A case of skin lesions of basal cell nevus syndrome

Long Chen; Hui Sun; Meng Zuo; Qing Xi

doi:10.5114/ada.2021.108902

eISSN: 2299-0046
ISSN: 1642-395X

Advances in Dermatology and Allergology/Postępy Dermatologii i Alergologii

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abstract:

Letter to the Editor

A case of skin lesions of basal cell nevus syndrome

Long Chen

¹

,

Hui Sun

²

,

Meng Zuo

²

,

Qing Xi

²

Department of Stomatology, Mancheng District People’s Hospital, Baoding City, Hebei Province, China
Stomatology Department, Chinese PLA Hospital, China

Adv Dermatol Allergol 2021; XXXVIII (4): 706–707

DOI: https://doi.org/10.5114/ada.2021.108902

Online publish date: 2021/09/14

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Basal cell nevus syndrome (BCNS) is a rare autosomal dominant disorder, and is believed to be caused by mutations in the PTCH gene at locus 9Q22.3-Q31. It is a complex syndrome and consists of multiple odontogenic keratocysts, multiple basal cell nevus (cancer) of the face and neck, punctate depression of the palm and sole, abnormal skeleton and rib, calcification in the brain and various other defects [1, 2]. Now we are reporting a case of BCNS seen in our department recently as follows: female, 20 years old, with typical clinical manifestations of BCNS: multiple and scattered pigmented nevus on the face (Figure 1 A), small depression on the palm (Figure 1 B), multiple cysts on the upper and lower jaw, calcification of cerebral falx and tentorium, sternum deformity, rib bifurcations, scoliosis, deviation of the right eye with decreased vision. The mother presented with multiple cysts of the upper and lower jaws, bifurcated ribs, scoliosis and scattered small depressions in the palm (Figure 2).

A case of skin lesions of basal cell nevus syndrome

Long Chen 1 , Hui Sun 2 , Meng Zuo 2 , Qing Xi 2

Long Chen

¹

,

Hui Sun

²

,

Meng Zuo

²

,

Qing Xi

²