Hyperkeratosis Lenticularis Perstans (Flegel Disease) Differential Diagnoses

Updated: Jan 31, 2017
  • Author: Daniel Roling, MD; Chief Editor: William D James, MD  more...
  • Print
DDx

Diagnostic Considerations

Consider disseminated superficial actinic porokeratosis (DSAP). Porokeratoses are progressive, hyperkeratotic, irregular plaques with a characteristic and prominent peripheral keratotic ridge. The superficial disseminated form occurs as a generalized symmetric process confined exclusively to sun-exposed areas. Lesions are small, 1- to 3-mm, keratotic lesions, with a central dell and a peripheral keratotic ridge that may number in the hundreds. DSAP is a relatively common condition occurring in the third and fourth decades, and it slowly progresses over years. Histologically, the peripheral, raised, keratotic ridge translates into a hyperkeratotic invagination in the epidermis termed cornoid lamellae. Cornoid lamellae are abrupt well-defined stacks of parakeratin confined to the periphery of the lesion. The underlying epidermis lacks a granular layer and frequently shows dyskeratosis.

Numerous reports exist in the literature that associate hyperkeratosis lenticularis perstans (Flegel disease) with an endocrinopathy. At a minimum, 4 reports were associated with adult-onset diabetes and 2 with hyperthyroidism.

A relationship between hyperkeratosis lenticularis perstans (Flegel disease) and malignancies has been suggested. One report describes epithelial tumors (basal and squamous cell carcinomas) occurring at distant, uninvolved, and nonlesional sites in patients with a familial hyperkeratosis lenticularis perstans (Flegel disease) lineage. A possible association between cancers of the digestive system and hyperkeratosis lenticularis perstans (Flegel disease) also has been implied.

The association of hyperkeratosis lenticularis perstans (Flegel disease) with endocrinopathies, skin, and GI neoplasms is weak at best. Certain factors (including rarity of hyperkeratosis lenticularis perstans (Flegel disease) and high prevalence of associated diseases) suggest a reporting bias.

Differential Diagnoses