PMC4314883_01
Plantar keratoderma
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F1
On examination, both palms and soles had diffuse scleroatrophy with fissuring and hyperpigmentation [Figure 1].
Genralised atrophy, dyschromia and xerosis; the hallmark features of poikiloderma
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F2
The skin had reduced pinchability on the distal extremities with generalized atrophy, dyschromia, telangiectasia, and xerosis [Figure 2].
Nodule over the palmar aspect of left hand (which later showed actinic keratosis)
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F3
A raised tender nodular lesion measuring around 1 cm x 1.5 cm was present on the left thenar eminence; there was no ulceration or bleeding from the lesion [Figure 3].
Hypoplastic nails with longitudinal ridging
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F4
Nail changes were characteristic with minimal growth of the third and fifth toenail bilaterally and absence of nail of the second right toe [Figure 4].
H and E, x40 showing epidermal hyperkeratosis and acanthosis and dermis showing a sparse mononuclear cell infiltrate
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F5
Biopsy of palms and soles revealed massive hyperkeratosis and acanthosis and that of the sclerosed skin on the left forearm showed acanthosis with an accentuated granular layer, orthohyperkeratosis with sparse mononuclear cell infiltrate [Figure 5].
Immunohistochemical analysis showed a marked reduction in the number of S100+ epidermal Langerhans cells
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F6
Immunohistochemistry analysis showed a marked reduction in CD1a, Lag+ and S-100 epidermal Langerhans cells [Figures 6 and 7], which is a characteristic finding of Huriez syndrome.
Immunohistochemical analysis showing marked reduction in CD1a+ epidermal Langerhans cells
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F7
Immunohistochemistry analysis showed a marked reduction in CD1a, Lag+ and S-100 epidermal Langerhans cells [Figures 6 and 7], which is a characteristic finding of Huriez syndrome.