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Miliary fibromas in tuberous sclerosis complex.

A M Cartron^1,2, D Buccine², A M Treichel^1,2

¹Pulmonary Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD, USA.

Journal of the European Academy of Dermatology and Venereology : JEADV

|February 10, 2021

Summary

This summary is machine-generated.

Miliary fibromas (MiF) are distinct skin lesions found in 14% of adults with tuberous sclerosis complex (TSC). These benign papules have unique clinical and histological features, differentiating them from other TSC-related skin manifestations.

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Area of Science:

Dermatology
Genetics
Pathology

Background:

Tuberous sclerosis complex (TSC) is a genetic disorder causing hamartoma syndrome.
Key skin manifestations include angiofibromas, shagreen patches, and miliary fibromas (MiF).

Purpose of the Study:

To define the clinical and histological characteristics of miliary fibromas (MiF) in adults with TSC.

Main Methods:

Retrospective analysis of 133 adult TSC patients.
Clinical data and photographic evidence of MiF were collected.
Histopathological examination of skin samples from four patients.

Main Results:

Miliary fibromas (MiF) were identified in 19 out of 133 (14%) TSC patients.
MiF present as 1-3 mm skin-colored sessile papules, typically on the back, with symmetric or asymmetric distribution.
Histology reveals papillary and periadnexal dermal expansion with hamartomatous changes.

Conclusions:

Miliary fibromas (MiF) are a distinct cutaneous finding in tuberous sclerosis complex (TSC).
Understanding MiF features aids in defining the TSC spectrum and reassuring patients about lesion benignity.