CLASSICAL PRESENTATION OF DARIER’S DISEASE: A RARE DISORDER OF KERATINISATION
Abstract
Keratosis follicularis or Darier’s disease (DD), a rare autosomal dominant disorder ischaracterised clinically by appearance of multiple, prurutic, discrete, scaly papules affectingseborrheic areas coupled with palmar pits, nail changes and mucosal involvement. Histologicalythe lesions show suprabasal clefts with acantholytic and dyskeratotic cells. We report a case of 35years old woman with typical clinical and histological features of Darier’s disease.Keywords: Autosomal dominant, palmar pits, suprabasal clefts, Darier’s diseaseReferences
Cardoso CL, Freitas P, Taveira LAA, Consolaro A. Darier
disease: case report with oral manifestations. Med Oral Patol Oral
Cir Bucal 2006;11(5): E404–6.
Munro CS. The phenotype of Dairer’s disease: penetrance and
expressivity in adults and children. Br J Dermatol
;127:126–30.
Judge MR, McLean WHI, Munro CS. Disorders of
keratinization. In : Burns T, Breathnach S, Cox N, Griffiths, eds.
Rook’s Textbook of Dermatology, 8th edition, Vol. 1.
Massachusetts, Oxford, Victoria: Blackwell Science; 2010.
p.19.81–6.
Kwok PY, Bhutani T, Liao W. Keratosis follicularis (Darier
Disease). 2010. Available at: http://emedicine.medscape.com/
article/1107340-overview#a0101
Singh S, Pandey SS. Unilateral Darier’s disease. Indian J
Dermatol Venerol Leprol 1996;62:390–1.
Fong G, Capaldi L, Sweeney SM, Wiss K, Mahalingam M.
Congenital Darier’s disease. J Am Acad Dermatol
;59(2Suppl 1):S50–1.
Dortzbach KL, Seykora JT, Werth VP. Darier’s disease
associated with an underlying neoplasm in combination with a
J Ayub Med Coll Abbottabad 2010;22(3)
http://www.ayubmed.edu.pk/JAMC/PAST/22-3/Atiya.pdf 233
nodular fibroproliferative disease. J Am Acad Dermatol
;49:S237–9.
Okada E, Nagai Y, Motegi S, Tamura A, Ishikawa O. Fatal case
of Darier’s disease with recurrent severe infections. Acta Derm
Venerol.2009;89:408–9.
Cordeiro Q Jr, Werebe DM, Vallada H. Darier’s disease: a new
paradigm for genetic studies in psychiatric disorders. Sao Paulo
Med J 2000;118:201–3.
Dhitavat J, Fairclough RJ, Hovnanian A, Burge SM. Calcium
pumps and keratinocytes: lessons from Darier’s disease and
Hailey-Hailey disease. Br J Dermatol 2004;150:821–8.
Muller EJ, Caldelari R, Kolly C, Williamson L, Baumann D,
Richard G, et al. Consequences of depleted SERCA2-gated
calcium stores in the skin. J Invest Dermatol 2006;126:721–31.
Bechetnia M, Charfeddine C, Kassar S, Ziribi H, Guettiti HT,
Ellouze F, et al. Clinical and mutational heterogeneity of Darier’s
disease in Tunisian families. Arch Dermatol 2009:145:654–6.
Kassar S, Tounsi-Kettiti H, Charfeddine C, Zribi H, Bchetnia M,
Jerbi E, et al. Histological characterization of Darier’s disease in
Tunisian families. J Eur Acad Dermatol Venerol 2009;23:1178–83.
Brodell DW, Frisch LE, Brodell RT. Robert B, David W.
Brodell, Lawrence E. Frisch. Debilitating Darier’s disease. Signa
Vitae 2007;2:1:33–4.
Darjani A, Ramezanpour A. Darier’s disease: Report of a new
case with a rare clinical appearance. Int J Dermatol 2002;1(2).
Konotochristopoulos G, katsavou AN, Kalogirou O, Agelidis S,
Zakopoulou N. Letter: Botulinum toxin type A: an alternative
symptomatic management of Darier’s disease. Dermatol Surg
;33:882–3.
Toombs EL, Peck GL, Electrosurgical treatment of etretinateresistant Darier’s disease. J Dermatol Surg Oncol
;15:1277–80.
Minsue Chen T, Wanitphakdeedecha R, Nguyen TH. Carbon
dioxide laser abalation and adjunctive destruction for DarierWhite disease (Keratosis follicularis). Dermatol Surg
;34:1431–4.
Brown VL, Kelly SE, Burge SM, Walker NP. Extensive
recalcitrant Darier’s disease successfully treated with laser
abalation. Br J Dermatol 2010;162:227–9.
Avery HL, Hughes BR, Coley C, Cooper HL. Clinical
improvement in Darier’s disease with photodynamic therapy.
Australas J Dermatol 2010;51(1):32–5.
Ahcan U, Dolenc-Voljc M, Zivec K, Zorman P, Jurcic V. The
surgical treatment of hypertrophic intertrigenous Darier’s disease.
J Plast Reconstr Aesthet Surg 2009;62:e442–6.
