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Year : 2022  |  Volume : 23  |  Issue : 2  |  Page : 179-180

Bullous variant of aplasia cutis congenita: A rare entity

Department of Dermatology, Venereology and Leprology, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India

Date of Submission25-Jun-2021
Date of Decision26-Dec-2021
Date of Acceptance10-Jan-2022
Date of Web Publication30-Mar-2022

Correspondence Address:
Dr. Sheetanshu Kumar
Department of Dermatology, Venereology and Leprology, All India Institute of Medical Sciences, Rishikesh - 249 203, Uttarakhand
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijpd.ijpd_100_21

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How to cite this article:
Dhiman A, Anuragi RP, Kumar S, Hazarika N. Bullous variant of aplasia cutis congenita: A rare entity. Indian J Paediatr Dermatol 2022;23:179-80

How to cite this URL:
Dhiman A, Anuragi RP, Kumar S, Hazarika N. Bullous variant of aplasia cutis congenita: A rare entity. Indian J Paediatr Dermatol [serial online] 2022 [cited 2022 Jun 29];23:179-80. Available from: https://www.ijpd.in/text.asp?2022/23/2/179/341452

Dear Editor,

Aplasia cutis congenita (ACC) is a congenital anomaly usually localized to the scalp and characterized by the absence of the skin with or without the absence of underlying structures such as bone or dura. It can be associated with other genetic syndromes and diseases, but in most of the cases, it is sporadic with an estimated incidence of 3 in 10,000 births. Bullous aplasia cutis congenita (BACC) is a rare clinical subtype of ACC with fewer than 20 reported cases so far.[1] Herein, we describe a case of BACC presenting on the scalp.

Dermatological consultation was sought for a male for a single fluid-filled lesion on a hairless area of the scalp noticed at birth. He was born after an uncomplicated full-term unassisted vaginal delivery, weighing 5.9 pounds. There was no history of significant illness or medication during pregnancy. On examination, a single well-defined around 8 mm × 10 mm slightly tense bulla with turbid fluid and overlying alopecia was appreciated on the vertex region of the scalp surrounded by a collar of dense black hair, which were thicker than surrounding hair [Figure 1]. On ultrasonography, there was no underlying skull defect. General physical and systemic examination did not reveal any other abnormalities.
Figure 1: Well-defined slightly tense bulla with turbid fluid and overlaying alopecia on the vertex region of the scalp surrounded by a collar of dense black hair, which were thicker than the surrounding hair

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A diagnosis of BACC was reached on the basis of the above findings, and parents were reassured about the benign nature and course of the disease. Topical antibiotic (mupirocin 2%) was given in case bullae rupture.

ACC is characterized by the absence of epidermis, dermis, and decreased subcutaneous tissue. The defect can extend as deep as muscle and bone and usually later transforms into an atrophic, flat scar covered by a thin epithelium.[1] BACC is a rare subtype of aplasia cutis congenita and usually presents as a single cystic or bullous lesion on the scalp at birth. Rarely, BACC can also present as multiple bullous lesions on the scalp.[2] It is commonly surrounded with collarette of dark, coarse hair around the defect on the vertex known as "hair collar sign."[3] On trichoscopy, aggregates of hair which are curved at one end are seen under the bulla is known as "golf club set" sign.[4]

The exact cause of ACC is not known. The proposed etiopathogenetic factors include genetic elements, teratogens, compromised vasculature to the skin, and trauma.[1] The diagnosis of ACC is mainly clinical and based on findings such as presence since birth, localization to the scalp, and presence of hair collar sign (collarette of dark, coarse hair around lesion) which can be an indicator of underlying neural tube defect.[3] Usually, sonography of the lesion and a transfontanellar sonography are recommended to rule out underlying skull defects or cerebral abnormalities.[5] Prognosis of ACC is based on associated anomalies. While conservative management and reassurance is sufficient in most cases, surgery may be required if the defect is large or it extends into the dura mater.[5]

In conclusion, BACC is rare and only a few cases are reported, so awareness among dermatologists, pediatricians, and neonatologists is imperative for early diagnosis clinically and investigations to rule out associated defect, particularly in case hair collar sign is present so that further complications can be prevented.

Declaration of consent

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

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Garcia-Romero MT, Narvóez-Rosales V, Hojyo-Tomoka MT. Bullous aplasia cutis congenita: Case report and review of the literature. Indian J Dermatol 2011;56:337-8.  Back to cited text no. 1
[PUBMED]  [Full text]  
Prcic S, Matic A, Jablanovic S, Matić M, Gajinov Z, Stasuk N. Bullous aplasia cutis congenita: A report of two cases and brief review of the selected literature. Vojnosanit Pregl 2017;76:76.  Back to cited text no. 2
Moros Peña M, Labay Matías M, Valle Sánchez F, Valero Adán T, Martín-Calama Valero J, Muñoz Albillos M. Aplasia cutis congenita in a newborn: Etiopathogenic review and diagnostic approach. An Esp Pediatr 2000;52:453-6.  Back to cited text no. 3
Cutrone M, Grimalt R. The trichoscopic "Golf Club Set" sign for bullous aplasia cutis congenita. Skin Appendage Disord 2018;4:320-2.  Back to cited text no. 4
Browning JC. Aplasia cutis congenita: Approach to evaluation and management. Dermatol Ther 2013;26:439-44.  Back to cited text no. 5


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