Case report
Dermoscopy of angioma serpiginosum: a case report Alireza Ghanadan1, MD, Kambiz Kamyab-Hesari1, MD, Homayoun Moslehi2, MD, and Ata Abasi3, MD
1 Department of Dermatopathology, Razi Hospital, Tehran University of Medical Sciences, Tehran, Iran, 2Department of Pathology, University of California, San Francisco, CA, USA , and 3Department of Pathology, Cancer Institute, Imam Khomeini Complex Hospital, Tehran University of Medical Sciences, Tehran, Iran
Correspondence Alireza Ghanadan, MD Department of Dermatopathology Razi Hospital Vahdate Eslami Ave PO Box: 1199663911 Tehran, Iran E-mail: [email protected] Funding: All Funding sources are provided by Tehran University of Medical Sciences. Conflicts of interest: None.
Introduction Angioma serpiginosum (AS) is an uncommon vascular nevus involving the superficial capillaries. It is characterized by progressive proliferation of ectatic capillaries in the papillary and upper dermis.1 Beginning in childhood, it usually stabilizes in adulthood after the initial growth and sometimes completely or partially regresses. Females are more affected, and lesions are predominantly distributed over the lower limbs or buttocks grouped in serpiginous or linear arrangement. Sometimes, the distribution of lesions may even be reminiscent of the lines of Blaschko.2 The most important histopathological finding is the presence of dilated and tortuous capillaries in the papillary dermis.1 We present a case of AS with linear distribution accompanying dermoscopic findings as red lagoons and comma hairpin vessels. Case report A 31-year-old man was admitted to our outpatient clinic for linear asymptomatic red-colored areas covering the ª 2014 The International Society of Dermatology
upper right arm and left thigh since childhood. The lesion had gradually extended along the lower arm and trunk during the past several years. Clinical examination revealed multiple, nonblanchable, red to violaceous macules involving his entire right arm being grouped in patches forming a net-like pattern. The lesions were arranged in linear and serpiginous patterns following the lines of Blaschko. The lesions with similar morphology were distributed on the upper trunk and upper back. Fundoscopic examination was performed, and no vascular abnormality was identified. No other family members had similar disorders. There was no accompanying pruritus and excoriation. Dermoscopy Dermoscopic images were obtained by means of Microderm (Visiomed AG, Bielefeld, Germany) with a 10- and 30-fold magnification of the image. Dermoscopic images were analyzed by an experienced observer (HM) applying the modified pattern analysis, searching for global and local dermoscopic features. In dermoscopy, numerous International Journal of Dermatology 2014, 53, 1505–1507
1505
1506
Case report
Dermoscopy of angioma serpiginosum
Ghanadan et al.
(a)
(a)
(b)
(b)
Figure 1 (a) Relatively well-demarcated, round to oval red lagoons. (b) Comma, hairpin-like vessels among red lagoons
small, relatively well-demarcated, round to oval red lagoons were detected (Fig. 1a). Accompanied with them were comma, hairpin-like vessels and patchy pigmentation dispersed through the background (Fig 1b). Histopathology A skin biopsy was performed from a typical lesion on the back. Histopathologic examination revealed lobular proliferation of dilated capillary vessels arranged in the papillary dermis. The epidermis was normal, and finger-like rete ridges embraced the dilated capillary vessels (Fig. 2a,b). No extravasation of erythrocytes, hemosiderin, or inflammation was seen. Discussion First described by Hutchinson in 1889 as a form of serpiginous and infective nevoid disease, Radcliffe-Crocker proposed the term of angioma serpiginosum in 1893.3 International Journal of Dermatology 2014, 53, 1505–1507
Figure 2 (a) Dilated capillary vessels arranged in papillary dermis (magnification 9 4, hematoxylin and eosin). (b) Finger-like rete ridges embracing the dilated capillary vessels (magnification 9 10, hematoxylin and eosin)
Beginning in childhood, AS is a rare asymptomatic condition with a predilection to females, presenting with a vascular nevus or malformation.4 Most cases occur sporadically, but an autosomal dominant pattern of inheritance has been reported in two families.5 Clinically, the lesion is characterized by erythematous to violaceous multiple, grouped, punctate macules growing in serpiginous and gyrate patterns. Usually, it appears on the lower limb, often starts unilaterally on Blaschko lines, but progresses widely.2,6,7 Infrequently, extensive involvement of the trunk and extremities has been reported.8 AS often shows a segmental arrangement strongly suggesting mosaicism.2 Dermoscopy is a useful procedure for evaluating vascular lesions. Characteristic findings are demarcated red lagoons due to dilated vascular spaces within the papillary or superficial reticular dermis. Red lagoons are also described in other vascular tumors, including ª 2014 The International Society of Dermatology
Ghanadan et al.
hemangioma and angiokeratoma.9 To the best of our knowledge, there are only two reports of dermoscopy as a procedure for the diagnosis and evaluation of AS.10,11 Although pigmented purpuric dermatoses and Henoch– Schoenlein purpura (HSP) are in clinical differentiation, dermoscopy could distinguish them from AS. Ohnishi et al.11 reported typical red lagoons in two cases of AS. In addition to this typical dermoscopic feature, we found comma, hairpin-like vessels scattered among red lagoons (Fig. 1b). Pinpoint and hairpin vessels are variants of the same basic vascular structure of loop-like vessels.12 Vascular loops are encountered in many lesions mainly in melanoma and keratinizing tumors. Comma vessels are encountered in compound and dermal melanocytic nevus, but hairpin vessels are seen in melanocytic nevus and seborrheic keratosis.12,13 Therefore, the spectrum of dermoscopic features of the comma, hairpin-like vessels should be evaluated in other comparative studies. The differential diagnoses of AS are pigmented purpura, unilateral nevoid telangiectasia, port-wine stain, and angiokeratoma corporis diffusum.2,6,14 All these diseases have dilated capillaries in the dermis, except for pigmented purpuric dermatoses. AS can be differentiated from pigmented purpura by the absence of extravasated erythrocytes, hemosiderin pigment, and inflammation. Demarcated red lagoons are not seen in pigmented purpura. Histopathologically, there are solitary or grouped dilated thick-wall capillary vessels in the papillary dermis. This histopathologic feature could be seen in unilateral nevoid telangiectasia and port-wine stain, but clinically they could be distinguishing from AS. Angiokeratoma corporis diffusum is an X-linked disorder showing hyperkeratosis and papillomatosis along with electron-dense material in the lysosomes of endothelial cells in electron microscopy. Most authors believe that AS is not a simple telangiectasia of pre-existing vessels but represents a type of capillary nevus, which has a tendency to manifest itself as capillary dilatation and proliferation.4,8,15 In conclusion, we presented a case of AS with unilateral distribution of the lesions arranged in linear and serpiginous patterns following the lines of Blaschko occurring on the right upper trunk and lower limbs.
ª 2014 The International Society of Dermatology
Dermoscopy of angioma serpiginosum
Case report
References 1 Calonje E. Vascular tumors. In: Elder DE, Elenitsas R, Johnson BL Jr, eds. Lever’s Histopathology of the Skin, 10th edn. Philadelphia, PA: Lippincott Williams & Wilkins, 2008: 1012. 2 Chen W, Liu TJ, Yang YC, et al. Angioma serpiginosum arranged in a systematized segmental pattern suggesting mosaicism. Dermatology 2006; 213: 236–238. 3 Stevenson MJR, Lincoln CS. Angioma serpiginosum. Arch Dermatol 1967; 95: 16–22. 4 Requena L, Sangueza O. Vascular anomalies of the skin. J Am Acad Dermatol 1997; 37: 887–920. 5 Marriott PJ, Munro DD, Ryan T. Angioma serpiginosum: familial incidence. Br J Dermatol 1975; 93: 701–706. 6 Al Hawsawi K, Al Aboud K, Al Aboud D, et al. Linear angioma serpiginosum. Pediatr Dermatol 2003; 20: 167– 168. 7 Poenitz N, Koenen W, Utikal J, et al. Angioma serpiginosum following the lines of Blaschko – an effective treatment with the IPL technology. J Dtsch Dermatol Ges 2006; 4: 650–653. 8 Katta R, Wagner A. Angioma serpiginosum with extensive cutaneous involvement. J Am Acad Dermatol 2000; 42: 384–385. 9 Stolz W, Braun-Falco O, Bilek P, et al. Color Atlas of Dermoscopy. Oxford: Blackwell Science, 1994. 10 Ilknur T, Fetil E, Akarsu S, et al. Angioma serpiginosum: dermoscopy for diagnosis, pulsed dye laser for treatment. J Dermatol 2006; 33: 252–255. 11 Ohnishi T, Nagayama T, Morita T, et al. Angioma serpiginosum: a report of 2 cases identified using epiluminescence microscopy. Arch Dermatol 1999; 135: 1366–1368. 12 Kreusch J. Vascular patterns in skin tumors. Clin Dermatol 2002; 20: 248–254. 13 Argenziano G, Zalaudek I, Corona R, et al. Vascular structures in skin tumors: a dermoscopy study. Arch Dermatol 2004; 140: 1485–1489. 14 Tsuruta D, Someda Y, Sowa J, et al. Angioma serpiginosum with extensive lesions associated with retinal vein occlusion. Dermatology 2006; 213: 256–258. 15 Long CC, Lanigan SW. Treatment of angioma serpiginosum using a pulsed tunable dye laser. Br J Dermatol 1997; 136: 631–632.
International Journal of Dermatology 2014, 53, 1505–1507
1507
Dermoscopy of angioma serpiginosum: a case report Alireza Ghanadan1, MD, Kambiz Kamyab-Hesari1, MD, Homayoun Moslehi2, MD, and Ata Abasi3, MD
1 Department of Dermatopathology, Razi Hospital, Tehran University of Medical Sciences, Tehran, Iran, 2Department of Pathology, University of California, San Francisco, CA, USA , and 3Department of Pathology, Cancer Institute, Imam Khomeini Complex Hospital, Tehran University of Medical Sciences, Tehran, Iran
Correspondence Alireza Ghanadan, MD Department of Dermatopathology Razi Hospital Vahdate Eslami Ave PO Box: 1199663911 Tehran, Iran E-mail: [email protected] Funding: All Funding sources are provided by Tehran University of Medical Sciences. Conflicts of interest: None.
Introduction Angioma serpiginosum (AS) is an uncommon vascular nevus involving the superficial capillaries. It is characterized by progressive proliferation of ectatic capillaries in the papillary and upper dermis.1 Beginning in childhood, it usually stabilizes in adulthood after the initial growth and sometimes completely or partially regresses. Females are more affected, and lesions are predominantly distributed over the lower limbs or buttocks grouped in serpiginous or linear arrangement. Sometimes, the distribution of lesions may even be reminiscent of the lines of Blaschko.2 The most important histopathological finding is the presence of dilated and tortuous capillaries in the papillary dermis.1 We present a case of AS with linear distribution accompanying dermoscopic findings as red lagoons and comma hairpin vessels. Case report A 31-year-old man was admitted to our outpatient clinic for linear asymptomatic red-colored areas covering the ª 2014 The International Society of Dermatology
upper right arm and left thigh since childhood. The lesion had gradually extended along the lower arm and trunk during the past several years. Clinical examination revealed multiple, nonblanchable, red to violaceous macules involving his entire right arm being grouped in patches forming a net-like pattern. The lesions were arranged in linear and serpiginous patterns following the lines of Blaschko. The lesions with similar morphology were distributed on the upper trunk and upper back. Fundoscopic examination was performed, and no vascular abnormality was identified. No other family members had similar disorders. There was no accompanying pruritus and excoriation. Dermoscopy Dermoscopic images were obtained by means of Microderm (Visiomed AG, Bielefeld, Germany) with a 10- and 30-fold magnification of the image. Dermoscopic images were analyzed by an experienced observer (HM) applying the modified pattern analysis, searching for global and local dermoscopic features. In dermoscopy, numerous International Journal of Dermatology 2014, 53, 1505–1507
1505
1506
Case report
Dermoscopy of angioma serpiginosum
Ghanadan et al.
(a)
(a)
(b)
(b)
Figure 1 (a) Relatively well-demarcated, round to oval red lagoons. (b) Comma, hairpin-like vessels among red lagoons
small, relatively well-demarcated, round to oval red lagoons were detected (Fig. 1a). Accompanied with them were comma, hairpin-like vessels and patchy pigmentation dispersed through the background (Fig 1b). Histopathology A skin biopsy was performed from a typical lesion on the back. Histopathologic examination revealed lobular proliferation of dilated capillary vessels arranged in the papillary dermis. The epidermis was normal, and finger-like rete ridges embraced the dilated capillary vessels (Fig. 2a,b). No extravasation of erythrocytes, hemosiderin, or inflammation was seen. Discussion First described by Hutchinson in 1889 as a form of serpiginous and infective nevoid disease, Radcliffe-Crocker proposed the term of angioma serpiginosum in 1893.3 International Journal of Dermatology 2014, 53, 1505–1507
Figure 2 (a) Dilated capillary vessels arranged in papillary dermis (magnification 9 4, hematoxylin and eosin). (b) Finger-like rete ridges embracing the dilated capillary vessels (magnification 9 10, hematoxylin and eosin)
Beginning in childhood, AS is a rare asymptomatic condition with a predilection to females, presenting with a vascular nevus or malformation.4 Most cases occur sporadically, but an autosomal dominant pattern of inheritance has been reported in two families.5 Clinically, the lesion is characterized by erythematous to violaceous multiple, grouped, punctate macules growing in serpiginous and gyrate patterns. Usually, it appears on the lower limb, often starts unilaterally on Blaschko lines, but progresses widely.2,6,7 Infrequently, extensive involvement of the trunk and extremities has been reported.8 AS often shows a segmental arrangement strongly suggesting mosaicism.2 Dermoscopy is a useful procedure for evaluating vascular lesions. Characteristic findings are demarcated red lagoons due to dilated vascular spaces within the papillary or superficial reticular dermis. Red lagoons are also described in other vascular tumors, including ª 2014 The International Society of Dermatology
Ghanadan et al.
hemangioma and angiokeratoma.9 To the best of our knowledge, there are only two reports of dermoscopy as a procedure for the diagnosis and evaluation of AS.10,11 Although pigmented purpuric dermatoses and Henoch– Schoenlein purpura (HSP) are in clinical differentiation, dermoscopy could distinguish them from AS. Ohnishi et al.11 reported typical red lagoons in two cases of AS. In addition to this typical dermoscopic feature, we found comma, hairpin-like vessels scattered among red lagoons (Fig. 1b). Pinpoint and hairpin vessels are variants of the same basic vascular structure of loop-like vessels.12 Vascular loops are encountered in many lesions mainly in melanoma and keratinizing tumors. Comma vessels are encountered in compound and dermal melanocytic nevus, but hairpin vessels are seen in melanocytic nevus and seborrheic keratosis.12,13 Therefore, the spectrum of dermoscopic features of the comma, hairpin-like vessels should be evaluated in other comparative studies. The differential diagnoses of AS are pigmented purpura, unilateral nevoid telangiectasia, port-wine stain, and angiokeratoma corporis diffusum.2,6,14 All these diseases have dilated capillaries in the dermis, except for pigmented purpuric dermatoses. AS can be differentiated from pigmented purpura by the absence of extravasated erythrocytes, hemosiderin pigment, and inflammation. Demarcated red lagoons are not seen in pigmented purpura. Histopathologically, there are solitary or grouped dilated thick-wall capillary vessels in the papillary dermis. This histopathologic feature could be seen in unilateral nevoid telangiectasia and port-wine stain, but clinically they could be distinguishing from AS. Angiokeratoma corporis diffusum is an X-linked disorder showing hyperkeratosis and papillomatosis along with electron-dense material in the lysosomes of endothelial cells in electron microscopy. Most authors believe that AS is not a simple telangiectasia of pre-existing vessels but represents a type of capillary nevus, which has a tendency to manifest itself as capillary dilatation and proliferation.4,8,15 In conclusion, we presented a case of AS with unilateral distribution of the lesions arranged in linear and serpiginous patterns following the lines of Blaschko occurring on the right upper trunk and lower limbs.
ª 2014 The International Society of Dermatology
Dermoscopy of angioma serpiginosum
Case report
References 1 Calonje E. Vascular tumors. In: Elder DE, Elenitsas R, Johnson BL Jr, eds. Lever’s Histopathology of the Skin, 10th edn. Philadelphia, PA: Lippincott Williams & Wilkins, 2008: 1012. 2 Chen W, Liu TJ, Yang YC, et al. Angioma serpiginosum arranged in a systematized segmental pattern suggesting mosaicism. Dermatology 2006; 213: 236–238. 3 Stevenson MJR, Lincoln CS. Angioma serpiginosum. Arch Dermatol 1967; 95: 16–22. 4 Requena L, Sangueza O. Vascular anomalies of the skin. J Am Acad Dermatol 1997; 37: 887–920. 5 Marriott PJ, Munro DD, Ryan T. Angioma serpiginosum: familial incidence. Br J Dermatol 1975; 93: 701–706. 6 Al Hawsawi K, Al Aboud K, Al Aboud D, et al. Linear angioma serpiginosum. Pediatr Dermatol 2003; 20: 167– 168. 7 Poenitz N, Koenen W, Utikal J, et al. Angioma serpiginosum following the lines of Blaschko – an effective treatment with the IPL technology. J Dtsch Dermatol Ges 2006; 4: 650–653. 8 Katta R, Wagner A. Angioma serpiginosum with extensive cutaneous involvement. J Am Acad Dermatol 2000; 42: 384–385. 9 Stolz W, Braun-Falco O, Bilek P, et al. Color Atlas of Dermoscopy. Oxford: Blackwell Science, 1994. 10 Ilknur T, Fetil E, Akarsu S, et al. Angioma serpiginosum: dermoscopy for diagnosis, pulsed dye laser for treatment. J Dermatol 2006; 33: 252–255. 11 Ohnishi T, Nagayama T, Morita T, et al. Angioma serpiginosum: a report of 2 cases identified using epiluminescence microscopy. Arch Dermatol 1999; 135: 1366–1368. 12 Kreusch J. Vascular patterns in skin tumors. Clin Dermatol 2002; 20: 248–254. 13 Argenziano G, Zalaudek I, Corona R, et al. Vascular structures in skin tumors: a dermoscopy study. Arch Dermatol 2004; 140: 1485–1489. 14 Tsuruta D, Someda Y, Sowa J, et al. Angioma serpiginosum with extensive lesions associated with retinal vein occlusion. Dermatology 2006; 213: 256–258. 15 Long CC, Lanigan SW. Treatment of angioma serpiginosum using a pulsed tunable dye laser. Br J Dermatol 1997; 136: 631–632.
International Journal of Dermatology 2014, 53, 1505–1507
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