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Angioma Serpiginosum

Updated : February 22, 2024





Background

Angioma serpiginosum is an uncommon, benign skin illness characterized by violaceous to red punctate lesions on an erythematous base. Classical pinpoint puncta are generated by ectasia or hyperplasia of preexisting superficial cutaneous capillaries.

Angioma serpiginosum was first reported by Hutchinson in 1889 as a rare kind of serpiginous angioma and was later dubbed as an “infective” nevoid illness. In 1893, Radcliffe-Crocker coined the term “angioma serpiginosum.”

Epidemiology

Females are 9 times more likely to be affected with angioma serpiginosum than males. This skin infection is extremely rare, affecting only 1 out of a million individuals.

80% of cases present early, generally before the age of 20, and most cases are reported in early childhood. Infants might also be affected since birth.

Anatomy

Pathophysiology

Angioma serpiginosum originates from the rapid multiplication of endothelial cells and the development of new capillaries, not from the dilatation of existing capillaries alone.

Due to the absence of progesterone and estrogen receptors on the connected blood arteries, researchers have disproven the involvement of hormones in the pathophysiology of vascular endothelial proliferation.

A second hypothesized etiology is an irregular vascular response to cold temperatures which results in the creation and accumulation of freshly formed capillaries, which therein leads to the production of big ectatic vessels in the papillary dermis.

Etiology

The majority of angioma serpiginosum cases are sporadic. Yet, the dominance of both x-linked inheritance, and autosomal dominant genes have been observed. Reports of isolated cases imply that an Xp11.23 deletion involving the PORCN gene is one of the potential causes of this condition.

Nevertheless, this was later refuted by other employees. Later investigations have also refuted the notion that angioma serpiginosum constitutes a moderate type of focal dermal hypoplasia, which is related with PORCN gene alterations.

Genetics

Prognostic Factors

Angioma serpiginosum is rarely associated with major complications, however, there have been reports of CNS, and ocular involvement. Rare manifestations include familial occurrences, a delayed onset, and significant skin involvement.

Angioma serpiginosum is also rarely linked with conditions such as esophageal papillomatosis, cherry angioma, retinal vein occlusion, and vulval angiokeratomas.

Clinical History

Physical Examination

Age group

Associated comorbidity

Associated activity

Acuity of presentation

Differential Diagnoses

Laboratory Studies

Imaging Studies

Procedures

Histologic Findings

Staging

Treatment Paradigm

by Stage

by Modality

Chemotherapy

Radiation Therapy

Surgical Interventions

Hormone Therapy

Immunotherapy

Hyperthermia

Photodynamic Therapy

Stem Cell Transplant

Targeted Therapy

Palliative Care

Medication

Media Gallary

References

https://www.ncbi.nlm.nih.gov/books/NBK459213/

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Angioma Serpiginosum

Updated : February 22, 2024




Angioma serpiginosum is an uncommon, benign skin illness characterized by violaceous to red punctate lesions on an erythematous base. Classical pinpoint puncta are generated by ectasia or hyperplasia of preexisting superficial cutaneous capillaries.

Angioma serpiginosum was first reported by Hutchinson in 1889 as a rare kind of serpiginous angioma and was later dubbed as an “infective” nevoid illness. In 1893, Radcliffe-Crocker coined the term “angioma serpiginosum.”

Females are 9 times more likely to be affected with angioma serpiginosum than males. This skin infection is extremely rare, affecting only 1 out of a million individuals.

80% of cases present early, generally before the age of 20, and most cases are reported in early childhood. Infants might also be affected since birth.

Angioma serpiginosum originates from the rapid multiplication of endothelial cells and the development of new capillaries, not from the dilatation of existing capillaries alone.

Due to the absence of progesterone and estrogen receptors on the connected blood arteries, researchers have disproven the involvement of hormones in the pathophysiology of vascular endothelial proliferation.

A second hypothesized etiology is an irregular vascular response to cold temperatures which results in the creation and accumulation of freshly formed capillaries, which therein leads to the production of big ectatic vessels in the papillary dermis.

The majority of angioma serpiginosum cases are sporadic. Yet, the dominance of both x-linked inheritance, and autosomal dominant genes have been observed. Reports of isolated cases imply that an Xp11.23 deletion involving the PORCN gene is one of the potential causes of this condition.

Nevertheless, this was later refuted by other employees. Later investigations have also refuted the notion that angioma serpiginosum constitutes a moderate type of focal dermal hypoplasia, which is related with PORCN gene alterations.

Angioma serpiginosum is rarely associated with major complications, however, there have been reports of CNS, and ocular involvement. Rare manifestations include familial occurrences, a delayed onset, and significant skin involvement.

Angioma serpiginosum is also rarely linked with conditions such as esophageal papillomatosis, cherry angioma, retinal vein occlusion, and vulval angiokeratomas.

https://www.ncbi.nlm.nih.gov/books/NBK459213/

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