Basaloid Follicular Hamartoma Clinical Presentation

Updated: Dec 15, 2017
  • Author: Kara Melissa T Torres, MD, DPDS; Chief Editor: Dirk M Elston, MD  more...
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Presentation

History

In general, basaloid follicular hamartoma (BFH) lesions are asymptomatic small papules that remain stable for many years. [22] In hereditary basaloid follicular hamartoma, the lesions may gradually increase in size throughout childhood and then stabilize and become static upon reaching adulthood. [9, 31]

Generalized basaloid follicular hamartoma syndrome (GBFHS) is most commonly associated with alopecia and myasthenia gravis. [2, 36] Other associations reported with the syndrome are systemic lupus erythematosus (SLE), [2] cystic fibrosis, [4] and chondrosarcoma. [11]

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Physical Examination

The skin lesions of basaloid follicular hamartoma (BFH) may present as macules, papules, nodules, plaques, or skin tags. [2, 9, 37]

Hereditary basaloid follicular hamartoma

Lesions most often manifest on the face and scalp. The lesions also commonly develop diffusely on the skin of the neck, back, chest, and extremities. [9] Patients may have few to hundreds of lesions. Most commonly, they appear as 1- to 2-mm flesh-colored papules. However, hyperpigmented or hypopigmented lesions are not uncommon. [9, 22] Palmar pitting has been documented in some cases of hereditary basaloid follicular hamartoma. [9, 22, 24]

Additional clinical features associated with generalized basaloid follicular hamartoma syndrome (GBFHS), especially those with no systemic affection, include milia and comedonelike papules, [14] hypohidrosis, [36] alopecia or hypotrichosis, and dermatosis papulosa nigra–like growths. [9] Generalized basaloid follicular hamartoma syndrome has also been reportedly associated in a patient with acrochordons, seborrheic keratoses, and a history of chondrosarcoma. [11]

Linear unilateral basaloid follicular hamartoma (LUBFH) displays multiple flesh-colored to pigmented lesions in a linear, bandlike pattern running along the lines of Blaschko. [2] In their early stages, the lesions may be hypopigmented, smooth, and striaelike. Lesional skin in an atypical case of linear unilateral basaloid follicular hamartoma was described as an atrophic, depressed patch. [25] A patient with linear unilateral basaloid follicular hamartoma with an unusual and extensive proliferation of trichoblastoma has also been reported. [38]

Acquired basaloid follicular hamartoma

The papules or plaques of basaloid follicular hamartoma are found on the eyelids, face, and scalp. [23, 39] The lesions may range from 0.1 cm to 3 cm. They may appear hypopigmented, hyperpigmented, or flesh colored. Hypotrichosis or alopecia may develop at sites of solitary basaloid follicular hamartoma. A case of a localized basaloid follicular hamartoma arising in an epithelioid blue nevus has been reported. [40]

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Complications

Although rare, malignant transformation in the basaloid follicular hamartoma (BFH) lesions in generalized basaloid follicular hamartoma syndrome (GBFHS) [41] and within a localized basaloid follicular hamartoma [42] has been reported. Continual monitoring is imperative.

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