ABSTRACT
Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare benign disease of unknown etiology, characterized by abnormal vascular proliferation. ALHE usually presents with painful, itchy, or asymptomatic lesions in the neck region, particularly around the ears. Clinically, it is usually around the ear, at the border of the scalp. It can be seen as single or multiple nodules or papules lesions on the neck and neck. Medium-sized peripheral muscular artery involvement is rare. A 41-year-old male patient, presented to a rheumatology clinic for 1 year history of a soft painless swelling in his bilateral temporal region. He sustained no preceding trauma to the area and had no additional symptoms. On physical examination, temporal arteries were prominent, but there was no tenderness. The patient had no clinical signs of temporal arteritis. Temporal artery Doppler ultrasound was performed, which revealed an echogenic asymmetric vessel wall thickening in the bilateral superficial temporal arteries. Temporal artery biopsy yielded the final diagnosis of ALHE, a rare vascular involvement of unknown etiology. ALHE is a condition that should be kept in mind in the differential diagnosis of temporal arteritis.
Introduction
Angiolymphoid hyperplasia with eosinophilia (ALHE) is a benign vasoproliferative disorder of unknown etiology.[1] The majority (70%) of affected patients are women in their third to the fifth decade of life. Affected patients may have a family history. ALHE frequently involve the skin of the head and neck, particularly in the periauricular area and the scalp. Systemic involvement is rare in ALHE.[2-5]
Being a rare disorder that may mimic several conditions such as lipoma, sebaceous cyst, Dupuytren’s disease, or giant cell arteritis[2,6,7], ALHE may impose a diagnostic challenge with a risk of misdiagnosis or delayed diagnosis.[8-11]
Here, we present a case of intra-arterial ALHE mimicking temporal arteritis with prominent temporal arteries.
Case Report
A 41-year-old male patient was referred to the rheumatology outpatient clinic with the suspected diagnosis of temporal arteritis due to bilateral swelling in the temporal region. The patient described a painless, non-pruritic swelling in the temporal region bilaterally lasting for 1 year along with no history of trauma, bruising, bleeding, or a chronic disease. The patient had no signs/symptoms related to polymyalgia rheumatica, while jaw claudication or visual symptoms were also absent.
Physical examination revealed a painless subcutaneous nodule on the surface of prominent temporal arteries bilaterally in the temporal region. No murmurs were noted in the temporal and carotid arteries, and there was no significant pulsation in the temporal arteries (Figure 1). Previous cranial computed tomography had revealed the thickening of the vessel wall in the superficial frontal branches of both temporal arteries, which was in the foreground on the right side. Temporal artery Doppler ultrasound was performed, which revealed an echogenic asymmetric vessel wall thickening in the bilateral superficial temporal arteries (Figure 2). Hemogram findings, eosinophil count, erythrocyte sedimentation rate, C-reactive protein levels and blood biochemistry findings were within the normal limits. An excisional biopsy of the temporal artery was performed. In the histological examination, an intravascular proliferation was noted that was composed of epithelioid endothelial cells, lymphocytes, and many eosinophils. There were no multinucleated giant cells or granulomatous inflammation. Immunohistochemical staining showed positive immunoreactivity for CD 68 (focal), CD 31, CD 34 and elastin (focal) expression. Histological analysis of the lesion revealed a diagnosis of eosinophilic angiolymphoid hyperplasia involving branches of the superficial temporal artery (Figures 3 and 4). Although the patient was referred to the relevant departments for surgical excision, medical treatment, laser therapy, or cryotherapy, he refused the treatment.
Discussion
ALHE is an uncommon disorder with its exact prevalence remains unknown, while several cases have been reported worldwide.[12,13] Although some authors consider ALHE a reactive process, it has also been suggested to represent a neoplastic process.[14,15] ALHE lesions usually present as single or multiple nodules in the head and neck region, especially in the periauricular area.[1,3] The lesions may be associated with pruritis, pain and ulceration, or an overlying pulsation.[16-19] In a review by Adler et al.[5], no gender-based differences were noted in the prevalence of ALHE along with an asymptomatic course in 15.4% of patients, while the most common localizations were considered as ear and periauricular area (36.3%), followed by face (28.2%) and scalp (17.3%).
Our patient had bilateral superotemporal lesions with no accompanying symptoms and was referred to our clinic with a suspected diagnosis of temporal arteritis due to bilateral prominent temporal arteries. In the current examination, Doppler ultrasound also revealed an echogenic asymmetric localized vessel wall thickening in the bilateral superficial temporal arteries, while the definitive diagnosis was made by the histopathological evaluation.
For ALHE cases involving the temporal region, the temporal arteritis is suggested to be considered in the differential diagnosis.[12] ALHE lesions can be seen adjacent to an artery, their development within the muscular artery is very rare.[20] Previous case reports indicated that the involved arteries comprise the temporal artery[2,6,12,14,20-31], radial artery[32-34], facial artery[35], post-auricular artery[36], popliteal artery[37], brachial artery[38,39], occipital artery[39], ulnar artery[9,40] and axillary artery[41] among these, cases of ALHE developed within the temporal arteries (Table 1).
Temporal arteritis is a vasculitis that can involve large- and medium-sized vessels. Presenting symptoms in patients with temporal arteritis may include tenderness in the temporal region, jaw claudication, localized headache, skin necrosis, eye involvement (i.e., ischemic optic neuropathy), fever, malaise, constitutional symptoms, polymyalgia rheumatica-like symptoms, arthralgia, myalgia and skin necrosis, whereas these symptoms are not expected in intra-arterial ALHE.[6,20,28] Nonetheless, temporal arteritis and intra-arterial ALHE may have some common manifestations, and the histopathological examination is required for definitive diagnosis.[2] Histologically, ALHE show varying degrees of nodular and diffuse lymphocytic infiltrates with eosinophils and an angio-proliferative lesion characterized by plump endothelial cells.[4] Mimicking vascular conditions include epithelioid hemangioendothelioma, Kimura’s disease, angiosarcoma, eosinophilic granulomatosis with polyangiitis. Conditions which may involve the scalp and subcutaneous tissue in the head region, such as Kaposi’s sarcoma, metastasis, lymphoma and pyogenic granuloma, should also be considered in the differential diagnosis.[2,4]
In this case, temporal arteritis was suspected because of the prominence of the temporal arteries. The patient’s age, normal acute phase response parameters, the absence of fever and constitutional symptoms, as well as lack of visual symptoms or symptoms related to polymyalgia rheumatica were the findings not supportive for the temporal arteritis diagnosis. In this study, although the halo sign in the temporal arteries was not typically seen on Doppler ultrasound imaging, there was echogenic asymmetric vessel wall thickening in the bilateral superficial temporal arteries. In fact, the halo sign, which is hypoechoic thickening of the temporal artery wall in Doppler ultrasound imaging, is frequently used in clinical practice in the diagnosis of temporal arteritis. Halo sign can also be evident, but less frequently, in other conditions such as infection, polymyalgia rheumatica and malignancy.[8,42] The finding of halo sign on ultrasound in ALHE has been previously reported in one case report.[8] The halo sign in temporal arteritis is not always related to an inflammatory process and may refer to an edema in the vessel wall rather than an inflammatory process.[8,43] In the case of intra-arterial ALHE, the halo sign can be seen if the vessel lumen is not completely obliterated. Indeed, the halo sign in intra-arterial ALHE has been associated with the presence of marked thickening of the vessel wall due to perivascular infiltrate.[8]
Conclusion
In conclusion, our findings indicate that ALHE, as a rare disorder, should be considered in the differential diagnosis of temporal arteritis, as it may mimic temporal arteritis in terms of clinical appearance and Doppler ultrasound findings.
Ethics
Informed Consent: Written informed consent was obtained from the patient for the anonymized information to be published in this article.
Peer-review: Externally peer-reviewed.
Authorship Contributions
Concept: S.İ., A.E., Design: S.İ., A.E., Data Collection or Processing: S.İ., A.E., G.K., Ç.T., Analysis or Interpretation: S.İ., A.E., G.K., Ç.T., Literature Search: S.İ., A.E., G.K., Ç.T., Writing: S.İ., A.E., G.K., Ç.T.
Conflict of Interest: No conflict of interest was declared by the authors.
Financial Disclosure: The authors declare that they have no relevant financial disclosures.
