Rare diseases · Sign or symptom
Urticaria
Hives
HP:0001025
What it means
Raised, well-circumscribed areas of erythema and edema involving the dermis and epidermis. Urticaria is intensely pruritic, and blanches completely with pressure.
In everyday care
Most people with this sign do not have a rare disease — it appears in common conditions far more often. These general reference pages cover the same topic:
Rare diseases that can present with this38
Very common80–99%
19- Ataxia-photosensitivity-short stature syndrome
- Bullous diffuse cutaneous mastocytosis
- Bullous pemphigoid
- CINCA syndrome
- Congenital lethal erythroderma
- Cutaneous mastocytosis-deafness-microtia syndrome
- Cutaneous small vessel vasculitis
- Dermatitis herpetiformis
- Eosinophilic granulomatosis with polyangiitis
- Familial cold urticaria
- Hereditary acrokeratotic poikiloderma
- Hereditary angioedema type 1
- Infantile systemic hyalinosis
- Pemphigus vulgaris
- Schnitzler syndrome
- Solar urticaria
- Spastic paraplegia-facial-cutaneous lesions syndrome
- Spinocerebellar ataxia type 34
- Xeroderma pigmentosum-Cockayne syndrome complex
Common30–79%
6Sometimes5–29%
11- Aggressive systemic mastocytosis
- Autoimmune lymphoproliferative syndrome
- Homocystinuria due to cystathionine beta-synthase deficiency
- Ichthyosis follicularis-alopecia-photophobia syndrome
- Idiopathic hypereosinophilic syndrome
- Immune dysregulation-polyendocrinopathy-enteropathy-X-linked syndrome
- Immunoglobulin A vasculitis
- Netherton syndrome
and 3 more in this range
The range is how often this sign appears among people diagnosed with each disease — it is not a probability of having any of them. Many of these signs also occur in common, non-rare conditions, and Orphanet separately records 3 diseases where this sign is specifically absent. A sign on this page is a conversation to have with a clinician, not a conclusion.
Part of the broader category
Tracking symptoms like this for someone? Eleplan keeps symptoms, diagnoses, medications and every specialist in one plan.
Start free with EleplanThis page uses the Human Phenotype Ontology (2026-06-23) — Human Phenotype Ontology Consortium, hpo.jax.org. Disease associations from Orphanet (INSERM), CC BY 4.0. Content is educational and is not medical advice.