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LETTER TO EDITOR
Year : 2019  |  Volume : 20  |  Issue : 4  |  Page : 347-349

Defining the dermoscopic characteristics in aplasia cutis congenita on the body


1 Department of Dermatology, Rita Skin Foundation, Kolkata, West Bengal, India
2 Department of Dermatology, Cloudnine Hospitals, Bengaluru, Karnataka, India

Date of Web Publication30-Sep-2019

Correspondence Address:
Dr Samipa Samir Mukherje
Department of Dermatology, Cloudnine Hospitals, Bengaluru, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijpd.IJPD_111_17

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How to cite this article:
Malakar S, Mukherje SS. Defining the dermoscopic characteristics in aplasia cutis congenita on the body. Indian J Paediatr Dermatol 2019;20:347-9

How to cite this URL:
Malakar S, Mukherje SS. Defining the dermoscopic characteristics in aplasia cutis congenita on the body. Indian J Paediatr Dermatol [serial online] 2019 [cited 2019 Nov 14];20:347-9. Available from: http://www.ijpd.in/text.asp?2019/20/4/347/268386



Sir,

Aplasia cutis congenita (ACC), reported by Cordon in 1767,[1] has an incidence of 1/10,000 births and is characterized by the absence of a skin fragment at birth most commonly present on the scalp but may be seen on the face, trunk, and limbs. Diagnosis of this entity is generally made clinically; however, in recent times, dermoscopy has served to be an important tool to differentiate between sebaceous nevus and posttraumatic lesion on the scalp. Although the characteristics of ACC have been described on the scalp,[2],[3] to the best of our knowledge, there are no reports describing its dermoscopic feature on the body (nonhairy areas). Herein, we report a case of ACC located bilaterally symmetrically over the lateral aspect of the neck.

A 4-year-old male child was brought to the dermatology outpatient department with lesions located symmetrically over both sides of the neck since birth [Figure 1], slowly improving with age. The mother gave a history of the lesion being like a whitish membrane at birth which is slowly improving to gain back the normal skin color. There was no history suggestive of any other systemic involvement. The lesion was treated as a posttraumatic ulcer at birth and managed conservatively. The child was off treatment for the past 2 years.
Figure 1: A discolored patch with a smooth surface and visible erythema symmetrically located on the lateral aspect of the neck

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Clinical examination revealed a discolored patch with a smooth surface and visible erythema symmetrically located on the lateral aspect of the neck. Palpation of the affected area revealed thinned out skin. The patch was characterized by areas of healing proceeding from the lower end of the lesion to the upper end demonstrated by hyperpigmentation of the lesion, decrease in erythema, and dilated vasculature.

Dermoscopy of the lesion on the left side revealed a healed lower two-third and an upper one-third which was still undergoing healing. The healed part revealed an intensified melanin pigment network suggestive of postinflammatory hyperpigmentation with no perceptible erythema. The healing part, on the contrary, demonstrated thinned out whitish membrane suggestive of a loss of epidermis, with visible linear vessels with increased caliber and a perceptible erythema. The periphery of this area demonstrated pigment network encroaching into the center of the lesion. Furthermore, there was an absence of follicular and eccrine openings suggestive of scarring [Figure 2].
Figure 2: The healing part demonstrated thinned out whitish membrane with visible linear vessels with increased caliber and a perceptible erythema. The periphery shows pigment network encroaching into the center of the lesion and an absence of follicular and eccrine openings suggestive of scarring

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The right-sided lesion demonstrated a healed lower half and an upper half which was still undergoing the process of healing. The healed area demonstrated a similar picture as on the left side with increased melanin pigment network. The healing part demonstrated similar features of whitish membrane, loss of follicular and eccrine openings, linear vessels with increased caliber, and a mild erythema [Figure 3].
Figure 3: The healing part demonstrated similar features of whitish membrane, loss of follicular and eccrine openings, linear vessels with increased caliber and a mild erythema and an increased melanin pigment in the periphery

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Histopathology of the lesion revealed thin layer of dermal collagen without overlying epithelium and adnexal structures.

A diagnosis of ACC was made based on history, clinical examination, dermoscopic finding, and histopathology.

ACC generally presents on the scalp as a solitary lesion, however, the possibility of multiple lesions, on the other areas of body, and even symmetric distribution has been documented. The absence of skin in ACC varies in depth with involvement of the epidermis and upper dermis with minimal alopecic scarring and can extend up to deep dermis to subcutaneous tissue and very rarely up to periosteum. The diagnosis is mostly clinical where biopsy is rarely needed.

Dermoscopic features of ACC on the scalp have been well described in the literature. Rudnicka et al. described a radial arrangement of hair shafts, visible elongated hair bulbs with darkly pigmented hair suggestive of anagen hair follicles visible through the translucent membrane and prominent vessels with increased caliber along with the loss of follicular opening both of which correspond to scarring.[4] Trichoscopy may be useful in newborns to differentiate this lesion from nevus sebaceous. Neri et al. have described that ACC shows a complete lack of skin appendages and a translucent appearance, while nevus sebaceous shows sebaceous gland (yellow dots) not associated with hair follicles.[5] VerzÌ et al. described a starburst appearance on the scalp formed by radially oriented, horizontal hair follicles surrounding a patch of scarring alopecia, and hypothesized that this might be due to the neural tube defects producing aberrant shearing forces during the formation of the follicles forcing them to point outward from the defect.[6]

Although dermoscopic features of ACC on the scalp have been well documented in the literature, there has been no documentation of its feature on the body. On dermoscopy in our patient, the healed part revealed an intensified melanin pigment network suggestive of postinflammatory hyperpigmentation with no perceptible erythema. The healing part, on the contrary, demonstrated thinned out whitish membrane suggestive of a loss of epidermis, with visible linear vessels with increased caliber and perceptible erythema. The periphery of this area demonstrated pigment network encroaching into the center of the lesion. The linear vessels would correspond to the visible superficial vascular plexus in the upper dermis. Furthermore, there was an absence of follicular and eccrine openings suggestive of scarring. In contrast to the lesions on the scalp, there were no hair changes that could be demonstrated in the form of classical hair collar sign which is used to establish the diagnosis of ACC on the scalp.

In our opinion, a constellation of changes in the pigment network, encroaching pigment network into the white structureless area, increased the caliber of the telangiectatic blood vessel, and absence of follicular and eccrine opening could point toward the possibility of dermoscopic diagnosis of ACC. To the best of our knowledge, the dermoscopic features of ACC on the body except scalp have not been described so far. Further continuous observations need to be made to establish the dermoscopic diagnostic features of ACC on the body.

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.
Cordon M. Extract from a letter describing three children from the same mother born with parts of the extremities devoid of skin. J Med Chir Pharm 1767;26:556-7.  Back to cited text no. 1
    
2.
Pinheiro AM, Mauad EB, Fernandes LF, Drumond RB. Aplasia cutis congenita: Trichoscopy findings. Int J Trichology 2016;8:184-5.  Back to cited text no. 2
    
3.
Damiani L, Aguiar FM, da Silva MV, Miteva MI, Pinto GM. Dermoscopic findings of scalp aplasia cutis congenita. Skin Appendage Disord 2017;2:177-9.  Back to cited text no. 3
    
4.
Rudnicka L, Olszewska M, Rakowska A. Atlas of Trichoscopy: Dermoscopy in Hair and Scalp Disease. London: Springer; 2012. p. 191-202.  Back to cited text no. 4
    
5.
Neri I, Savoia F, Giacomini F, Raone B, Aprile S, Patrizi A, et al. Usefulness of dermatoscopy for the early diagnosis of sebaceous naevus and differentiation from aplasia cutis congenita. Clin Exp Dermatol 2009;34:e50-2.  Back to cited text no. 5
    
6.
VerzÌ AE, Lacarrubba F, Micali G. Starburst hair follicles: A dermoscopic clue for aplasia cutis congenita. J Am Acad Dermatol 2016;75:e141-2.  Back to cited text no. 6
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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