Spontaneous regression of angiolymphoid hyperplasia with eosinophilia of lacrimal gland in an HIV-positive patient.

A 38-year-old retro-positive female presented with a sudden onset protrusion of the left eye for 2 weeks. She was a known case of diabetes mellitus for the past 2 years on treatment, and her blood sugar levels were uncontrolled. She is also a known case of HIV infection for 3 years on anti-retroviral treatment and CD4 count was 450 cells per cubic ml.

On examination of the left eye, there was moderate proptosis and inferior dystopia [Fig. 1a and b]. Left upper eyelid showed edema and mechanical ptosis. The conjunctiva was chemosed with diffuse congestion, discharge and cornea had exposure keratitis. She had total ophthalmoplegia in the left eye. Right eye anterior segment and both eyes dilated fundus examinations were within normal limits. Best corrected visual acuity of the right eye was 6/6, and left eye was 6/36. Gadolinium-enhanced MRI of the brain and orbits suggested the presence of a well-defined homogenous intensely enhancing mass measuring 3.2 × 1.7 × 2 cm3, which was T1 iso/T2 iso-hyperintense, non-suppressing on FLAIR with a few prominent flow voids. It was present in the superolateral aspect of the left extraconal space extending up to preseptal region involving the anterior part of superior rectus, lateral rectus, and lacrimal gland [Fig. 1c and d]. The patient received a course of intravenous antibiotics after which an incisional biopsy from the left lacrimal gland mass was done which showed dense inflammatory infiltrate composed of sheets of mature lymphocytes, a few plasma cells, and histiocytes with a prominence of eosinophils admixed with rich thin-walled blood vessels lined by histiocytoid endothelial cells. No evidence of granuloma or malignant cells observed. IHC with CD 34 highlights blood vessels and CD1a and S100 negative, so Langherhans cell histiocytosis was ruled out. It was consistent with the diagnosis of angiolymphoid hyperplasia with eosinophilia (ALHE) [Fig. 2a-e]. Blood eosinophilic count was normal. Surgical excision of the mass was deferred owing to the ill-defined, vascular nature of the lesion and risk of recurrence in the absence of incomplete resection. The patient underwent a lateral cantholysis to decompress the orbital pressure. On follow up after 4 months, the proptosis reduced in size and swelling regressed spontaneously with an improvement of ptosis [Fig. 3a and b]. The cornea was clear and extra-ocular movements were full. Visual acuity improved to 6/6 in the left eye.

Figure 1

(a and b) Clinical photograph of the patient at presentation showing proptosis and dystopia of the left eye. (c and d) Axial and coronal-section MRI brain with orbit showing a well-defined mass in the left extraconal space, superolateral aspect extending to preseptal space, causing infero-medial displacement of the globe with resultant non-axial proptosis

Figure 2

(a-e) Photomicrographs of biopsy shows (a) fibrous tissue infiltrated by dense inflammatory infiltrate (H and EX100); (b) composed of sheets of mature lymphocytes admixed with prominence of eosinophils and blood vessels lined by reactive plump histiocytoid endothelial cells (H and EX200); (c) IHC with CD34 shows positivity only in endothelial cell (arrow head) (IHCX400); (d and e) IHC with CD1a and CD 68 negative (IHCX200 and IHCX400)

Figure 3

(a and b) Clinical picture of the patient after 4 months showing regression of orbital mass on follow up

Discussion

ALHE is characterized by a florid proliferation of blood vessels lined by plump endothelial cells and admixed with a dense inflammatory infiltrate of lymphocytes, eosinophils, and mast cells. Most commonly, it affects the face and the periauricular area especially, where it usually manifests as papules or nodules. Pathogenesis includes either a reactive or neoplastic phenomenon in response to trauma, increased estrogen or a vascular malformation. Association with possible human immunodeficiency virus has been reported previously in extraorbital sites.[12]

However, this is the first reported case of ALHE involving the lacrimal gland in an HIV-sero-reactive individual.

ALHE must be histologically and clinically differentiated from Kimura disease, which is a chronic inflammatory condition, characterized by large subcutaneous nodules in the head and neck region.[3] The natural course of ALHE tends to be chronic and non-remitting over months to years, and spontaneous regression is very rarely seen.[4] A variety of treatment modalities have been discussed for ALHE. Surgical treatment remains the treatment of choice, but recurrences are seen in 30% of cases.[5]

AHLE is an entity in HIV-positive individuals. It is most commonly associated with tumors involving mucocutaneous area, buccal areas as well as head and neck region. Dermatological manifestations are commonly seen in all age groups. Although surgical excision is the treatment of choice, a conservative approach can be considered in view of its spontaneous regression.

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.