Multiple follicular hamartomas with sweat gland and sebaceous differentiation, vermiculate atrophoderma, milia, hypotrichosis, and late development of multiple basal cell carcinomas.
We describe a 46-year-old woman with multiple symmetrical papulocystic lesions on the face, neck, chest, back, and upper arms since childhood. Vermiculate atrophoderma, entropion, and progressive loss of eyelashes was also noted. Since the age of 30, 12 basal cell carcinomas have developed on the face. Histopathologic examination revealed multiple anastomosing nests and strands that arose from the lower part of a hair follicle in early lesions. In more advanced lesions, multiple keratinizing microcysts within a moderately sclerotic stroma were noted in the upper and mid dermis. In some biopsy specimens, focal areas showing sweat gland (ductal) and sebaceous differentiation were also observed. Basal cell carcinomas developed from the basaloid component of the hamartomatous proliferation.
Pujol
R M
RM
Department of Dermatology, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain.
Nadal
C
C
Matias-Guiu
X
X
Peyrí
J
J
Ferrándiz
C
C
Palou
J
J
de Moragas
J M
JM
D001284:Atrophy; D002280:Basal Cell Carcinoma / Q000473:pathology*; D004774:Entropion / Q000473:pathology; D004814:Epidermal Cyst / Q000473:pathology*; D005140:Eyelashes / Q000473:pathology; D005141:Eyelid Diseases / Q000473:pathology; D005153:Facial Neoplasms / Q000473:pathology*; D005260:Female; D005500:Follow-Up Studies; D006201:Hair Diseases / Q000473:pathology; D018859:Hair Follicle / Q000473:pathology*; D006222:Hamartoma / Q000473:pathology*; D006801:Humans; D007039:Hypotrichosis / Q000473:pathology*; D008875:Middle Aged; D012598:Sclerosis; D012627:Sebaceous Glands / Q000473:pathology*; D012867:Skin / Q000473:pathology*; D012871:Skin Diseases / Q000473:pathology; D012878:Skin Neoplasms / Q000473:pathology*; D013543:Sweat Gland Diseases / Q000473:pathology*