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Year : 2020  |  Volume : 21  |  Issue : 3  |  Page : 200-202

Recurrent urinary retention: An unusual presentation of laryngo-onycho-cutaneous syndrome

1 Department of Dermatology, Base Hospital, Lucknow, Uttar Pradesh, India
2 Consultant Dermatologist, Military Hospital, Dehradun, Uttarakhand, India
3 Department of Urology, Armed Forces Medical College, Pune, Maharashtra, India
4 Consultant Dermatologist, Base Hospital, Delhi Cantt, New Delhi, India

Correspondence Address:
Dr. Aradhana Sood
Department of Dermatology, Base Hospital, Lucknow - 226 002, Uttar Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijpd.IJPD_15_19

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Laryngo-onycho-cutaneous (LOC) first described by Shabbir in 1986 in Muslim families from Punjab is a rare autosomal recessive disorder characterized by increased skin and mucous membrane fragility associated with exuberant granulation. The 4th International Consensus Meeting on “Diagnosis and Classification of Epidermolysis Bullosa” classifies it as a localized form of Junctional Epidermolysis Bullosa (JEB-LOC Syndrome) with mutations in α3 chain of Laminin 332. While laryngeal, ocular, and cutaneous involvement in these cases has been widely reported, urinary tract involvement is relatively rare. We report a case of JEB-LOC syndrome in a child from South India with extensive urethral involvement.

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