PMC8358431_01
Dynamic changes of erythema multiforme major in an 8-year-old Chinese girl. (A,B) Initiation (A) and progression (B) of erythema multiforme with painful blistering spread in the chest, back, and limbs of the child. (C-E) Cutaneous erythema was further exacerbated (C,D) and new rashes occurred on her hands and feet (E). (F) Local ulcerations on lips and oral mucosa and systemic rash improved after 4 weeks of treatment. Rashes on the lower extremities and abdomen were scab-free. (G) Pigmentation persisted on her arms and shoulders where erythema multiforme had occurred during the follow-up visit after discharge.
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F1
Sporadic miliary rashes with raised target (three concentric rings) were observed on her trunk (Figure 1A). Two days after admission, progressive erythema with painful blistering and raised typical target lesions with three-concentric rings spread on her chest, back, and limbs (Figure 1B), indicating severe erythema multiforme. After 3 days of admission, erythema developed progressively and further invaded her feet and perineum, and erosion on the lips and oral mucosa was observed (Figure 1C). However, cutaneous erythema was further exacerbated (Figure 1D) and new rashes occurred on her hands and feet on the 6th day after admission (Figure 1E). On the 30th day of therapy, rashes on the upper and lower extremities and abdomen were completely scab-free (Figure 1F). At the sites of erythema multiforme, pigmentation persisted on her arms and shoulders (Figure 1G).
Pedigree of the three-generation (I-III) patient's family. Squares denote males while circles denotes females. Shadow symbols indicate affected individuals, while open symbols represent unaffected individuals. Diagonal lines are used to show that a person is died. Information regarding HLA genotypes associated with EM is also included in the pedigree. II-2 without a history of EM shows the same haplotypes (HLA-DQB1*03:01) as the patient with EMM (III-2).
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F2
The pedigree chart of the patient's family is presented in Figure 2. The HLA genotypes overlapped between the patient and her parents were marked and mapped into a genetic pedigree (Figure 2).