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Year : 2021  |  Volume : 12  |  Issue : 3  |  Page : 433-438  

Management of naevoid conditions following the lines of blaschko in paediatric age group


1 Sakhiya Skin Clinic, 2nd Floor, Ayush Doctor House, Station-Lal Darwaja Road, Surat, India
2 B.J. Medical College, New Civil Hospital Asarwa, Ahmedabad, Gujarat, India

Date of Submission29-Aug-2020
Date of Decision20-Oct-2020
Date of Acceptance14-Dec-2020
Date of Web Publication12-May-2021

Correspondence Address:
Jagdish Jadavbhai Sakhiya
Sakhiya Skin Clinic, 2nd Floor, Ayush Doctor House, Station-Lal Darwaja Road, Surat - 395 003, Gujarat
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/idoj.IDOJ_681_20

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How to cite this article:
Sakhiya JJ, Sakhiya DJ, Gajjar T, Virmani N, Gandhi J, Purohit M, Bhalala K, Khambhati R, Daruwala F. Management of naevoid conditions following the lines of blaschko in paediatric age group. Indian Dermatol Online J 2021;12:433-8

How to cite this URL:
Sakhiya JJ, Sakhiya DJ, Gajjar T, Virmani N, Gandhi J, Purohit M, Bhalala K, Khambhati R, Daruwala F. Management of naevoid conditions following the lines of blaschko in paediatric age group. Indian Dermatol Online J [serial online] 2021 [cited 2021 Jun 24];12:433-8. Available from: https://www.idoj.in/text.asp?2021/12/3/433/315886



Dear Editor,

Blaschko's lines also termed as the lines of Blaschko, are believed to represent normal cell development in the skin. This concept was first introduced by the German dermatologist Alfred Blaschko at the 7th Congress of German Dermatological Society in 1901.[1] These lines create a surface pattern which is distinct from other morphological lines of the skin and skin dermatomes. They do not correspond to nervous, muscular, or lymphatic systems.[2] They are invisible under normal conditions and become apparent when some diseases of the skin or mucosa manifest themselves according to these patterns.[1] They follow a “V”-shape over the upper spine, an S-shape on the abdomen, and an inverted U-shape from the breast area over to the upper arm. These are perpendicular down the front and back of the lower extremities.[1] Pictorial view of the distribution of these lines is displayed in [Figure 1]a and [Figure 1]b. Because of their linear distribution, historically, these were assumed to represent Koebner's phenomenon, but the curvature of the lesions does not advocate this theory. These are not clearly defined over head and neck. Happle et al.[3],[4] pronounced these lines to the posterior scalp, whereas, Bolognia et al.[5] further reported them over the lateral aspect of the face and neck. Brown and Gorlin introduced vertical striations on the lips, linear midline lesions on the hard and soft palate, and linear unilateral and/or midline bands over the tongue in patients with epidermal nevi. The anatomic equivalent of Blaschko's lines have also been reported over the teeth as well as in the eyes.[6]
Figure 1: Distribution of the line of Blaschko in human body (a) front view (b) back view

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Naevus is the Latin word for “maternal impression” or “birthmark” and designates a circumscribed, non-neoplastic skin or mucosal lesion, commonly present at or soon after birth, and fixed. The term should always be qualified according to the cell or tissue origin, for instance, “connective tissue naevus” and “vascular naevus”. Confusingly, “naevus”, “naevo-” and “naevoid” are often used to refer to melanocytic naevus. The term 'naevus' is identical with cutaneous hamartoma (e.g., smooth muscle naevus/hamartoma), both containing an abnormal mixture of a usual tissue components (the word “hamartoma” originates from the Greek word hamartia, meaning “to err”).[7]

Lines of Blaschko are believed to represent pathways of epidermal cell migration and proliferation during the development of the fetus. Hitherto, the embryological basis of the distribution pattern of these lines is a riddle. Many, probably all, naevi express clones of genetically modified cells emerging from mosaicism.[8],[9],[10]

Genetic mosaicism implies the presence of two or more genetically different cell populations in an individual derived from a single zygote. The variations can be between single genes, groups of genes or entire chromosomes. Chimerism denotes the presence of two or more genetically distinct cell populations in an individual derived from two different zygotes. Chimeras can result from the fusion of dizygotic twin embryos or the fertilization by two spermatozoa, and consequent splitting of an ovum comprising a polar body. Mosaicism typically involves an abnormal clone within a normal individual whereas, chimerism includes two different normal clones. Cutaneous anomalies due to mosaicism affect any skin cell type, but the cutaneous abnormalities observed in human chimeras are always pigmentary.[11],[12]

Successive hypotheses have been proposed to describe Blaschko 's lines and each one is more convincing than the previous one, but till date, mosaicism remains the most widely accepted one.[13] Nonetheless, no conclusive decision was drawn, so the room for study remains therein.

Various congenital and acquired dermatological conditions are recognized to follow the lines of Blaschko such as X-linked dominant skin disorders, epithelial naevi, pigmentary disorders. These dermatoses have been classified into three groups: genodermatoses, congenital and/or naevoid conditions, and acquired conditions. Of these , naevoid skin lesions are most common and are present at birth or have a later onset. The term 'naevoid” is referred to mosaic forms of inherited skin conditions following these lines such as naevoid psoriasis[8] epidermal nevi (naevus unius lateris, linear porokeratosis), adnexal nevi (linear sebaceous nevus, linear basal cellnevus), pigmented lesions (systematized linear achromicnevus) and intricated nevi of the connective tissue (angio-lipomatous nevus).[14],[15],[16]

Naevoid conditions appear frequently in the pediatric population. Usually, they are developmental defects, means those originating in the embryo and fetus, that is, in the prenatal period. Given the increased general awareness of melanoma, however, caregivers often express concerns about the development or appearance of their child's nevus. An understanding of the more common types of nevus seen in children and the associated risk for the development of melanoma is essential to properly counsel caregivers and patients about management recommendations. Here, we explore potential treatment options involving either specific laser or combined lasers to tackle the majority of the naevoid condition to get more cosmetically acceptable benefits.


   Management of Naevoid Condition in the Pediatrics Age Group Top


As adhering to the concept, if the lesion is small, less energy, and reduced number of sessions are required resulting in a less painful procedure. Final cosmetic appearance is more acceptable. Though lesion is small and benign, it has a great psychological impact on the child and, hence, early clearance will minimize the effect on the self-esteem of the child. Before starting the procedure, informed consent should be obtained from all the patients. Position the patient according to the area of a lesion so that the site to be treated is close to the laser. Gloves mask and cap should be used by operators and assistants. Clean the area with a cleanser. All mentioned procedure to be done under topical anesthesia (eutectic Mixture of Local Anesthesia (EMLA 2.5%) cream). Apply 2mg/cm2 topically under occlusion for 45-60 min. The occlusion should be removed just before the procedure. Patient's eye should be protected with the eye shield or with wet gauze. Dermatologist and assistants should use wavelength-rated spectacles. Various laser specific parameters and concern regarding individual treatment for naevoid conditions are tabulated in [Table 1]. [Figure 2]a, [Figure 2]b, [Figure 3],[Figure 4],[Figure 5],[Figure 6],[Figure 7],[Figure 8],[Figure 9],[Figure 10],[Figure 11],[Figure 12],[Figure 13],[Figure 14],[Figure 15],[Figure 16]a and [Figure 16]b show the results after treatment in different naevoid conditions.
Table 1: Laser specification for various nevoids condition and special concern

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Figure 2: Angiofibroma (a) before laser (b) after laser

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Figure 3: Photograph of neurofibromatosis (a) pre-treatment (b) post treatment

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Figure 4: Nevus achromicus (a) before laser (b) effectively cleared with laser

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Figure 5: Compound Nevi on nose (a) before laser (b) after treatment

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Figure 6: Epidermal melanocytic nevus (a) pre-treatment (b) post treatment

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Figure 7: Lymphangioma circumscriptum (a) before treatment (b) after treatment

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Figure 8: Sebaceous nevus (a) before laser (b) improved with laser

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Figure 9: Freckles (a) pre-treatment (b) post treatment

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Figure 10: Lentigines (a) pre-treatment (b) post treatment

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Figure 11: Faun tail nevus (a) before treatment (b) after treatment

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Figure 12: Zosteriform vitiligo (a) before treatment (b) improved cosmetic appearance after treatment

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Figure 13: Zosteriform Eczema (a) before treatment (b) after treatment

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Figure 14: Nevus of ota (a) pre-treatment (b) post treatment

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Figure 15: Capillary haemangioma (a) pre-treatment (b) post treatment

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Figure 16: Verrucous epidermal nevus (a) before treatment (b) after treatment

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Confusingly, Blaschko's lines are difficult to diagnose with dermatomes because distribution patterns for both are delineated by a striking demarcation of cutaneous lesions at the midline. But on the close comparison, the two do not appear to be related, though on the upper extremities there is a resemblance to the anatomical location of the motor nerves. Similarly, Blaschko's lines can be differentiated from Voigt's lines (the boundaries of the areas of distribution of the main cutaneous nerve stems) as these lines do not explain the V-shape on the back or the S-shape on the abdomen.[1],[5]

In the end, each naevoid cell is genetically capable for recurrence of the lesion if treated partially. Some dermatological practitioners believe that patients should wait for the treatment of naevoid conditions until the child grows to be an adult. But our practical experience recommends that it should be treated as soon as possible regardless of the age of the child. The treatment thumb rule, therefore, is 'strike hard strike early'. To sum up, we have tried to bust the age-old myth that many diseases just cannot be cured or helped.

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.
Jackson R. The lines of Blaschko: A review and reconsideration: Observations of the cause of certain unusual linear conditions of the skin. Br J Dermatol 1976;95:349-60.  Back to cited text no. 1
    
2.
Harper JI. Genetics and genodermatoses. In: Champion RH, Burton JL, Burns DA, Breathnech SM, editors. Rook, Wilkinson/Ebling Textbook of Dermatology. 6th ed. Oxford: Blackwell Science; 1998, p. 360-1.  Back to cited text no. 2
    
3.
Happle R, Fuhrmann RA, Fuhrrnann W. Wiever-laufen die Blaschko-Linien am behaarten Kopf? Hautarzt 1984;35:366-9.  Back to cited text no. 3
    
4.
Happle R. Absence of bipolarity in Blaschko's lines. Ann Dermatol Venereol 1990;117:397.  Back to cited text no. 4
    
5.
Bolognia JL, Orlow SJ, Glick SA. Lines of Blaschko. J Am Acad Dermatol 1994;31:157-90; quiz 190.  Back to cited text no. 5
    
6.
Brown HM, Gorlin RJ. Oral mucosal involvement in nevus unius lateralis (ichthyosis Hystrix): A review of the literature and report of a case. Arch Dermatol 1960;8:509-15.  Back to cited text no. 6
    
7.
Albrecht E. Über hamartome. Verh Dtsch Ges Pathol 1904;7:153-7.  Back to cited text no. 7
    
8.
Moss C, Shahidullah H. Nevi and other developmental defects. In: Burns T, Breathnach S, Cox N, Griffiths C, editors. Rooks Textbook of Dermatology. 8th ed. Oxford, United Kingdom: Blackwell Publishing Ltd; 2010. p. 18.1-30.  Back to cited text no. 8
    
9.
Happle R, Mittag H, Küster W. What is a Nevus? A proposed definition of a common medical term. Dermatology 1995;191:1-5.  Back to cited text no. 9
    
10.
Moss C, Jones DO, Blight A, Bowden PE. Birthmark due to cutaneous mosaicism for keratin 10 mutation. Lancet 1995;345:596.  Back to cited text no. 10
    
11.
Paller AS. Expanding our concepts of mosaic disorders of skin. Arch Dermatol 2001;137:1236-8.  Back to cited text no. 11
    
12.
Stosiek N, Ulmer R, von den Driesch P, Claussen U, Hornstein OP, Rott HD. Chromosomal mosaicism in two patients with epidermal verrucous nevus. Demonstration of chromosomal breakpoint. J Am Acad Dermatol 1994;30:622-5.  Back to cited text no. 12
    
13.
Findlay GH, Moores PP. Pigment anomalies of the skin in the human chimaera: Their relation to systematized naevi. Br J Dermatol 1980;103:489-98.  Back to cited text no. 13
    
14.
Goudie RB, Jack AS, Goudie BM. 1985. Genetic and developmental aspects of pathological pigmentation patterns. In: Berry CL, editor. Dermatopathology. Current Topics in Pathology. Vol. 74. Berlin, Heidelberg: Springer. Available from: https://doi.org/10.1007/978-3-642-69574-2_3. [Last accessed on 23 2020 July]  Back to cited text no. 14
    
15.
Thomas IT, Frias JL, Cantu ES, Lafer CZ, Flannery DB, Graham JG Jr. Association of pigmentary anomalies with chromosomal and genetic mosaicism and chimerism. Am J Hum Genet 1989;45:193-205.  Back to cited text no. 15
    
16.
Tagra S, Talwar AK, Walia RS. Lines of Blaschko. Indian J Dermatol Venereol Leprol 2005;71:57-9.  Back to cited text no. 16
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