Home About us Editorial board Search Ahead of print Current issue Archives Submit article Instructions Subscribe Contacts Login 
  • Users Online: 354
  • Home
  • Print this page
  • Email this page

 Table of Contents  
Year : 2020  |  Volume : 7  |  Issue : 2  |  Page : 96-99

Zosteriform lymphangioma circumscriptum and angiokeratoma: A rare co-occurrence

Department of Dermatology, Venereology and Leprology, Government Medical College and Hospital, Nagpur, Maharashtra, India

Date of Submission18-Aug-2020
Date of Decision18-Oct-2020
Date of Acceptance01-Dec-2020
Date of Web Publication22-Dec-2020

Correspondence Address:
Dr. Vaishali H Wankhade
Department of Dermatology, Venereology and Leprology, Government Medical College and Hospital, Nagpur, Maharashtra
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijdpdd.ijdpdd_96_20

Rights and Permissions

Lymphangioma circumscriptum (LC) is a rare benign hamartomatous malformation of lymph vessel manifesting at birth or during early childhood. Angiokeratomas (AK) are vascular ectasia of papillary dermal blood vessels with secondary epidermal changes. AK and/or LC are one of the common vascular malformations associated with Klippel–Trenaunay syndrome (KTS). The simultaneous appearance of LC and AK has been reported rarely, and the occurrence of LC, AK, and port-wine stain in a patient of KTS is even rarer. Thereby, we report a case of co-occurrence of LC and AK in the same patient without systemic involvement.

Keywords: Angiokeratoma, cryotherapy, lymphangioma circumscriptum, zosteriform

How to cite this article:
Kowe PA, Malpani SS, Wankhade VH, Singh RP. Zosteriform lymphangioma circumscriptum and angiokeratoma: A rare co-occurrence. Indian J Dermatopathol Diagn Dermatol 2020;7:96-9

How to cite this URL:
Kowe PA, Malpani SS, Wankhade VH, Singh RP. Zosteriform lymphangioma circumscriptum and angiokeratoma: A rare co-occurrence. Indian J Dermatopathol Diagn Dermatol [serial online] 2020 [cited 2021 Sep 27];7:96-9. Available from: https://www.ijdpdd.com/text.asp?2020/7/2/96/304343

  Introduction Top

Morris in 1889 coined the term “lymphangioma circumscriptum (LC)” which is the most common type of localized lymphangioma.[1] They constitute around 4% of all vascular malformation and 26% of all benign vascular tumors.[2] The term “angiokeratoma (AK)” was coined by Mibelli in 1889, and solitary or multiple AK is the most common type.[3] Clinically, the two conditions look similar creating a diagnostic difficulty. There are very few case reports of the coexistence of these two entities. There is a single case of simultaneous occurrence of LC, AK, and Klippel–Trenaunay syndrome (KTS) in the same patient reported by Supekar et al., in 2020.[4] We highlight a young female with zosteriform LC and AK which occurred in the same region without systemic involvement, thus ruling out underlying KTS.

  Case Report Top

A 22-year-old female presented with asymptomatic fluid-filled lesions over the left side of the neck extending up to the left shoulder and left upper arm since childhood. There was no history of trauma before the onset of lesions. Her family history was insignificant. On dermatological examination, multiple translucent superficial vesicles coalescing to form bulla giving the characteristic appearance of “frogspawn” were present over the left shoulder region in the distribution of third and fourth cervical (C3, C4) dermatomes ranging from 3 mm to 15 mm in size [Figure 1]a. These lesions were associated with black-colored crusted papules present in the same area which had a history of occasional bleeds [Figure 1]b. Due to varied morphology, a differential diagnosis of LC and AK was kept for both. Dermoscopy using 3Gen Dermlite DL4 (CA, USA) ×10 polarized mode, from vesicles and bulla revealed multiple well-circumscribed rounded whitish-to-yellow areas (lacunae), surrounded by pale septa. Some of these lacunae contained blood characteristically accumulated at the bottom of the lacuna resembling the appearance of “hypopyon sign” (“half-and-half lacuna”) suggestive of cutaneous LC [Figure 2]. Dermoscopy from hyperpigmented crusted lesion revealed dark blue to red lacunae with a whitish veil suggestive of AK [Figure 3]. General and systemic examination was normal. Histopathological examination from the vesicle showed thinning of the epidermis and dermis with dilated endothelial-lined vascular channels with focal lymphocytes in the dermis confirmative of cutaneous LC [Figure 4]a and [Figure 4]b. Skin biopsy from pigmented crusted papule revealed epidermis with hyperkeratotic squamous epithelium with few dilated thin-walled blood vessels in the papillary dermis consistent with a diagnosis of AK [Figure 5]a and [Figure 5]b. X-ray of both shoulder girdle showed no asymmetry and local ultrasonography was unremarkable. Based on history, clinical findings, dermoscopy, and histopathology, a final diagnosis of zosteriform lymphangioma circumsrciptum with AK was made. The patient was started on cryotherapy with only mild improvement after one session and she is still under treatment.
Figure 1: (a) Multiple translucent vesicles and bullae with the characteristic appearance of “frogspawn” over the left shoulder in the distribution of third and fourth cervical (C3, C4) dermatomes (b) hyperpigmented crusted papules in the same region

Click here to view
Figure 2: Multiple well-circumscribed rounded whitish to yellow lacunae (blue arrow) surrounded by pale septa. Lacuna resembling the appearance of “hypopyon sign” (“half-and-half lacuna”) (green arrow)

Click here to view
Figure 3: Dark-blue to red lacunae (yellow and red arrow) with whitish veil (blue arrow) suggestive of angiokeratoma

Click here to view
Figure 4: (a) Skin biopsy from vesicle showing thinning of epidermis and dermis (H and E, ×10) (b) multiple dilated endothelial-lined vascular channels (red arrow) with focal lymphocytes in the dermis (H and E, ×40)

Click here to view
Figure 5: (a) Biopsy from crusted papule showing compact hyperkeratosis, acanthosis (H and E, ×10) (b) multiple dilated vascular spaces in the papillary dermis containing erythrocytes in their lumen (blue arrow) (H and E, ×40)

Click here to view

  Discussion Top

Lymphangiomas also referred to as localized congenital lymphatic malformations (LMs) can be categorized into microcystic (diameter <1 cm) or superficial and macrocystic (diameter >1 cm) or deep. LMs which are macrocystic are usually present at birth in the neck and are called as cystic hygroma.[2] LMs that are localized, microcystic, and superficial within the skin, subcutaneous tissue, and occasionally muscles are called as “LC” which is a misnomer since they communicate with deeper subcutaneous lymphatic cisterns through small channels (especially if the lesion is at the root of a limb). They manifest as fluid-filled pseudovesicles (lymph blister) which may be discrete or may be clustered resembling “frog spawn.” Recurrent oozing is the most common symptom which discharges clear fluid (lymph). Occasionally, the discharge may be red to blue when blood predominates. Commonly affected sites are extremities, abdomen, thigh, etc. Rare sites mentioned in the previous reports are vulva, perineum, scrotum, tongue, and oral mucosa.[5] Dermoscopically, LC shows two patterns: yellow lacunae with surrounding pale septa without the inclusion of blood in it and dark red to blue lacunae filled with blood. In recent years, a new characteristic dermoscopic sign of cutaneous LC has been described and is called “half-and-half lacuna.” It implies dark red to blue lacunae, wherein blood is settled at the bottom of the lacuna due to the gravity of the cellular component of the blood, whereas the serum part remains at the top, resulting in a color transition from light to dark.[6] Histopathology of LC shows some degree of hyperkeratosis with acanthosis and contains multiple dilated lymphatics in the papillary dermis with clear fluid in their lumina. This dermoscopically correlates with yellow lacunae. Less frequently, there may be red blood cells in their lumen corresponding to red-blue lacunae on dermoscopy. Dermal stroma shows lymphocytes as a predominant infiltrate.[7] Differential diagnosis of LC includes AK, viral warts, herpes zoster, verrucous hemangioma, and melanoma.[3],[8],[9],[10] Rarely bacterial superinfection, and malignant transformation such as lymphangioendothelioma, lymphangiosarcoma can complicate LC. Surgical excision is the most definitive treatment for a localized lesion with the lowest recurrence rate. Other modalities of treatment include laser therapy, cryosurgery, radiofrequency ablation, sclerotherapy, and imiquimod 5% cream.[11] AK represents a group of benign cutaneous vascular ectasia with secondary epidermal changes such as hyperkeratosis or acanthosis. It may be localized or diffuse. The localized variants include AK of Mibelli, solitary or multiple AKs, AK circumscriptum, and AK of Fordyce (involvement of scrotum or vulva). The diffuse form is known as AK corporis diffusum commonly seen in Fabry's disease.[12] The exact etiology of AK is unknown but suggested that etiological factors include local trauma and underlying arteriovenous fistula.[12] It manifests clinically as solitary or multiple dark red-blue-colored keratotic papules that tend to bleed. Zaballos et al. demonstrated six dermoscopic structures of AK which include dark lacunae, red lacunae, erythema, whitish veil, peripheral erythema, and hemorrhagic crusts.[3] Histopathology of AK shows hyperkeratosis and acanthosis which corresponds to whitish veil on dermoscopy, wide and dilated vascular spaces in the papillary dermis correlates to red lacunae, vascular spaces which are partially or completely thrombosed corresponds to dark lacunae, and bleeding and erythema corresponds with hemorrhagic crusts and inflammation in the lesion with erythrocyte extravasation in the papillary dermis represents peripheral erythema on dermoscopy.[3] Pyogenic granuloma, seborrheic keratosis, malignant melanoma, pigmented basal cell carcinoma, dermatofibroma, melanocytic nevi, hemangioma, and Spitz-Reed nevi are the differential diagnosis of AK.[9],[10],[13],[14],[15],[16] Treatment options for AK include surgical excision, curettage, diathermy, electrocautery, cryosurgery, and laser therapy.[17] Both LC and AK are common vascular malformations associated with KTS which is a rare congenital disorder characterized by a triad of vascular malformations, bony or soft-tissue hypertrophy involving an extremity and varicose veins which are associated with significant morbidity and mortality. Recently, Supekar et al. in 2020 reported a case of KTS associated with LC and AK which is the only case manifesting as capillary, venous, and arterial malformation.[4] Kemeriz et al. reported the coexistence of cutaneous LC and AK in the same area which postulated that AK might evolve from underlying LC by repeated trauma.[5] In our case, the two entities might have developed independently from one another as the patient denied a history of trauma.

  Conclusion Top

Distinguishing between LC and AK may be challenging clinically but can be easily differentiated with dermoscopy. Thus, dermoscopy plays an important bedside tool that helps in diagnosing similar-looking conditions. Furthermore, one should be vigilant to rule out underlying KTS when LC and AK coexist as these are common associations of KTS.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that name and initials will not be published, and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.


We would like to thank Dr Dharitri Bhat (Associate professor, Department of Pathology, Government Medical College and Hospital Nagpur) for helping us with histopathology report.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Morris M. Lymphangioma circumscriptum. In: Unna PG, Morris M, Duhring LA, Leloir H, editors. International Atlas of rare Skin Diseases. London: Lewis; 1889. p. 1-4.  Back to cited text no. 1
Peter S, Mortimer, Gordon K. Disorders of the lymphatic vessels. In: Griffiths C, Barker J, Bleiker T, Chalmer R, Creamer D, editors. Rook's Textbook of Dermatology. 9th ed. Oxford: Wiley Blackwell; 2016. p. 105.35.  Back to cited text no. 2
Zaballos P, Daufı C, Susana P, Argenziano G, Ramirez D, Cabo H, et al. Dermoscopy of solitary angiokeratoma. Arch Dermatol 2007;143:318-25.  Back to cited text no. 3
Supekar BB, Chopkar AD, Wankhade VH, Singh RP, Bhat DM, Suresh P. Klippel-Trenaunay Syndrome with Arterio-veno-lymphatic Malformation: A rare presentation. Indian Dermatol Online J 2020;11:404-8.  Back to cited text no. 4
  [Full text]  
Kemeriz F, Muzeyyen G, Aysun G, Murat A. Coexistence of lymphangioma circumscriptum and angiokeratoma in the same area: A case report. Dermatol Case Rep 2019;314:153  Back to cited text no. 5
Jha AK, Lallas A, Sonthalia S. Dermoscopy of cutaneous lymphangioma circumscriptum. Dermatol Pract Concept 2017;7:37-8.  Back to cited text no. 6
Calonje E. Vascular tumors: Tumors and tumor-like conditions of blood vessels and lymphatics. In: Elder DE, editor. Lever's Histopathology of the Skin. 10th ed. Philadelphia: Lippincott Williams & Wilkins; 2008. p. 1044.  Back to cited text no. 7
Nayak SS, Mehta HH, Gajjar PC, Nimbark VN. Dermoscopy of general dermatological conditions in Indian population: A descriptive study. Clin Dermatol Rev 2017;1:41-51.  Back to cited text no. 8
  [Full text]  
Prabhakar V, Kaliyadan F. A case of verrucous hemangioma and its dermoscopic features. Indian Dermatol Online J 2015;6:S56-8.  Back to cited text no. 9
Kato J, Horimoto K, Sato S, Minowa T, Uhara H. Dermoscopy of melanoma and non-melanoma skin cancers. Front Med 2019;6:180.  Back to cited text no. 10
Puri N. Treatment options of lymphangioma circumscriptum. Indian Dermatol Online J 2015;6:293-4.  Back to cited text no. 11
[PUBMED]  [Full text]  
Goldsmith PC. Dermatoses resulting from disorders of the veins and arteries. In: Griffiths C, Barker J, Bleiker T, Chalmer R, Creamer D, editors. Rook's Textbook of Dermatology. 9th ed. Oxford: Wiley Blackwell; 2016. p. 103-13.  Back to cited text no. 12
Camara MF, Pinheiro PM, Jales RD, da Trindade Neto PB, Costa JB, de Sousa VL. Multiple dermatofibromas: Dermoscopic patterns. Indian J Dermatol 2013;58:243.  Back to cited text no. 13
[PUBMED]  [Full text]  
Cengiz FP, Emiroglu N, Ozkaya DB, Su O, Onsun N. Dermoscopic Features of Small, Medium, and Large-Sized Congenital Melanocytic Nevi. Ann Dermatol 2017;29:26-32.  Back to cited text no. 14
Oiso N, Kawada A. Dermoscopy of infantile hemangioma. Pediatr Dermatol 2011;28:592-3.  Back to cited text no. 15
Lallas A, Apalla Z, Ioannides D, Lazaridou E, Kyrgidis A, Broganelli P, et al. Update on dermoscopy of spitz/reed naevi and management guidelines by the International Dermoscopy Society. Br J Dermatol 2017;177:645-55.  Back to cited text no. 16
Das A, Mondal AK, Saha A, Chowdhury SN, Gharami RC. Angiokeratoma circumscriptum neviforme: An entity, few and far between. Indian Dermatol Online J 2014;5:472-4.  Back to cited text no. 17
  [Full text]  


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


Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)

  In this article
Case Report
Article Figures

 Article Access Statistics
    PDF Downloaded73    
    Comments [Add]    

Recommend this journal