Angioma serpiginosumL81.7

Rare, superficial, diffuse but also segmentally distributed vascular neoplasms. Occasional associations with systemic diseases have been described (Sjögren's syndrome, primary biliary cirrhosis, paraproteinemia) without a pathogenetic explanation for this association.

AS is a rare disease, estimated at less than 1 in 1,000,000 people. Although both sexes are affected at any age, 90% of cases occur in girls under the age of 16. The disease is usually sporadic, but familial occurrence has also been reported (Omim 106050).

The pathogenesis of angioma serpiginosum is unknown. The association of an Xp11.23 deletion of the PORCN gene is being discussed. The association of angioma serpiginosum-like skin changes and Goltz syndrome is noteworthy (Fernandez-Flores A et al. 2022; Tewari A et al. 2019). The combination with oesophageal papillomatosis has also been reported (Blinkenberg EO et al. 2007) and the extent to which increased oestrogen levels play a role in the development of angioma serpiginosum remains to be seen. Rapid progression has been reported during pregnancy (Das D et al. 2016).

Mostly occurring in childhood and adolescence (80% of patients are affected before the age of 18). Rarely present at birth. Angioma serpiginosum has been observed less frequently in newborns.

The lower extremities and buttocks are most commonly affected; atypical areas such as the chest and soles of the feet and mucous membranes can also be affected.

Disseminated, 1-2 mm large, bright red to purple spots, some of which cannot be pushed away. Occasionally also telangiectasias. Grouped arrangement results in serpiginous, gyrated or linear patterns. The mucous membranes close to the skin remain free. The arrangement and extent of the lesions can result in a serpiginous pattern. Rarely, the eyes and nervous system may be affected (Gautier-Smith PC et al. 1971). Manifestation along the Blaschko lines has been described but is rather rare.

Pressure causes incomplete blanching. However, the lesions are not hemorrhagic.

Dermatoscopy: The spots represent dilated, non-inflamed capillaries in the dermal papillae. These can be visualized very well with dermoscopy. Dermoscopy shows numerous small, relatively well demarcated, round to oval red lagoons (Ilknur T et al. 2006), which are due to irregularly dilated capillaries in the papillary or superficial reticular dermis. The dermoscopic findings of angioma serpiginosum have also been described as a "school of goldfish in a pond". In particular, dermoscopy can help to differentiate angioma serpiginosum from purpura diseases.

Convolute dilated capillaries with or without thickening of the vessel wall in the papillary stratum, sometimes also capillary proliferation and erythrocyte extravasations. No inflammatory infiltrates.

Angioma serpiginosum has characteristic clinical features that help to distinguish this disease from similar dermatological anomalies. As a rule, patients are asymptomatic and show no bleeding, pain or inflammation.

The following diseases should be considered in the differential diagnosis:

• Spider veins
• Purpura pigmentosa progressiva
• Angiokeratoma corporis diffusum
• Teleangiectasia hereditaria haemorrhagica.

Cosmetic coverage. Studies have shown that various lasers are effective in the treatment of angioma serpiginosum, including argon lasers, pulsed dye lasers (PDL) and intense pulsed light (IPL). PDL is the most commonly used laser for angioma serpiginosum and usually requires multiple sessions. Case reports of patients treated with the potassium titanyl phosphate (KTP) laser with a shorter wavelength of 532 nm have also shown good results. The advantages of shorter wavelength lasers are reduced depth of penetration, as this is not required for most angioma serpiginosum, reduced energy output (i.e. fluence) and reduced damage to melanocytes, which may reduce the risk of hypopigmentation (Anjaneyan G et al. 2024).

Healing without atrophy is possible, usually after years.

The disease often develops progressively and begins with small, asymptomatic lesions that enlarge and merge at the edges, resolving in the center. After an initial growth phase in childhood, the lesions usually stop growing during puberty and remain stable in adulthood. Conditions associated with AS include vulval angiokeratomas. Retinal vein occlusions have also been observed.

Kanji A et al. (2023): A 15-year-old female patient presented with multiple asymptomatic lesions on the first two fingers of the right hand that first appeared at the age of 2 years. On examination, there were erythematous circumscribed patches on the right hand (dorsal and volar) corresponding to a capillary malformation in the dermis with normal blood flow on ultrasound. As cosmetics were not an issue for the patient, treatment was not indicated.

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