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LETTER TO EDITOR
Year : 2020  |  Volume : 21  |  Issue : 3  |  Page : 245-247

Dermoscopic dirty scalp of darier's disease


1 Consultant Dermatologist, Rita Skin Foundation, Kolkata, West Bengal, India
2 Associate Dermatologist, Skin Secrets, Mumbai, Maharashtra, India

Date of Submission20-Nov-2019
Date of Decision24-Dec-2019
Date of Acceptance21-Feb-2020
Date of Web Publication30-Jun-2020

Correspondence Address:
Dr. Harshal Ranglani
Skin Secrets, Pedder Road, Mumbai - 400 026, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijpd.IJPD_113_19

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How to cite this article:
Malakar S, Ranglani H, Malakar S. Dermoscopic dirty scalp of darier's disease. Indian J Paediatr Dermatol 2020;21:245-7

How to cite this URL:
Malakar S, Ranglani H, Malakar S. Dermoscopic dirty scalp of darier's disease. Indian J Paediatr Dermatol [serial online] 2020 [cited 2020 Aug 13];21:245-7. Available from: http://www.ijpd.in/text.asp?2020/21/3/245/288483



Sir,

Darier's disease is an autosomal dominant disorder clinically presenting as dirty warty papules, mainly in the seborrheic areas. Characteristic palmar pits and punctate keratosis along with nail changes are present in most cases.[1] Here, we highlight the trichoscopic findings in a case of Darier's disease, which can be used as a diagnostic marker.


  Case Top


A 13-year-old boy presented with lesions over the face, neck, and arms, for the past 10 months. They had progressively increased in number. There were also nail changes present. The patient reported having a greasy scalp, with occasional foul smell. Clinical examination revealed numerous brown, warty papules over the forehead, neck, and upper arms [Figure 1]. The nails showed splinter hemorrhages along with V-shaped nicking and alternating longitudinal white and red bands. Presence of palmoplantar pitting was noted. There were yellowish-brown greasy scales on the scalp. Based on the history and clinical examination, a diagnosis of Darier's disease was suspected.
Figure 1: Dirty warty papules over the forehead of a 13-year-old boy with Darier's disease. Similar lesions were present over other seborrheic areas

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Dermoscopic examination of the warty brown papules revealed pseudocomedones with a surrounding whitish halo on a diffuse, pink background [Figure 2]. On trichoscopic examination, there were thick, brownish gray scales present in both perifollicular and interfollicular regions, giving the surface of the scalp a dirty look [Figure 3]. Histopathology revealed presence of acantholysis, dyskeratosis, and a few corps ronds [Figure 4].
Figure 2: Dermoscopy showing pseudocomedones. They are polygonal, yellow-brownish areas often surrounded by whitish halo. A diffuse pinkish background is also noted. In this particular field, peudocomedones appear conglomerated

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Figure 3: “Dermoscopic dirty scalp” of Darier's disease: Presence of thick, grayish-brown greasy scales interspersed with black-dotted dirt particles covering the scalp surface. The hair shafts are also studded with scales

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Figure 4: Histopathological examination showing acantholysis, dyskeratosis, and a few corps ronds

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  Discussion Top


Darier's disease is an autosomal dominant disorder, occurring as a result of a defect in the ATP2A2 gene, resulting in SERCA2 defects in keratinocytes. It is a part of the spectrum of acantholytic dyskeratotic disorders.

The characteristic finding on the dermoscopy of lesional skin of Darier's disease is the presence of “pseudocomedones,” consisting in dilated openings with raised or flat borders and central polygonal brownish or yellowish star-shaped areas. These central brownish areas of the pseudocomedones correspond histopathologically to compact hyperkeratosis,[2],[3] whereas the whitish halo corresponds to acanthosis. The pinkish background along with the presence of vessels of varying patterns indicates dermal inflammation.[3]

There are several variants of Darier's disease including linear, segmental, and comedonal. In addition to skin involvement in Darier's disease, scalp involvement also occurs as scalp is also a seborrheic region. In fact, there have been reports of isolated involvement of the scalp as well.[4],[5] This may occur in the form of irregular plaques formed from coalescing papules or even tinea amiantacea.[5],[6] Most frequently, the scalp is covered in greasy scales. Patients even complain of the scalp being difficult to clean and the hair being matted. Some individuals may complain of a “damp dog” smell emanating from the scalp.[6] Scalp hair is usually preserved in Darier's disease, but scarring alopecia may occur in severe cases. On trichoscopic examination, the scalp shows an overall dirty look because of the presence of excess scales and sebum trapping the dirt and dust (black-brown dotted particles) from the environment, giving it a brownish-gray appearance. Hence, the term “dermoscopic dirty scalp” has been coined for this distinct appearance of the scalp in Darier's disease.

This may be particularly useful in cases presenting with isolated scalp involvement. A diagnosis of Darier's disease, which would otherwise have been missed, could be suspected on the basis of trichoscopy.

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.
Paller AS, Mancini AJ. Hereditary Disorders of Cornification, Hurwitz Clinical Pediatric Dermatology. Canada, Elsevier; 2011. p. 92-114.  Back to cited text no. 1
    
2.
Errichetti E, Stinco G, Lacarrubba F, Micali G. Dermoscopy of Darier's disease. J Eur Acad Dermatol Venereol 2016;30:1392-4.  Back to cited text no. 2
    
3.
Lacarrubba F, Verzì AE, Errichetti E, Stinco G, Micali G. Darier disease: Dermoscopy, confocal microscopy, and histologic correlations. J Am Acad Dermatol 2015;73:e97-9.  Back to cited text no. 3
    
4.
Wheeland RG, Donaldson ML, Bulmer GS. Localized Darier's disease of the scalp complicated by Trichophyton tonsurans infection. Arch Dermatol 1985;121:905-7.  Back to cited text no. 4
    
5.
Hussain W, Coulson IH, Salman WD. Pityriasis amiantacea as the sole manifestation of Darier's disease. Clin Exp Dermatol 2009;34:554-6.  Back to cited text no. 5
    
6.
Yang MY, Park SM, Lee HJ, Jin H, You HS, Shim WH, et al. Darier's disease appearing as pityriasis amiantacea. Ann Dermatol 2018;30:729.  Back to cited text no. 6
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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