PMC9393491_01
(a and b) Facial and truncal blistering, and hemorrhagic oral mucositis at initial presentation (20% BSA involvement).
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Multiple well-defined tense clear fluid-filled vesicles/bullae (1-10 mm in size) presented on the face, trunk, upper/lower proximal extremities, and labia majora (Figure 1(a) and (b)), affecting 20% of the body surface area (BSA).
(a-d) Progression of tense cutaneous blisters and hemorrhagic oral mucositis 24 h post-IVIG on day 4 (35% BSA involvement), prior to which she had received methylprednisolone and one dose of ETN.
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Her mucocutaneous lesions rapidly worsened within 24 h (to 35% BSA involvement post-IVIG (Figure 2(a)-(d)) from 20% pre-IVIG) prompting a second higher dose of ETN (0.8 mg/kg SC) on day 5.
Resolution of tense blisters, drying of erosions, and halted progression after receiving the second dose of ETN subsequent to IVIG administration given 48 h prior. (a) Facial blistering with erosions and hemorrhagic oral mucositis 24 h after the second dose of ETN on day 6. (b-d) Hemorrhagic oral mucositis and diffuse vesicles and bullae on the trunk with skin sloughing 48 h after the second dose of ETN on day 7.
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The following day, she demonstrated improvement with drying of erosions and halted progression (Figure 3(a)-(d)).
Follow-up post-discharge 3 weeks after receiving second dose of ETN. (a and b) There is almost complete resolution of mucocutaneous lesions with only mild desquamation of vermillion lip remains. (c and d) Re-epithelialization has occurred in areas of previous blisters/erosions with minimal dyspigmentation.
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One-week post-discharge, she had almost complete recovery with minimal dyspigmentation and only mild vermillion lip desquamation remaining (Figure 4(a)-(d)).