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Schopf‐Schulz‐Passarge syndrome: a rare ectodermal dysplasia with a delayed diagnosis

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A 58-year-old female of German and English ancestry, born of a nonconsanguineous marriage, presented with bilateral eyelid cysts (Fig. 1), palmoplantar keratoderma (Fig. 2), nail dystrophy, and hypodontia (Fig. S1).… Click to show full abstract

A 58-year-old female of German and English ancestry, born of a nonconsanguineous marriage, presented with bilateral eyelid cysts (Fig. 1), palmoplantar keratoderma (Fig. 2), nail dystrophy, and hypodontia (Fig. S1). She was diagnosed with an uncharacterized ectodermal dysplasia 25 years prior. No family members were affected. Palmoplantar keratoderma first appeared at age 31. Multiple eyelid cysts appeared at the age of 39. She reported abnormal dentition from childhood, with conical primary teeth and agenesis of permanent teeth. She has worn complete dentures since the age of 13. She recalls dystrophic nail changes from childhood. Additional features noted on examination included palmoplantar hyperhidrosis, perifollicular hyperkeratosis on her shins, and scaling on the tip of her nose. There was no associated hair disorder. At age 59, she had a nodular basal cell carcinoma excised from her neck. A diagnosis of Schopf-Schulz-Passarge syndrome (SSPS) was suspected, and histopathology from a punch biopsy taken

Keywords: schopf schulz; passarge syndrome; schulz passarge; age; ectodermal dysplasia

Journal Title: International Journal of Dermatology
Year Published: 2019

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