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Vascular lesions in infants

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01.   |   Théo

Théo, 16 weeks old.

Coll. Dr D. Wallach - Dermaweb Théo, 16 weeks old, is taken to the doctor because of a red papule-nodule on the right shoulder.
This lesion appeared 3 weeks earlier. The child was born 4 weeks before term and is in perfect health.

Questions >

What are the arguments in favor of an infantile hemangioma diagnosis?

Several possible answers.






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Points to remember

Infantile hemangioma (IH or immature angioma) is a benign vascular tumor created from a proliferation of capillaries.

A superficial IH diagnosis is usually clinical when presented with a raised, red or purplish lesion that has formed after a treatment-free interval. The non-congenital nature of IH is a key factor in the diagnosis.

IH is a very common lesion (affects approximately 1 in 20 infants), and is even more common in premature infants. However, these last two factors are not essential for diagnosis!

Spontaneous IH involution and the benign location of the lesion in Theo's case are not favorable for systemic treatment.

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02.   |   Justine

Justine, 22 weeks old.

Image bank Pierre Fabre Dermatologie Justine, 22 weeks old, was brought in by her parents acting on advice from the family pediatrician. Justine has a large, erythematous, poorly delimited patch with a vascular appearance on the right temporal region. These lesions were not present at birth or during her doctor's visit during her first month. When she was between 8 and 16 weeks old, the lesions spread rapidly during the following visits to the pediatrician. They have been generally stable since. You mention and infantile hemangioma (IH).

Questions >

Which of the following statements are
true?

Several possible answers.






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Points to remember

Some IH lesions are widespread and distributed based on a segmented topography which was initially considered to be metameric. The area of skin involved is probably a sign of mosaicism of embryological origin.

In the case presented, IH topography could focus the discussion on part of the area of the first branch of the trigeminal nerve (V1). A search must be done for Malformative PHACES syndrome (Posterior fossa malformations, Hemangiomas, Arterial anomalies, Cardiac defect, Eye anomalies, Sternal cleft and supraumbilical raphe syndrome) via cerebral and thoracic MRI, in addition to an ophthalmological exam and a cardiac echography including the great vessels.

Systemic treatment of IH in cases of PHACES syndrome is debatable due to the theoretic risk of intracerebral arterial spasm. A multidisciplinary discussion can be held after further exams are conducted.

This observation highlights the importance of early examination by pediatric dermatologists for all IH cases that could benefit from systemic treatment in order to initiate rapid management.

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03.   |   Julien

Julien, 6 months old.

Coll Dr D. Wallach - Dermaweb Julien, 6 months old, has a congenital erythematous mark on the frontal region.

This lesion becomes more visible when he cries, but its size has not changed since birth.

Questions >

What are the arguments against infantile hemangioma in this case?

Several possible answers






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Points to remember

The congenital and planar nature of this apparently vascular lesion rules out infantile hemangioma diagnosis. This is a port-wine stain (capillary malformation).

Port-wine stains are very common in infants. They are stable in size and tend to fade with time, without disappearing completely.

Certain areas of the skin are frequently affected by port-wine stain, such as the interbrow region shown here. Here, the port-wine stain is called "flammeus angioma." Other common sites for port-wine stain are the nape of the neck and the lumbar region.

Systemic treatment has no indication in capillary malformations. For strongly marked lesions, vascular laser treatment can be discussed.

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04.   |   Kevin

Kevin, 6 weeks old.

Coll Dr D. Wallach - Dermaweb Kevin is 6 weeks old. He has had an erythematous nodule on the left lower eyelid for three weeks.
This lesion has been growing progressively. You suspect infantile hemangioma (IH).

Questions >

Which of the following statements are true?

Several possible answers.






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Points to remember

Certain IH cases occur on the eyelids or in the canthal regions. They must be monitored carefully because they can lead to prolonged palpebral occlusion (pending their involution lasting several months or years) and a functional rest period for the eye. This amblyopia can be definitive.

In this case, systemic treatment should be discussed after a few weeks of monitoring to affirm palpebral fissure closure. This treatment rapidly reduces IH volume and prevents amblyopia. In Kevin's case, the diagnosis of a PHACES-type malformation syndrome and association with glaucoma are ruled out since the IH is not located in the frontal region (see Justine's case) and it is not segmented.

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05.   |   Julie

Julie, 3 weeks old.

Coll. Dr D. Wallach – Dermaweb Julie is 3 weeks old.
She has a congenital lesion on her right thigh, objectified during the last prenatal ultrasound.
When the infant was examined by you, the lesion had already paled considerably and had decreased in thickness.

Questions >

What is your main diagnostic hypothesis?

Several possible answers.






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Points to remember

Some benign vascular tumors called "hemangiomas" are congenital. Their histogenesis is different to that of infantile hemangiomas (IH). Rapidly involuting congenital hemangiomas (RICH) are different to non-involuting congenital hemangiomas (NICH). RICH are found at the base of the limbs, like in the case presented and begin to regress spontaneously in the days after birth. No treatment is usually necessary.

If there is any doubt surrounding an IH diagnosis, a biopsy can be performed. RICH (and NICH) do not express the GLUT-1 marker in immunohistochemistry.

In some cases, RICH involution is incomplete and can require surgery. Such cases are referred to as partially involuting congenital hemangiomas (PICH).

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06.   |   Pénélope

Pénélope, 11 weeks old.

Image bank Pierre Fabre Dermatologie Pénélope was brought in at the age of 11 weeks. She was born at 33 weeks of amenorrhea. After a short stay in the neonatal unit for monitoring, Pénélope was sent home. Her parents observed the formation of several nodular lesions with a vascular appearance (two on the face, several on the torso), in addition to a tumefaction on the lower left eyelid.

Questions >

Which statements about Pénélope are true?

Several possible answers.






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Points to remember

The upper left palpebral nodule indicates a deep infantile hemangioma (IH). It can cause an occlusion in the left eye as well as an amblyopia. This requires close monitoring and prescription of systemic treatment can be discussed.

IH can be multiple ; they are very numerous at times (miliary hemangiomatosis). When there are more than 5 IH, a search must be systematically carried out for one or more hepatic hemangiomas potentially at the origin of cardiac insufficiency and/or hypothyroidism. If these complications are proven, a systemic treatment is also offered and combined with cardio-pediatric surveillance.

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07.   |   Maxime

Maxime, 4 months old.

Image bank Pierre Fabre Dermatologie Maxime, 4 months old, has a superficial IH located in the xyphoidian region. His parents are worried because the lesion has not shown any regression for several weeks.

Questions >

What do you do?

Several possible answers.






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Points to remember

Infantile hemangioma (IH) regresses spontaneously in all cases. Schematically, small lesions (such as those on Maxime) involute in few months. Voluminous lesions can take several years to disappear.

Maxime was examined again 3 months after the first consultation and the condition was beginning to improve.

Usually IH disappearance is complete and scar-less. In fact, the most voluminous IH in particular can leave telangiectasias, resembling a skin hernia or a fibrous-adipous nodule at the lesion site after regression of capillary proliferation.

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08.   |   Maud

Maud, 18 months old.

Coll Dr D. Wallach - Dermaweb Maud, 18 months old, was sent by her family doctor for an opinion concerning a vascular lesion on the left arm, considered up until then to be an infantile hemangioma. Patient history reveals that this lesion appeared during the first months after birth. Its surface slowly increased as the infant got older.

Questions >

What are the arguments in favor of arteriovenous malformation diagnosis?

Several possible answers.






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Points to remember

Infantile hemangioma diagnosis is ruled out here in the absence of involution since the lesion appeared. It is therefore important to consider a vascular malformation diagnosis.

Faced with a hot lesion presenting a thrill during palpation and blowing sounds during auscultation, the arteriovenous malformation diagnosis (AVM) is the most likely.

The diagnosis will be confirmed by a Doppler ultrasound and by MR angiography. Therapeutic treatment must be entrusted to a specialized team.

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09.   |   Solène

Solène, 2 years and 3 months old.

Image bank Pierre Fabre Dermatologie Solène, 2 years and 3 months old, has a large infantile hemangioma (IH) in the sus-umbilical region. Her parents were told that IH can now be treated systemically. They are seeking your advice on how to begin this treatment.

Questions >

Which of the following statements are
true?

Several possible answers.






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Points to remember

Solène's lesion is rather characteristic of a superficial infantile hemangioma (IH) with involution underway. The IH has become sunken and paler, changing from red to a very indicative grayish-pink. A biopsy (not useful for diagnosis) will show a small number of capillaries in the process of fading.

An IH of this size can be treated systematically, due to the risk of unaesthetic skin dystrophy sequelae. However, the treatment must be started when the lesion is in proliferation phase (most often before the age of 5 months). Experience shows that if the treatment is started later, efficacy in terms of reducing lesion volume and duration of evolution is very modest. Initiation of a treatment should be discussed in Solene’s situation. .

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09.   |   Blandine

Blandine, 4 months old.

Image bank Pierre Fabre Dermatologie Blandine, 4 months old, is brought in because of a red plaque on the right upper limb. This lesion appeared when she was 3 weeks old. The child is perfectly healthy.

Questions >

What are the arguments in favor of an infantile hemangioma diagnosis?

Several possible answers.






< Previous To remember >

Points to remember

Infantile hemangioma (IH or immature angioma) is a benign vascular tumor created from a proliferation of capillaries.

A superficial IH diagnosis is usually clinical when presented with a raised, red or purplish lesion ("strawberry") that has formed after a treatment-free interval. The non-congenital nature of IH is a key factor in the diagnosis.

The majority of IH lesions are small in size, but some cover a large area of skin (like in this case) and are sometimes segmental. The segmental distribution is not absolutely necessary for an IH diagnosis in large lesions.

The spontaneous IH involution and the benign location of this lesion in Blandine's case are not favorable for automatically recommending systemic treatment. However, given the common occurrence of cutaneous sequelae (hernia, fibro-fatty residue, etc.), after the involution of IH of this size, a treatment like this can be considered.

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11.   |   Marion

Marion, 20 weeks old.

Coll Dr D. Wallach - Dermaweb Marion, 20 weeks old, is brought in by her parents because of erythematous marks on the face. These lesions were present at birth, but they have been clearly visible since the child lost her normal erythrosis after the first weeks.

Questions >

What is/are your diagnosis/diagnoses?

Several possible answers.






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Points to remember

The port-wine stain (PWS) diagnosis is retained here due to the existence of congenital macular erythematous vascular lesions. The ISSVA classification likens PWS to a capillary malformation.

The appearance presented here is a particular widespread form of PWS recently individualized and named medio-facial PWS. This variety of PWS exceeds the surface affected by the commonplace "flammeus angioma." It can affect the nasolabial folds, the philtrum and even the chin. In some cases, medio-facial PWS runs in the family. This type of PWS becomes lighter in most cases. Vascular laser treatment, for esthetic purposes, can be discussed on a case-by-case basis.

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12.   |   Marietou

Marietou, 3 months old.

Image bank Pierre Fabre Dermatologie Marietou is 3 months old. Her parents are from Benin. The child was brought in because of a reddish-brown nodule with an uneven surface, located on the lateral surface of the neck. This lesion appeared during the first week after birth. The child is in a generally good state of health.

Questions >

What is your diagnosis?

Several possible answers.






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Points to remember

Infantile hemangioma (IH) is a benign vascular tumor that rarely affects black skin. The reason for this rarity is unknown.

The main risk factors recognized in the onset of an IH are female sex, prematurity and low birth weight as well as white skin.

In Marietou's case, diagnostic uncertainty linked to the atypical presentation and the onset on black skin, leads to surgical exeresis. The IH diagnosis was confirmed histologically and via immunohistochemistry with marking of GLUT1-positive endothelial cells.

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13.   |   Xavier

Xavier, 7 months old.

Coll Dr D. Wallach - Dermaweb Xavier has had a vascular nodule on the right antihelix since he was four weeks old. His doctor reached a diagnosis of infantile hemangioma (IH). When the boy's parents bring him in at age 7 months, they inform you that the lesion is beginning to turn grayish in color.

Questions >

What are the possible therapeutic options?

Several possible answers.






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Points to remember

This is an IH with no expected functional impacts. However, it is likely that after capillary proliferation disappears, sequelae will remain on the skin, particularly cutaneous hernia and/or a fibro-fatty nodule with ear deformation.

We will suggest systemic treatment with the aim of limiting the size of the IH. Beginning this treatment at Xavier's age will probably be too late. Therapeutic abstention is possible if discussed, after complete involution and corrective surgery on sequelae.

Early surgical exeresis is recommended by some teams, before any deformation occurs, in cases where an ear lesion is pendular on the lobe. Such an option is not considered here.

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14.   |   Paul

Paul, 5 months old.

Coll Dr D. Wallach - Dermaweb Paul, 5 months old, is sent by the family doctor because of a large erythematous plaque on the lower limb, observed since birth. The child is perfectly healthy.

Questions >

Which of the following statements are true?

Several possible answers.






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Points to remember

These congenital macular erythematous lesions with geographical edges form a port-wine stain (capillary malformation). Distribution is segmented here on a lower limb, probably indicating a somatic mosaicism for the mutation of a gene involved in vascular growth.

Certain segmented PWS on a lower limb occurs as part of Klippel-Trenaunay syndrome (KTS). A repeated search would therefore have to be carried out for asymmetry in length and/or circumference of the lower limbs, as well as vascular anomalies such as varicose veins or arteriovenous fistulae.

KTS treatment is complex and multidisciplinary. If the PWS is isolated, vascular laser treatment can be discussed.

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15.   |   Aurélie

Aurélie, 16 weeks old.

Image bank Pierre Fabre Dermatologie Aurélie is 16 weeks old. She was brought in by her parents because of a large angiomatous patch on the left malar region. It was discreet at first, but has been spreading quickly over the last 4 weeks. You diagnose Aurélie with superficial infantile hemangioma (IH).

Questions >

Which of the following statements are true?

Several possible answers.






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Points to remember

This is a case of infantile hemangioma (IH) occupying a segmented region of embryological origin, almost superimposable upon the second branch of the trigeminal nerve.

Diagnosis of Sturge-Weber syndrome is ruled out because the lesion is not a port-wine stain on the upper part of the face. A PHACES syndrome diagnosis, associated with an IH is improbable in this topography respecting the frontal region. Nonetheless, cases of PHACES syndrome have been described with IH far from the frontal region, for example on the nape of the neck.

No lesion assessment necessary. The problem is mainly aesthetic in this case. Systemic treatment is indicated.

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16.   |   Chaïma

Chaïma, 16 weeks old.

Image bank Pierre Fabre Dermatologie Chaïma is 16 weeks old and was sent to the pediatric emergency room because of a red papule-nodule on her right arm that very suddenly became painful. This lesion appeared 6 weeks earlier. The child was born 3 weeks before term and had been in perfect health until then.

Questions >

Which diagnoses should be discussed in light of this suddenly painful vascular lesion?

Several possible answers.






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Points to remember

The two main diagnoses to be considered are Kasabach-Merritt syndrome (KMS) and ulcerated infantile hemangioma (IH).

KMS is defined by an intravascular coagulation located within a rare variety of hemangioma (kaposiform hemangioendothelioma or tufted angioma, both GLUT-1 negative). It is accompanied by deep thrombopenia. Urgent, specialized treatment is required.

The diagnosis here was more basically that of IH complicated by painful ulceration and by necrosis. This occurrence is not exceptional, particularly when IH is cephalic or perineal. Treatment is a combination of analgesics, mild localized care and systemic treatment to accelerate healing.

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17.   |   Johnson

Johnson, 13 weeks old.

Image bank Pierre Fabre Dermatologie Johnson, 13 weeks old, was brought in because of a bluish lesion on the tip of his nose. It was observed from when he was three weeks old.

Questions >

What is your diagnosis?

Several possible answers.






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Points to remember

Infantile hemangioma (IH or immature angioma) is a benign vascular tumor created from a proliferation of capillaries. It can be superficial ("strawberry" red), subcutaneous (Deep) or combined.

Johnson has a purely subcutaneous IH. The tip of the nose is a classic location. The functional prognosis of this lesion is benign. On the other hand, it is not rare for IH in this location to cause significant deformation of the tip of the nose. There is therefore an indication here to suggest early systemic treatment.

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18.   |   Christian

Christian, 3 months old.

Coll Dr D. Wallach - Dermaweb Christian is 3 months old. He was brought to the pediatric dermatologist because of a bluish lesion on the plantar region of the right foot. It is difficult to specify exactly how old this lesion is. It is not mentioned in the health record booklet filled out at the end of the maternity ward stay.

Questions >

Which anamnestic and clinical arguments should be upheld in favor of venous malformation?

Several possible answers.






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Points to remember

A venous malformation (VM) is occasionally included in the differential diagnosis to infantile hemangioma (IH) when the latter is purely dermal.

The child's parents can spontaneously indicate a change in VM volume, and this information is useful in diagnosis. Such a change in volume does not exist in dermal IH.

A lesional assessment with medical imaging must be conducted in order to appreciate the lesion's anatomical limits, which is sometimes wider and more poorly defined than a simple skin inspection implies. Therapeutic treatment is specialized and usually entrusted to vascular radiologists.

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19.   |   Martine

Martine, 3 months old.

Image bank Pierre Fabre Dermatologie Martine is 3 months old. Her parents have come to you multiple times seeking an opinion, particularly because systemic treatment and surgical treatment were suggested. Martine has an infantile hemangioma (IH) on her upper lip.

Questions >

Which of the following statements about treating this IH are true?

Several possible answers.






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Points to remember

Infantile hemangioma (IH, or immature hemangioma) of the upper lip is particularly difficult to treat, especially when the lesion is at the border between the skin and the vermillion zone as in Martine's case.

Treatment has not yet been properly codified, but it seems that the best solution is very early systemic treatment in order to limit labial deformation.

Surgery is difficult and runs the risk of retractile and unaesthetic scarring of the skin. Surgery is to be considered if systemic treatment fails or to correct residual IH sequelae.

A systemic treatment was suggested to the parents, and plastic surgery only in the event of residual lesions (cutaneous-mucosal hernia, fibrous-lipoma).

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