what-when-how
In Depth Tutorials and Information
skin products and practices that increase the risk of
trauma, skin sensitivity and infection.
36
Bath or shower
times should be limited with the use of warm rather
than hot water to prevent stripping of natural oils from
the skin. After washing, skin should be patted dry with
immediate application of emollients to seal in moisture.
While physicians should encourage careful sun protec-
tion in all patients, this message is especially important
in patients with OI as UV irradiation furthers dermal col-
lagen damage.
37
Sun protection includes routine use of
broad-spectrum sunscreens with UVA and UVB cover-
age and avoidance of sun exposure during peak hours (10
am to 4 pm). Protective clothing, wide-brimmed hats and
sunglasses are also advisable to decrease UV skin dam-
age. One caveat to rigorous sun protection is an increased
risk of vitamin D deficiency.38
38
Susceptible patients should
take oral vitamin D supplementation as a safe and effec-
tive option to sustain appropriate vitamin D levels.
39
Several therapies exist for hyperhidrosis depending
on the severity and areas affected. Patients who experi-
ence mild to moderate hyperhidrosis may benefit from
prescription strength antiperspirants containing 20%
aluminum chloride in ethanol (Drysol) or 6.25% alumi-
num tetrachloride (Xerac).
40
Alternatively, iontophoresis
treats hyperhidrosis by introducing ionized substances
through intact skin using a direct current and temporar-
ily blocking sweat glands.
41
Other options for hyperhi-
drosis include botulinum toxin,
42
oral glycopyrrolate
43
and surgery.
43
Unfortunately, clinical trials evaluating the effi-
cacy of specific interventions in OI skin are lacking.
Mechanistically, topical retinoids may benefit OI patients
with thin, fragile skin. Retinoids are a derivative of vita-
min A that stimulates collagen synthesis by fibroblasts.
This drug class has been established as the gold standard
in treating chronologically aged and photoaged skin.
44-50
Similar to intrinsically and extrinsically aged skin, OI skin
has deficiencies in dermal collagen. It would be interest-
ing to study the ability of topical retinoids to increase der-
mal volume in individuals with OI. Other options to treat
the aging skin including iller injections, chemical peels
and ablative laser resurfacing may be too harsh for use in
OI skin although definitive studies are pending.
Several treatments for EPS have been studied, includ-
ing liquid nitrogen cryotherapy,
51
topical retinoids
(tazarotene gel),
52
systemic retinoids (isotretinoin),
53
imiquimod,
54
laser resurfacing
55
and cellophane tape
stripping.
56
Choosing among these options is largely
based on physician and patient preferences. Intralesional
corticosteroids
52
as well as curettage and electrodessica-
tion
31
have been found to be ineffective in treating EPS.
We are still in the early phases of understanding OI
skin pathologies and potential treatments. Preliminary
observational studies have characterized the clini-
cal presentation of skin in patients with OI. However,
future studies are necessary to appreciate how OI skin
responds to environmental and pharmacologic chal-
lenges such as UV irradiation and topical retinoids.
The mechanistic changes of the integument in OI are an
exciting area of future dermatologic research.
SUMM
ARY AND RECOMMENDAT
IONS
●
The normal structure of the skin is made of the
epidermis, dermis and subcutaneous fat. Skin
protects us from external insults, maintains internal
homeostasis and facilitates social interactions.
●
Cutaneous manifestations of OI include thinness,
translucency, easy bruisability and impaired
elasticity. EPS has also been associated with OI.
●
The pathophysiology of OI involves mutations
in genes encoding α-chains of type I collagen, the
main component of the dermis. Deficiencies in the
quantity and structure of collagen lead to various
presentations of OI.
●
The histopathology of OI shows a decrease in dermal
collagen.
●
Histologic findings of EPS observed in OI include
focal areas of hyperplastic epidermis with
perforating channels containing fragmented elastic
tissue and nuclear debris.
●
The wide differential for the dermatologic features
of OI includes chronologic aging, photoaging,
steroid-induced atrophy and other connective tissue
diseases.
●
Management of cutaneous symptoms of OI involves
gentle skin care, sun protection, off-label use of
topical retinoids and targeted treatments for EPS and
hyperhidrosis.
●
Future studies are required to determine the efficacy
of dermatologic interventions in OI-associated skin
conditions.
References
