Recurrent corneal ulcerations with perforation in keratosis follicularis (Darier-White disease)
نویسندگان
چکیده
منابع مشابه
Rare ocular manifestations in keratosis follicularis (Darier–White disease)
Keratosis follicularis (Darier's disease) is a rare (1 in 30,000-100,000) genetic autosomal-dominant predominantly dermatological disorder characterized by hyperkeratosis and acantholysis due to a defective calcium transport in the cells. Ocular findings, if present, are very rare in this condition. Here, we are reporting a case of keratosis follicularis (Darier's disease) with ocular manifesta...
متن کاملDermoscopy of keratosis follicularis squamosa
Although the characteristic clinical appearance and the differences in distribution, it is often difficult to differentiate keratosis follicularis squamosa (Dohi) from other keratotic disorders. Here, we describe the case of a 5-year-old boy with Dohi in the first time that by using dermoscopy. Dermoscpoy of the lesion showed typical lotus leaves on the water appearance and follicular plug in t...
متن کاملSubstance P in keratosis follicularis spinulosa decalvans
KFSD: Keratosis folicularis spinulosa decalvans SP: Substance P INTRODUCTION Keratosis follicularis spinulosa decalvans (KFSD) is an inherited rare disorder characterized by diffuse keratosis pilaris and scarring alopecia. Palmoplantar keratoderma, ocular abnormalities, and atopy can also be present. Most cases occur in males and have a X-linked pattern of inheritance, although autosomal domina...
متن کاملKeratosis follicularis spinulosa decalvans: case report.
Keratosis follicularis spinulosa decalvans is a rare disease, with genetic transmission either X-linked or sporadic, characterized by follicular hyperkeratosis and cicatricial alopecia. The disease usually begins in early childhood exacerbating throughout adolescence. The therapies are somewhat effective, with frustrating treatment when there are changes which are predominantly cicatricial. It ...
متن کاملIn vivo confocal microscopy of the cornea in Darier-White disease.
Darier-White disease (also known as Darier disease or keratosis follicularis spinulosa decalvans) is a rare dominantly inherited skin disorder characterized by firm, scaly, cutaneous papules and plaques distributed over various regions of the body. Histopathologic and electron microscopic studies of biopsied skin specimens have revealed a loss of cell to cell adhesion and abnormal differentiati...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
ژورنال
عنوان ژورنال: British Journal of Ophthalmology
سال: 2002
ISSN: 0007-1161
DOI: 10.1136/bjo.86.10.1192