REVIEW ARTICLE
BLASCHKOLINEAR DERMATOSES - AN OVERVIEW OF
INTERESTING PATTERNED DERMATOLOGICAL DISORDERS
Arfan ul Bari
ABSTRACT
A large number of congenital/nevoid and acquired skin diseases on the human skin and mucosae assume a characteristic
morphological pattern following the Blaschko lines. This pattern recognition is important for dermatologists so as to have an idea
about the etiopathogenesis as well as to categorize various such disorders. This article is an attempt to overview and classify a
variety of blaschkoid disorders described so far in the literature.
KEY WORDS : Lines of Blaschko. Genetic mosaicism. Blaschkolinear dermatoses. Blaschkitis.
INTRODUCTION
In 1901, on the occasion of the 7th Congress of the German
Dermatological Society held in Breslau, by a German
dermatologist, Alfred Blaschko presented his observations on
various linear dermatoses.1 He pointed out that the lines
described by these conditions did not only correspond to any
known anatomical basis, but were remarkably consistent both
from patient to patient and even from one disease to another.
He observed this interesting pattern in various nevoid and
acquired linear skin diseases and carefully transposed the
lines onto dolls and statues. Subsequently, a composite
diagram was drawn by schematically transferring these
distribution patterns to the anterior and posterior aspect of the
human body. Since the original description by Blaschko, a
century ago, the concept of this patterned lines has been
potentiated greatly by Jackson, Bolognia and Happle. 1-3
EMBRYOLOGICAL BASIS OF BLASCHKOID
PATTERNS
Initially, Blaschko proposed an embryonic origin for these
lines, but he could not elaborate his concept. He described
that linear and zoniform eruptions are not a unitary
phenomenon and should be subdivided into different
categories. Blaschko was among the first to note that the
grouped eruption of Herpes zoster correlated with
dermatomes.1 However, he was unable to offer an explanation
for the peculiar distribution of the other linear dermatoses
following a set pattern lines, except calling those the lines of
embryogenesis.
Subsequently, numerous hypotheses were advanced to
explain these patterned lines. It was suggested that the
lines might run superficial to the course of blood-vessels
or lymphatics, or peripheral nerve-trunks; they might
Department of Dermatology, CMH, Muzaffarabad, A.K. / Pak
Field Hospital-IV, UNAMSIL, Kenema, Sierra Leone (West Africa).
Correspondence: Maj. Dr. Arfan ul Bari, Consultant Dermatologist, Pak Field
Hospital-IV, UNAMSIL, (United Nations Mission in Sierra Leone), Kenema, Sierra
Leone (West Africa). E-mail: albariul@gmail.com
Received October 7, 2005; accepted: March 28, 2007.
JCPSP 2007, Vol. 17 (5): 299-302
correspond to the distribution of a particular cutaneous nerve;
they might follow the boundaries of distribution of nerves;
(Voight's lines) or the lines of junction between dermatomes.
They were also thought to be related to embryonic patterns in
the skin such as the dermatomes, lines of cleavage of the skin
(Langer's lines) or hair-stream lines. An inborn error of
morphogenesis due to a single mutant gene or stretching of
the skin during embryogenesis were also considered possible
mechanisms to explain the basis of origin of these lines but
none stood fast.
The basis of distribution pattern of these lines remained an
enigma, but with time, it was established that these lines were
certainly distinct from other known linear patterns as they did
not correspond to any known nervous, vascular or lymphatic
structures and possibly represented the developmental growth
pattern of the skin.2-6 The characteristic distribution pattern of
these lines is now believed to represent two different clones of
cells in early embryogenesis. This concept greatly relies on the
hypothesis that disorders following Blaschkos lines are
caused by genetic mosaicism where two or more genetically
distinct cell populations are present in an individual derived
from a single zygote. This human mosaicism manifesting
along Blaschkos lines may result from lyonization (random
inactivation of 1 of the 2 X-chromosomes in women), somatic
postzygotic mutation during early embryogenesis or gametic
half chromatid mutation occurring before fertilization. This
concept of genetic mosaicism has been proved only in cases
of Blaschko-linear nevoid or X-linked diseases. It is still less
obvious in acquired inflammatory diseases expressing along
the lines of Blaschko. The epidermis and its appendageal
structures such as melanocytes, vascular system, fatty
hypoderm, all, separately or in combination, may be involved
in the morphological manifestations which follow the
Blaschko's lines.2, 4-8
TOPOGRAPHICAL PATTERNS EXHIBITED BY
BLASCHKOS LINES
These are V-shape lines over the upper spine (forming linear
lesions), S-shape on the anterolateral abdomen (forming
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Arfan ul Bari

whorled or swirled lesions) , inverted U-shape from the breast

area onto the upper arm (forming arches) and perpendicular
lines down the front and back of the lower extremities (forming
streaks) as described by Blaschko in his early schematic
diagram.2,3,5,7,9 All of these patterns characteristically respect
midline of the body (Figure 1, a-b). Blaschkos lines over scalp
assume spiral (splashes) morphology. When these lines and
bands are contiguous, they may exhibit one of the following
surface morphology like narrow band, large band,
checkerboard and phylloidal appearance (Figure 2, a-d)
respectively.

COMPLETING THE BODY MAP

Figure 1a: Lines of Blaschko on front
of the body.
Figure 1b: Lines of Blaschko on back
of the body..
In his schematic drawing, Blaschko left the scalp as a blank
area because of lack of information, and on the face and the
ventral aspect of the neck the nevus lines were drawn in a
rather cursory way. These blank areas were gradually filled by
other researchers. On the face, they show an hourglass-like
configuration converging on the nasal root. However, in
several areas, these lines intersected at an angle of almost 90
degrees. On the scalp, they formed a spiral configuration.
This elaborated work of describing system of Blaschko's lines
on the head and neck, in an attempt to complete the
Blaschkoid body surface map, was done by Happle3 and
Bolognia 2 and it showed definite crossing of lines in this
region. The anatomic equivalent of Blaschko's lines has been
described in the teeth and eyes as well.2, 10

DERMATOSES FOLLOWING BASCHKOID

Figure 2a: Narrow band pattern of
lines of Blaschko.
Figure 2c: Checkerboard pattern of
lines of Blaschko.
Figure 2b: Broad band pattern of lines
of Blaschko.
Figure 2d: Phylloid pattern of lines of
Blaschko.
PATTERN
Several congenital or acquired dermatoses, either inherited or
sporadic, have a linear distribution following these embryonic
lines of Blaschko. Most of them are nevoid skin lesions
present at birth or having a later onset. 2,9,11 These include
epidermal nevi (naevus unius lateris, linear porokeratosis),
adnexal nevi (linear sebaceous nevus, linear basal cell
nevus), pigmented lesions (systematized linear achromic
nevus) and intricated nevi of the connective tissue (angio-
lipomatous nevus). Rarely, genodermatoses with X-
chromosomal mosaicism that occurs in females only such as
incontinentia pigmenti, focal dermal hypoplasia, etc. exhibit
also a linear arrangement following Blaschko's lines. This
pattern is also obvious in some cases of very common
inflammatory skin diseases like lichen planus, lichen nitidus,
scleroderma, vitiligo, fixed drug eruption and chronic lupus
erythematosus 1 etc. These dermatoses have broadly been
grouped as congenital and/or nevoid (non acquired)
(Table I)2,9,11-36 and acquired conditions (Table II). 2,9,37-50
There are anecdotal reports of the occurrence of the
erythematous exanthem of scarlet fever along these lines in a
child with hypomelanosis of Ito 49 , and of eosinophilic
cellulitis 50 along these lines. Contrary to earlier belief,
recently, cases of linear scleroderma, both of the fronto-
parietal type and those occurring on limbs, have been
reported in a Blaschko pattern 44 and the spectrum is
continuously expanding. Few cases of unique self-limiting
inflammatory skin condition, called blaschkitis, have been
reported in recent past, corresponding to the established lines
of Blaschko and are categorized as acquired Blaschkolinear
dermatoses.45,46 However, some of the linear dermatoses,
initially thought to occur in blaschkoid pattern, (Becker's

300 JCPSP 2007, Vol. 17 (5): 299-302

Blaschkolinear dermatoses
Table I: Classification of non acquired Blaschkolinear dermatoses 2,9,11-36
No Groups Disorders
1. X-linked dominant skin l Incontinentia pigmenti
disorders2,9,11-16 l Focal dermal hypoplasia
l X-linked dominant chondrodysplasia
punctata (Conradi-Hunermann syndrome)
l Oral-facial-digital syndrome type 1
l MIDAS syndrome
l CHILD syndrome
l X-linked hypohydrotic ectodermal
hypoplasia*
l IFAP syndrome (ichthyosis follicularis with
atrichia and photophobia).*
2. Pigmentary l Nevus achromicus (including
disorders 2,9,17-21 hypomelanosis of Ito)
l Nevus depigmentosis
l Linear and whorled nevoid hypermelanosis
l Linear hyperpigmentation arranged in
broad bands (McCune-Albright syndrome)
l Segmental ash leaf spots
l Segmental caf au lait spots
3. Epithelial nevi / l Sebaceous nevus of Jaddasohn
tumors 2,9,22-26 l Epidermal nevus (epidermolytic or
nonepidermolytic type)
l Inflammatory linear verrucous epidermal
nevus
l Linear epidermolytic hyperkeratosis
l Linear nevus comedonicus
l Nevus corniculatus
l Unilateral basal cell carcinoma syndrome
l Linear basal cell nevus
l
l
Linear eccrine nevus
Multiple familial basal cell carcinoma
l Eccrine spiradenoma
4. Miscellaneous nevoid/ l Linear porokeratosis of Mibelli
congenital/ l Linear Dariers disease
genodermatoses 2,9,27-36 l Grovers disease (Transient acantholytic
dermatosis)
l Relapsing linear acantholytic dermatosis
l Syringocystadeoma papilliferrum
l Bart syndrome
l Familial cutaneous amyloidosis
(Partington type)
l Menkes syndrome
l Linear atrophoderma of Moulin
l Atrophoderma vermiculata
l Nevoid psoriasis
l Hailey-Hailey disease
* Carrier state in females
nevus, segmental neurofibromatosis, zosteriform leukemia
cutis, cutaneous metastases and angiolipomas) do not seem
to follow the lines of Blaschko.2,9
DIFFERENTIATION FROM OTHER PATTERNS
It is important to differentiate Blaschkos lines from other body
surface patterns. Blaschkoid dermatoses may have a delayed
onset after birth and they have to be differentiated from
acquired dermatoses exhibiting the same linear pattern.
Blaschko's lines have been most commonly confused
with dermatomes as both the distribution patterns are
characterized by a striking demarcation of cutaneous lesions
at the midline. But on close observation, the two do not seem
JCPSP 2007, Vol. 17 (5): 299-302
Table II: Classification of acquired Blaschkolinear dermatoses 2,9,37-50
No Categories Disorders
1 Inflammatory disorders 2,9,37-40 Linear psoriasis
Linear lichen planus
Lichen striatus
2 Connective tissue Cutaneous lupus erythematosus
disorders 2,9,41-44 Lupus profundus
Scleroderma
Extragenital lichen sclerosis
3 Pigmentary disorders 2,9,44-47 Vitiligo
Pigmented fixed drug eruption
Drug induced blaschkitis
Generalized lichenoid drug eruption
4 Cutaneous deposit Mucinosis
disorders2,9,48 Mycosis fungoides
5 Miscellaneous Idiopathic blaschkitis
Linear exanthema of scarlet fever
Eosinophilic cellulitis
to be different, 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.2,4,5,9
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