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CASE REPORT
Year : 2016  |  Volume : 5  |  Issue : 4  |  Page : 295-298

Angiolymphoid hyperplasia with eosinophilia during pregnancy: A study of three cases with a brief review of literature


1 Department of DVL, GSL Medical College, Rajahmundry, Andhra Pradesh, India
2 Chief Radiologist, Seven Hills Hospital, Visakhapatnam, Andhra Pradesh, India

Date of Web Publication23-Dec-2016

Correspondence Address:
G Raghurama Rao
Department of DVL, GSL Medical College, Rajanagaram, Rajahmundry - 533 296, Andhra Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2277-8632.196592

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  Abstract 

Angiolymphoid hyperplasia with eosinophilia (ALHE) is an uncommon and benign vascular disorder characterized by angiomatous, pink-red papulonodular lesions located on the scalp, neck, and peri and post-auricular areas. The exact cause of ALHE being unknown, several etiological factors such as trauma, insect bites, hyperestrogen states, and immunological factors have been implicated in the pathogenesis of the disease. A few cases of pregnancy-related ALHE have been reported in the literature. In this study, we report three cases of ALHE, which were aggravated during the first trimester of pregnancy, along with their management. The relation between ALHE and pregnancy is discussed with a brief review of the literature.

Keywords: ALHE, hormonal factors, pregnancy


How to cite this article:
Haritha K, Satya S, Rao G R, Durga Prasad B K, Chowdary A P. Angiolymphoid hyperplasia with eosinophilia during pregnancy: A study of three cases with a brief review of literature. J NTR Univ Health Sci 2016;5:295-8

How to cite this URL:
Haritha K, Satya S, Rao G R, Durga Prasad B K, Chowdary A P. Angiolymphoid hyperplasia with eosinophilia during pregnancy: A study of three cases with a brief review of literature. J NTR Univ Health Sci [serial online] 2016 [cited 2020 Apr 10];5:295-8. Available from: http://www.jdrntruhs.org/text.asp?2016/5/4/295/196592


  Introduction Top


Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare benign vascular proliferative disorder, characterized by solitary or multiple red-to-brown papules or nodules found mainly on the head and neck of young adults. [1]

The exact etiology of ALHE is not known. Infections (HTLV, HHV-8), hormonal background, trauma, and insect bites are proposed causative factors. [2] So far, only a few studies of ALHE have been reported in association with pregnancy. [3],[4],[5],[6] We report three cases of ALHE with pregnancy and their management and prognosis are discussed.


  Case Reports Top


Case-1

A 27-year-old, 12-week pregnant woman presented with multiple, papulonodular lesions of 4 years duration on the right parieto-occipital region. There was no history of contraceptive intake. The initial lesions developed during her first pregnancy three years ago, and the lesions increased in size and number, and she observed slight regression of lesions after the delivery. Again, during her second pregnancy, the number and size of lesions increased and she developed severe pruritus and foul smelling discharge. On physical examination, there were numerous skin-colored glistening, nonpulsatile papules and nodules of 3 mm to 1 cm in size over the post-auricular region extending onto the right parieto-occipital region. The lesions had a tendency to bleed on minor trauma and after scratching. There was a large nevus flammeus present over the right pre-auricular area extending onto the post-auricular area [Figure 1]a and b. Systemic examination was normal. Cervical lymph nodes were present. All hematological and biochemical investigations were within normal limits. HBsAg and human immunodeficiency virus (HIV) tests were negative. Absolute eosinophil count in the peripheral blood was normal. Serum IgE levels were normal. Histological examination showed an increased number of thick-walled dilated blood vessels (capillaries/venules) involving whole of the reticular dermis. These vessels were lined by endothelial cells with perivascular and interstitial infiltration, primarily of lymphocytes and eosinophils. The lining of these vessels showed thick endothelial cells that project into the lumen giving a hob-nail appearance [Figure 2]a and b. A diagnosis of ALHE was made. Immunohistochemical studies of estrogen and progesterone receptors and CD31 were not done. She was asked to report 6 months after the delivery for surgical management. She reported for surgical intervention as there was no regression of the lesions. Before surgery, computed tomography angiogram of the neck vessels showed angiomatous malformation at superficial soft tissues of the right frontoparieto-occipital scalp with supply, predominantly from the right superficial temporal and occipital arteries, and to a lesser extent from the contralateral occipital artery. Cervical lymphadenopathy was present [Figure 3]a and b. Patient underwent wide surgical excision with split thickness graft. The wound healed nicely. No recurrence was observed during the follow-up period of 6 months.
Figure 1: (a) Nevus flammeus with multiple nodules. (b) Multiple glistening nodular lesions of ALHE

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Figure 2: (a) Prominent proliferation of small vessels with lymphocytes and eosinophils (Hematoxylin and eosin 100×). (b) Numerous enlarged endothelial cells in the lumen of abnormal blood vessels (Hematoxylin and eosin 400×)

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Figure 3: (a) CECT of the brain shows contrast enhancing nodular lesions in soft tissues of right parietal region. (b) CECT of the base of the skull with multiple engorged vessels and contrast enhancing lymph node in the right posterior triangle

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Case 2

A 25-year-old lactating woman presented with multiple pruritic, nonpulsatile, painful nodules of 7 years duration on the scalp. There was no history of contraceptive usage. Initially she had 2-3 lesions. Subsequently, during her first pregnancy, the number and size of the lesions increased, and the lesions did not regress even after delivery. On examination, there were numerous skin-colored nodules of variable sizes over the occipital region with prominent follicular orifices. Some of the lesions ruptured with bleeding spots [Figure 4]. Left cervical lymphadenopathy was present. Systemic examination was normal. All hematological and biochemical tests were normal except for peripheral eosinophilia. HBsAg and HIV tests were negative. Serum IgE levels were normal. Histopathological examination of the lesions was consistent with ALHE. Wide surgical excision of the lesions was advised.
Figure 4: Large nodular lesions of ALHE with dilated follicular orifices

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Case 3

A 32-year-old lactating woman reported with numerous skin-colored, nonpulsatile, papulonodular lesions over the right occipital region. She initially developed a few lesions 10 years ago and the number of lesions increased during pregnancy. She observed regression of the lesions after delivery. On physical examination, there were 5-10 skin-colored nontender nodular lesions over the right occipital region [Figure 5]. There was no regional lymphadenopathy. Systemic examination revealed no abnormality. All hematological and biochemical tests were normal except for peripheral eosinophilia. HBsAg and HIV tests were negative. Serum IgE levels were normal. Histopathology features were consistent with ALHE. The lesions were treated with cryotherapy.
Figure 5: Multiple skin colored nodules of ALHE

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  Discussion Top


ALHE was first described by Wells and Whimster in 1969 who coined the term "subcutaneous angiolymphoid hyperplasia." They considered it to be a late stage of kimura's disease. It is now generally accepted that these are two separate entities. [1] ALHE occurs in young-to-middle aged adults without gender predilection. [7] ALHE is a benign proliferation of capillaries and venules with head and neck region being the most frequent site of occurrence. The precise etiology of this condition is not known. Prior trauma, hyperestrogenemia, pregnancy, oral contraceptive pills (OCP), infectious agents, and atopy may play a role in the pathogenesis of ALHE. Cases have been described in association with arteriovenous fistula and malformations. [8] It is unclear whether this entity represents a reactive proliferation or low grade neoplastic disease, secondary to various stimuli. Cutaneous lesions are usually asymptomatic but may be pruritic, painful, hemorrhagic, and manifest as papules, nodules, and plaques. Peripheral eosinophilia is an inconsistent feature seen in 21% of the cases. [9] Diagnosis is based on characteristic histological features of small vessel proliferation lined by endothelial cells projecting into lumen of the vessel wall and mixed inflammatory infiltrate of lymphocytes and eosinophils. [1],[4],[8]

The number of ALHE cases reported during pregnancy or involving the use of oral contraceptive pills is limited. All these studies showed occurrence and worsening of ALHE during pregnancy and regression of the tumors during the postpartum period. These findings suggest that sex hormones may play a role in the pathogenesis of ALHE. The relationship between pregnancy and ALHE has not yet been established. Olsen and Helwig [8] suggested an association of hyperestrogen states in the pathogenesis of ALHE in their study of 116 cases. Four patients had development of nodules in their first trimester of pregnancy. The fifth patient who had stable disease exhibited increase in nodular size during pregnancy. Moy et al. [5] demonstrated increased level of estrogen and progesterone receptors in the lesions of ALHE during pregnancy and suggested that ALHE was a hormonal responsive tumor. Pregnancy may also enhance the occurrence or recurrence of lobular capillary hemangioma, including pyogenic granuloma of gingiva. Nichols et al. [10] in a study of 21 lobular capillary hemangiomas including lesions from 6 pregnant women observed that estrogen hormones may play a role in the lobular capillary hemangioma by indirect activity in the absence of estrogen and progesterone receptors. Other case reports [2],[3],[4] also demonstrated the aggravation of ALHE in the first or second trimester of pregnancy, without any overexpression of estrogen and progesterone in the lesional skin. These studies suggested that elevated levels of estrogen during the first trimester of pregnancy may have an important role in aggravating ALHE by an indirect hormonal action. Parimalam and Thomas [6] recently reported a case of ALHE developed in a 33-year-old pregnant woman with history of usage of oral contraceptive pills for 1 year.

In the present study also, there is development of new lesions and worsening of the disease observed in all the three cases, however, in 1 case, there is regression of lesions during the postpartum period. Although triggering factors such as previous history of oral contraceptive pill use, trauma, insect bites, or infections were not present in our cases, aggravation of the disease during pregnancy suggests a potential role of sex hormones in the pathogenesis of ALHE. Thus, we demonstrated a possible hormonal relationship between ALHE and pregnancy, which corroborates the previous reports. However, further studies are needed to evaluate the causes of ALHE during pregnancy.

Spontaneous regression of ALHE rarely occurs. Therefore, treatment is generally necessary. Surgical excision is the most common therapeutic option and approximately one-third of lesions recur after excision due to incomplete excision of nidus of arteriovenous malformation. Various other therapeutic options such as pulse dye laser, electrodessication, cryotherapy, sclerotherapy, interferon alpha 2, and cytotoxic intralesional steroids have been tried with variable success. [7] We successfully managed one case with cryotherapy. In another case, wide surgical excision of lesions with split thickness grafts was performed 6 months after the delivery, and there was no recurrence during the 6-month follow-up period. Wide surgical excision was planned for another case.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Briggs PL. Kimura disease is not angiolymphoid hyperplasia with eosinophilia: Clinical and pathological correlation with literature review and definition of diagnostic criteria. An Bras Dermatol 2006;2:167-73.  Back to cited text no. 1
    
2.
Ceyhan AM, Akkaya VB, Yildirium M, Karahan N. A case of angiolymphoid hyperplasia with eosinophilia aggravated during pregnancy. Balkan Med J 2011;28:319-21.  Back to cited text no. 2
    
3.
Hollo P, Marschalko M, Sikos G, Harsing J, Horvath A. Angiolymphoid hyperplasia with eosinophilia in pregnancy. J Eur Acad Dermatol Venereol 2005;19:645-6.  Back to cited text no. 3
    
4.
Zarrin- Khamesh N, Spoden JE, Tran RM. Angiolymphoid hyperplasia with eosinophilia associated with pregnancy: A case report and review of literature. Arch Pathol Lab Med 2005;129:1168-71.  Back to cited text no. 4
    
5.
Moy RL, Luftman DB, Nguyen QH, Amenta JS. Estrogen receptors and the response to sex hormones in angiolymphoid hyperplasia with eosinophilia. Arch Dermatol 1992;128:825-8.  Back to cited text no. 5
    
6.
Parimalam K, Thomas J. Angiolymphoid hyperplasia with eosinophilia associated with pregnancy-A rare report. Indian J Dermatol 2016;61:125.  Back to cited text no. 6
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7.
Adler BL, Krausz AE, Minuti A, Silverberg JI, Lev-Tov H. Epidemiology and treatment of angiolymphoid hyperplasia with eosinophilia: A systematic review. J Am Acad Dermatol 2016;74:506-12.  Back to cited text no. 7
    
8.
Oslen TG, Helwig EB. Angiolymphoid hyperplasia with eosinophilia: A clinicopathologic study of 116 patients. J Am Acad Dermatol 1985;12:781-96.  Back to cited text no. 8
    
9.
Lade NR, Banode P. Dhope N, Tharafdar P, Saoji V. Angiolymphoid hyperplasia with arterial ectasia and arteriovenous malformation: Response to intra-arterial embolization. Indian J Dermatol Venereol Leprol 2016;82:413-5.  Back to cited text no. 9
[PUBMED]  Medknow Journal  
10.
Nichols GE, Gaffey MJ, Mills SE, Weiss LM. Lobular capillary hemangioma: An immunohistochemical study including steroid hormone receptor status. Am J Clin Pathol 1992;97:770-5.  Back to cited text no. 10
    


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  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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