title: Two Egyptian cases of lipoid proteinosis successfully treated with acitretin.
abstract: BACKGROUND
  Lipoid proteinosis (Urbach-Wiethe disease) is a rare progressive autosomal recessive disorder, characterized histologically by deposition of periodic acid Schiff-positive, diastase resistant, hyaline-like material into the skin, upper aerodigestive tract, and internal organs.
  
  
  METHODS
  We report two cases of lipoid proteinosis. A 2-year-old girl presented with vesiculobullous skin lesions on her face, trunk, extremities and scalp, inability to protrude the tongue and hoarseness of voice that appeared few months after birth. The other case is a 4-year-old girl, who presented with waxy papules on face and trunk, hoarseness of voice and enlarged lips and tongue. The lesions healed leaving pitted scars in both cases. Based on clinical, histopathological and laryngoscopy findings, lipoid proteinosis was diagnosed in both cases. Acitretin was started in a dose of 0.5 mg/kg/day in every child. Complete remission of cutaneous lesions and improvement of the hoarseness was observed after one year.
  
  
  CONCLUSIONS
  Acitretin may be benificial for treatment of mucosal and cutaneous lesions in lipoid proteinosis.
authors.affiliation: Department of Dermatology, Andrology and S.T.Ds, Faculty of Medicine, Menoufiya University, Shebin El Kom, Menoufiya, Egypt;
authors.forename: Ola Ahmed
authors.initials: OA
authors.lastname: Bakry
authors.affiliation: Department of Pathology, Faculty of Medicine, Menoufiya University, Shebin El Kom, Menoufiya, Egypt.
authors.forename: Rehab Monir
authors.initials: RM
authors.lastname: Samaka
authors.affiliation: Department of Pathology, Faculty of Medicine, Menoufiya University, Shebin El Kom, Menoufiya, Egypt.
authors.forename: Nanees Shawky
authors.initials: NS
authors.lastname: Houla
authors.affiliation: Department of Dermatology, Andrology and S.T.Ds, Faculty of Medicine, Menoufiya University, Shebin El Kom, Menoufiya, Egypt;
authors.forename: Mohamed Ahmed
authors.initials: MA
authors.lastname: Basha
keywords: acitretin; blister; genodermatoses; hoarseness; lipoid proteinosis; pharynx; throat
references.citation: Br J Ophthalmol. 1997 Mar;81(3):253
references.pmid: 9135394
references.citation: J Invest Dermatol. 2000 Apr;114(4):718-24
references.pmid: 10733679
references.citation: Hautarzt. 1997 Jul;48(7):477-81
references.pmid: 9333627
references.citation: Am J Dermatopathol. 2014 Jan;36(1):1-48
references.pmid: 23249837
references.citation: J Eur Acad Dermatol Venereol. 2009 Apr;23(4):482-3
references.pmid: 18808438
references.citation: Eur J Dermatol. 2005 Sep-Oct;15(5):344-6
references.pmid: 16172042
references.citation: Clin Exp Dermatol. 2002 Nov;27(8):624-9
references.pmid: 12472532
references.citation: Case Rep Dermatol Med. 2012;2012:324506
references.pmid: 23259080
references.citation: Hum Genet. 1984;68(4):342-5
references.pmid: 6210239
references.citation: Dermatol Online J. 2008 Jul 15;14(7):16
references.pmid: 18718200
references.citation: J Dermatolog Treat. 2011 Jun;22(3):178-83
references.pmid: 20666665
references.citation: Dermatologica. 1989;179(1):14-7
references.pmid: 2527768
references.citation: J Am Acad Dermatol. 1992 Dec;27(6 Pt 2):S8-14
references.pmid: 1460124
references.citation: Br J Dermatol. 1988 Oct;119(4):541-4
references.pmid: 3191019
references.citation: Br J Dermatol. 2004 Aug;151(2):413-23
references.pmid: 15327549
references.citation: Clin Exp Dermatol. 1991 Sep;16(5):350-4
references.pmid: 1794188
references.citation: Clin Exp Dermatol. 2008 Jul;33(4):484-90
references.pmid: 18462443
references.citation: Exp Dermatol. 2007 Nov;16(11):881-90
references.pmid: 17927570
references.citation: Pediatr Dermatol. 2002 Jul-Aug;19(4):359-62
references.pmid: 12220287
references.citation: Acta Derm Venereol. 1996 Mar;76(2):154-5
references.pmid: 8740275