PMC4868932_01
The patient presented hyperpigmented and rough bands following a Blaschko-linear pattern on the right side of the body and generalized follicular hyperkeratosis on the 'background' skin. Both histological sections showed hyperkeratosis with basket-woven configuration in the stratum corneum, irregular epidermal acanthosis with papillomatosis and dilated blood vessels surrounded by scattered lymphocytic infiltrates. A Thinned and dystrophic hair shafts in the section of the 'background' skin. B Orthokeratotic stratum corneum, hair follicles clustered with an evident follicular hyperkeratosis, infundibular keratinic plugs and irregular distribution of melanin pigment in the basal cell layer in the section of the nevus.
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F1
On physical examination, a follicular hyperkeratosis was noted on his entire body, more pronounced in the darker skin patches (fig. 1). In the papillary and perifollicular dermis, the blood vessels were dilated and surrounded by scattered lymphocytic infiltrates (fig. 1A, fig. 1B). In addition, in the section obtained from the 'background' skin, thinned and dystrophic hair shafts could be noted (fig. 1A). The section derived from the darker bands showed instead an orthokeratotic stratum corneum, more pronounced hair follicles clustered with an evident follicular hyperkeratosis, infundibular keratinic plugs and irregular distribution of melanin pigment in the basal cell layer (fig. 1B).
Tufted hair folliculitis on the scalp, a patch of alopecia characterized by perifollicular erythema and follicular hyperkeratosis.
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F2
On his scalp, an area of scarring alopecia characterized by perifollicular erythema and follicular hyperkeratosis was noted with a tufted-like folliculitis pattern (fig. 2).
The patient showed clinodactyly of the fifth finger of both hands (A), more evidently on the left side, confirmed by X-ray examination (B).
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F3
On X-ray examination, clinodactyly was confirmed, while no other skeletal abnormalities were found (fig. 3).