Hypotrichosis-osteolysis-periodontitis-palmoplantar keratoderma syndrome

Orpha code: 307936OMIM code: 607658

Definition

Hypotrichosis-osteolysis-periodontitis-palmoplantar keratoderma syndrome is an extremely rare ectodermal dysplasia syndrome characterized by hypotrichosis universalis with mild to severe scarring alopecia, acro-osteolysis, onychogryphosis, thin and tapered fingertips, periodontitis and caries leading to premature teeth loss, linear or reticular palmoplantar keratoderma and erythematous, scaling, psoriasis-like skin lesions on arms and legs. Lingua plicata and ventricular tachycardia have also been observed.

Disease data
Classification

Disease

Synonyms
HOPP syndrome
Zespół hipotrichoza-osteoliza-zapalenie przyzębia-hiperkeratoza dłoniowo-podeszwowa
Zespół HOPP
Hypotrichosis-osteolysis-periodontitis-palmoplantar hyperkeratosis syndrome
Hypotrichosis-striate palmoplantar hyperkeratosis-acroosteolysis-periodontitis syndrome
Hypotrichosis-striate palmoplantar keratoderma-acroosteolysis-periodontitis syndrome
ORPHA code
307936
OMIM code
607658
ICD10 code
Q82.8
ICD11 code
-

No additional description.

Orphanet - interntowa baza danych dotyczących rzadkich chorób i sierochych leków. ©INSERM 1999 - Dostępna na stronie www.orphanet.pl