DERMOSCOPY
Dermoscopic Features of Cutaneous Lymphangioma Circumscriptum NICOLA ARPAIA, MD, NICOLETTA CASSANO, MD,
AND
GINO ANTONIO VENA, MD
BACKGROUND Cutaneous lymphangioma circumscriptum (CLC) is clinically characterized by clusters of translucent vesicles that may be filled by blood. OBJECTIVE
To evaluate the dermoscopic features of CLC.
MATERIALS AND METHODS Dermoscopic examination was performed in two cases presenting with discrete translucent and blood-tinged lesions. RESULTS Lesions filled with clear fluid were dermoscopically characterized by light brown lacunas surrounded by paler septa. Lesions tinged with blood were associated with different dermoscopic features depending on the amount of blood content: focal reddish areas inside the lagoons, pink diffuse coloration, reddish to violaceous lacunar structures. CONCLUSIONS On dermoscopy, CLC was characterized by a lacunar pattern. Lesions with a marked hematic content show dermoscopic findings indistinguishable from those of hemangioma. Nicola Arpaia, MD, Nicoletta Cassano, MD, and Gino Antonio Vena, MD, have indicated no significant interest with commercial supporters.
C
utaneous lymphangioma circumscriptum (CLC) is an uncommon benign disorder of the lymphatic channels that is usually congenital, although it may arise at any age, especially during childhood.1–3 Clinically, CLC manifests with discrete or grouped translucent vesicles resembling frog spawn; lesions are asymptomatic and can develop in any part of the body, most frequently in proximal areas of the limbs, buttocks, shoulders, perineum, groin, and axillae. The clear fluid contained in the vesicles can be tinged with blood and the surface of the lesions may show a warty hyperkeratosis.2,4
Removal of CLC frequently results in recurrence or persistence because the extent of involvement is commonly deeper than expected, as shown by magnetic resonance imaging.3,5,6 We report the results of dermoscopic examination of CLC in two patients.
Case Reports Case 1 A 14-year-old girl was referred to our unit for the evaluation of an asymptomatic skin lesion
localized on the proximal area of the right thigh. The mother had noted this lesion 2 years before and reported that, during the last year, its original clear color repeatedly became reddish, causing notable anxiety. Clinical examination showed a dark red papule close to a pale translucent lesion with scanty hematic content (Figure 1). The two lesions had distinct dermoscopic features (Figure 2): the former was characterized by dark red/ bluish lagoons, the latter by light brown lacunas surrounded by paler septa and focally containing mottled reddish areas.
All authors are affiliated with 2nd Unit of Dermatology, MIDIM Department, University of Bari, Italy & 2006 by the American Society for Dermatologic Surgery, Inc. Published by Blackwell Publishing ISSN: 1076-0512 Dermatol Surg 2006;32:852–854 DOI: 10.1111/j.1524-4725.2006.32174.x 852
A R PA I A E T A L
ferential diagnosis can be established by histopathology.
Figure 1. Case 1: Presence of a dark red papule and a pale translucent lesion with scanty blood content (clinical image).
Case 2 A 22-year-old man presented with two coalesced asymptomatic papules on his right buttock, which developed approximately 10 years before. The color of both lesions was initially pale to pink; over the last year, one of these lesions became darker. No substantial modifications of size were observed. On dermoscopy, a double component was noted: one lesion consisted of red violaceous and reddish black lacunar structures (Figure 3), and the other had a diffuse pink color with a few central reddish areas (Figure 4).
behavior. CLC should be differentiated from a great variety of cutaneous lesions, including molluscum contagiosum, warts, vascular and lymphatic lesions (such as hemangioma, angiokeratoma, lymphangioendothelioma, low-grade angiosarcoma),7–9 and adnexal tumors (such as eccrine hidrocystoma).10 In cases of uncertain clinical diagnosis, a dif-
Histologically, CLC is characterized by dilated spaces containing lymphatic fluid and/or red blood cells; dilated lymphatic channels are not only confined to the superficial dermis but frequently extend to the lower dermis and even to the subcutaneous tissue.2–4 The mechanisms responsible for the extravasation of erythrocytes into the dilated lymphatics are still unknown; a possible hypothesis is that microshunts between lymphatic channels and small blood vessels may exist.11 Dermatoscopy of CLC shows the presence of a lacunar/saccular pattern. CLC containing clear fluid is dermoscopically characterized by light brown lacunas delimited by pale septa (Figure 2). Scattered red areas may be seen inside some lagoons, when the intraluminal content of blood is scanty and focal. (Figure 2). In
Discussion CLC is characterized by clusters of translucent small vesicles; their color can vary from clear and pink to dark red or blue depending on the amount of blood that invades the lymphatic channels. Diagnosis is usually simple and based on the clinical aspect and
Figure 2. Case 1: Dermoscopic image of a lesion was characterized by dark red/bluish lagoons, and the other by light brown lacunas surrounded by paler septa and focally containing mottled reddish areas.
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circumscriptum: an example of Whimster’s hypothesis. Pediatr Dermatol 2004;21:652–4. 4. Calonje E, Wilson-Jones E. Vascular tumor. In: Elder D, Elenitsas R, Jaworsky C, Johnson B, editors. Lever’s histopathology of the skin, 8th ed. Philadelphia: Lippincott-Raven, 1997:p. 921–5. 5. McAlvany JP, Jorizzo JL, Zanolli D, et al. Magnetic resonance imaging in the evaluation of lymphangioma circumscriptum. Arch Dermatol 1993;129:194–7. 6. Gupta S, Radotra BD, Javaheri SM, Kumar B. Lymphangioma circumscriptum of the penis mimicking venereal lesions. J Eur Acad Dermatol Venereol 2003;17:598–600.
Figure 3. Case 2: Dermoscopic image of a lesion. Presence of red violaceous and reddish black lacunar structures.
7. Drachman D, Rosen L, Sharaf D, Weissmann A. Postmastectomy low-grade angiosarcoma. An unusual case clinically resembling a lymphangioma circumscriptum. Am J Dermatopathol 1988;10:247–51. 8. Kim JH, Nam TS, Kim SH. Solitary angiokeratoma developed in one area of lymphangioma circumscriptum. J Korean Med Sci 1988;3:169–70. 9. Yiannias JA, Winkelmann RK. Benign lymphangioendothelioma manifested clinically as actinic keratosis. Cutis 2001;67:29–30. 10. Blugerman G, Schavelzon D, D’Angelo S. Multiple eccrine hidrocystomas: a new therapeutic option with botulinum toxin. Dermatol Surg 2003;29:557–9.
Figure 4. Case 2: Dermoscopic image of a lesion in which a diffuse pink color with central reddish areas was observed. The lacunar pattern is suggested by the presence of convex polycyclic borders at the periphery and of pale septa inside the lesion.
case of a homogeneous distribution of modest amounts of blood, the lesion can have a diffuse pink hue that somehow makes the lacunar structures less evident, although the lacunar pattern can be suggested by the presence of convex polycyclic borders at the periphery and of pale septa inside the lesion (Figure 4). When extravasation of blood is marked, dermoscopic findings of CLC are indis-
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tinguishable from those of hemangioma (Figures 2 and 3).12–14 References 1. Flanagan BP, Helwig EB. Cutaneous lymphangioma. Arch Dermatol 1977;113:24–30. 2. Caputo R, Gelmetti C, Annessi G. Pediatric dermatology and dermatopathology, Vol. III. Baltimore Philadelphia: Williams & Wilkins, 1995:p. 269–78. 3. Martinez-Menchon T, Mahiques-Santos L, Febrer-Bosch I, et al. Lymphangioma
11. Franke FE, Steger K, Marks A, et al. Hobnail hemangiomas (targetoid hemosiderotic hemangiomas) are true lymphangiomas. J Cutan Pathol 2004;31:362–7. 12. Kenet RO, Kang S, Kenet BJ, et al. Clinical diagnosis of pigmented lesions using digital epiluminescence microscopy. Grading protocol and atlas. Arch Dermatol 1993;129:157–74. 13. Wang SQ, Katz B, Rabinovitz H, et al. Lessons on dermoscopy #7. The diagnosis was thrombosed hemangioma. Dermatol Surg 2000;26:891–2. 14. Wolf IH. Dermoscopic diagnosis of vascular lesions. Clin Dermatol 2002;20:273–5.
Address correspondence and reprint requests to: Prof. Gino A. Vena, MD, 2nd Unit of Dermatology, MIDIM Department, University of Bari, PoliclinicoFPiazza Giulio Cesare 11, 70124 Bari, Italy, or e-mail:
[email protected].
