Erythrokeratodermia variabilis - Skin
Initial migratory plaques of erythema last minutes to weeks; these may be exacerbated by trauma or temperature change. Fixed plaques of erythema with or without hyperkeratosis also occur. Half of patients have a peeling palmar-plantar keratoderma. The disorder progresses through childhood, stabilizes after puberty, and may regress as the patient ages.
Periodic improvement may occur, sometimes noted after prolonged sun exposure. Worsening has been seen in pregnancy and with oral contraceptive use. Health is otherwise normal. The patient may complain of burning, stinging, or itching.
For more information, see OMIM.
L53.8 – Other specified erythematous conditions
70041004 – Erythrokeratodermia variabilis
- Netherton's syndrome presents with associated hair abnormality.
- Outshoorn disease presents with keratolytic winter erythema.
- KID syndrome (keratitis, ichthyosis, deafness)
- Progressive symmetric erythroderma (atrophic dermal papules of dermatomyositis [formerly called Gottron papules]) – In this the plaques are fixed.
- Giroux-Barbeau syndrome presents with erythrokeratoderma and ataxia.
- Greither disease typically has later onset erythrokeratoderma of hands/knees/elbows.
- Schnyder's syndrome has asymmetric erythrokeratoderma with deafness, peripheral neuropathy, muscle atrophy, and mental retardation.