Lymphangioma Circumscriptum Associated with Milroy\'s Disease

EJVES Extra 5, 26–27 (2003) doi: 10.1016/S1533-3167(03)00023-2, available online at http://www.sciencedirect.com on 1

55

2

56

3

57

4

58

5

59

6

SHORT REPORT

7 8 9 10 11 12

Lymphangioma Circumscriptum Associated with Milroy’s Disease

13

16 17

61 62 63 64 65 66

B. Aksakal and M. O. Oztas*

67

Fatih University School of Medicine, Istanbul, Turkey

69

14 15

60

Key Words: Lymphangioma circumscriptum; Milroy’s disease; Skin.

68 70 71

18

72

19

73

20 21

Introduction

22 23 24 25 26 27 28 29 30 31 32 33 34

The term lymphangioma circumscriptum (LC) was first used by Morris in 1889. It is a benign disorder of lymphatic channels which is localized to an area of skin, subcutaneous tissue or sometimes muscle. It is considered to be a circumscribed developmental defect of lymphatic tissue in the dermis. In Milroy’s disease (MD), there is primary lymphoedema due to the total absence of skin lymphatics and it is characterized by peripheral œdema of the lower extremities at birth or in early childhood. The occurrence of LC and MD was thought to be interesting and is presented here.

varying sizes from 2 mm to 1 cm were seen (Fig. 2). The translucent papules were slightly pinkish or skin colored and the first impression was of a viral infection. Histopathologically, there were numerous dilated lymphatics in the superficial and papillary dermis and a diagnosis of LC was made.

74 75 76 77 78 79 80 81 82 83 84 85 86 87 88

35

89

36

90

37 38 39 40 41 42 43 44 45 46 47 48 49 50 51 52 53 54

91

Case Report

92 93

A 14 year old male patient visited our clinic suffering from severe bilateral lower limb oedema and clear papules localized on the scrotum from which a serous leakage occurred with trauma. There were no subjective symptoms. Dynamic lymphangioscintigraphy (LAS) of the lower limbs was performed and the absence of lymphatic channels was demonstrated; a case of MD was diagnosed. Dynamic LAS of the inguinal lymphatics was normal. There was no family history of lymphoedema. On dermatological examination, severe lymphoedema of the lower limbs (Fig. 1) and numerous translucent papules localized mainly on the scrotal area and partly on the glans penis with *Corresponding author. Dr Pinar Oztas, Fatih University School of Medicine.

94 95 96 97 98 99 100 101 102 103 104 105 106

Fig. 1. Lymphœdema located on the lower limbs of the patient.

1533–3167/03/000026 + 02 $35.00/0 q 2003 Elsevier Science Ltd. All rights reserved.

107 108

Lymphangioma Circumscriptum Associated with Milroy’s Disease

109 110 111 112 113 114 115 116 117 118 119 120 121 122 123 124 125 126 127 128 129 130 131 132 133 134 135 136 137 138 139 140 141 142 143 144 145 146 147 148 149 150 151 152 153 154

Fig. 2. Scrotal and penile shiny lymphangiomata.

Discussion LC may be present at any age and may or may not be associated with lymphœdema of the lower limb. It is accepted as a developmental malformation in infancy but it can appear at any age. In adulthood, identical lesions with LC can be seen in chronic lymphœdema cases or following radiotherapy. Such lesions are best identified as lymphangiectasia. Acquired lymphangiectasis is a dilatation of lymphatic vessels that can be encountered as a complication of surgical intervention or radiation therapy for malignancy. Acquired lymphangiectasis shares clinical and histologic features with the congenital lesion, LC.1 We accepted our case as LC because of the early onset of lesions with compatible histopathology. The lesions of LC present as numerous, vesicle-like, often verrucous lesions. Some lesions are purplish because of a hemangiomatous component. Lesions vary between less than 1 cm2 to larger than many square centimeters and are usually asymptomatic. LC may occur on any anatomic site, but has a predilection for the chest, thigh, buttock, or axilla.1 In our case, the papules were verrucous and localized on the scrotum and penis without any subjective symptoms. MD is characterized by peripheral œdema of the lower extremities at birth or in early childhood which is due to the complete aplasia of skin lymphatics. The diagnosis of MD can be made by imaging the

27

lymphatic channels. There is no need for histopathologic examination.1 The ætiology of LC is still obscure, but it is noteworthy that it usually follows an obstruction. The skin lymphatic system consists of the initial lymphatics, lymphatic precollectors, collectors and lymphatic ducts; these in turn convey the lymph to the regional lymph nodes. In lymphœdema, including cases of MD, various types of congenital and acquired abnormalities can lead to lymphatic hypertension and lymphostasis. LC primarily affects the subcutaneous tissue in the form of cystic dilatation of lymphatic channels without general lymphatic communication and the skin lesions in LC are probably secondary to increased intraluminal pressure due to obstruction. There is no systemic lymphatic communication in LC. A high recurrence rate after surgical excision suggests the possible pathology; lymphostasis at higher levels of the lymphatic system as in MD.2 In our case we believe that high pressure caused by MD led to the formation of the lesions of LC. Many isolated cases of MD and LC appear in the literature.3,4 No association between these two conditions has previously been reported in the current literature except for a case report describing vulval LC lesions associated with unilateral leg oedema.5 Further studies are still needed to elucidate the precise underlying pathological cause of LC.

163 164 165 166 167 168 169 170 171 172 173 174 175 176 177 178 179 180 181 182 183 184 185 186 187 188 189 190 191 192 193

References 1 Calonje E, Wilson-Jones E. Vascular tumors. In: Elder D, Elenitsas R, Jaworsky C, Johnson B, eds. Lever’s Histopathology of the Skin, 8th edn. Lippincott-Raven: Philadelphia, 1997: 889–932. 2 Szuba A, Rockson SG. Lymphedema: anatomy, physiology and pathogenesis. Vasc Med 1997; 2(4): 321–326. 3 Schwab J, Baroody F. Lymphangioma circumscriptum: an unusual oral presentation. Clin Pediatr (Phila) 1999; 38(10): 619–620. 4 Bardazzi F, Orlandi C, Antuono D, Patrizi A. Lymphangioma circumscriptum of the penis. Sex Transm Infect 1998; 74(4): 303–304. 5 Esquivias Gomez JI, Miranda-Romero A, Cuadrada Valles C, Bajo del Pozo C, Sanchez Sambucety P, Martinez Fernandez M, Aragoneses Fraile H, Munoz M. Lymphangioma circumscriptum of the vulva. Cutis 2001; 67: 229 –232.

194 195 196 197 198 199 200 201 202 203 204 205 206 207

Accepted 13 November 2002

208

155

209

156 157

210 211

158

212

159

213

160

214

161

215

162

216 EJVES Extra, 2003