A patient, aged 50 years, presented with complaints of bumps on her chest, behind her ears, and in her ear conchae. She has had this eruption since she was a teenager, and reported it worsened in hot weather. The patient reported two of her four children had the same affliction.
On exam, she had warty papules on her sternum, neck, post-auricular sulcus, axilla, and ear conchae. There were small pits on her palms and soles. Her nails demonstrated V-shaped nicking at the distal margin.
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Darier disease (DD), or keratosis follicularis, is a dermatologic condition caused by an autosomal dominant mutation in the ATP2A2 gene.
DD presents in childhood with warty papules and plaques on seborrheic surfaces and sometimes mucosal surfaces. DD may be exacerbated by sweat, warmth, sunlight, and trauma. Infection of the lesions may also occur during the course of this disease.
Nails may display red and white lines and V-shaped nicking of the tips. Palms and soles may have punctate keratosis.
Epilepsy, mental retardation, bipolar disorder and other neuropsychiatric conditions have been associated with DD, possibly due to co-segregation with ATP2A2 or associated increased susceptibility to these illnesses.
Treatment typically includes use of corticosteroids and emollients and oral and topical retinoids. Keeping the lesions free from sweat and heat is also important to therapy, as is control of any infection of the lesions that may be present.
Yasmin Qaseem, BS, is a medical student at Baylor College of Medicine
Adam Rees, MD, a graduate of the David Geffen School of Medicine at UCLA, practices dermatology in Los Angeles.
- Buteica E et al. “Darier disease and Hailey-Hailey disease.” Romanian Journal of Morphology and Embryology. 2011; (4): 423-426.
- Gordon-Smith K et al. British Journal of Dermatology. 2010; doi: 10.1111/j.1365-2133.2010.09834.x
- Letulé V et al. Clinical and Experimental Dermatology. 2013; doi: 10.1111/ced.12078
- Szigeti R et al. “Autosomal-Dominant Calcium ATPase Disorders.” Journal of Investigative Dermatology. 2006; 126: 2370-2376.