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Arch Dermatol. 2008;144(7):933-938.

	Since this article does not have an abstract, we have provided the first 150 words of the full text and any section headings.

Diagnosis: Generalized basaloid follicular hamartoma syndrome (GBFHS).

MICROSCOPIC FINDINGS

Medium-power microscopy demonstrated circumscribed dermal tumors consisting of cords and lobules of folliculocentric basaloid cells with minimal palisading (Figure 2). Structures resembling small keratinous cysts and immature hair follicles were appreciated. Higher magnification revealed vellus hairs within some of the tumor lobules, minimal cytologic atypia, and no mitotic figures (Figure 3).

Figure appears in full text version. Figure 2.

Figure appears in full text version. Figure 3.

DISCUSSION

Basaloid follicular hamartoma (BFH) is a benign adnexal tumor that presents clinically as a small skin-colored papule. These skin appendage tumors show hair follicle differentiation and may present as solitary or multiple lesions.^1-2 Although solitary lesions usually are not hereditary, generalized forms typically exhibit autosomal dominant inheritance.^1-4 Generalized BFH syndrome is an autosomal, dominantly inherited disease consisting of multiple BFHs, hypotrichosis, hypohidrosis, and palmoplantar pitting.^{3, 5-6} Although rare, multiple BFHs have been reported with autoimmune disorders such as myasthenia gravis, alopecia universalis, systemic lupus erythematosus, and antiphospholipid antibody syndrome.^4-7

Usually, GBFHS presents . . . [Full Text of this Article]

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Arch Dermatol. 2008;144(7):933-938.
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