[Manifestation and treatment of lipoid proteinosis in larynx].
OBJECTIVE
To investigate the manifestation, characteristics and treatment of lipoid proteinosis (LP) in larynx.


METHODS
Sixteen cases of LP with hoarseness as the first sign were reported. The onset was in the newborn period in 10 cases. The upper eyelids were infiltrated in all cases, 13 of them reported an age of onset older than 6 - 8 years. The laryngeal behaviors and its treatment, multisystem manifestations, and characteristics of histopathology were evaluated.


RESULTS
One patient had moderate hoarseness, and the others had severe hoarseness. Videolaryngoscopy revealed thickening of the interarytenoid region and irregularities along the rims and the surface of the vocal folds due to yellowish papules. Oral tissues showed yellow-white infiltrates in 13 cases (81.3%). Whitish moniliform papules on the upper eyelids were found in all cases. Pock-like or acneiform scars were particularly evident on the face and the limbs in 14 cases (87.5%). Histologically, it was characterized by deposition of hyaline material in the submucosa and dermis of the eyelid or larynx with periodic acid-Schiff-positive and Congo red stain-negative. Eight patients underwent microlaryngosurgery for the excision of deposits in the vocal folds. The vocal function was significantly improved after surgery.


CONCLUSIONS
One of the most common features of lipoid proteinosis is hoarseness since infancy with laryngeal involvement. LP patients also display skin or mucosa infiltration of multiple systems. Microlaryngosurgical excision of the deposits in the vocal fold could improve the voice.
Xu
Wen
W
Department of Otorhinolaryngology Head and Neck Surgery, Beijing Tongren Hospital, Capital Medical University, Key Laboratory of Otorhinolaryngology Head and Neck Surgery, Ministry of Education, Beijing 100730, China. entwen@trhos.com
Wang
Lei
L
Zhang
Li
L
Liu
Hong-gang
HG
Zan
Fang
F
Hu
Rong
R
Liu
Wen-bin
WB
Han
De-min
DM
D000293:Adolescent; D000328:Adult; D002648:Child; D005260:Female; D006801:Humans; D007818:Laryngeal Diseases / Q000175:diagnosis / Q000473:pathology* / Q000601:surgery; D007830:Larynx / Q000473:pathology* / Q000601:surgery; D008065:Lipoid Proteinosis of Urbach and Wiethe / Q000175:diagnosis / Q000473:pathology* / Q000601:surgery; D008297:Male; D012867:Skin / Q000473:pathology; D055815:Young Adult