Download The Early Onset Case of Terra Firma Forme Dermatosis

Terra Firma Forme Dermatosis
T AD
CASE REPORT
The Early Onset Case of Terra
Firma Forme Dermatosis
Erken Başlangıçlı Terra Firma
Forme Olgusu
Pınar Özuğuz1 , Seval Doğruk Kaçar1 , Gulcan Saylam Kurtipek2 , Fatma Akyurek 2
1
Afyon Kocatepe University, Faculty of Medicine, Department of Dermatology, Afyon, Turkey
2
Konya Training and Research Hospital, Department of Dermatology, Konya, Turkey
Abstract
Terra firma-forme dermatosis (TFFD) is an idiopathic and uncommon disorder of keratinization in which
patients present with acquired, dirt-like lesions that are resistant to washing. TFFD consists of 55% of the
pediatric and 45% of the adult patients. It commonly involves neck, trunk and umbilicus. Although the affected
patients present with typical lesions, the disorder is not well-known by pediatrician and dermatologists. The
lesions can be easily removed with gentle swabbing with 70 % isopropyl alcohol pads. We describe a 3-monthold girl with TFFD, whose lesions started when she was one month old, therefore is the youngest patient thus far
reported with this condition.
Key Words: Terra firme forme dermatosis, early onset, dirty neck syndrome, acantosis nigricans
Özet
Terra firma-forme dermatoz (TFFD), yıkamaya dirençli olan kir-benzeri lezyonlar ile mevcut olan nadir
görülen idyopatik bir keratinizasyon hastalığıdır. TFFD\'nin %55\'ini pediyatrik olgular, %45\'ini erişkin
hastalar oluşturur. Genellikle boyun, gövde ve göbekte görülür. Hastalığın lezyonları tipik olsa da, hastalık
Pediyatrist ve dermatologlar tarafından iyi bilinmemektedir. Lezyonlar% 70 izopropil alkollü pamukla hafif
silme ile temizlenir. Biz burada, bir aylık iken başlayan 3-aylık bir kız hastayı, şimdiye kadar bildirilen en
küçük olgu olmasından dolayı, sunuyoruz.
Anahtar Kelimeler: Terra firme forme dermatozu, erken başlangıç, kirli boyun sendromu, akantozis nigrikans
T
erra firma-forme dermatoz (TFFD) is a
benign dermatosis whose name derives from
the Latin words terra firma, meaning “solid
ground.” The condition was first described in 1987
by Duncan et al. (1). Synonym of this condition is
“Duncan’s dirty dermatosis”. The etiology of TFFD
is unknown. Lesions may appear verrucous,
reticulate, and stuck-on, often affecting the neck,
trunk, and umbilicus. Affected patients are often
adolescents or young adults, with no gender
predilection (2). The lesions can be easily removed
with gentle swabbing with isopropyl alcohol pads.
Although only of cosmetic significance, if
unrecognized, may cause anxiety and lead to
unnecessary investigations. Here we present a 3month-old girl with TFFD, which is the youngest
reported patient.
*
A 3-month-old healthy girl presented with brown
lesions on the knee and dorsum of foot and outer
malleolus for two months (Figure 1). The child was
otherwise well, with no history of atopic eczema
but she had mild dryness of the skin. She was
bathed every other day, and various soaps had been
tried. General physical examination revealed no
other significant skin lesions. The family reported
only minimal improvement with aggressive
attempts at peeling and scraping. On physical
The case report was presented in 8
during 3-7 May 2013 in İzmir
th
Ege Dermatology Days
Corresponding Author:
Asist Prof Pınar Özuğuz
Afyon Kocatepe University, Faculty of Medicine, Department
of Dermatology 03100 Afyonkarahisar, Turkey
Phone number: 02722463333
Fax number: 02722463300
e-mail address: [email protected]
Tıp Araştırmaları Dergisi; 2015: 13(3):137-139
Case Report
137
Özuğuz et al
Figure 1. The brown lesions on the knee and dorsum of
foot and outer malleolus.
examination, she had brown, hyperkeratotic
plaques approximately 2x2 cm and 1x1 cm in size
involving bilateral knee, dorsum of foot and outer
malleolus (Figure 1). Firm rubbing of a lesion with
a gauze pad saturated with 70% isopropyl alcohol
completely removed the pigmentation (Figure 2),
confirming the diagnosis of TFFD. We suggested
treatment with a moisturizer without urea and a
mixture of 1% salicylic acid and petroleum jelly.
Discussion
TFFD is a recently described cutaneous
pigmentation disorder and is easily curable for a
physician if aware of its existence. The condition is
an uncommon disorder of keratinization in which
patients present with dirt-like lesions that are
resistant to washing. The cause of TFFD is
unknown, but is not thought to be caused by a lack
of personal hygiene. Fungal and bacterial studies in
previous cases have been unhelpful (3). TFFD is a
disorder of retantion keratinization with an
unknown etiology (4). Although urea containing
emollients are thought to play role in the
development of this disorder, our patient had no
history of usage of a topical agent containing urea
and she started lesions when she was only a month
old. The disease appears to be more frequent in
children and young adults; in other instances, it
may start during childhood or early adolescence but
present at a later age (5).
Clinically, TFFD is characterized by brown-grey
and pigmented patches or plaques and macular
discoloration located most commonly on the neck
and trunk, but also reported on scalp, lips, axilla,
chest, umblical area, chest, pubis and arms (4,6-8).
The distribution may be localized, generalized or
symmetrical (7). Acanthosis nigricans, confluent
and reticulate papillomatosis, tinea versicolor,
ichthyosis, and granular parakeratosis and
inadequate cleansing as the cause of the lesions,
Tıp Araştırmaları Dergisi; 2015: 13(3):137-139
Figure 2. The lesion after cleaning with alcohol.
such as those seen in dermatosis neglecta and ‘dirty
neck syndrome’’ of atopic dermatitis should be
excluded (8). In dermatosis neglecta, simple
washing with soap and water can remove the
pigmentation, whereas in TFFD swabbing with
isopropyl alcohol is the only effective method to
wipe it off. Therefore, when this condition is
suspected, firm, persistent pressure should be
applied while rubbing the skin with isopropyl
alcohol (4).
Because TFFD is readily identified and treated,
biopsies have rarely been performed, limiting the
availability of histopathological analyses that
might shed light on why this condition occurs (9).
The reported skin biopsies have revealed a lamellar
hyperkeratosis with absence of parakeratosis, but
with focal orthokeratosis in whorls, and increased
melanin in the hyperkeratotic areas and basal layer.
It has been suggested that the condition arises from
partial maturation and incomplete development of
keratin squames, with retention of melanin (1,10).
This
histopathological
picture
may
be
indistinguishable from other benign papillomatous
conditions, such as confluent and reticulated
papillomatosis, acanthosis nigricans and epidermal
nevi. So biopsy was unnecessary in this condition
and we didn’t perform a biopsy from our patient. It
is important for pediatricians and dermatologists to
recognize TFFD. Recognizing and treating this
condition can be gratifying for physicians, who can
provide almost instant relief (9).
Although TFFD is not harmful, it is critical for
both clinicians and dermatopathologists to be
aware of this condition in order to avoid
misdiagnosis and expensive, painful, unnecessary
evaluation, such as blood testing to investigate
possible insulin resistance in patients with
acanthosis nigricans and biopsy for other
differential diagnosis. Furthermore, future studies
are required to understand the etiology of TFFD.
138
Terra Firma Forme Dermatosis
CASE REPORT
References
1.
2.
3.
4.
5.
Duncan WC, Tschen JA, Knox JM. Terra firmaforme dermatosis. Arch Dermatol. 1987;
123:567–569.
Akkash L, Badran D, Al-Omari AQ. Terra firma
forme dermatosis. Case series and review of the
literature. J Dtsch Dermatol. 2009;7: 102–107.
Pinder VA, Eriyagama S, Saracino A, Moosa Y.
Terra firma-forme dermatosis:another cause of
reticulate pigmentation. Clin Exp Dermatol.
2012; 37(4):446-447.
Browning J, Rosen T. Terra firmaforme
dermatosis revisited. Dermatol Online J. 2005;
11: 15.
Oztürk F, Kocabaş E, Ertan P, Ermertcan AT.
Terra firma-forme dermatosis. Cutan Ocul
Toxicol. 2010 Dec; 29(4):303-305.
Tıp Araştırmaları Dergisi; 2015: 13(3):137-139
6.
7.
8.
9.
10.
Mallari R, Sinclair RD. Terra firma-forme
dermatosis of the scalp. Br J Dermatol. 2002;
147:1019.
Guarneri C, Cannavò SP. Is this skin diseased, or
is it just dirty? JAAPA 2009; 22:17.
Berk DR, Bruckner AL. Terra firma‑forme
dermatosis in a 4-month-old girl. Pediatr
Dermatol 2011; 28:79-81.
Berk DR. Terra firma-forme dermatosis: a
retrospective review of 31 patients. Pediatr
Dermatol. 2012; 29(3): 297-300.
Berk DR, Mutizwa MM. Comment regarding the
histopathology of terra firma-forme dermatosis. J
Cutan Pathol 2012; 39:300-301.
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