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Basaloid Follicular Hamartoma

Background

Basaloid follicular hamartoma (BFH) is a rare, benign adnexal tumor. A variety of clinical patterns have been noted with identical histopathologic features and possible associations with numerous disorders. The tumor is morphologically similar to infundibulocystic basal cell carcinoma.

In general, basaloid follicular hamartomas occur in two forms: hereditary and acquired. Hereditary types of basaloid follicular hamartoma can be either generalized or localized. Acquired types of basaloid follicular hamartoma can be localized, solitary, or multiple.

Hereditary basaloid follicular hamartomas

Generalized basaloid follicular hamartoma syndrome (GBFHS) subtype is inherited in an autosomal dominant pattern and has additional cutaneous features that include milia, comedonelike lesions, alopecia or hypotrichosis, and hypohidrosis. The syndrome is usually associated with some autoimmune disease,
but widespread lesions with no associated systemic disorders also have been reported.
When multiple basaloid follicular hamartomas are associated specifically with myasthenia gravis and diffuse alopecia, the syndrome is known as Brown-Crounse syndrome.

Linear unilateral basaloid follicular hamartoma (LUBFH) subtype is also known as linear unilateral basal cell nevus with comedones, linear unilateral basal cell nevus, and basal cell and linear unilateral adnexal hamartoma.
The lesions associated with linear unilateral basaloid follicular hamartoma occur along the lines of Blaschko in a limited, mosaic pattern.

Familial multiple basaloid familiar hamartoma type of is an autosomal dominant disease that may or may not have the clinical features of hypotrichosis, hypohidrosis, and palmoplantar pitting.

Other hereditary syndromes are recognized. Multiple basaloid follicular hamartomas have been reported to be associated with the nevoid basal cell carcinoma syndrome (NBCCS), also known as Gorlin syndrome or basal cell nevus syndrome,
and Bazex-Dupré-Christol syndrome.
Unilateral and segmentally arranged basaloid follicular hamartomas are associated with Happle-Tinschert syndrome.

Acquired basaloid follicular hamartomas

Solitary/localized lesions are the usual presentations of acquired basaloid follicular hamartoma.

There are few reported cases of multiple basaloid follicular hamartomas with no evidence of autosomal dominance inheritance
and nonfamilial systematized unilateral epithelial nevus.

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