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LETTER TO THE EDITOR
Year : 2020  |  Volume : 11  |  Issue : 4  |  Page : 645-647  

Pleuri-segmental ipsilateral nonsyndromic infantile hemangioma responding favorably to oral propranolol


Department of Dermatology, Venereology and Leprology, PGIMER, Chandigarh, India

Date of Web Publication13-Jul-2020

Correspondence Address:
Rahul Mahajan
Department of Dermatology, Venereology and Leprology, PGIMER, Chandigarh - 160 012
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/idoj.IDOJ_455_19

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How to cite this article:
Ashraf R, Mahajan R, Handa S, De D. Pleuri-segmental ipsilateral nonsyndromic infantile hemangioma responding favorably to oral propranolol. Indian Dermatol Online J 2020;11:645-7

How to cite this URL:
Ashraf R, Mahajan R, Handa S, De D. Pleuri-segmental ipsilateral nonsyndromic infantile hemangioma responding favorably to oral propranolol. Indian Dermatol Online J [serial online] 2020 [cited 2020 Aug 8];11:645-7. Available from: http://www.idoj.in/text.asp?2020/11/4/645/289624



Sir,

Infantile hemangiomas (IH) may manifest as focal, multifocal, segmental, or indeterminate lesions. Segmental hemangiomas have been associated with an 11-fold associated risk of complications when compared with localized forms and are frequently associated with syndromes.[1]

A 6-month old girl (preterm with a birth weight of 2.5 kg) was brought to the dermatology outpatient with red vascular lesions on the right side of the forehead, buttock, perineum, and lower limb. At birth, parents noticed faint erythema simultaneously at the abovementioned sites, which progressively increased in size after 1 week. The perineal lesion ulcerated 2 weeks prior to presentation. There was no history to suggest a developmental delay, hypotonia, syncopal attacks, or urogenital abnormalities. Examination revealed well-defined grouped, superficial red vascular papules 0.3–0.8 cm in diameter on the right S1 segment of face [Figure 1]a. Similar lesions present on the right lower limb had a deeper component showing a lacing pattern of blue vascular structures [Figure 1]b. Corresponding labia majora and buttock showed similar lesions with superficial ulceration and scarring at places [Figure 1]c. Ophthalmological examination was normal. Radiologic investigations, including abdominal sonography and magnetic resonance imaging (MRI) of the brain, spine, and pelvis were normal, as were electrocardiogram (ECG), and thyroid function tests (TFTs). She was started on oral propranolol 2 mg/kg/day (crushed tablets), advised local care for ulceration, and showed significant improvement in lesions within 1 month of starting therapy and complete involution after 9 months [Figure 2]a, [Figure 2]b, [Figure 2]c.
Figure 1: (a) Pretreatment image of segmental hemangioma in the right facial S1 (frontotemporal) region. (b) Pretreatment image of lesions on the right lower limb. (c) Pretreatment image of lesions on the right labia majora and perineal region

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Figure 2: (a) Posttreatment images of segmental hemangioma in the right facial S1 (frontotemporal) region. (b) Posttreatment images of lesions on the right lower limb. (c) Posttreatment images of segmental hemangioma in right labia majora and perineal region (Note the healing with scarring reminiscent of previous ulceration)

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Segmental hemangiomas constitute around 13–18% of hemangiomas.[1] As with other segmental lesions, mosaicism has been the proposed mechanism for the occurrence of such hemangiomas.[2] However, others believe that patterns of segmental hemangiomas (especially of the face) are usually neither blaschkoid/dermatomal nor do they correspond to sensory nerve distributions/known embryonic segments.[3] Large segmental IHs are often associated with underlying malformations, particularly PHACES and PELVIS/LUMBAR syndromes. Large segmental IH in a ''beard'' distribution may mark a subglottic hemangioma. Large IHs and hepatic hemangiomas may cause hypothyroidism and may be associated with local complications like ulceration, bleeding, orificial obstruction and cosmetic disfigurement as was seen in our patient.

The occurrence of hemangiomas in multiple segments, as seen in the index case is a rare occurrence. Khanna et al. reported the occurrence of large segmental hemangiomas of the right upper limb and shoulder as well as contralateral thigh with high-output cardiac failure. Improvement was seen in cutaneous and hemodynamic symptoms after propranolol therapy.[4] Dakshayini et al. reported a case with segmental hemangiomas on the left side of the face, left upper and lower limbs with PHACES syndrome.[5] Fortunately, no such syndromic associations or systemic complications were seen in the present case.

Although all segmental hemangiomas per se may not always necessitate medical intervention, it is indicated in patients with complicated IH. Propranolol has surpassed oral steroids in becoming the drug of choice in treating IHs. Our patient responded favorably to the same without any serious adverse effects. Concluding, pleuri-segmental hemangiomas are a rare presentation of IH. Although they often raise a strong suspicion of local and/or systemic complications, they may be nonsyndromic and respond favorably to oral propranolol.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Leaute-Labreze C, Prey S, Ezzedine K. Infantile haemangioma: Part I. Pathophysiology, epidemiology, clinical features, life cycle and associated structural abnormalities. J Eur Acad Dermatol Venereol 2011;25:1245-53.  Back to cited text no. 1
    
2.
Yu X, Zhang J, Wu Z, Liu M, Chen R, Gu Y, et al. LUMBAR syndrome: A case manifesting as cutaneous infantile hemangiomas of the lower extremity, perineum and gluteal region, and a review of published work. J Dermatol 2017;44:808-12.  Back to cited text no. 2
    
3.
Phan TA, Adams S, Wargon O. Segmental haemangiomas of infancy: A review of 14 cases. Australas J Dermatol 2006;47:242-7.  Back to cited text no. 3
    
4.
Khanna D, Chakravarty P, Arora P, Jain R, Mittal M. Large multifocal cutaneous hemangioma along lines of Blaschko with cardiac failure treated with propranolol. Pediatr Dermatol 2015;32:e18-22.  Back to cited text no. 4
    
5.
Dakshayani B, Benakappa A. Multiple segmental hemangiomas over one half of the body-A rare feature of PHACES syndrome. Indian J Pediatr 2016;83:83-4.  Back to cited text no. 5
    


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