Congenital Insensitivity to Pain Syndrome With Hidrosis in a 17-Year-Old Female

Congenital insensitivity to pain syndrome (CIP) is a rare hereditary sensory and autonomic neuropathy (HSAN) characterized by impaired pain perception, recurrent injuries, chronic infections, and varying degrees of autonomic involvement. Among its subtypes, HSAN-V is distinguished by preserved cognition and sweating function but with a complete absence of pain sensation and progressive joint degeneration. We present the case of a 17-year-old female with suspected HSAN-V who developed orthopedic complications, including a chronic foot ulcer and an infected finger wound. Imaging revealed soft tissue swelling of the finger, necessitating surgical amputation, followed by a second procedure for wound closure and tendon sheath debridement. Postoperative cultures guided antibiotic therapy, and the patient recovered without complications. Genetic testing has not yet established a definitive diagnosis. This case highlights the orthopedic challenges associated with HSAN-V and underscores the importance of early recognition, multidisciplinary management, and patient-family education in optimizing outcomes and quality of life.

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Publication Details

Journal
Cureus
Published
2025-09-02
DOI
https://doi.org/10.7759/cureus.91496
Primary Topic
Sympathectomy and Hyperhidrosis Treatments
Type
article
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article

Congenital Insensitivity to Pain Syndrome With Hidrosis in a 17-Year-Old Female

Molly M. Jeffery, Robert Paul, Allan M. Parnell, Grace E Corrier
Cureus
Sympathectomy and Hyperhidrosis Treatments
article

Congenital Insensitivity to Pain Syndrome With Hidrosis in a 17-Year-Old Female

Molly M. Jeffery, Robert Paul, Allan M. Parnell, Grace E Corrier
article en

Abstract

Congenital insensitivity to pain syndrome (CIP) is a rare hereditary sensory and autonomic neuropathy (HSAN) characterized by impaired pain perception, recurrent injuries, chronic infections, and varying degrees of autonomic involvement. Among its subtypes, HSAN-V is distinguished by preserved cognition and sweating function but with a complete absence of pain sensation and progressive joint degeneration. We present the case of a 17-year-old female with suspected HSAN-V who developed orthopedic complications, including a chronic foot ulcer and an infected finger wound. Imaging revealed soft tissue swelling of the finger, necessitating surgical amputation, followed by a second procedure for wound closure and tendon sheath debridement. Postoperative cultures guided antibiotic therapy, and the patient recovered without complications. Genetic testing has not yet established a definitive diagnosis. This case highlights the orthopedic challenges associated with HSAN-V and underscores the importance of early recognition, multidisciplinary management, and patient-family education in optimizing outcomes and quality of life.

CureusVol. 17(9)
University of Louisiana at Monroe (US), Louisiana State University Health Sciences Center New Orleans (US), Edward Via College of Osteopathic Medicine (US)
Openalex Percentile: Top 71%
Sympathectomy and Hyperhidrosis Treatments
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Congenital Insensitivity to Pain Syndrome With Hidrosis in a 17-Year-Old Female — Molly M. Jeffery, Robert Paul, et al. · Cureus (2025) | TGRS Research Map | TGRS