Papular-purpuric gloves and socks syndrome
Painful purpuric swelling of the hands and feet stopping at the wrists and ankles - usually parvovirus B19, and unlike slapped cheek the patient may still be infectious.
Red flags
- The infectivity is the opposite of erythema infectiosum. In slapped cheek the child is no longer infectious once the rash appears; in this syndrome antibodies develop later, after the eruption begins, while viraemia is still present - so assume the patient may still be infectious
- Exposure of a pregnant woman: parvovirus B19 in pregnancy risks fetal loss and hydrops. Ask, and act on the answer
- A patient with a haemoglobinopathy, hereditary spherocytosis or any chronic haemolysis - parvovirus B19 causes transient aplastic crisis. Check the full blood count and reticulocytes
- Immunocompromise - B19 can cause persistent anaemia rather than a self-limiting illness
Recognise
Oedema and bright erythema of the palms and soles with petechiae and purpuric papules, ending abruptly at the wrist and ankle. The hands and feet are sore, burning or itchy rather than merely marked.
This entry is mostly purpura, and purpura is the sign that darker skin hides best. The erythema may be absent or violaceous, and the petechiae that give the syndrome its name can be very hard to see against brown or black skin. Rely on what pigment does not conceal: the palms and soles, where pigmentation is least and where this eruption happens to live, the oral mucosa for the enanthem, and the conjunctivae. Rely also on the tone-independent features - the swelling, the pain, and above all the sharp cut-off at the wrist and ankle, which is a border you can see and feel regardless of skin tone. NICE NG240 states directly that rashes can be hard to detect on brown, black or tanned skin, and asks you to check all over the body and to look for petechiae in the conjunctivae.
- A sharp cut-off at the wrists and ankles - the single most specific feature
- Painful or burning swelling of hands and feet, not just a visible rash
- An oral enanthem - erosions, petechiae or vesicles - is common
- Fever and systemic symptoms are usual and precede or accompany the rash
- A strikingly uniform clinical picture from case to case, which is why it is a named syndrome
Distribution
Mimics
- Parvovirus B19 (slapped cheek disease) — The same virus, a different syndrome - and the infectivity rule is reversed between them, which is the point worth carrying away
- Hand, foot and mouth disease — Also acral with an enanthem, but vesicular rather than purpuric and without the sharp wrist and ankle border
- Meningococcal septicaemia and purpura fulminans — Any purpura demands this is excluded first. Meningococcal purpura is not confined to hands and feet and the patient is septic
- IgA vasculitis (Henoch-Schonlein purpura) — Also purpuric with painful swelling, but the purpura is on the buttocks and extensor legs, not palms and soles
- Kawasaki disease — Also painful swollen hands and feet with peeling, but with five days of fever, conjunctivitis, mucosal change and lymphadenopathy
- Erythema multiforme major — Also acral with mucosal involvement, but with true target lesions rather than confluent purpura
What to do in the ED
- Exclude meningococcal disease before anything else. Purpura in a febrile patient is meningococcal sepsis until the distribution and the well patient in front of you say otherwise
- Send parvovirus B19 IgM and IgG. Unlike erythema infectiosum, serology taken at the time of the rash may still be negative while the patient is viraemic, so a negative early IgM does not exclude it
- Ask directly about pregnant contacts and about haemoglobinopathy in the patient and the household. This is the action that changes an outcome
- Check a full blood count and reticulocyte count where there is any haemolytic disorder or immunocompromise
- Advise on isolation from pregnant women and from anyone immunosuppressed until the eruption resolves
Disposition
Discharge the well patient with serology sent and GP follow-up for the result. Admit or discuss urgently where there is haemolytic disease, immunocompromise, or significant anaemia on the blood count.
Safety-netting
Local variation
Sources
- Vargas-Diez E, Buezo GF, Aragues M, Dauden E, De Ory F. Papular-purpuric gloves-and-socks syndrome. Int J Dermatol 1996;35(9):626-32 - 21 patients; antibodies develop later than in erythema infectiosum while viraemia persists
- Frieden IJ. Childhood exanthems. Curr Opin Pediatr 1995;7(4):411-4 - PPGSS is usually, but not invariably, caused by parvovirus B19
- Chuh A, Zawar V, Law M, Sciallis G. Gianotti-Crosti syndrome, pityriasis rosea, asymmetrical periflexural exanthem, unilateral mediothoracic exanthem, eruptive pseudoangiomatosis, and papular-purpuric gloves and socks syndrome: a brief review and arguments for diagnostic criteria. Infect Dis Rep 2012;4(1):e12 - B19 risks in haemoglobinopathy and pregnancy
- NICE NG240 - Meningitis (bacterial) and meningococcal disease: recognition, diagnosis and management. Recommendation 1.1.12: check all over the body (including nappy areas), check for petechiae in the conjunctivae, and note that rashes can be hard to detect on brown, black or tanned skin