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A Case Report
Rahmeh Fayez MD*
ABSTRACT
We report the case of a 16 year old male patient who presented to the dermatology clinic with spiny
hyperkeratosis in flexural areas and palmoplantar keratoderma. The patient gave history of occasional
localized blisters formation. Clinical findings and the histopathological picture fit the diagnosis of Bullous
Congenital Ichthyosiform Erythroderma. Family history is also positive for the same disease.
Introduction
Case Report
*From Department of Dermatology, Prince Rashed Bin Al-Hassan Hospital, (PRHH), Irbid-Jordan
Correspondence should be addressed to Dr. R. Fayez, (PRHH), E- mail: rahmehfyz@yahoo.com
Manuscript received December 1, 2009. Accepted March 11, 2010
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Discussion
BCIE is a type of erythroderma that affects infants
and children. BCIE clinically presents at birth with
generalized erythema, blisters and erosions with or
without focal areas of hyperkeratosis.(8) Sepsis and
electrolytes imbalance can occur as secondary
events during neonatal period.(8) In subsequent
months erythema and blistering improve but patient
goes on to develop hyperkeratosis and scaling.
During childhood and adulthood, the patient usually
presents
with
localized
or
generalized
hyperkeratosis with rare focal bullae secondary to
bacterial infections. The scales are characteristically
linear, warty, with spiny ridges mainly in the
flexures and the nape of the neck. Thick scales
especially in the intertrignous areas may shed with
full thickness stratum corneum leaving tender and
denuded areas. Scale shedding and maceration in
conjunction with secondary bacterial infection
produce a foul odor. Involvement of the palms and
soles occur in about 60% of the patients, resulting in
recurrent painful fissures and contractures.(9)
Although typically those patients with K1 mutation
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Conclusion
Treatment
Treatment is mostly symptomatic and is aimed at
the ichthyotic skin as well as the bacterial infection.
Treatment depends on the age: neonatal period,
childhood, and adulthood.
On the whole, emollients, keratolytics and
antibiotics are the mainstay of treatment. In the
neonatal period the patients require management in
the intensive care unit to provide isolation and to
prevent or to treat dehydration, temperature
instability and cutaneous infection. To decrease
blisters formation and hasten erosions healing, the
affected neonates should be handled gently,
protective padding and lubricants should be used.
During childhood and adulthood the treatment is
aimed at managing the ichthyotic skin by hydration,
lubrication and keratolysis.(11)
Keratolytic creams and lotions may contain urea,
salicylic acid, alphahydroxy acids or propylene
JOURNAL OF THE ROYAL MEDICAL SERVICES
Vol. 18
No. 1
March 2011
References
1. Sudip Das, Alok Kumar Roy, Chinmoy Kar,
Arunasis Maiti. Epidermolytic hyperkeratosis with
a rare digital contracture. Indian J Dermatol
Venereol Leprol 2007; 73(4): 280.
2. Rajiv S, Rakhesh SV. Ichthyosis bullosa of
Siemens: Response to topical tazarotene. Indian J
Dermatol Venereol Leprol 2006; 72(1): 43- 46.
3. Bogenrieder T, Landthaler M, Stolz W. Bullous
congenital ichthyosiform erythroderma: safe and
effective topical treatment with calcipotriol
ointment in a child. Acta Derm Venereo 2003;
83(1): 52- 54.
4. Guardiano RA, Ryan M, Liotta EA. Bullae in a
20-year-old man. Arch Dermatol. 2001; 137(11):
1521- 1526.
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