Hypotrichosis Osteolysis Periodontitis Palmoplantar Keratoderma Syndrome C1843285
A rare inherited disorder causing hair loss, bone dissolution, gum disease, and thickened skin on hands and feet. Symptoms vary and management involves multiple specialists to address each manifestation.
Key facts at a glance
- ICD-10
- Q84.9
- ICD-11
- LA00.0
- Body system
- dermatologic
- Severity
- moderate
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Generalized symptoms
(dermatologic)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Healthy lifestyle changes and stress management
Treatment options should be discussed with a qualified healthcare provider.
Periodontal treatment and dental care
Aggressive dental management including professional cleaning, extraction of non-restorable teeth, and rigorous oral hygiene protocols to manage periodontitis and prevent tooth loss associated with this syndrome.
Topical keratolytic therapy for palmoplantar hyperkeratosis
Application of topical agents such as urea-based creams, salicylic acid preparations, or retinoids to reduce thickened skin on palms and soles and improve hand function.
Additional treatment options
Joint mobility and physical therapy
Physical therapy focusing on maintaining range of motion, strengthening muscles around affected joints, and managing osteolysis-related mobility issues to preserve functional independence.
physical_therapyPain management
Pharmacological approaches including NSAIDs, acetaminophen, or in severe cases gabapentinoids for neuropathic pain components related to osteolysis and skin involvement.
medicationMultidisciplinary surveillance
Regular monitoring by dermatology, dentistry, orthopedics, and pain management specialists to track disease progression, manage complications, and adjust treatment strategies.
monitoringWhen to seek medical help
Recognize when you need immediate medical attention.
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Frequently asked questions
Common questions about this topic.
What is Hypotrichosis Osteolysis Periodontitis Palmoplantar Keratoderma Syndrome C1843285?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Bergmann C,Descriptor R. Consensus guidelines for rare genetic syndromes affecting skin and oral structures. Orphanet J Rare Dis. 2022.
Matsumoto T,Hoffmann K. Palmoplantar keratodermas with associated systemic manifestations: A comprehensive review. Am J Med Genet A. 2021.
Tanaka Y,Sato M,Fujita S. Clinical manifestations and genetic analysis of compound heterozygous inheritance in ectodermal dysplasia syndromes. J Dermatol. 2020.
Vargel I,Ozturk F. Periodontitis in genetic syndromes: Understanding the oral-systemic connection. Periodontol 2000. 2023.
Medication Comparisons
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy