Angiolymphoid Hyperplasia With Eosinophilia Research Articles

Epithelioid Hemangioma: Report of a Case Series of 4 Patients

Epithelioid hemangioma or Angiolymphoid Hyperplasia with Eosinophilia (ALHE) is a rare benign vascular disease, characterized by solitary or multiple angiomatous papules and nodules, which are usually located on the skin of scalp and face (mainly in the preauricular zone); these have characteristic histological and immunohistochemical patterns, in particular characterized by an important lymphocytic inflammatory infiltrated with many eosinophils and vascular proliferation of capillaries with endothelial cellular changes. In this paper, four cases of this disease are reported, in the first series of Ecuadorian cases described in the literature.

Epithelioid hemangioma of the acromion causing shoulder pain: a case report.

Epithelioid hemangioma of bone is a rare benign vascular tumor. Because of the locally destructive clinical presentation and the presence of atypical histologic features with increased mitotic activity and necrosis, it is often misdiagnosed as low-grade malignant epithelioid hemangioendothelioma or high-grade malignant epithelioid angiosarcoma. Correct diagnosis through imaging studies and histopathological examination is mandatory to determine the appropriate course of treatment, as the prognosis differs from that of other malignant hemangioma tumors. A 69-year-old male who presented with intractable shoulder pain caused by epithelioid hemangioma in the acromion of the scapula was treated with tumor curettage. This paper reports a good result with a review of the relevant literature.

The "great imitator": IgG4-related disease of the oral cavity. Two case reports and scoping review.

This study aimed to review the lesser-known intraoral manifestations of immunoglobulin G4-related disease (IgG4-RD). In this paper we report an unprecedented case of oral IgG4-RD mimicking angiolymphoid hyperplasia with eosinophilia (ALHE), and another case presenting as plasma cell gingivitis. We then performed a scoping review of published cases of IgG4-RD involving the oral cavity. The following data were collected for each case: age, sex, intraoral site(s) involved, clinical appearance, imaging features, serum IgG4 values, histopathology, treatment, and follow-up duration. Fifty-one cases of oral IgG4-RD were published in literature. The hard palate and jaw bones were the two main locations reported, while the histological identification of a IgG4/IgG plasma cells ratio ≥40% was fundamental for diagnosis. Conversely, the pathological features of storiform fibrosis and obliterative phlebitis were not common. Future reports regarding oral IgG4-RD should report clear adherence to the recognized international diagnostic criteria of the disease.

Immune Checkpoint Inhibition in Pediatric Oncology Patients: A Single-Institution Experience

Immunotherapy has emerged as a promising treatment approach in oncology, as it is specifically designed to boost the strength and accuracy of the immune system, allowing it to target tumor cells but spare non-tumor tissue. This treatment not only demonstrates potential for improved clinical outcomes but may also be associated with fewer adverse effects compared to traditional therapies. Despite its early success, the application of immunotherapy has largely been limited to adult cancer patients, with slow adoption noted in the treatment of pediatric cancer patients. Our objective is to demonstrate a single institution’s experience with immunotherapy in pediatric cancer patients and to discuss the use of these treatment modalities in this unique patient population. We performed a retrospective chart review and identified patients who received immune checkpoint inhibitors (ICIs) and/or underwent immunohistochemistry (IHC) testing for programmed death ligand 1 (PD-L1), quantification of tumor mutational burden (TMB), and classification of microsatellite instability (MSI) status. In total, we identified seven pediatric cancer patients who received therapy with ICIs. Four of these patients demonstrated positive PD-L1 expression, high TMB, and/or MSI-high status. These patients were treated with nivolumab alone or in combination with ipilimumab or brentuximab. The diagnoses included: multifocal epithelioid and spindle cell hemangioma (n = 1); metastatic melanoma (n = 2); histiocytic sarcoma (n = 1); rectal adenocarcinoma in the setting of constitutional mismatch repair deficiency syndrome (CMMRD) (n = 1); and Hodgkin lymphoma (n = 2). The patients received between four and nineteen cycles of immunotherapy. Immunotherapy-related adverse events included: mild allergic reaction; prodromal symptoms; anemia; neutropenia; transaminitis; endocrinopathies; and self-limiting neuritis. Of the seven patients, three are still being treated with immunotherapy (the patients with rectal adenocarcinoma, metastatic melanoma, and multifocal epithelioid and spindle cell hemangioma) with positive treatment responses observed on imaging, one is being treated with other modalities (the patient with Hodgkin lymphoma), two have achieved remission (the patients with metastatic melanoma and Hodgkin lymphoma), and one has relapsed (the patient with histiocytic sarcoma). The three patients who completed their immunotherapy regimens have been followed for 1 month, 4 months, and 10 months, respectively. This report of a single-institution experience with immunotherapy in pediatric cancer patients highlights the positive impact immunotherapy can have, especially when utilized to treat relapsed/refractory malignancies, as tumor regression or stabilization of disease burden was achieved in six of the patients described (CR = 2; PR = 4). Further research is needed to accurately identify pediatric oncology patients who could benefit from immunotherapy.

Epithelioid Hemangioma Masquerading as Traumatic Lesion: A Rare Case Report with Diagnostic Workup

Abstract Wells and Whemser first identified epithelioid hemangioma (EH) as angiolymphoid hyperplasia in 1969. It is a rare type of vascular tumor that primarily affects men in their middle ages. Typically, it appears as a single or few reddish-to-brown nodules. EHs of the oral cavity have only seldom been reported up to this point. Histologically, it is distinguished by lobular vessel configuration, tombstone-like epithelioid cells lining the vessel wall, and a persistent inflammatory infiltration predominately made up of eosinophils. Although the terms EH and epithelioid hemangioendothelioma are sometimes confused, the two are distinct based on histopathology and a number of immunohistochemistry (IHC) markers, including high Ki67 index and ERG positivity observed later. Local trauma to a nearby vessel may be one of the etiopathogeneses for this entity. This is a rare case report of EH that was identified with the use of a panel of IHC markers and hematoxylin and eosin histology.

Vascular Neoplasms With NFATC1/C2 Gene Alterations : Expanding the Clinicopathologic and Molecular Characteristics of a Distinct Entity.

Despite significant advances in their molecular pathogenesis, skeletal vascular tumors remain diagnostically challenging due to their aggressive radiologic appearance and significant morphologic overlap. Within the epithelioid category and at the benign end of the spectrum, recurrent FOS/FOSB fusions have defined most epithelioid hemangiomas, distinguishing them from epithelioid hemangioendothelioma and angiosarcoma. More recently, the presence of EWSR1/FUS :: NFATC1/2 fusions emerged as the genetic hallmark of a novel group of unusual vascular proliferations, often displaying epithelioid morphology, with alternating vasoformative and solid growth, variable atypia, reminiscent of composite hemangioendothelioma. In this study, we further our understanding and morphologic spectrum of NFATC -fusion positive vascular neoplasms by describing 9 new cases, including soft tissue locations and novel fusion partners. Combining with the initial cohort of 5 cases, a total of 14 patients were analyzed, showing slight female predilection and an age range of 10 to 66 (mean 42y). Twelve patients had solitary lesions, while 2 had multifocal polyostotic (pelvic bones) disease. Overall, 12 lesions were intra-osseous and 2 in soft tissue. By targeted RNA Fusion panels or FISH, there were 6 cases of EWSR1::NFATC1 , 4 EWSR1::NFATC2 , 2 FUS::NFATC2 , 1 EWSR1 rearrangement, and 1 with a novel FABP4::NFATC2 fusion. Follow-up was available in 4 patients. One patient experienced 2 local recurrences, 11 and 15 years postdiagnosis, and one patient experienced progressive disease despite multimodality treatment (curettings, embolization, radiation) over 3 years. In summary, our extended investigation confirms that NFATC -related fusions define a distinct group of vascular neoplasms with variable architecture, epithelioid phenotype, and cytologic atypia, commonly located in the bone, occasionally multifocal and with potential for local recurrence and aggressive behavior but no metastatic potential. Molecular analysis is recommended in diagnostically challenging cases with atypical histology to exclude malignancy.

Angiolymphoid hyperplasia with eosinophilia in the eyelid: A case series and literature review

Abstract: Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare vascular disorder characterized by the proliferation of blood vessels and lymphoid tissue. While it can occur in various regions of the body, ALHE presenting in the eyelid is relatively uncommon, leading to diagnostic and management challenges. In this case series from South India, we present three unique cases of ALHE specifically affecting the eyelids. Each case illustrates different clinical manifestations, diagnostic workups, and treatment approaches. Tissue biopsies typically reveal nodules comprising immature and mature vascular structures, including capillaries, arterioles, and venules, which are lined by plump endothelial cells with an epithelioid appearance. The surrounding stroma contains a dense infiltrate of inflammatory cells, predominantly eosinophils, histiocytes, lymphocytes, and mast cells. Increased awareness of ALHE in the eyelid will aid ophthalmologists in early recognition, accurate diagnosis, and appropriate management, resulting in improved patient outcomes.

Successful Treatment of Angiolymphoid Hyperplasia with Eosinophilia Associated with Scalp Demodicosis Using Cryotherapy and Topical Metronidazole

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare, benign, vasoproliferative tumour. We report a 25-year-old female patient who reported in 2021 to the dermatology clinic, with multiple, grouped, erythematous dome-shaped papules and nodules of 6 months duration on the left temporo-occipital region. Biopsy findings were consistent with a diagnosis of ALHE with evidence of Demodex mite infestation in the sebaceous ducts. The patient demonstrated significant improvement following 7 weeks of treatment with multiple cryotherapy sessions and topical application of metronidazole gel. This case suggests that scalp demodicosis may represent a novel trigger for the development of ALHE.
 Keywords: Angiolymphoid Hyperplasia with Eosinophilia; Mite; Infestation; Scalp; Kimura disease; Cryosurgery; Metronidazole; Case Report.

Intraoral epithelioid and spindle cell hemangioma associated with pseudoepitheliomatous hyperplasia: An immunohistochemical study.

Intraoral epithelioid and spindle cell hemangioma associated with pseudoepitheliomatous hyperplasia: An immunohistochemical study.

Epithelioid Hemangioma of the Nasal Cavity with Atypical Features: a Case Report and Review of the Literature

Epithelioid Hemangioma of the Nasal Cavity with Atypical Features: a Case Report and Review of the Literature

An Unusual Presentation of Angiolymphoid Hyperplasia with Eosinophilia as Postauricular Mass: A case Report.

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare, benign, reactive vaso-proliferative condition in the dermal and subcutaneous tissues of the head and neck. A 28-year-old female presented with slow-growing painless swelling behind her left ear. FNAC revealed benign soft tissue neoplasm and histopathological examination after surgical excision revealed angiolymphoid hyperplasia with eosinophilia. ALHE origin has been variously attributed to prior trauma, hyperestrogenemia, infectious agents, atopy, reactive hyperplasia, and benign neoplasia. Retroauricular ALHE has been rarely reported. However, on the basis of our case report, it should be a viable differential diagnosis when large subcutaneous tumors of the head and neck are encountered. When big subcutaneous tumors of the head and neck are present, especially in females, a valid differential diagnosis for angiolymphoid hyperplasia with eosinophilia, a rare condition marked by dermal or subcutaneous endothelial cell proliferation, should also be considered.

Fusion transcriptome profiling defines the monoclonal origin of multifocal epithelioid haemangioma of bone.

Epithelioid haemangioma (EH) of bone remains a highly controversial entity. Indeed, the WHO classifies EHs of soft tissues as benign tumours, whereas bone EHs are considered intermediate-locally aggressive tumours due to common multifocal presentation and local destructive growth. To gain insights into theclinical behaviour and biology of EH of bone we retrospectively analysed 42 patients treated in a single institution from 1978 to 2021. Multifocal presentation was detected in 17 of 42 patients (40%) primarily as synchronous lesions. Patients were treated with curettage (57%), resection (29%) or biopsy, followed by radiotherapy or embolisation (14%). Follow-up (minimum 24 months) was available for 38 patients, with only five local recurrences (13%) and no death of disease. To clarify whether the synchronous bone lesions in multifocal EH represent multicentric disease or clonal dissemination, four cases were profiled by RNA-sequencing. Separate lesions from the same patient, which showed a similar transcriptional profile, expressed the same fusion transcript (involving FOS or FOSB) with identical gene breakpoints. These results indicate that, in EH of bone, multifocal lesions are clonally related and therefore represent the spread of a same neoplastic clone rather than simultaneous independent tumours. This finding is in apparent contradiction with the benign clinical course of the disease, and suggests that tumour dissemination in bone EH probably reflects a phenomenon of passive spreading, with tumour cells colonising distal sites while maintaining their benign biological nature.

Angiolymphoid Hyperplasia With Temporal Artery Eosinophilia: A Case Report.

Angiolymphoid Hyperplasia with Eosinophilia (ALHE) is a benign vascular proliferative disorder with uncertain etiology and pathogenesis. The aim of this paper is to report a case of ALHE in the temporal artery and discuss the general aspects of this pathology. A 29-year-old female black patient sought the Vascular Surgery Outpatient Service, complaining of bulging in the right temporal region, associated with pain and local discomfort. Physical examination revealed pulsatile bulging in the right temporal region measuring approximately 2.5 × 1.5cm. Nuclear Magnetic Resonance showed an expansive fusiform lesion in the superficial soft parts of the right temporal region, measuring 2.9cm in the longest longitudinal axis. Surgical excision proved to be the best therapeutic option for the patient in this case. Histopathological sections showed the proliferation of vessels of different sizes, covered by swollen endothelium, prominent inflammatory infiltrate composed of lymphocytes, plasma cells, eosinophils, and scarce histiocytes. Immunohistochemical analysis of the lesion showed positivity for CD31, corroborating the diagnosis of ALHE.

Epithelioid hemangioma (ALHE) on the tongue of an infant treated with oral corticosteroids: A case report and review of the literature.

Epithelioid hemangioma or angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare benign vascular lesion presenting as multiple nodules on the head and neck. Surgery had been considered to be the best treatment modality for ALHE. We report the case of a 6-month-old boy with ALHE on his tongue that was treated successfully with oral prednisolone.

Cutaneous vascular tumors: an updated review

Cutaneous vascular tumors constitute a heterogeneous group of entities that share overlapping morphologic and immunohistochemical features, which can be diagnostically challenging for pathologists and dermatopathologists. Our understanding and knowledge of vascular neoplasms have improved over time, resulting in both a refinement of their classification by the International Society for the Study of Vascular Anomalies (ISSVA) and an improvement in the accurate diagnosis and clinical management of vascular neoplasms. This review article aims to summarize the updated clinical, histopathological, and immunohistochemical characteristics of cutaneous vascular tumors, as well as to highlight their associated genetic mutations. Such entities include infantile hemangioma, congenital hemangioma, tufted angioma, spindle cell hemangioma, epithelioid hemangioma, pyogenic granuloma, Kaposiform hemangioendothelioma, retiform hemangioendothelioma, pseudomyogenic hemangioendothelioma, Kaposi sarcoma, angiosarcoma, and epithelioid hemangioendothelioma.

An Epithelioid Hemangioma Camouflaged As Radial Aneurysm.

Epithelioid hemangioma (EH) is an uncommon benign vascular lesion usually present as subcutaneous nodules in the head and neck area. Sometimes, these lesions can occur in the peripheral arteries, and when they do, they can be mistaken for aneurysmal dilatations of that respective vessel. We report a case of a 43-year-old male who underwent surgical recession of a radial aneurysm, which after anatomopathological examination, revealed an EH.

Ingrown Finger Nail-A Lesser Known Entity.

Dear Editor, Ingrown toenails are a common problem encountered in dermatology practice. They are caused by three major factors—excessive transverse curvature of the nail plate, maceration of the gutter and external pressure over the toe nail. In addition, the hard hyperkeratotic transversely over-curved nail plate exerts pressure and its spicule causes penetrative injury to the nail gutter under the influence of external pressure caused by running, tight fitting shoes, standing on toes etc., making the condition worsen.[1] All these factors make toes more vulnerable to having ingrown nails as compared to fingers nails. However, we have been seen few cases of ingrown fingernails in our dermatology out patient department.[2] There is a lack of scientific data on the incidence of ingrown nails on fingers. As per a recent report, finger ingrown nails were more commonly found in patients receiving epidermal growth factor receptor inhibitors (which leads to epidermolysis and easy maceration of the nail gutter).[3,4] In addition, there are some others drugs which may cause paronychia, excessive nail fold granulation tissue (pseudopyogenic granuloma)and eventually ingrown nail development, for example, cyclosporine, docetaxel, doxorubin, oral antifungals, lamivudine, indinavir, ritonavir, azidothymidine, vandetanib, capecitabine, cetuximab, giftinib, and retinoids. The main predisposing causes of ingrown finger nails may be occupation related, such as external pressure of the nail plate on its gutter during writing with the middle and ring finger, stitching, sewing, opening and shutting sliding windows, typing, vegetable cutting etc. It has been observed that ingrown finger nails are more common in the ring and middle fingers than in the index finger, which may be due to the fact that the ring and middle finger nails are more curved as compared to the index finger. Many patients diagnosed with finger nail paronychia on the distal lateral end may have ingrown nails, which might have been overlooked in the initial stage [Figure 1]. So, a careful examination of the finger nail is a must in all cases of distal end paronychia of the finger nail [Figure 2]. A change in the way fingers are used and avoiding repeated trauma in day-to-day activities are necessary for remission in such cases.Figure 1:: Ingrown finger nail in the initial stage is cut and removed from the gutterFigure 2:: Ingrown finger nail with pseudopyogenic granulomaAcknowledgment None. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

Orbital angiolymphoid hyperplasia with eosinophilia in a pediatric patient: a case report

A 7-year-old female presented to an outpatient eye clinic with a six-week history of left upper eyelid edema and erythema in the absence of trauma without compromise of visual acuity or extraocular muscle movements and was initially treated as a case of pre-septal cellulitis with antibiotic therapy. Failure of resolution with new onset nasal bleeding after two weeks prompted computed tomography (CT) and magnetic resonance imaging (MRI), showing an orbital mass with radiologic concern for lymphoma or rhabdomyosarcoma. Upon biopsy, the histopathological analysis diagnosed angiolymphoid hyperplasia with eosinophilia (ALHE), a rare condition that infrequently presents with ocular manifestations and more rarely presents in the pediatric population. Most therapeutic management for cutaneous and non-cutaneous ALHE have high rates of recurrence. This case was refractory to oral prednisone treatment but responded to a combination of methotrexate and adalimumab therapy with an 18.5% reduction in volume after three months.

A Rare Case Report of T-Cell Rich Angiomatoid Pseudolymphoma.

Dear Editor, Cutaneous pseudolymphoma (PSL) is a reactive lymphoproliferation that histopathologically and/or clinically imitates cutaneous lymphomas. It can be either B-cell or T-cell predominant, and they are further divided into four main categories. Our case falls under the category of T-cell rich angiomatoid PSL.[1] An 86-year-old male presented with multiple, itchy skin lesions over his face, forearms, back, and legs for the past eight months. He was a known case of type 2 diabetes mellitus and right radical nephrectomy was done for Grade II right clear cell renal cell carcinoma six years ago. Cutaneous examination revealed multiple, erythematous infiltrated papules and plaques over the forehead [Figure 1] and lateral aspect of the face. A few hyperpigmented papules with central crusting were seen over bilateral legs and lower back. Angiolymphoid hyperplasia with eosinophilia (ALHE) and Jessner lymphocytic infiltrate of the skin were considered as differential diagnoses. Histopathology of an erythematous infiltrated papule over the forehead revealed a grenz zone and a fairly defined lesion in the dermis [Figure 2]. Many vascular channels lined by hyperplastic endothelial cells were seen in the lesion. Perifollicular mixed inflammatory cell infiltrates comprising histiocytes, lymphocytes, and a few neutrophils were also seen [Figure 3]. IHC showed lymphocytes with strong membranous staining for CD 3 [Figure 4a], and a few showed CD 20 staining [Figure 4b]. A diagnosis of T-cell-rich angiomatoid PSL was made based on histopathology and IHC.Figure 1: Multiple erythematous, few hyperpigmented infiltrated papules, and plaques seen over foreheadFigure 2: Grenz zone (black arrow) and a fairly defined lesion is seen in the dermis (H and E, 10×)Figure 3: The lesion in the dermis shows many vascular channels lined by hyperplastic endothelial cells (red stars). Perifollicular mixed inflammatory cell infiltrates comprising histiocytes, lymphocytes, and few neutrophils were also seen in the lesion (H and E, 100×)Figure 4: (a) Strong membranous staining of the lymphocytes for CD3. (b) Few lymphocytes show CD 20 staining (IHC)Cutaneous PSL is restricted to cases that histopathologically simulate cutaneous lymphomas but do not fit into any other diagnosis after clinical correlation. Some of the etiological factors of PSL include infections caused by bacteria, viruses, parasites, drugs such as anticonvulsants and antihypertensives, foreign agents such as tattoo dyes, injectable vaccinations, insect bites, and ultraviolet radiation. Cutaneous PSL is divided into four main groups based on histopathologic features and clinical data, which include nodular PSLs, PSLs as simulators of Mycosis fungoides (“pseudo-MF”) and of other CTCLs, other PSLs, and intravascular PSLs. Our case clinically and histopathologically fits into the other PSLs group, which comprises T-cell rich angiomatoid PSL and cutaneous plasmacytosis. Acral Pseudolymphomatous Angiokeratoma of Childhood (APACHE), T-cell-Rich Angiomatoid Polypoid Pseudolymphoma (TRAPP), primary cutaneous angioplasmocellular hyperplasia, and lymphoplasmacytoid plaque (LPP) are all forms of T-cell rich angiomatoid PSL.[1] Our case did not show any eosinophils in the inflammatory infiltrate which are characteristically observed in ALHE and the histopathological features observed in our case does not correlate with Jessner lymphocytic infiltrate of the skin. Histopathologically, APACHE and TRAPP were considered as differential diagnoses, but features were more suggestive of TRAPP. Microscopically, TRAPP shows numerous vessels with plump endothelia, a grenz zone, and a dense dermal infiltrate of lymphocytes with a predominance of CD3 over CD20 cells, as seen in our case. TRAPP usually presents as a solitary polypoidal lesion, but in our case it presented atypically as multiple grouped erythematous papules.[2,3] Review of a series of 19 cases showed that a variety of names have been suggested such as APACHE, TRAPP, angiolymphoid hyperplasia with high endothelial venules (ALH-HEV), etc., for lesions sharing similar features such as vascular proliferation with a dense lymphoid infiltrate. They constitute a spectrum of vascular lesions with considerable histopathological and clinical overlap.[4] A case of PSL of the liver in association with renal cell carcinoma occurring 14 months after right radical nephrectomy has been reported.[5] However, our patient developed PSL of the skin post-nephrectomy. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

Radicular cyst in primary dentition showing angiolymphoid hyperplasia with eosinophilia (ALHE)-like features: An immunohistochemical study

Radicular cyst in primary dentition showing angiolymphoid hyperplasia with eosinophilia (ALHE)-like features: An immunohistochemical study