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Journal of the American Academy of Dermatology
Volume 66, n° 4
pages 617-621 (avril 2012)
Doi : 10.1016/j.jaad.2011.04.002
accepted : 1 April 2011
Original Articles

Folliculocystic and collagen hamartoma of tuberous sclerosis complex
 

Antonio Torrelo, MD a, , Smail Hadj-Rabia, MD b, Isabel Colmenero, MD c, Robert Piston, MD d, Virginia P. Sybert, MD d, Helena Hilari-Carbonell, MD e, Angela Hernández-Martín, MD a, Joan C. Ferreres, MD f, Sergio Vañó-Galván, MD a, Daniel Azorín, MD c, Javier Enríquez de Salamanca, MD g, Luis Requena, MD h, Christine Bodemer, MD b, Rudolf Happle, MD i, Vicente García-Patos, MD e, Sylvie Fraitag, MD j
a Department of Dermatology, Hospital del Niño Jesús, Madrid, Spain 
c Department of Pathology, Hospital del Niño Jesús, Madrid, Spain 
g Department of Plastic Surgery, Hospital del Niño Jesús, Madrid, Spain 
b Department of Dermatology, Centre National de Référence des Maladies Génétiques à Expression Cutanée, Hôpital Necker-Enfants Malades, Assistance Publique-Hôpitaux de Paris (APHP), Université Paris-Descartes, Paris, France 
j Department of Pathology, Hôpital Necker-Enfants Malades, Assistance Publique-Hôpitaux de Paris (APHP), Université Paris-Descartes, Paris, France 
d Group Health Permanente and University of Washington School of Medicine, Seattle, Washington 
e Department of Dermatology, Hospital Universitari Vall d’Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain 
f Department of Pathology, Hospital Universitari Vall d’Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain 
h Department of Dermatology, Fundación Jiménez Díaz, Madrid, Spain 
i Department of Dermatology, Philipp University, Marburg, Germany 

Reprint requests: Antonio Torrelo, MD, Department of Dermatology, Hospital del Niño Jesús, Menéndez Pelayo 65, 28009-Madrid, Spain.
Abstract
Background

Tuberous sclerosis complex (TSC) is an autosomal dominant disorder characterized by tumors and hamartomas in several organs including the skin.

Objective

We sought to describe a new type of complex hamartoma in patients with TSC.

Methods

This was a retrospective clinical and histopathologic evaluation of 6 cases.

Results

The skin lesions consisted of large, painless, infiltrated plaques that were first noticed at birth or during early infancy on the abdomen, thigh, back, or scalp. In time, the plaques became studded with numerous follicular comedo-like openings and cysts containing and draining a keratinous or purulent material. The main histopathologic features were: abundant collagen deposition in the dermis and extending into the underlying fat; concentric, perifollicular fibrosis surrounding hair follicles; and comedones and keratin-containing cysts lined by infundibular epithelium, some of which were ruptured with secondary granulomatous reaction. Five of the 6 patients had a clinical diagnosis of TSC.

Limitations

Genetic testing was performed in only one patient.

Conclusion

This distinctive folliculocystic and collagen hamartoma has not been recognized previously in association with TSC.

The full text of this article is available in PDF format.

Key words : collagen nevus, hamartoma, tuberous sclerosis complex



 Funding sources: None.
 Conflicts of interest: None declared.



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