Pseudo Darier Sign: A Distinctive Finding for Congenital Smooth
Muscle Hamartoma
A
9-day-old girl presented with a congenital skin lesion
on the right proximal thigh with hypertrichosis. Her
parents reported a transient elevation and whitening
of the lesion by touching. On examination, a hypertrichotic,
poorly-defined, slightly raised, faintly erythematous to hy-
perpigmented plaque was elevated temporarily when it was
rubbed (Figure). Her medical history and remainder of the
physical examination were unremarkable. Because her
lesion was congenital, accompanied with hypertrichosis and
showed a strongly positive pseudo Darier sign, she was
diagnosed with a congenital smooth muscle hamartoma.
Smooth-muscle hamartoma is a benign, rare hamartoma-
tous proliferation of dermal smooth muscle fibers. It usually
presents as a congenital, solitary, skin-colored or hyperpig-
mented patch or plaque on the trunk or proximal extrem-
Figure. A, Hypertrichotic, poorly-defined, slightly raised,
faintly erythematous to hyperpigmented plaque on the right
thigh. B, Rubbing the affected area resulted in transient
induration and elevation of the plaque (pseudo-Darier sign).
J Pediatr 2016;169:318.
0022-3476/$ - see front matter. Copyright ª 2016 Elsevier Inc. All rights reserved.
http://dx.doi.org/10.1016/j.jpeds.2015.10.084
318
ities. Occasionally, multiple linear lesions or diffuse skin
involvement may occur.1-3 The surface of the lesion can
exhibit small follicular papules, variable hyperpigmentation,
and hypertrichosis. Stimulation of muscle activity (eg, rub-
bing of the plaque, cold exposure) may result in transient
elevation or increased induration of the affected area or pi-
loerection, referred to as a pseudo Darier sign. Though it
may be seen in up to 80% of patients,4 its intensity diminishes
with age. Histopathologically, numerous haphazardly ori-
ented, well-defined, and thickened bundles of smooth muscle
are seen in the dermis.
Differential diagnosis includes congenital melanocytic
nevus, Becker nevus, neurofibroma, solitary mastocytoma,
caf
e-au-lait macule, and pilar leiomyoma.1-3 Although soli-
tary mastocytomas, caf
e-au-lait macule, and pilar leiomyo-
mas are not hypertrichotic, congenital melanocytic nevus
and plexiform neurofibroma may be hypertrichotic and
require histopathologic examination. However, none of these
lesions exhibit a positive pseudo Darier sign and the recogni-
tion of this sign will protect children from unnecessary and
invasive approaches for diagnosis. Treatment is not necessary
for ordinary congenital smooth muscle hamartoma. If
desired, surgical excision or laser therapy may performed
for cosmetic concerns. n
€
Ozlem Bilgiç, MD
Fatma Tunçez Aky€urek, MD
Hilmi Cevdet Altınyazar, MD
Department of Dermatology
Selcuk University
School of Medicine
Konya, Turkey
References
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Am J Dis Child 1990;144:782-4.