|Year : 1992 | Volume
| Issue : 2 | Page : 120-121
Angiokeratoma circumscriptum : Some unusual features
S Talwar, MS Suresh, PK Jha
Source of Support: None, Conflict of Interest: None
A case of angiokeratoma circumscriptum in 55-year old female involving both breasts and intermammary area is reported. Late onset, unusual site, bilateral involvement and associated extensive telangiectasia are interesting features in this case.
Keywords: Angiokeratoma circumscriptum
|How to cite this article:|
Talwar S, Suresh M S, Jha P K. Angiokeratoma circumscriptum : Some unusual features. Indian J Dermatol Venereol Leprol 1992;58:120-1
|How to cite this URL:|
Talwar S, Suresh M S, Jha P K. Angiokeratoma circumscriptum : Some unusual features. Indian J Dermatol Venereol Leprol [serial online] 1992 [cited 2020 Apr 8];58:120-1. Available from: http://www.ijdvl.com/text.asp?1992/58/2/120/3769
| Introduction|| |
Angiokeratoma circumscriptum (AC) is a rare disorder. Telangiectasia of papillary capillaries acquired as a result of trauma is considered as basic aetiopathogenetic factor in these cases. Usually the lesions are present at birth,  in some it appears during childhood  or adulthood.  Usually the lesions measure a few centimeters and are unilateral.  Majority of the lesions are seen over lower leg and foot. We are reporting a case of AC with some unusual features.
| Case Report|| |
A 55-year-old female presented with purple coloured eruptions over both breasts and intermammary area of 4 years duration. On scratching, there was bleeding from some of these lesions occasionally. No family history of such illness could be elicited. Dermatological examination revealed extensive reticulate telangiectasia involving both the breasts and intermammary area. In between there were multiple purple coloured papular lesions and blood filled cystic nodules which had coalesced in the intermammary area to form a plaque [Figure - 1]. There was no evidence of haemorrhage in skin/ mucous membrane or vasomotor disturbance. Cerebellar functions were normal and examination of eye, oral mucosa, nasal mucosa and other systems did not reveal any abnormality. Haemogram, urine analysis and LFT were within normal limits. Skin biopsy revealed hyperkeratosis, irregular acanthosis, papillomatosis. Dilated capillary spaces lined by single layer of endothelial cells were seen both in papillary dermis and reticular dermis.
| Comments|| |
Late onset, unusual site, bilateral involvement, intermittent bleeding and associated extensive telangiectasia are certain unusual features observed in this case. Onset of AC in late age has been rarely reported.  Involvement of trunk in angiokeratoma was found to be 4 percent  while in AC trunk involvement is regarded still rarer. Intermittent bleeding following irritation of the lesions Is seen in 25 percent cases only.  Its association has been reported with naevus flammeus,  cavernous haemangioma,  Klippel-Trιnaunay syndrome,  angioma of spinal cord (Cobb syndrome)  sub and cornu cutaneum. sub Association with extensive telangiectasia as observed in our case has not been reported earlier. No specific history of trauma could be elicited. Breast feeding for long duration to her 6 children, youngest being 7 years, might have been a traumatizing factor.
| References|| |
|1.||Imperial R, Helwig EB. Angiokeratoma- a clinicopathologicai study. Arch Dermatol 1967; 95: 166-75. [PUBMED] |
|2.||Dammert K. Angiokeratosis naeviformis, a form of naevus telangiectaticus lateralis (naevus flammeus). Dermatologica 1965; 130: 17-39. [PUBMED] |
|3.||Knath W, Knath- Born RC, Boergen G. Uber das angiokeratoma corporis circumscriptum naeviforme der stammhaut. Hautarzt 1963; 14 : 452-62. |
|4.||Lynch PJ, Kosanovich M. Angiokeratoma circumscriptum. Arch Dermatol 1967; 96: 665-8. [PUBMED] |
|5.||Bruce DH. Angiokeratoma circumscriptum and angiokeratoma scroti. Arch Dermatol 1960; 81 : 388-93. [PUBMED] |
|6.||Fischer H, Friederich HC. Angiokeratoma corporis circumscriptum naeviforme mit venekitasien and Osteohypertrophic. Dermatol Wochenschr 1965; 151 : 297-306. [PUBMED] |
|7.||Zola L, Mumenthaler M. Cobb Syndrome. Dermatologica 1981; 163: 417-25. |
|8.||Srinivas CR, Ramnane M, Naik RPC, et al. Spontaneous bleeding, cornu cutaneum, nevus flammeus in angiokeratoma circumscriptum. Ind J Dermatol Venereal Leprol 1987; 53: 118-9. |
[Figure - 1]