Here, the authors present a rare condition with an unusual clinical and histopathological presentation that may correspond to a clinical manifestation of a mutation of the PTCH gene. [scielo.br]

Case Report A 45-year-old healthy woman presented with a history of asymptomatic papules in a Blaschkoid distribution since childhood. There was no family history of skin disease. [ijdvl.com]

They may be congenital (present from birth) or acquired (appear later). [dermnetnz.org]

Basaloid follicular hamartoma: a histologic diagnosis with diverse clinical presentations. Arch Dermatol. 1993 Jul. 129(7):915-7. [QxMD MEDLINE Link]. Toyoda M, Kagoura M, Morohashi M. Solitary basaloid follicular hamartoma. [emedicine.medscape.com]

• Weakness

  Special stains Bcl-2 demonstrated weak cytoplasmic positivity in the outermost tumor cells only (Fig. 5) and CD-34 was positive in stromal cells next to tumor cells (Fig. 6). [scielo.br]

• Skin Disease

  There was no family history of skin disease. The physical examination revealed multiple shiny brown papules over her left hemithorax, left shoulder and left arm [Figure - 1]. [ijdvl.com]

  BFH may present alone or in association with other hereditary skin diseases, such as Bazex-Dupre-Christol syndrome, Brown-Crounse syndrome, and Happle-Tinschert syndrome.11 Mills O, Thomas LB. Basaloid Follicular Hamartoma. [scielo.br]

• Skin Lesion

  Due to the varied clinical presentation of BFH, it is imperative to perform a skin biopsy to distinguish BFH from other common skin lesions. Currently, there are no standard treatments for BFH. [scielo.br]

  Del Barrio-Diaz et al have suggested that local inflammation of BFH lesions is a clinical sign of malignant transformation. [25] Diagnostics Skin biopsy is important for distinguishing basaloid follicular hamartoma (BFH) lesions from other lesions in [emedicine.medscape.com]

• Erythema

  Dermatoscopic findings on this inflamed area showed pigmented areas associated with erythema [Figure - 3]. [ijdvl.com]

For a full discussion, including histopathology, see Workup. [emedicine.medscape.com]

What is the treatment of basaloid follicular hamartoma? Basaloid follicular hamartoma is a benign tumour that usually requires no treatment. [dermnetnz.org]

Treatment Although no medications have been approved for basaloid follicular hamartoma (BFH) treatment, systemic isotretinoin has been reported to decrease the size of lesions and improve associated alopecia in a patient with multiple BFH and systemic [emedicine.medscape.com]

Currently, there are no standard treatments for BFH. Correct identification allows for periodic monitoring for malignant transformation while sparing patients unnecessary surgery.11 Mills O, Thomas LB. Basaloid Follicular Hamartoma. [scielo.br]

Published cases report the age of onset ranging from 20-88 years, with a median of 66 years and a mean of 63 years. [40] Prognosis The prognosis for basaloid follicular hamartoma (BFH) is usually excellent, unless associated systemic disorders and/or [emedicine.medscape.com]

[…] addition to findings on hematoxylin and eosin (H&E) staining, both entities were found to have positive CK20 staining, suggesting that basaloid follicular hamartoma and infundibulocystic basal cell carcinoma might actually represent the same lesion. [1] Etiology [emedicine.medscape.com]

This gene defect would therefore be present only in cells derived from the precursor cell that acquired the mutation, accounting for the mosaic, linear, and unilateral pattern following the lines of Blaschko. [42] Epidemiology Frequency Both hereditary [emedicine.medscape.com]

Background Pathophysiology Basaloid follicular hamartoma (BFH) is considered an abortive growth of secondary hair germs, with differentiation limited to the upper part of the follicles. [33] The causative gene for this hamartoma is still unknown. [emedicine.medscape.com]