Superficial epidermolytic ichthyosis

Superficial epidermolytic ichthyosis (SEI) is a rare keratinopathic ichthyosis (KI; see this term) characterized by the presence of superficial blisters and erosions at birth.

Erythroderma

An inflammatory exfoliative dermatosis involving nearly all of the surface of the skin. Erythroderma develops suddenly. A patchy erythema may generalize and spread to affect most of the skin. Scaling may appear in 2-6 days and be accompanied by hot, red, dry skin, malaise, and fever.


Total: 5

                       


(per page)
PMID (PMCID)
1517502
 MIXED_SAMPLE Adult
Annular epidermolytic ichthyosis: a unique phenotype.
Sahn EE, Weimer CE Jr, Garen PD.
J Am Acad Dermatol. 1992;27(2 Pt 2):348-55.
Electron microscopic examination of the annular plaques showed interrupted perinuclear tonofilament rings consistent with either bullous ichthyotic erythroderma of Brocq or ichthyosis bullosa of Siemens.
1836350
 MIXED_SAMPLE Adult
Ichthyosis bullosa of Siemens responds well to low-dosage oral retinoids.
Steijlen PM, van Dooren-Greebe RJ, Happle R, Van de Kerkhof PC.
Br J Dermatol. 1991;125(5):469-71.
Two patients with ichthyosis bullosa of Siemens (IBS) and one patient with bullous ichthyosiform erythroderma of Brocq (BIE) were treated with etretinate.
2178822
 FEMALE
Ichthyosis bullosa of Siemens and bullous ichthyosiform erythroderma--variants of the same disease?
Murdoch ME, Leigh IM.
Clin Exp Dermatol. 1990;15(1):53-6.
Ichthyosis bullosa of Siemens and bullous ichthyosiform erythroderma--variants of the same disease?
2178822
 FEMALE
Ichthyosis bullosa of Siemens and bullous ichthyosiform erythroderma--variants of the same disease?
Murdoch ME, Leigh IM.
Clin Exp Dermatol. 1990;15(1):53-6.
Bullous ichthyosiform erythroderma and ichthyosis bullosa of Siemens, occurring together in this patient may best be regarded as varying expressions of a single genodermatosis.
2138447
 MIXED_SAMPLE Adult
Ichthyosis bullosa of Siemens: further delineation of the phenotype.
Steijlen PM, Perret CM, Schuurmans Stekhoven JH, Ruiter DJ, Happle R.
Arch Dermatol Res. 1990;282(1):1-5.
Ichthyosis bullosa of Siemens can be clearly distinguished from bullous ichthyosiform erythroderma.