Familial dyskeratotic comedones L85.8

Rodin et al. 1967

Autosomal dominant, generalized cornification anomaly characterized by disseminated, innumerable, approximately 0.2-0.3 cm, black (follicular but also non-follicular) comedone-like papules with central hyperkeratosis. The entire integument is affected, except for the mucous membranes, palms, and capillitium. The keratoses can be expressed by lateral pressure without bleeding.

First appearance usually during puberty, sometimes together with acne vulgaris.

In the familial form, mutations in PEN-2 are detectable.

Proximal extremities, trunk, face.

Cyst-like follicular and interfollicular depressions of the surface epithelium with ortho- and parakeratotic keratinization. The cyst epithelium may be thinned in sections; dyskeratosis with acantholysis in places. Electron microscopy demonstrates similarities to Hailey-Hailey disease.

Generally caring with Hanrstoff-containing Externa. Experiment with systemic retinoids.

1. Carneiro SJ et al (1972) Familial dyskeratotic comedones. Arch Dermatol 105:249-251.
2. Cheng MJ et al. (2014) Familial disseminated comedones without dyskeratosis: report of an affected family and review of the literature. Dermatology 228:303-306.
3. Maddala RR et al (2016) Familial dyskeratotic comedones: A rare entity. Indian Dermatol Online J 7:46-48.
4. Prabha N et al (2018) Familial dyskeratotic comedones. Skinmed 16:273-274.
5. Rodin HH et al (1967) Diffuse familial comedones.Arch Dermatol 95: 145-146.