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CASE REPORT
Year : 1997  |  Volume : 63  |  Issue : 5  |  Page : 323-325

Unilateral Darier's disease




Correspondence Address:
M Manimegalai


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Source of Support: None, Conflict of Interest: None


PMID: 20944368

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  Abstract 

A 60-year old house-wife presented with mildly pruritic, over crusted, greasy, follicular and non-follicular papules over the forehead, retroauricular region and abdomen on the right side. There were numerous discrete hypopigmented macules scattered over the mammary region, abdomen, thigh and leg on the right side. Histopathological study of one of the papules over the forearems showed features of acrokeratosis verruciformis of Hopf.


Keywords: Darier′s disease, Keratosis follicularis, Unilateral dermatoses


How to cite this article:
Manimegalai M, Kumar P, Premalatha S. Unilateral Darier's disease. Indian J Dermatol Venereol Leprol 1997;63:323-5

How to cite this URL:
Manimegalai M, Kumar P, Premalatha S. Unilateral Darier's disease. Indian J Dermatol Venereol Leprol [serial online] 1997 [cited 2019 Dec 8];63:323-5. Available from: http://www.ijdvl.com/text.asp?1997/63/5/323/4607


Darier's disease (keratosis follicularis) is an inherited genodermatosis of autosomal dominant transmittence with variable penetrance, although many cases are caused by mutation. In 1889 James White and Jean Darier described this condition. Its clinical variants include the hypertrophic, vesicular, and acute eruptive types. Unilateral involvement in Darier's disease has been noted in 10% of cases.[1] The present case is reported because of the rare unilateral distribution of lesions of Darier's disease intermingled with hypopigmented macules.


  Case Report Top


A 60-year-old house-wife presented with mildly pruritic papules over the right side of the body of six years duration. Initially she developed the skin lesions over the right retroauricular region which gradually extended over the chest and abdomen on the right side. There was history of exacerbation of skin lesions during summer. There was no family history of the disease. There were no systemic complaints. Dermatological examination revealed crusted, greasy follicular and non-follicular papules of 1-3 mm size over the forehead, retroauricular region and abdomen including the flexures such as inframammary and inguinal region on the right side. There were numerous discrete hypopigmented macules of 1 mm diameter scattered over the mammary region, abdomen, thigh and leg on the right side. She also had small flat topped papular lesions of 2 mm size over the dorsum of right hand and forearm. Oral mucosa showed a few hyperpigmented papules over the palate and diffuse hyperpigmentation over the buccal mucosa. Multiple punctate keratoses and pits were seen over the right palm. Nails were not involved. Systemic examination was normal. Routine hematological and biochemical parameters were normal. Skiagram of the chest and long bones were also normal. Histopathological study of one of the crusted papules showed typical features of Darier's disease. Biopsy from one of the papules over the forearm showed features of acrokeratosis verruciformis of Hopf. The patient was treated with topical retinoic acid 0.025%. This resulted in flattening of the lesions in one month.


  Discussion Top


Unilateral involvement in Darier's disease was first reported in 1948 by Anderson.[2] It occurs in 10% of cases but this high percentage may be due to close resemblance between this type of keratosis follicularis and linear dyskeratotic epidermal naevi. Unlike the classical type of Darier's disease, the unilateral variety is thought to be of non genetic origin with a delayed onset.[3] A review of literature revealed that majority of cases had the onset in adult life. The unilateral form of disease differs from classic form in having linear (or) zosteriform distribution. A case of unilateral keratosis follicularis that followed the  Lines of Blaschko More Details's was reported in 1992.[4] In our case the distribution of lesions was. not linear or zosteriform. It was an unilateral (or) hemidistribution over the entire area of the chest and abdomen on the right side with predominant involvement of flexures such as inframammary region and inguinal region. The presence of hypopigmented macules is a rare manifestation in keratosis follicularis. They were first described by Goddal and Richmond in 1965.[5] In our case the hypopigmented macules did not occur at the site of previous papular lesions and these macules also showed unilateral distribution.



 
  References Top

1.Grifiths WAD, Leigh IM, Marks R. Disorders of keratinization. In: Rook A, Wilkinson DS, Ebling FJG, eds. Textbook of Dermatology. Oxford: Blackwell Scientific, 1992;1325.  Back to cited text no. 1    
2.Anderson A P. Darier's disease unilateral. Arch Dermatol Syphilol 1948;58:581-583.  Back to cited text no. 2    
3.Dermetree JW, Lang PG, St. Clair JT. Unilateral linear zosteriform epidermal nevus with acantholytic dyskeratosis. Arch Dermatol 1979;115:875-877.  Back to cited text no. 3    
4.Jackson R. The lines of Blaschko: A review and reconsideration. Br J Dermatol 1976;95:349-360.  Back to cited text no. 4    
5.Goddal JW, Richmond QM. A Case of Darier's disease. Br J Clin Pract 1965;19:475-476.  Back to cited text no. 5    




 

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