PMC4772537_01
Clinical images and microscopy. a, b Hands: significant hyperlinearity with desquamation as well as partial hyperkeratosis and fissures. c Inner thighs: hyperpigmentation. d Heels and soles of the feet: marked fissures and hyperkeratosis. e Histology with H&E staining: orthohyperkeratosis shifted upwards by a hyperparakeratotic layer, psoriasiform akanthopapillomatosis, partly with hypergranulosis and partly with reduced stratum granulosum. Keratinocytes were strikingly pale and show signs of disturbed maturation. Necrotic keratinocytes were also found. Discrete perivascular lymphocytic infiltration subepidermally. f Electron microscopy: intra- and extracellular lipid inclusions (not shown) as well as segmental amyloid deposits (unbranched fibers in image) in the upper dermis (bar = 200 nm).
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F1
On examination, the palms showed significant hyperlinearity with strong desquamation, hyperkeratosis and fissures (fig. 1). The heels and soles exhibited marked fissures and hyperkeratosis (fig. 1d). Furthermore, there was hyperpigmentation on the inner thighs (fig. 1c), the buttocks, the palms and the extensor surfaces of the metacarpophalangeal joints. In the upper dermis, discrete perivascular lymphocytic infiltrates were found (fig. 1e). Under electron microscopic analysis, intra- and extracellular lipid inclusions as well as segmental amyloid deposits in the upper dermis were detected (fig. 1f).