Syn.: Annular epidermolytic ichthyosis.
Def .: The erythrodermic form of ichthyosis from the group associated with epidermolysis of the epidermis.
Epid .: Rarer than classical ichthyosiform erythroderma
bullous. Exact frequency undetermined.
Etiol .: Probably focal keratin 10 or 1 mutation. Autosomal recessive inheritance.
Loc: Diffuse changes all over the skin, articular flexions.
Clinical: Mild form of bullous ichthyosis 7 with migratory and recurrent eruptions. Relapses last from a few weeks to months and then regress. Changes present at birth in the form of blisters and numerous ringed and multicolored gray hyperkeratotic plaques with an erythematous margin, articular flexions involved. Ease of blistering and superficial skin infections. May be accompanied by keratinization of the hands and feet.
Hist .: A similar picture as in epidermolytic hyperkeratosis. In the electron microscope, less keratohialin clusters.
DR: Hereditary epidermolysis bullosa, pigment incontinence, Siemens bullous ichthyosis.
But: As in? Epidermolytic hyperkeratosis.
Year: The course is milder than in epidermolytic hyperkeratosis.
Lit.: [1] Burns T., Breathnach S., Cox N., Griffi ths Ch.: Rook?fs Textbook of Dermatology. Blackwell Science 2004, t. I; 34.1-34.53. [2] Braun-Falco O., Plewig G., Wolff H.H., Burgdorf W.H.C.: Dermatologia, red. wyd. pol. Gli.ski W., Wolska H., Wydawnictwo Czelej, Lublin 2002, t. I; 671-686. [3] Harper J., Oranje A., Prose N.: Textbook of Pediatric Dermatology. Blackwell Publishing 2006, t. II; 19.5-19.6. [4] Irvine A.: Inherited defects in keratins. Clin Dermatol 2005, 23; 6-14.
Source: A. Kaszuba, Z. Adamski: “Lexicon of dermatology”; XNUMXst edition, Czelej Publishing House