Angiolymphoid Hyperplasia with Eosinophilia Associated with Pregnancy - A Rare Report.

Sir,

Angiolymphoid hyperplasia with eosinophilia (ALHE) was first described by Wells and Whimster as ‘subcutaneous angiolymphoid hyperplasia with eosinophilia’. ALHE also called as atypical or pseudo pyogenic granuloma is characterized by solitary or multiple red to brown papules or nodules seen commonly in women between 20 and 40 years of age. The lesions may rarely present as non-healing ulcer.[1] ALHE occurring during pregnancy is rare and may be attributable to the elevated levels of estrogen. Histological findings included proliferation of small vessels lined by plump endothelial cells, surrounded by inflammatory cells including lymphocytes, mastocytes, eosinophils, and sometimes formation of lymphoid follicles. We report a 33-year-old female patient with asymptomatic reddish nodules over the right ear since fourth month of her second pregnancy. The lesions increased in size and number even after delivery. The patient gave a history of having taken oral contraceptive pills for one year. There were multiple red, non-pulsatile smooth surfaced papules, over the pre auricular region extending on to the crus of the helix, upper portion of tragus, and into the meatus [Figure 1]. There was no lymphadenopathy. Other systems were normal. Differential count showed 18% eosinophils and her Ig E level was normal. The entire lesion was excised. Histology was suggestive of ALHE [Figure 2]. There has been no recurrence for two years following excision [Figure 3].

Figure 1

Angiomatous papules and nodules involving the skin over the pre auricular region and the ear

Figure 2

Histology showing the tumor mass with vascular proliferation some vessels showing plump endothelial cells, perivascular and interstitial infiltration of lymphocytes, histiocytes, and eosinophils (H and E, original magnification ×50) (a) Histology showing vascular proliferation some vessels showing plump endothelial cells, perivascular and interstitial infiltration of lymphocytes, histiocytes, and eosinophils (H and E, original magnification ×400)

Figure 3

Same patient as in Figure 1 two years after excision

The exact etiology of ALHE is not known. Infections (HTLV or HHV8), hormonal background, trauma, insect bites are proposed causative factors. So far, only few studies of ALHE have been reported in association with pregnancy.[2] ALHE in a male has been earlier reported in India.[3] Surgical excision has been reported to have the most favorable results. The predominance of T lymphocytes and a rearrangement of T Cell Receptor gene in some cases suggest that ALHE could be a low-grade neoplastic disease secondary to various stimuli.[4] In cases of ALHE showing regression following discontinuation of oral contraceptive pills and during postpartum, the tumors expressed a significant amount of estrogen and progesterone receptors. CD34 expression, a sensitive marker for endothelial differentiation has also been reported.[5] Kimura's disease is thought to be an allergic response which is histologically characterized by lymphoid nodules with germinal centers, vascular proliferations when seen are lined by flat endothelial cells. Systemic eosinophilia is almost always present. Whereas, ALHE is believed to be a true neoplasm of the endothelium. The high number of estrogen and progesterone receptors in the tumor cells of some patients and regression of the lesions after discontinuation of the effect of sex hormones are noteworthy findings to support sex hormones to be the etiological factor in ALHE of pregnancy.

The hyper-estrogenic state due to the long term use of oral contraceptive pills and pregnancy might have had an important role either directly or indirectly in inducing or aggravating lesion growth. Further studies are needed to evaluate the cause of ALHE during pregnancy and the neoplastic nature of the disease.