Version June 2024
| Stage | Clinical skin changes | Stage onset | Histopathologic skin findings | Differential diagnosis |
| Stage 1 Vesiculobullous stage | Erythema and blistering | Within the first few weeks of life; generally disappears by age 18 months | Eosinophilic spongiosis and intraepidermal vesicle containing eosinophils. Many apoptotic keratinocytes in the epidermis. |
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| Stage 2 Verrucous stage | Hypertrophic rash | Within the first few months of life; usually lasts for a few months | Papillomatosis, hyperkeratosis, and acanthosis of the epidermis. Many apoptotic cells in the epidermis forming squamous eddies. Major melanin incontinence. |
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| Stage 3 Hyperpigmented stage | Hyperpigmentation | Usually begins as stage 2 starts to resolve; persists into adulthood | Marked melanin incontinence with numerous melanophages in the dermis. No more epidermal hyperplasia. Scattered apoptotic cells in the epidermis. |
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| Stage 4 Atrophic/hypopigmented stage | Hypopigmentation and alopecia | The hyperpigmentation usually begins to fade in the teens and early twenties; does not occur in all patients | An atrophic epidermis; massive reduction of melanin in the basal layer; the persistence of apoptotic bodies in the epidermis or papillary dermis; the complete absence of pilosebaceous units and eccrine glands. |
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