Hyperkeratosis Lenticularis Perstans (Flegel Disease) Workup

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

Other Tests

Since several reports have associated hyperkeratosis lenticularis perstans (Flegel disease) with an endocrinopathy, obtain a thorough and complete systems review, as well as social and family histories. Laboratory, radiographic, or surgical tests are not needed, unless indicated by information gleaned from these sources.

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Procedures

Skin biopsy with hematoxylin and eosin staining shows characteristic findings of hyperkeratosis lenticularis perstans (Flegel disease). Electron microscopy is not essential.

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Histologic Findings

A discrete area of hyperkeratosis occurs (with areas of parakeratosis) overlying a thinned stratum malpighii and thinned-to-absent granular layer. Irregular acanthosis and some vascular dilatation are peripheral. A lymphoid infiltrate with occasional histiocytes in a bandlike pattern in the papillary dermis typically is seen. See the images below.

Hematoxylin and eosin-stained section, low magnifi Hematoxylin and eosin-stained section, low magnification. Epidermal hyperplasia with rete elongation surmounted by a thickened, compact, hyperkeratotic scale. A bandlike lymphoid infiltrate expands the papillary dermis.
Hematoxylin and eosin-stained section, medium magn Hematoxylin and eosin-stained section, medium magnification. The lateral edge of the lesion demonstrates abrupt hyperkeratosis and a combination of epidermal atrophy and acanthosis.
Hematoxylin and eosin-stained section, high magnif Hematoxylin and eosin-stained section, high magnification. The section shows mostly orthokeratotic scale, thinning of the epidermis with a diminished granular cell layer, and an infiltrate of lymphocytes in the superficial dermis, which approximate the dermal-epidermal interface.

Some evidence has indicated that older lesions may show some histologic differences compared with newer ones. [11] Older lesions can show absence of epidermal atrophy and may infiltrate the upper dermis. Ultrastructural studies also reveal the presence of many normal-appearing, membrane-coating granules in the keratinocytes of an old lesion, whereas these normal organelles were not found in the keratinocytes of earlier hyperkeratotic skin lesions. [12]

Electron microscopy

Several authors have reported an absence or decrease in the number of membrane-coating granules, or Odland bodies, within lesional keratinocytes. Although other authors have found Odland bodies to be present, most suggest that the membrane-coating granules may undergo some alterations in number or morphology. Perilesional skin uniformly shows normal keratinocyte differentiation.

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