Search
ichthyosis vulgaris (ichthyosis simplex)
Etiology:
- frequently associated with atopy
- atopic dermatitis, allergic rhinitis, intrinsic asthma
Epidemiology:
- no sex predilection
Pathology:
1) compact hyperkeratosis
- epidermis proliferated normally, but keratin is retained with a resultant thickened stratum corneum
2) reduced or absent granular layer
3) germinal layer flattened
4) electron microscopy:
- small, poorly formed keratohyalin granules
Genetics:
- autosomal dominant
- associated with defects in FLG
Clinical manifestations:
1) mild generalized hyperkeratosis with xerosis
2) distribution
a) generally diffuse
b) most pronounced on lower legs (fish scale pattern)
c) generally sparing of axillae, antecubital & popliteal fossa
d) generally sparing of face, but cheeks & forehead may be involved
3) keratosis pilaris
4) age of onset 3-12 months of age
5) ocular keratopathy is rare
Differential diagnosis:
1) xerosis
2) acquired ichthyosis due to paraneoplastic syndrome
3) drug-associated ichthyosis (triparanol)
4) X-linked ichthyosis
5) lamellar ichthyosis
6) generalized dermatophytosis
Management:
1) prognosis:
a) may improve in summer, humid climates & in adulthood
b) lesions on cheeks during childhood generally improve as an adult
2) emollients
3) keratolytics
a) propylene glycol 44-60% qhs after bathing with plastic occulsion (plastic suit worn as pajamas) weekly
b) salicylic acid
c) urea 10-20%
d) alpha-hydroxy acids (glycolic acid, lactic acid)
4) systemic retinoids
a) isotretinoin
b) acitretin
c) etretinate
Notes:
- Support group: Foundation for Ichthyosis & Related Skin Types (FIRST) [2]
Related
13-cis retinoic acid; isotretinoin (Accutane)
acitretin (Soriatane)
etretinate (Tegison)
hyperkeratosis
keratosis pilaris
salicylic acid
xerosis (dry skin)
General
ichthyosis; erythrokeratodermia variabilis
Database Correlations
OMIM 146700
References
- Color Atlas & Synopsis of Clinical Dermatology, Common
& Serious Diseases, 3rd ed, Fitzpatrick et al, McGraw Hill, NY,
1997, pg 96-101
- Foundation for Ichthyosis & Related Skin Types (FIRST)
P.O. Box 20921
Raleigh, NC 27619
(800) 545-3286; fax -> (919) 781-0679