Ann Dermatol.  2011 Dec;23(Suppl 3):S398-S401. 10.5021/ad.2011.23.S3.S398.

A Case of Familial Comedonal Darier's Disease

• Chung J
• Kim JY
• Gye J
• Namkoong S
• Hong SP
• Park BC
• Kim MH

• KMID: 2171882
• DOI: http://doi.org/10.5021/ad.2011.23.S3.S398

Abstract

Darier's disease is a genetic disorder of keratinization with autosomal dominant inheritance. Its appearance is usually in the form of greasy, crusted, keratotic yellow-brown papules and plaques found particularly on seborrheic areas of the body. However, there are some clinical variants showing atypical skin lesions. Here we report an unusual case of Darier's disease, which mainly showed prominent comedonal papules over the face.

Keyword

Comedonal type; Darier disease; Keratosis follicularis

MeSH Terms

Darier Disease
Keratins
Skin
Wills
Keratins

Figure

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  Fig. 1 Multiple skin colored or somewhat erythematous papules on the forehead, eyelids and areas along paranasal and nasolabial folds. The facial skin looked greasy (the arrow indicates the biopsy site).

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  Fig. 2 (A) Follicular epidermal invagination, diminished granular layer and lacunae formation. Inflammatory cellular infiltration in the superficial dermis (H&E, ×40 magnification). (B) Keratotic materials with parakeratotic cells in the dilated follicles. Suprabasal lacunae with villi structures (H&E, ×200 magnification). (C) Acantholytic dyskeratosis, corp ronds and grains (H&E, ×400 magnification).

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