|Year : 2017 | Volume
| Issue : 2 | Page : 274-275
Multiple reddish papules in the bathing trunk distribution
Premanshu Bhushan, Konchok Dorjay
Department of Dermatology, Dr. PN Behl Skin Institute and School of Dermatology, New Delhi, India
|Date of Web Publication||3-Feb-2017|
Dr. PN Behl Skin Institute and School of Dermatology, Greater Kailash-1, New Delhi - 110 048
Source of Support: None, Conflict of Interest: None
|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 Mar 25];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|
Click here to view
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)|
Click here to view
| What Is Your Diagnosis?|| |
| References|| |
Karen JK, Hale EK, Ma L. Angiokeratoma corporis diffusum (Fabry disease). Dermatol Online J 2005;11:8.
Germain DP. Fabry disease. Orphanet J Rare Dis 2010;5:30.
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.
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.
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.
[Figure 1], [Figure 2]