis (MCAPNON): Distinct entity, CAPNON variant, or old neurocysticercosis?

We report a case of multiple calcifying pseudoneoplasms of the neuraxis (MCAPNON) with associated multifocal perivascular microcalcifications and vascular calcinosis. Calcifying pseudoneoplasm of the neuraxis (CAPNON) is a very rare condition that may arise in extra-axial and occasionally, in intra-axial locations. Moreover, it is nearly always a solitary mass with only one case with two lesions reported. While the etiology and pathogenesis of CAPNON remains unclear, the histopathology findings of this entity have been well described. We report a case of a 62-year-old woman with 18 calcifying radiologic lesions involving bilateral cerebral hemispheres. Histologically, these lesions have features similar to that reported for CAPNON, including nodular calcification with fibro-osseous components and peripheral histiocytic reaction. The patient had a poorly documented diagnosis of neurocyticercosis 32 years prior, although without tissue confirmation. The lack of detectable cysticercus serum antibody titers, and absence of residual larval or cyst wall tissue render multifocal calcific involution of that parasite unprovable although still plausible. We also raise the possibility of a blood-brain barrier derangement and/or a metabolic disorder as an alternative etiology. Whether this case of MCAPNON shares the same pathogenesis as the usual solitary CAPNON is unclear.