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Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare vasoproliferative lesion of uncertain aetiology, involving the skin and subcutaneous tissue. The predilection of the tumour-like lesion is for the head and neck region. Radical surgical excision is still regarded as the most effective treatment. We present the case of a 33-year-old female with ALHE of the right hand. Preoperative MRI and angiography demonstrated involvement of the fourth and fifth rays, with complete occlusion of the ulnar artery, and a small lesion at the level of the metacarpophalangeal joint of the index finger. Complete tumour excision could not be achieved without resection of the fourth and fifth rays. One year postoperatively, there were no clinical signs of recurrence. The patient refused any further invasive diagnostic and follow-up examinations. Angiolymphoid hyperplasia of the hand is a rare disease, and patients should undergo early surgical treatment to achieve complete excision of the lesion.
A 33-year-old woman presented with a progressively tender swelling of her right palm and ring finger that had started 18 months previously. Over the past 6 months, she had noticed blue discoloration of the nail bed of her ring finger (Fig. 1) with decreasing finger temperature and increasing cold intolerance. Eight weeks before she had been operated on for misdiagnosed Dupuytren's disease. The operation had been aborted because of prolific bleeding. Histopathology of an intraoperative specimen had led to the diagnosis of angiolymphoid hyperplasia with eosinophilia (ALHE). There was no family history of cutaneous or vascular malformations.
Figure 1Preoperative appearance: the patient had noticed some blue discoloration of the nail bed of her ring finger.
On physical examination, the patient was found to have no pathological cutaneous lesions other than in the right hand. No bone lesions were seen on radiography. An MRI scan revealed a soft-tissue tumour-like lesion with concentric formation around the ulnar, metacarpal and digital arteries of the ring finger. Angiography showed hand perfusion through the radial artery, but neither orthograde nor retrograde filling of the ulnar artery (Fig. 2) . In the late arterial phase, significant vascular enhancement (a sign of vascular neoformation) was noted in the metacarpal region and in the tip of the ring finger, as well as in a smaller area next to the metacarpophalangeal joint of the index finger (Fig. 3) . Surgical excision of the vascular lesions was undertaken. The obliterated ulnar and fourth metacarpal arteries (Fig. 4) were removed en-bloc with the surrounding tumour-like tissue. The smaller lesion at the level of the second metacarpophalangeal joint was completely excised, including resection of the ulnar palmar digital artery. Complete excision of the vasoproliferative lesions of the fourth and fifth rays could not be achieved without resection of both rays. All specimens showed similar histological features in haematoxylin and eosin section (Figure 5, Figure 6) . The ulnar artery showed a central muscular arterial blood vessel proliferation lined with epithelioid endothelial cells obliterating the vessel lumen (Fig. 5). The picture is completed by the neoformation of smaller vascular cords surrounded by a mixed inflammatory infiltrate, predominantly consisting of eosinophils and lymphoid aggregates (Fig. 6).
Figure 2In the early arterial phase of the angiogram, there was neither orthograde nor retrograde filling of the ulnar artery (arrow). Blood supply to the hand was maintained through the radial artery alone.
Figure 3In the late arterial phase, significant vascular enhancement (a sign of vascular neoformation) was noted in the metacarpal region (black arrows), in the tip of the ring finger (framed white arrow) and in a smaller area next to the metacarpophalangeal joint of the index finger (grey arrow).
Figure 5In haematoxylin and eosin section, the ulnar artery showed central muscular arterial blood vessel proliferation obliterating the vessel lumen (marked with an encircled cross).
Three months postoperatively there were no clinical signs of recurrent disease. The patient refused further follow-up examinations.
2. Discussion
ALHE is a rare vasoproliferative disease. The aetiology is uncertain; possible explanations vary from benign vascular neoplasm to reactive inflammatory lesion.
It shows a predilection for the cutis and subcutis of the head and neck region, with a peak incidence in the third decade of life and a slight predominance in women.
which was initially regarded as a variant of ALHE. Kimura's disease is less proliferative, the new vessels are always canalised and vascular cords are sparse.
ALHE is a benign proliferative disease; the lesions grow slowly over several months, with no reported incidence of metastatic disease. ALHE of the hand is very rare and has previously been described only in conjunction with other lesions of the upper extremity.
In our patient we did not see any additional vasoproliferative lesions of the upper extremity on angiographic screening. This case is the first report of localised ALHE of the hand.
Complete surgical excision is the treatment of choice for ALHE, but may be mutilating in cases of extended lesions of the extremities, as in our case. Other therapies have been used with little success, including low-dose irradiation,
Early diagnosis is most important for efficient eradication of these lesions. As there is no risk of metastasis, biopsies are recommended once ALHE is suspected.
In conclusion, ALHE is a rare condition, especially in the hand as reported here. Diagnostic investigations should include MRI in combination with angiography, angio-MRI or angio-CT-scan to determine the extent of the lesion and the vascular involvement. Once the diagnosis of ALHE is suspected, early and complete surgical excision of the lesions should be undertaken.
References
Rosai J
Gold J
Landy R
The histiocytoid hemangiomas. A unifying concept embracing several previously described entities of skin, soft tissue, large vessels, bone, and heart.
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