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| CASE REPORT |
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| Year : 2022 | Volume
: 9
| Issue : 1 | Page : 108-110 |
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Angioma serpiginosum: Clinico-dermoscopic histopathology correlation
Pankaj Das1, Sandeep Arora1, Gautam Kumar Singh1, GB Prashantha1, Sanghita Barui2, Vikram Singh3
1 Department of Dermatology, Base Hospital Delhi Cantt and Army College of Medical Sciences, New Delhi, India
2 Department of Pathology, Base Hospital Delhi Cantt and Army College of Medical Sciences, New Delhi, India
3 Department of Pathology, Army Hospital Research and Referral, New Delhi, India
| Date of Submission | 02-Jul-2021 |
| Date of Acceptance | 28-Sep-2021 |
| Date of Web Publication | 23-Mar-2022 |
Correspondence Address:
Pankaj Das
Department of Dermatology, Base Hospital Delhi Cantt and Army College of Medical Sciences, New Delhi
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/ijves.ijves_73_21
Angioma serpiginosum (AS) is an uncommon vascular anomaly with unknown etiopathogenesis. It typically presents as asymptomatic unilateral red-colored macules and papules in linear serpiginous pattern during the first two decades of life. It most frequently affects lower limbs followed by gluteal area. We present a case of AS affecting primarily one-half of the trunk and the ipsilateral limb. The diagnosis was confirmed with dermoscopy that revealed characteristic well-defined round-to-oval red lagoons representing dilated capillaries in the papillary dermis on histopathology.
Keywords: Angioma serpiginosum, case report, dermoscopy, progressive patchy cutaneous malformation
How to cite this article:
Das P, Arora S, Singh GK, Prashantha G B, Barui S, Singh V. Angioma serpiginosum: Clinico-dermoscopic histopathology correlation. Indian J Vasc Endovasc Surg 2022;9:108-10 |
How to cite this URL:
Das P, Arora S, Singh GK, Prashantha G B, Barui S, Singh V. Angioma serpiginosum: Clinico-dermoscopic histopathology correlation. Indian J Vasc Endovasc Surg [serial online] 2022 [cited 2022 May 28];9:108-10. Available from: https://www.indjvascsurg.org/text.asp?2022/9/1/108/340507 |
| Introduction | |  |
Angioma serpiginosum (AS), also known as progressive patchy cutaneous malformation, is a rare vascular disorder affecting young individuals characterized by nonpurpuric erythematous punctate lesions on one-half of the body in linear or serpiginous pattern following Blaschko lines.[1] AS has a prevalence of less than 1: 000, 000 and is more commonly seen in females.[2]
| Case Report | | |
A 38-year-old male presented to our outpatient department with complaints of asymptomatic unilateral flat red-colored skin lesions on the left side of the trunk and left arm for the last 13 years. These lesions initially appeared over the left lateral aspect of the trunk, which gradually progressed to involve left forearm over a period of illness. There was no pruritus, pain, or bleeding. General physical and systemic examination was within normal limits. Dermatological examination revealed multiple, 1–3 mm erythematous macules arranged in linear, serpiginous, and geographic patterns over left side of the abdomen, chest, and inner aspects of left arm and forearm [Figure 1]. There was clear demarcation at midline of the trunk. Diascopy showed incomplete blanching of macules. Face, scalp, gluteal area, lower limbs, palms, soles, and mucous membranes were not involved. Diagnosis was confirmed with dermoscopy supplemented with skin biopsy. Dermoscopy revealed characteristic red round-to-oval lagoons at the center of normal reticular pigment pattern representing dilated capillaries in papillary dermis [Figure 2]. Histopathology revealed numerous dilated thin-walled blood vessels in the papillary dermis, confirming the clinical as well as dermoscopy findings [Figure 3]a and [Figure 3]b. | Figure 1: 1–3 mm sized discrete to confluent macules involving strictly the left side of abdomen, chest, and inner aspect of left arm and forearm in linear, serpiginous, and geographic patterns
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 | Figure 2: Dermoscopy (DermLite DL4, ×10, contact/polarized mode) showing characteristic round to oval red lagoons at the center of normal reticulate pigment network of the skin
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 | Figure 3: (a) Dilated capillaries in papillary dermis (H and E, ×10). (b) High-power view showing dilated capillaries in papillary dermis with the absence of inflammation, red blood cell extravasation, or hemosiderin deposition (H and E, ×40)
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| Discussion | | |
AS was first described by Hutchinson in 1889.[3] Four years later, Radcliffe–Crocker coined the term AS.[3] It is characterized by multiple, small, asymptomatic, nonpalpable, crimson-red, punctate telangiectatic macules organized in sheets and clusters arranged in gyrate and serpiginous patterns. A disease of vascular system, particularly capillaries, has a female preponderance of 9:1 (females: males).[4] Lesions classically begin in the first or second decade commonly on the lower limbs and buttocks. Most cases are unilateral along the lines of Blaschko, but rare cases with bilateral and extensive cutaneous involvement have been reported.[5] Truncal involvement is uncommon. Although pathogenesis of AS is unknown, female preponderance, postpubertal age of onset, and faster progression during pregnancy points to role of estrogen in its pathogenesis.[4] Dermoscopy shows characteristic well-circumscribed round-to-oval red lagoons due to dilated vascular spaces within superficial reticular or papillary dermis.[6] Histopathology shows characteristic features of AS with increased numbers of dilated and proliferated capillaries in papillary dermis without inflammation, red blood cell extravasation, or hemosiderin deposition. The clinical differential diagnoses considered in our case were angiokeratoma, unilateral nevoid telangiectasia (UNT), pigmented purpuric dermatosis (PPD), and telangiectatic macularis eruptiva perstans (TMEP). Angiokeratoma presents with scaly papules with histopathology showing dilated subepidermal vessels with epidermal acanthosis and hyperkeratosis. UNT manifests with blanchable telangiectatic macules which on dermoscopy show dilated linear and branched vessels. PPD presents with cayenne pepper-like discoloration of the skin primarily affecting the lower legs. The characteristic cayenne pepper pigmentation is derived from extravasation of erythrocytes in the skin with marked hemosiderin deposition. The lesions of TMEP, a subtype of cutaneous mastocytosis, usually are small, irregular reddish-brown telangiectatic macules overlying a brown-to-tan background.[7] The telangiectatic lesions blanch on diascopy. Darier's sign, although weak due to lesser number of mast cells in the lesions, is positive in TMEP.[8] Histologically, there is only a slight increase in mast cells with infiltration of the superficial dermis and epidermal hyperpigmentation.[9] Mast cells are mainly concentrated around dilated capillaries and venules of the superficial venous plexus found in the upper dermis. A simple dermoscopic examination helps to clinch the diagnosis of AS from other morphologically similar looking conditions. Our case showed typical clinical presentation, histopathological correlation, and characteristic red round-to-oval lagoons on dermoscopy consistent with the diagnosis of AS. AS has been categorized into two subtypes – autosomal dominant diffuse nonsegmental type and sporadic zosteriform or segmental type.[10] Our patient belongs to the latter subtype. AS sometimes regress partially or completely on its own. Persistent AS can be managed with pulse dye laser and 532 nm potassium-titanyl-phosphate lasers have been used in the treatment of AS.
Acknowledgments
We sincerely thank the Department of Pathology, Base Hospital Delhi Cantt, who helped us in diagnosis and management of the case.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given his consent for images and other clinical information to be reported in the journal. The guardian understands that names and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| References | | |
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| 7. | Costa DL, Moura HH, Rodrigues R, Pineiro-Maceira J, Ramos-E-Silva M. Telangiectasia macularis eruptiva perstans: A rare form of adult mastocytosis. J Clin Aesthet Dermatol 2011;4:52-4. |
| 8. | Galen BT, Rose MG. Darier's sign in mastocytosis. Blood 2014;123:1127. |
| 9. | Watkins CE, Bokor WB, Leicht S, Youngberg G, Krishnaswamy G. Telangiectasia macularis eruptiva perstans: More than skin deep. Dermatol Reports 2011;3:e12. |
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[Figure 1], [Figure 2], [Figure 3]
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