Niger J Paed 2014; 41 (4): 390 - 392
Henshaw EB
Dyschromatosis symmetrica
Ntia HU
Archibong JE
hereditaria: Report of a sporadic
case in a Nigerian child
Accepted: 24th July 2014
metricahereditaria (DSH) is one
dyschromic lesions on the dorsa of
Henshaw EB (
of a group of reticulate pigment
the hands and feet, with no family
Dermalology unit,
history of similar lesions. The di-
Department of Medical,
disorders of the skin. It is a rare
Faculty of Medicine / Dentistry
autosomal dominantly inherited
agnosis was confirmed by the typi-
University of Calabar, Nigeria.
cal histologic finding of basal hy-
mottled admixtures of hypopig-
perpigmentation with normal num-
Ntia HU
ber of melanocytes and absence of
Department of Paediatric,
macules on the dorsa of the ex-
melanin incontinence from a hy-
perpigmented lesion
Archibong JE
found among persons of Oriental
Department of Medicine,
origin. We hereby document the
Keywords: DyschromatosisSym-
University of Calabar Teaching Hospi-
metricaHereditaria, Nigerian,
tal Calabar, Nigeria.
first case of dyschromatosissym-
metricahereditaria in a four year
old Nigerian boy who presented
melanotoxic chemicals nor any preceding inflammatory
skin disorder such as pityriasis alba, pityriasisliche-
Dyschromatosissymmetricahereditaria (DSH) (aka.
noideschronica or psoriasisin the affected area prior to
Reticulate acropigmentation of Dohi) is a rare genoder-
the appearance of the pigmentary changes. The lesions
matoses which was first described in Japan and has been
were limited to the dorsa of the hands and feet, did not
more widely reported among Asian populations . There
affect the palms, soles, face, axilla, trunk or mucous
have also been few reports among Caucasians and in
membrane. (Figure 1) There were no similar lesions in
some Middle Eastern countries . It presents with as-
parents, siblings or other known family members and he
ymptomatic symmetrical reticular admixtures of hyper-
is the product of a non-consanguinous marriage. His
pigmented and hypopigmented macules on the dorsa of
delivery was normal, with attainment of normal devel-
the hands and feet, and sometimes with freckle-like
opmental milestones. There were no other systemic
macules on the face. The condition usually starts in in-
symptoms, no evidence of photosensitivity, and his gen-
fancy or childhood and progressively increases until
eral health was good with a weight of 84.4% of the ex-
adolescence when it stops spreading. Various therapeu-
pected. Examinationof the hands and feet revealed re-
tic agents have been employed in the treatment of this
ticulate hyper and hypopigmented macules extending
condition including topical steroids, calcipotriol and
proximally from the dorsal surfaces of the fingers and
psoralen plus UVA (PUVA), but none has been effec-
toes to the wrists and ankles respectively, the skin tex-
tive. This report aims to acquaint clinicians on the exis-
ture was normal and there were no pits or breaks in the
tence of DSH in Nigeria, thus increasing the list of dif-
linear ridges of the palms. There were no similar lesions
ferential diagnoses of pigmentary disorders in our envi-
on other parts of the body. Systemic examination was
ronment. It will also add to the existing global body of
essentially normal, full blood count and urinalysis
yielded no abnormality and biopsy of a hyperpigmented
macule showed focal reticulated papillomatosis alternat-
Case Report
ing with epidermal atrophy. There was basal hyperpig-
mentation with normal number of melanocytes and ab-
A four year old boy of the Igbo tribe in South-eastern
sence of melanin incontinence. (Figure 2) Based on the
Nigeria was brought by an overtly anxious mother to the
typical clinical presentation and suggestive histologic
paediatric dermatology clinic of the University of Cala-
finding a diagnosis of Dyschromatosissymmetrica-
bar Teaching Hospital (UCTH)with a history of progres-
hereditaria was made. The family was informed about
sively increasing symmetrical mottled hypopigmented
the natural history of DSH and counselled on their ex-
and hyperpigmented lesions on the dorsa of both hands
pectations regarding treatment and resolution, and pa-
and feet which started when he was one year old. There
tient was given a yearly appointment
was no associated pruritus, localized abnormality of
Fig 1: Mottled hyper- and
pits and breaks in dermatoglyphics; Dowling-Degos
hypopigmentation on the dorsa
disease which has a similar clinical presentation with
of the feet
RAK, but lesions are found in the flexures and large
body folds and Galli-Galli disease with a distinctive
histologic finding of suprabasal acantholysis . The typi-
cal histopathologic finding in DSH is, increased melanin
pigment in the basal layer and throughout the epidermis
in hyperpigmented lesions, and reduced or absent mela-
nin in hypopigmented lesions, all with normal number
Fig 2: Photomicrograph of
of melanocytes. In addition to the above findings our
biopsied lesion from dorsum
of the hand. (H & EX 10)
patient also had epidermal atrophy which is not a dis-
tinctive feature of DSH, however Peng et al reported a
case of DSH, howbeit with focal epidermal atrophy on
histology, mutation analysis confirmed the diagnosis of
DSH. Epidermal atrophy is more commonly found in
acropigmentation of Kitamura (RAK), however, there is
often an increase in the number of basal melanocytes
(which was not observed in our patient’s histology) and
the clinical picture is also different. DSH has been asso-
ciated with a number of disease entities, including
dystonia, mental retardation, neurofibromatosis, seizure,
Dyschromatosis symmetrica hereditaria is a rare genetic
autism, urticaria, mood disorder, β thalassemia and poly-
disorder of skin pigmentation, which was initially
but none of these associations have been
thought to be a Japanese-specific genodermatoses. It is
consistent. There was no similar or novel association in
often shown to have an autosomal dominant pattern of
our patient. Treatment of DSH has included the use of
inheritance ; however, there have been reports of auto-
topical steroids, pimerolimus, calcipotriol and Psoralen
somal recessive and sporadic cases . The latter which is
plus UVA (PUVA) all of which have not led to resolu-
the mode seen in our index patient, with no known fam-
tion of the condition .
ily history of the condition. Mutations occur in the RNA
-specific adenosine deaminase gene (ADAR1), and more
than 122 of such mutations in this gene have been re-
ported . It phenotypically presents as pinpoint or pea-
sized symmetrical mottled hypo- and hyperpigmented
macules, typically in an acral distribution. There may be
In conclusion, this report has documented the existence
associated freckle-like macules on the face, extension of
of dyschromatosis symmetrica hereditariain Nigeria; a
acral lesions proximally and lesions on the chest . On-
condition which may be misdiagnosed as vitiligo, lead-
set of DSH is usually in infancy and childhood, with
ing to the unnecessary use of potentially harmful and/or
progression and eventual stabilization prior to adoles-
expensive therapies by clinicians .
cence and this lasts for life, however, Gaiewski re-
It is hoped that our report has increased the list of differ-
ported a late onset presentation, with acral lesions begin-
entials of pigmentary disorders in our environment,
ning at the age of 26 years.
where most hypopigmentary lesions are often consid-
ered to be leprosy by the lay public. (a condition associ-
A number of other reticulate pigmentary disorders exist
ated with a high degree of stigmatization). We have also
which must be differentiated from DSH, they include:
highlighted the need for follow-up of children with DSH
dyschromatosis universalis hereditaria (DUH), which in
for the future documentation of known or novel associa-
contrast to DSH has a more widespread distribution;
reticulate acropigmentation of Kitamura (RAK) which
presents with mottled solely hyperpigmented lesions
Conflict of interest: None
also on the dorsal extremities with associated palmar
Funding: None
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