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Year : 2018  |  Volume : 9  |  Issue : 6  |  Page : 448-451

Schopf–Schulz–Passarge syndrome

Department of Dermatology, Seth G.S. Medical College and King Edward Memorial Hospital, Mumbai, Maharashtra, India

Correspondence Address:
Kinjal D Rambhia
House No. 574, Gulati Bhavan, Mukundraj Lane, Walker Road, Mahal, Nagpur - 440 032, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/idoj.IDOJ_26_18

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Schopf–Schulz–Passarge syndrome (SSPS) is a rare type of ectodermal dysplasia that has autosomal recessive inheritance. It is characterized by palmoplantar keratoderma, hypodontia, hypotrichosis, nail dystrophy, and multiple periocular and eyelid apocrine hidrocystomas. A 36-year-old male presented with multiple eyelid and periocular apocrine hidrocystomas, ichthyosis, palmoplantar keratoderma, hypodontia, nail dystrophy, and thin scalp hair. Skin biopsy from a periocular lesion revealed cyst lined with smooth, thin epithelium, and few areas revealed foci of decapitation secretion consistent with apocrine hidrocystoma. The patient was diagnosed with SSPS.

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