Corneal subepithelial mucinous dystrophy

Classification according to ICD-10
H18.5	Hereditary corneal dystrophies
ICD-10 online (WHO version 2019)

The subepithelial corneal mucinous (SMCD) is a very rare congenital form of a superficial corneal dystrophy with onset of the disease in the first decade of life with both sides occurring veil-like haze in the center of the cornea .

Diffuse haze in the papillary region of the cornea with discrete opacity. (Reproduced in Klintworth et al. With permission from Feder et al.)

The first description comes from the year 1993 by the American ophthalmologist Robert S. Feder et al.

distribution

The cause and frequency are not known, so far one family has been reported, the inheritance is probably autosomal dominant .

Clinical manifestations

Clinical criteria are:

• Onset of illness in the first decade of life
• Painful, recurrent erosions of the cornea
• less frequent erosions during puberty , but increasing loss of vision
• ( "haze") on both sides cloudiness and fog over the entire cornea, especially centrally, below the epithelium located

diagnosis

Deposits of glycosaminoglycans ( chondroitin and dermatan sulfate) can be detected.

Differential diagnosis

Other forms of corneal dystrophy are to be distinguished.

Individual evidence

1. ↑ ^{a b} IC3D classification
2. ^ GK Klintworth: Corneal dystrophies. In: Orphanet Journal of Rare Diseases. Vol. 4, February 2009, p. 7, doi: 10.1186 / 1750-1172-4-7 , PMID 19236704 , PMC 2695576 (free full text) (review).
3. ↑ RS Feder, M. Jay, BY Yue, EL Stock, RB O'Grady, SI Roth: Subepithelial mucinous corneal dystrophy. Clinical and pathological correlations. In: Archives of ophthalmology. Volume 111, Number 8, August 1993, pp. 1106-1114, PMID 8352693 .
4. ↑ SMCD.  In: Online Mendelian Inheritance in Man . (English)

This article is about a health issue. It is not used for self-diagnosis and does not replace a diagnosis by a doctor. Please note the information on health issues !