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DERMA-QUEST
Year : 2006  |  Volume : 51  |  Issue : 2  |  Page : 151
Pruritic papulo-nodular lesion on face with tendency of bleeding on minor trauma


Department of Dermatology, Medical College Calcutta, Kolkata-73, India

Correspondence Address:
Nilay Kanti Das
Devitala Road, Majerpara, Ishapore, North 24 Paraganas - 743144
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.26944

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How to cite this article:
Das NK, Datta PK. Pruritic papulo-nodular lesion on face with tendency of bleeding on minor trauma. Indian J Dermatol 2006;51:151

How to cite this URL:
Das NK, Datta PK. Pruritic papulo-nodular lesion on face with tendency of bleeding on minor trauma. Indian J Dermatol [serial online] 2006 [cited 2019 Nov 15];51:151. Available from: http://www.e-ijd.org/text.asp?2006/51/2/151/26944


A 28 year female presented with red swollen lesion on the tip of the nose, which was noted for the last 5 months and was gradually increasing in size. She also complained of eruption of fresh new lesion with similar appearance by the side of the nose. She could not remember any history of trauma preceding the development of disease. The lesions were essentially asymptomatic except for slight pruritus and bleeding on minor trauma. She gave no history of systemic complaints. No significant family or drug history was revealed.

On examination erythematous non-tender shiny papulo-nodular lesions ranging in size from 1cm to 4cm were noted on the tip of nose and right naso-labial furrow [Figure - 1]. There was no significant lymphadenopathy, enlarged salivary glands or hepato-splenomegaly. Systemic examination and examination of peripheral nervous system revealed no abnormality.

CBC showed Hb-11.5%, TLC-8000/mm 3sub , P-58%, L-25%, E-15% and ESR-8 mm. Mantoux test (with 10 TU) showed an induration of 11mm and chest screening showed no abnormality. Liver function test and renal profile were found to be within normal limits. Slit skin smear was negative for both acid-fast bacilli and LD bodies.

Histopathological examination with H and E showed prominent cellular infiltrate in the dermis and proliferation of vascular element without any evidence of granuloma formation [Figure - 2]A, B, C. Reticulum stain showed no ring of reticulum outlining the basement membrane of vascular endothelial cells [Figure - 2]D.

Questions

1. What is the diagnosis?

2. Which are histopathological mimickers of this clinico-pathological entity?

3. What are the proposed mechanisms behind its etio pathogenesis?

4. How can the condition be managed?


   Answer to Derma-Quest - I Top


Ans 1. Angiolymphoid hyperplasia with eosinophilia (ALHE)

The clinical presentation points towards several clinical entities but the diagnosis is essentially histopathological. The disorder is uniquely characterized by vascular and cellular component as described in 1969 by Wells and Whimster.[1]

One of the hallmark of this disorder being the hyperplasia of the vascular element with enlarged endothelial cells which projects into the lumen of the vessels producing "cobble-stone" appearance[2] [Figure - 2]B with thickening of their wall and occlusion of the lumen at places[3] [Figure - 2]C. Histiocytoid appearance of the endothelial cells has prompted this disorder to be referred to as "Histiocytoid hemangioma".[4] In same year 1969 another term "Pseudogranuloma pyogenicum" was coined for lesions presenting as dermal nodules[5] but soon it was found that both of them represented the same disease process.[6]

Other distinctive feature of this clinico-pathological entity is the peri-vascular infiltrate of lymphocytes and eosinophils [Figure - 2]B, C and tendency of the infiltrating lymphocytes towards follicle formation [Figure - 2]A.[2] The local tissue eosinophilia is sometimes associated with systemic eosinophilia,[7] as in this case.

Ans 2. ALHE is often histologically misdiagnosed as angiosarcoma because of its vascular proliferation associated with lymphocytic infiltrate. Presence of eosinophils and absence of nuclear atypia with mitotic figures in ALHE distinguishes it from the latter.2 With reticulum stain, a ring of reticulum outlining the basement membrane of endothelial cells can be seen with angiosarcoma,8 which is absent in the present case [Figure - 2]D.

Retiform hemangioendothelioma can be distinguished by its typical "hobnail pattern" of endothelial cells where the cells are tall, narrow and protrude inside the lumen[9] instead of being plump and swollen and occluding the lumen as in ALHE.

Insect bite reaction sometimes is difficult to distinguish from ALHE but the former is never associated with the intense vascular proliferation, which can serve to distinguishing the two.[2] Changes similar to ALHE may also be encountered following injection of vaccine[10] but as in case of insect bite reaction the vascular hyperplasia is not prominent; and the site of lesion gives the clinical clue.

One of the most intriguing entities to separate from ALHE, is Kimura's disease, which was described in 1948 by Kimura from Japan, as quoted by Kawada et al .[11] There is controversy regarding whether to describe it as separate disease entity or consider them as part of one disease spectrum. A very recent report describes the coexistence of the two diseases in the same patient considering them as spectrum illness.[12] But those in favor of considering it a separate entity highlighted a few distinguishing points for Kimura's disease , which include male predominance, extensive nodular lesions, involvement of salivary glands and lymph nodes.[13] Histological differences comprise of less extensive vascular proliferation and eosinophilia, and absence of uncanalized blood vessel in Kimura's disease. Associated renal disease is also described with Kimura's disease[14] and sometimes ALHE masquerade as Kimura because of renal involvement.[15]

Ans 3. Presently no uniform consensus is formed regarding the etio-pathogenesis of ALHE. Some believe that ALHE is a regenerative change representing healing response or an inflammatory reaction arising secondary to immunological injury.16,17 Reports of spontaneous regression of ALHE supports the view of reactive condition, which reflects its response with the cessation of exciting stimulus.18 Presence of systemic eosinophilia, tissue mast cells and eosinophils suggest type I hypersensitivity, but definite proof of stimulus like trauma or infection is still lacking.

Few other consider it to be a true-benign tumour with inflammatory component, considering the multicentricity and tendency to recur following excision as points favouring the neoplastic nature.[19] Even the name histiocytoid hemangioma is suggested to refer the tumour nature.[4] Recently ALHE was reported to develop in pregnancy, suggesting the role of sex hormones in its etiogenesis.[20]

Ans 4. Angiolymphoid hyperplasia is a benign disease and it is suggested that early superficial lesion be observed for 3-6 months pending spontaneous regression.18 Various treatment modalities are suggested for ALHE ranging from surgical excision, local irradiation and systemic corticosteroid therapy.19 Because of high recurrence rates with standard excision, Mohs micrographic surgery with complete margin examination is suggested as a treatment option.21 Both Carbon-dioxide22 and Pulsed-Dye23 Lasers are also used for treatment of ALHE.

Various medical agents are also been tried in ALHE with reported success; which includes tacrolimus,[24] imiquimod,[25] anti-interleukin-5 antibody (mepolizumab)[26] and antiallergic agent suplatast tosilate.[27]

 
   References Top

1.Wells GC, Whimster IW. Subcutaneous angiolymphoid hyperplasia with eosinophilia. Br J Dermatol 1969;81:1-15.   Back to cited text no. 1  [PUBMED]  
2.Colonje E, Wilson-Jones E. Vascular tumours:tumour and tumour-like conditions of blood vessels and lymphatics. In : Elder D, Elenitsas R, Jaworsky C, Johnson Jr. B editors. Lever's Histopathology of the Skin, 8th ed. Lippincott Williams and Wilkins: Phladelphia; 1997. p. 891-5.  Back to cited text no. 2    
3.Grimewood R, Swinehart JM, Aveling JL. Angiolymphoid hyperplasia with eosinophilia. Arch Dermatol 1979;115:205-7.  Back to cited text no. 3    
4.Rosai J. Angiolymphoid hyperplasia with eosinophilia of the skin. Am J Dermatopathol 1982;4:175-84.  Back to cited text no. 4  [PUBMED]  
5.Wilson JE, Bleehen SS. Inflammatory angiomatous nodules with abnormal blood vessels occurring about the ears and scalp (pseudo or atypical pyogenic granuloma). Br J Dermatol 1969;81:804-16.  Back to cited text no. 5    
6.Kandil E. Dermal angiolymphoid hyperplasia with eosinophilia versus pseudopyogenic granuloma. Br J Dermatol 1970;83:405-8.  Back to cited text no. 6    
7.Mehregan AH, Shapiro L. Angiolymphoid hyperplasia with eosinophilia. Arch Dermatol 1971;103:50-7.  Back to cited text no. 7  [PUBMED]  
8.Mach K. Zur Frage des Lymphangioendotheliomas. Arch Klin Exp Dermatol 1966;226:318-26.  Back to cited text no. 8  [PUBMED]  
9.Calonje E, Fletcher CD, Wilson-Jones E, Rosai J. Retiform hemangioendothelioma. A distinctive form of low-grade angiosarcoma delineated in a series of 15 cases. Am J Surg Pathol 1994;18:115-25.  Back to cited text no. 9    
10.Hallam LA, MacKinlay GA, Write AM. Angiolymphoid hyperplasia with eosinophilia: Possible aetilogical role for immunization. J Clin Path 1989;42:944-9.  Back to cited text no. 10    
11.Kawada A, Takahashi H, Anzai T. Eosinophilic lymphofoiliculosis of the skin (Kimura's disease). Jpn J Dermatol 1966;76:61-72.  Back to cited text no. 11    
12.Chong WS, Thomas A, Goh CL. Kimura's disease and angiolymphoid hyperplasia with eosinophilia: Two disease entities in the same patient:case report and review of the literature. Int J Dermatol 2006;45:139-45.  Back to cited text no. 12  [PUBMED]  [FULLTEXT]
13.Kawada A, Morbus Kimura. Darstellung der erkrankung und ihre differential diagnose. Hautarzt 1976;27:309-17.  Back to cited text no. 13    
14.Dede F, Ayli D, Atilgan KG, Yuksel C, Duranay M, Sener D, et al . Focal segmental glomerulosclerosis associating Kimura disease. Ren Fail 2005;27:353-5.  Back to cited text no. 14    
15.Ramchandani PL, Sabesan T, Hussein K. Angiolymphoid hyperplasia with eosinophilia masquerading as Kimura disease. Br J Oral Maxillofac Surg 2005;43:249-52.  Back to cited text no. 15    
16.Castro C, Winkelmann RK. Angiolymphoid hyperplasia with eosinophilia in the skin. Cancer 1974;34:1696-705.  Back to cited text no. 16    
17.Grimewood R, Swinehart JM, Aveling JL. Angiolymphoid hyperplasia with eosinophilia. Arch. Dermatol 1979;115:205-7.   Back to cited text no. 17    
18.Satpathy A, Moss C, Raafat F, Slator R. Spontaneous regression of a rare tumour in a child: Angiolymphoid hyperplasia with eosinophilia of the hand: Case report and review of the literature. Br J Plast Surg 2005;58:865-8.  Back to cited text no. 18    
19.Shroff CP, Pandit SP, Deodhar KP. Angiolymphoid hyperplasia with eosinophilia of the scalp. (A case report). J Postgrad Med 1983;29:113-6.  Back to cited text no. 19    
20.Zarrin-Khameh N, Spoden JE, Tran RM. Angiolymphoid hyperplasia with eosinophilia associated with pregnancy: A case report and review of the literature. Arch Pathol Lab Med 2005;129:1168-71.  Back to cited text no. 20    
21.Miller CJ, Ioffreda MD, Ammirati CT. Mohs micrographic surgery for angiolymphoid hyperplasia with eosinophilia. Dermatol Surg 2004;30:1169-73.  Back to cited text no. 21    
22.Kaur T, Sandhu K, Gupta S, Kanwar AJ, Kumar B. Treatment of angiolymphoid hyperplasia with eosinophilia with the carbon dioxide laser. J Dermatolog Treat 2004;15:328-30.  Back to cited text no. 22    
23.Angel CA, Lewis AT, Griffin T, Levy EJ, Benedetto AV. Angiolymphoid hyperplasia successfully treated with an ultralong pulsed dye laser. Dermatol Surg 2005;31:713-6.  Back to cited text no. 23    
24.Mashiko M, Yokota K, Yamanaka Y, Furuya K. A case of angiolymphoid hyperplasia with eosinophilia successfully treated with tacrolimus ointment. Br J Dermatol 2006;154:803-4.  Back to cited text no. 24    
25.Redondo P, Del Olmo J, Idoate M. Angiolymphoid hyperplasia with eosinophilia successfully treated with imiquimod. Br J Dermatol 2004;151:1110-1.  Back to cited text no. 25    
26.Braun-Falco M, Fischer S, Plotz SG, Ring J. Angiolymphoid hyperplasia with eosinophilia treated with anti-interleukin-5 antibody (mepolizumab). Br J Dermatol 2004;151:1103-4.  Back to cited text no. 26    
27.Harada K, Kambe Y, Takeda H, Nakano H, Hanada K. Angiolymphoid hyperplasia with eosinophilia:successful treatment with the antiallergic agent suplatast tosilate. Dermatology 2004;208:176-7.  Back to cited text no. 27    


    Figures

[Figure - 1], [Figure - 2]



 

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