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Year : 2017  |  Volume : 83  |  Issue : 2  |  Page : 274-275

Multiple reddish papules in the bathing trunk distribution


Department of Dermatology, Dr. PN Behl Skin Institute and School of Dermatology, New Delhi, India

Date of Web Publication3-Feb-2017

Correspondence Address:
Konchok Dorjay
Dr. PN Behl Skin Institute and School of Dermatology, Greater Kailash-1, New Delhi - 110 048
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0378-6323.190872

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How to cite this article:
Bhushan P, Dorjay K. Multiple reddish papules in the bathing trunk distribution. Indian J Dermatol Venereol Leprol 2017;83:274-5

How to cite this URL:
Bhushan P, Dorjay K. Multiple reddish papules in the bathing trunk distribution. Indian J Dermatol Venereol Leprol [serial online] 2017 [cited 2017 Jul 26];83:274-5. Available from: http://www.ijdvl.com/text.asp?2017/83/2/274/190872


A 20-year-old boy presented with multiple, discrete, 2-4 mm sized, red-purple papules on the arms, trunk, penis, scrotum and legs, becoming confluent around the umbilicus [Figure 1]. The papules started appearing 3 years ago on his thighs and occasionally bled on trauma, without any other symptoms. The patient had episodic burning pain in both hands and feet, heat intolerance and decreased sweating. There was no history of abdominal pain, bone pain, edema of the feet, auditory or neurological complaints or significant drug intake. No other family member had similar features. Systemic and ophthalmic evaluations revealed no abnormalities.
Figure  1: Multiple, red to purple, hyperkeratotic papules on anterior trunk with grouping around umbilicus

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Routine hematological tests, coagulation profile, electrocardiography and chest radiographs were normal. Renal function analysis showed mild proteinuria, up to 0.46 g/L. The α-galactosidase enzyme assay on dried blood spots was 0.55 nmol/h/ml (normal 3–20 nmol/h/ml). Biopsy of a papule showed hyperkeratosis, acanthosis and papillomatosis with dilated and congested vessels, lined by endothelium within dermal papillae [Figure 2].
Figure  2: Hyperkeratosis of epidermis with acanthosis and papillomatosis, and dilated and congested vessels lined by endothelium in the dermal papillae  (H  and  E, ×100)

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  References Top

1.
Karen JK, Hale EK, Ma L. Angiokeratoma corporis diffusum (Fabry disease). Dermatol Online J 2005;11:8.  Back to cited text no. 1
    
2.
Germain DP. Fabry disease. Orphanet J Rare Dis 2010;5:30.  Back to cited text no. 2
    
3.
Orteu CH, Jansen T, Lidove O, Jaussaud R, Hughes DA, Pintos-Morell G, et al. Fabry disease and the skin: Data from FOS, the Fabry outcome survey. Br J Dermatol 2007;157:331-7.  Back to cited text no. 3
    
4.
Roos JM, Aubry MC, Edwards WD. Chloroquine cardiotoxicity: Clinicopathologic features in three patients and comparison with three patients with Fabry disease. Cardiovasc Pathol 2002;11:277-83.  Back to cited text no. 4
    
5.
De Schoenmakere G, Chauveau D, Grünfeld JP. Enzyme replacement therapy in Anderson-Fabry's disease: Beneficial clinical effect on vital organ function. Nephrol Dial Transplant 2003;18:33-5.  Back to cited text no. 5
    


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