PMC5034917_01
At presentation, there was linear thickening of skin of lower limbs with small discrete papules and areas of hyperpigmentation.
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Closer inspection of his skin showed that the skin was thickened in a linear distribution with the papules being skin-colored with scattered hyperpigmentation (Figure 1).
Magnetic resonance imaging of pelvis and legs showed bilateral lower limb subcutaneous edema with prominence of the dermal thickness and patchy tethering with increased T2 signal of the quadriceps and adductor muscle groups.
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Magnetic resonance imaging of pelvis and legs showed widespread lower abdominal trunk, pelvic girdle, and bilateral lower limb subcutaneous edema with prominence of the dermal thickness (Figure 2).
(A) Skin biopsy - leg (x40 magnification), (B) skin biopsy - abdomen (x40 magnification), (C) deep skin biopsy - leg (x100 magnification), and (D) perivascular and thickened collagen - abdomen (x200 magnification).. Note: The histology shows normal epidermis with proliferation of angular fibroblasts in the reticular dermis accompanied by disorganized collagen fibres (A-C). Increased interstitial connective tissue mucin in the dermis associated with perivascular lymphocytic infiltrate, there was thickening of the dermal collagen bundles with loss of adipose tissues around the eccrine glands (D).
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There was thickening of the dermal collagen bundles with loss of adipose tissues around the eccrine glands (Figure 3A-D).
Progress at 9 months: spontaneous regression of skin changes.
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Clinically, his lower limb skin was less tethered (Figure 4).