PMC5803943_01
(a) Cyanosis on the left hand and skin ulcer on the fourth finger. (b) Subtle livedo reticularis in fingers dorsum, without cuticle involvement. (c) Intense livedo reticularis lesions in right palm, together with cyanosis in distal phalange. (d) Erythematous-violaceous papules over left knuckles, one of them also hyperqueratotic due to a previous ulcer
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F1
Physical examination revealed an incomplete reticulated erythema overlying the acral surfaces, namely the hands, thighs, and feet, with a ruddy-to-violaceous hue [Figure 1a-c]. Thin violaceous plaques were noted on the metacarpophalangeal joints [Figure 1d], bilateral eyelids [Figure 2a], and patellar surface. Discrete reticulated ulcerations were present on the palmar surfaces, extensor surface of the forearms, and distal toes, identified in various stages of evolution [Figures 1a-c and 2b].
(a) Violet erythema in both eyelids, without involvement of nasal dorsum. (b) Erythematous plaque on the right elbow with central desquamative and hyperkeratotic area from a previous ulcer. (c) Right dorsum foot with erythematous warm and tender nodule
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F2
Thin violaceous plaques were noted on the metacarpophalangeal joints [Figure 1d], bilateral eyelids [Figure 2a], and patellar surface. Discrete reticulated ulcerations were present on the palmar surfaces, extensor surface of the forearms, and distal toes, identified in various stages of evolution [Figures 1a-c and 2b]. Erythematous nodules were noted on the thighs and dorsal feet [Figure 2c].
(a) Superficial perivascular infiltrated of lymphocytes, with epidermal atrophy and dilated papular vessels with prominent endothelial cells (biopsy from the right-hand dorsum). (b) Dense, mostly septal, neutrophilic infiltrate with necrosis of fat lobules and calcium deposition, without dermal or epidermal involvement (biopsy from the right foot). Hematoxylin and eosin stain, original magnification: (a) x10, (b) x2
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F3
The first biopsy from the right dorsal hand demonstrated a sparse superficial perivascular infiltrated of lymphocytes, a muted rete ridge pattern, and dilated papillary dermal vessels with swollen endothelial cells in the superficial and deep plexus [Figure 3a]. The second skin biopsy harvested from the right dorsal foot showed a predominantly septal neutrophilic infiltrates and necrosis without vascular involvement [Figure 3b].