Reference No. Of Cases Country Age Sex Family History Sites Associations Treatment Response       Of Onset Of Diagnosis     Comedones Open &/or Closed Classic Darier’s Disease       Derrick et al, 1995 [3] 2 UK 61 65 M Negitive *Face,Scalp Nails, Palms Pruritus Topical steroid, Emolients Initial improvement then recurrence 10s 55 M Positve Face,Trunk Nails,Palms,Trunk Scalp nodules Etretinate 50mg/day Improved, except for persistent scalp nodules Song et al, 1997 [6] 1 Korea 16 43 M Negitive Face, Scalp,Trunk Trunk,Nails None Etretinate 30mg/day Improved Lee et al, 2002 [5] 2 Korea 25 47 M Negitive Face,Trunk Trunk, Face Greasy skin, Pruritus, Facial ice-pick scars & nodules  with a leonine appearance Oral Isotretinion 30 mg/day Stopped  after 4 weeks due to side effects 24 34 M Negitive Face None Facial nodules Minocyclin No response after several months Aliagaoglu et al, 2006 [9] 1 Turkey 18 42 M Negitive Face,Neck, Trunk,Legs None Cornifying& hypertrophic variants Acitretin 1mg/kg/day No response after 4 months Long-term antibiotics No response Topical adapalene for comedonal&cornifying lesions No response after 4 months Yegin et al, 2007 [7] 1 Turkey 7 42 M Negitive Face,Scalp Trunk Face,Scalp Trunk, Palms, Soles, Nails, Oral Scalp syringocyst-adenoma papilliferum& scarring alopecia, Pitted scars on face, scalp, trunk, Malodor Isotretinion 1mg/kg/day for 3 months Poor response Tsuruta et al, 2010 [4] 1 Japan 10s 22 M Negitive Face,Trunk None Ice-pick scars on face Oral: biotin, Korean ginseng, anti-histamins No response Topical: bufexamac, calcipotriol, steroid No response Etretinate 10 mg/day & topical adapalene Mostly improved Chung et al, 2011 [1] 1 Korea Late 20s 31 F Positve Face None Greasy skin Oral minocycline & Topical tacrolimus Little improvement Goel et al, 2012 [8] 1 India 66 70 M Negitive Face Scalp, Palms None NM NM Buchanan & Strutton , 2013 [13] 1 Australia 22 52 M Negitive Not defined NMSC NM NM               Face,Neck,Chest,Abdomen,Back       Lora et al, 2013 [11] 2 Italy NM 46 M Negitive Scalp Trunk, Palms NM NM NM       25 68 F Negitive Face, Trunk Palms NM NM NM Our case 1 Egypt 16 19 M -ve Face,Trunk Trunk, Scrotum, Palms, Nails Greasy skin, Ice-pick scars, Facial nodules Surgical removal of large nodulo-cystic facial lesions & then oral Isotretinion 40 mg/day Improved cosmetic results after removal of the nodules. No response after 2 months

Table 1: Summary of the reported cases of comedonal Darier’s disease