PMC9815528_01
(A-C) Clinical features on admission at age 5-months: erythematous plaques with central necrosis on the tip of the nose and the cheeks bilaterally (A). Ulceration on the helix of the left ear (B) Right knee with erythematous plaques with yellow-brown central crusts (C).
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F1
Similar lesions were seen on the knees and extensor surfaces of the arms (Figures 1A-C).
(A,B) Histopathological findings of skin biopsies: epidermal necrosis, a mononuclear perivascular and periadenxal infiltrate, fat necrosis with hyalinization. Changes compatible with lipodystrophy in the dermis and subcutaneous tissue. (Figure A: H&E, x50, Figure B: H&E, x100).
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F2
No thickening of the basement membrane was observed (Figures 2A,B).
PMC9815528_02
(A-C) Clinical features on his last follow up at the age of 9-years. Dyspigmented atrophic plaques on the cheeks, nose, chin and lateral upper eyelidswith deformity and scarring. Dystrophic and wasted saddle nose (A). Right hand showing ulcerated plaques covered with thick crust and scale, dyspigmented atrophic scars and contractures of fingers (B). Muscle wasting and dyspigmented atrophic scars on the lower limbs, ulcerated plaques and swelling of the knees (C).
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F3
Sadly, on his recent follow up in our clinic in November 2022 he presented a similar but more strikingly debilitating picture (Figures 3A-C).