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Angioma Serpiginosum
Introduction
Angioma serpiginosum is a rare dermal pathology characterized by pinpoint violaceous to coppery-red punctate maculopapular eruptions that cluster together in linear, serpiginous (ie, snake-like), or gyrate (ie, ring) patterns on an erythematous background. Angioma serpiginosum affects the lower limbs more commonly than other areas and is more frequently seen in females. The exact pathophysiology of this nevoid condition is not entirely known. Experts theorize that the condition may evolve from the proliferation of endothelial cells and the formation of new capillaries, not merely the dilation of preexisting capillaries.[1][2][3][4]
Angioma serpiginosum is not associated with malignancy or physically debilitating symptoms, but the condition can have a significant psychological impact on patients due to perceived disfigurement of their appearance. The primary indication for treatment is the cosmetic concerns of the patient. Therefore, the ability to differentiate this dermatologic pathology from similar conditions and familiarity with available treatments is essential for clinicians to obtain because proper management of angioma serpiginosum involves tailoring therapy to the patient's needs.[5]
Etiology
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Etiology
The exact cause of angioma serpiginosum is unknown. Some experts have theorized that there may be a genetic component involved, while others have proposed that angioma serpiginosum may result from pathophysiologic changes precipitated by cold temperatures; however, there is a paucity of evidence for either postulation.[6]
Epidemiology
Females are affected more frequently than males, with a reported ratio of 9 to 1. AS is a rare condition, estimated to be less than 1 per 1,000,000 individuals. Furthermore, angioma serpiginosum occurs more frequently in children and adolescents, with 80% of patients diagnosed before age 20.[7] Less commonly, angioma serpiginosum has been observed in neonates.[2]
Pathophysiology
The pathophysiology of angioma serpiginosum is not entirely known. Histological studies have demonstrated abnormal capillary dilation in the papillary dermis of individuals with the condition. The underlying mechanism of the pathophysiologic change is unknown, though. Some experts have proposed that angioma serpiginosum may result from the formation of capillaries in response to the cold, leading to increased dilation of vessels within the dermis; however, there is little evidence to support this.[6]
Histopathology
Characteristic histologic findings in patients with angioma serpiginosum that differentiate the condition from other differential diagnoses include dilatated capillaries with thickened walls within the papillary dermis and subpapillary regions without any inflammatory changes, signs of hemorrhage, or hemosiderin depositions.[8][9]
History and Physical
Angioma serpiginosum has characteristic clinical features that help to differentiate this condition from similar dermatologic abnormalities. Typically, patients are asymptomatic without any bleeding, pain, or inflammation associated with the lesions, predominately involving the lower limbs. However, there have been isolated reports of AS in areas including the upper limbs, face, palms, soles, and mucous membranes.[10][11] AS is commonly described as having a purple to coppery-red punctate appearance that clusters together in serpiginous or gyrate patterns on an erythematous background.[12][13][14][15]
The condition is frequently found to develop progressively, beginning as small asymptomatic lesions that enlarge and merge around the margins with central clearing.[12][13][14][15] Furthermore, following an initial growth period during childhood, the lesions usually stop growing during puberty and remain stable through adulthood.[4] Rarely extensive areas are involved and have a later onset.[16] Recently, cases of a zosteriform or acral distribution have been reported.[17][18][19] Conditions associated with AS include vulval angiokeratomas, overlying cherry angioma, and retinal vein occlusion, which have also been observed.[20][21][22]
Evaluation
A clinically suspected AS diagnosis can be further evaluated with diascopy and dermoscopy, but confirmation is achieved through skin biopsy. Primary care clinicians and dermatologic specialists use these modalities to enhance visual assessment of skin conditions. Serum and imaging studies are usually unnecessary unless indicated to investigate an underlying etiology.
Diascopy
Diascopy is used to assess whether lesions blanch with the application of pressure. This characterizes dermatologic pathologies as vascular, which blanchs, or nonvascular or hemorrhagic, which do not blanch. Clinicians can use these findings to differentiate vascular (eg, angiomas) from nonvascular or hemorrhagic (eg, purpura and petechiae) diagnoses.[23][24]
Dermoscopy
Dermoscopy is another technique used to visually assess skin lesions using a microscope with or without polarized light that has been shown to improve diagnostic accuracy.[25] Using dermoscopy, clinicians can see the characteristic appearance of AS, including red clusters, "lacunae," due to irregularly dilated capillaries in the papillary or superficial reticular dermis.[26] The dermoscopy findings in angioma serpiginosum have also been described as a "school of redfish in a pond." Dermoscopy can especially help distinguish angioma serpiginosum from purpuric dermatoses.[27][28] Reflectance confocal microscopy can be used in addition to dermoscopy to improve diagnostic accuracy. When used to evaluate angioma serpiginosum, reflectance confocal microscopy similarly demonstrates multiple dilated vascular spaces aligned perpendicularly within the upper dermis.[29][30][29]
Skin Biopsy
Skin biopsy confirms an AS diagnosis through histopathologic features, including dilatated capillaries with thickened walls within the papillary dermis and subpapillary regions without any inflammatory changes, hemorrhagic signs, or hemosiderin depositions, which distinguishes the condition from other differential diagnoses.[8][9]
Treatment / Management
AS is a benign, asymptomatic condition; however, patients frequently suffer adverse psychosocial effects due to the noticeable changes in their appearance. AS may partially resolve spontaneously, but the complete disappearance of the condition has not been reported.[5] Cosmetics may be used to conceal lesions temporarily. Though there is no evidence of effective topical treatments, experts have recommended laser therapy as the treatment of choice.[22] Consequently, the primary indication for laser treatment is a patient's desire to undergo therapy after informed consent is obtained. (B3)
Studies have found various lasers effective in treating angioma serpiginosum, including Argon laser, tunable pulsed dye laser (PDL), and intense pulsed light (IPL).[31] The PDL has been the most reported laser used in angioma serpiginosum, with multiple sessions usually required.[32] Case reports of patients treated with the potassium titanyl phosphate (KTP) laser using a shorter 532 nm wavelength have shown good results also.[5] The advantages of lasers with shorter wavelengths are decreased penetration, since the significant depth of penetration is not required for most cases of angioma serpiginosum, reduced amounts of energy delivered (ie, fluence), and decreased melanocyte damage, which may help reduce the risk of hypopigmentation.[33][34](B3)
Differential Diagnosis
Angioma serpiginosum can sometimes be mistaken for other conditions, such as angiokeratomas,[35] purpura, or cutaneous metastases. Differentiating angioma serpiginosum from other vascular lesions is critical to ensure appropriate management and treatment. A few differential diagnoses for angioma serpiginosum include:[6]
- Angiokeratoma circumscriptum
- Capillary Malformation
- Hemangioma
- Majocchi Granuloma
- Pigmented Purpuric Dermatoses
- Unilateral Nevoid Telagiectasia
- Port-wine stain
Prognosis
The prognosis of angioma serpiginosum is generally considered to be good. As a benign vascular disorder, there typically is no concern for significant health risks. Additionally, the lesions are usually asymptomatic and do not cause any functional limitations. Cosmetically, the prognosis varies according to the patient's concerns and desired outcomes. While the condition does not resolve spontaneously, various treatment options can be explored to help minimize the appearance of the lesions and improve cosmetic outcomes. Laser therapy, such as pulsed dye laser or intense pulsed light (IPL), has shown promising results in selectively targeting and reducing the vascular lesions associated with angioma serpiginosum. However, the effectiveness of all these treatments may vary among individuals, and continuous sessions may be required to achieve the desired improvement.[5]
Complications
There are a few potential complications associated with angioma serpiginosum, including:
-
Bleeding: Although rare, there have been reports of bleeding from the lesions of angioma serpiginosum. Trauma or injury to the affected area can cause the blood vessels to rupture, leading to bleeding. If bleeding occurs, it is usually minimal and self-limiting, but medical intervention may be required to control it in rare cases.
- Psychological impact: While it is not a direct physical complication, angioma serpiginosum can emotionally impact individuals, mainly if the lesions are prominent or located in visible areas. The appearance of these skin lesions may cause distress and self-consciousness, leading to a negative body image affecting an individual's quality of life and self-esteem.[5]
- Ocular and nervous system involvement: There are sporadic reports of patients with angioma serpiginosum involving the nervous or ocular system.[36][37] Patients with extensive cutaneous lesions have been reported to present with ocular pathologies such as retinal vein occlusion.[38]
- Adverse effects of treatment: Laser therapies used on the skin have several reported potential complications depending on the number of treatments a patient undergoes, including pain, skin atrophy, erythema, and hypopigmented and hyperpigmented lesions.[39]
Consultations
Depending on a primary care clinician's training, experience, and access to required equipment, patients with dermatologic conditions may be referred to a dermatology specialist for evaluation and treatment.[40]
Deterrence and Patient Education
Patient education and shared decision-making are key in managing a patient with angioma serpiginosum. Clinicians should educate their patients about this condition's benign, non-life-threatening nature. However, clinicians should counsel patients that the lesions may persist for a long and typically do not spontaneously resolve. Furthermore, for patients to make an informed decision, they should be made aware of their options for treatment, the risks and benefits of therapy, and that multiple visits for treatment may be required. Clinicians should also discuss the condition's potential impact on the patient's self-esteem with the patient and any involved caregivers, offering psychological support and counseling if needed. Through well-imparted patient education and support, healthcare professionals can help patients understand their clinical condition, manage their expectations, and make informed decisions about management options, including addressing their cosmetic concerns.[5]
Enhancing Healthcare Team Outcomes
Healthcare clinicians encounter patients with various skin lesions, particularly in primary care. Patients may be evaluated within the primary care clinic or referred to a dermatological specialist based on their comfort level. While angioma serpiginosum is benign, it severely impacts a patient's self-esteem and confidence. After guiding the patient about the condition and management options, dermatologists may need to refer the patient to a psychologist for proper counseling sessions. Although complications are rare, some underlying pathologies need to be assessed for which physicians' opinions may be needed. Specific difficulties will require an expert opinion from the relevant specialty doctor. In patients with neurological and ocular involvement, services from ophthalmologists and neurologists are often needed. Therefore, timely interprofessional collaboration and detailed management plans can help improve patients' condition and quality of life.
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