Open AccessSchopf–Schulz–Passarge syndrome
Author(s) -
Kinjal Deepak Rambhia,
Vidya Kharkar,
Sunanda Mahajan,
Uday Khopkar
Publication year - 2018
Publication title -
indian dermatology online journal
Language(s) - Uncategorized
Resource type - Journals
eISSN - 2249-5673
pISSN - 2229-5178
DOI - 10.4103/idoj.idoj_26_18
Subject(s) - apocrine , palmoplantar keratoderma , hypotrichosis , medicine , keratoderma , dermatology , ectodermal dysplasia , hypodontia , pathology , ichthyosis , anatomy , hyperkeratosis , biology , genetics , dentistry , gene
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.