Indian Journal of Dermatology
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CASE REPORT
Year : 2009  |  Volume : 54  |  Issue : 5  |  Page : 49-50
Reticulate variant of Darrier's disease


Department of Dermatology, Venerology and Leprosy, Nilratan Sircar Medical College, Kolkata, India

Correspondence Address:
Sudip Das
P-103 Bosenagar, Madhyamgram, 24 Parganas (N), Kolkata - 700 129
India
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Source of Support: None, Conflict of Interest: None


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   Abstract 

Darrier disease belong to acantholytic group of disorders with loss of intraepidermal coherence. Darrier disease has an onset in puberty in the form of greasy papules, skin coloured to yellow in nature often progressing to plaques. Sites of predeliction being seborrhic areas of body. Nail abnormalities are a constant feature with a V shaped nicking.Histopathology shows orthohyperkeratoses with focal dyskeratoses, corps and ronds in stratum spinosum and grains in upper epidermis. Variable patterns have been described like bullous, haemorrhagic and linear but reticulate pattern have not been described earlier. We describe a case of reticulate variant of Darrier responding excellently to acitretin therapy.


Keywords: Acitretin, Darrier′s disease, reticulatevariant


How to cite this article:
Das S, Roy AK, Kar C, Giri PP. Reticulate variant of Darrier's disease. Indian J Dermatol 2009;54, Suppl S1:49-50

How to cite this URL:
Das S, Roy AK, Kar C, Giri PP. Reticulate variant of Darrier's disease. Indian J Dermatol [serial online] 2009 [cited 2019 Dec 12];54, Suppl S1:49-50. Available from: http://www.e-ijd.org/text.asp?2009/54/5/49/45455



   Introduction Top


Darrier White disease (DWD) is an autosomal dominant disorder associated with altered keratinisation of epidermis, nail, mucus membrane. [1] Positional cloning studies have localized gene to be defective in DWD to a site on long arm of Chromosome 12 within the region of 12 q 23 - 24.1. Characteristic site of predilection are face, forehead, scalp, chest and back (site of sebaceous gland predilection), butcan occur on non-sebaceous site - palm and sole. Disease worsens in summer with heat and humidity and exacerbated by UVB exposure.

Classic lesions present as multiple, discrete scaling; crusted, rough skin papules with or without malodourer disease involving mainly the seborrheic areas of body. The lesions first appear as skin colored papules that become yellowish to tan, with scaly crust and roughening of skin texture. At times they become thick and warty.

Uncommon form of Darrier's disease - hypertrophic form, [2] alopecic pattern, zosteriform distribution, vescicobullous form - has been described; but we did not come across a Reticular variant pattern of Darrier's disease in literature. [3]


   Case History Top


Our patient a 26-year-old and a shop-keeper by profession presented to us with a serious disorder of crusted, scaly, rough papules on face, neck, back, upper chest, progressing to form reticulate hyperpigmented plaques with greasy papules in it [Figure 1],[Figure 2]. The disease worsened in summer and with exposure to sunlight. There were no flat, wart like papules seen on dorsum of hands and feet, no palmar pits, but nail was thin and showed 'V' shaped scalloping subungual thickening. Mucus membranes were normal. No hemorrhegic lesions were seen. Histopathological studies of the back lesions showed abnormal keratinisation and loss of adherence with acantholysis [Figure 3]. There were also eosinophillic dyskeratotic cells in Spinous and Stratum Corneum. There was no reticulate pigmentation on axilla, oral cavity etc.

We treated the patient with Acetretin - 25mg. (Body Wt.-45Kg.) and within six to eight months of therapy there was total clearing of skin lesions. Nail lesions also improved by eight weeks. After 12 weeks of Acitretin therapy, we decreased the dose to 10mg/day for three months and have put him off therapy after another three months without any recurrences.

His lipid profile and hematologic profiles were within normal limits during and after Acitretin therapy; though mild chelitis and episodes of joint pain did occur.


   Discussion Top


We report a rare new case of Darrier's disease - reticulate variant- though a hypertrophic form, zosteriform pattern are all well described, [4],[5],[6] this reticulate pattern is not well described and focuses a new light on Darrier White disease.

 
   References Top

1.Baclen PH. Fredburg Darrier White disease (keratoses follicularis) acrokeratotic verrucioformis. In: Goldsmith LP, editor. Text book of Dermatology. p. 614-9.  Back to cited text no. 1    
2.Eisbach EM, Nater JP. The hypertrophic form of Darrier. Dis Dermatol 1960;120:93.  Back to cited text no. 2    
3.Pendrod JN. Observation on keratoses follicularis: And Dermatology 82.  Back to cited text no. 3    
4.Pendrod JN. Unilateral lien to verrucous epidermal naevus with acantholytic dyskeratoses. Arch Devolop 1979;115:875.  Back to cited text no. 4    
5.Forseman PL. Haemorrhegic Darrier disease. Arch Dermatol 1493;129:511.  Back to cited text no. 5    
6.Telter NR. Vessicobullous Darrier disease. By J Deabl 1990;122:831.  Back to cited text no. 6    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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    Abstract
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    Case History
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    References
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