Papular purpuric gloves and socks syndrome

Author: Kevin Zheng. Medical Student, University of Auckland. Chief Editor: Hon A/Prof Amanda Oakley, Dermatologist, Hamilton, New Zealand, October 2015.


What is papular purpuric gloves and socks syndrome?

Papular purpuric gloves and socks syndrome is a distinctive viral rash characterised by painful redness and swelling of the feet and hands. It is sometimes abbreviated PPGSS.

What is the cause of papular purpuric gloves and socks syndrome?

Papular purpuric gloves and socks syndrome is usually caused by an erythrovirus, EVB19 or Parvovirus B19. This is a single-stranded DNA virus that targets red cells in the bone marrow. It spreads via respiratory droplets, and has an incubation period of 7–10 days.

The syndrome has also been noted to have seasonal variation, often occurring during spring and summer. Papular purpuric gloves and socks syndrome has also been associated with:

Who gets papular purpuric gloves and socks syndrome?

Papular purpuric gloves and socks syndrome typically occurs in young adults. It also sometimes affects older adults and children.

What are the symptoms of purpuric gloves and socks syndrome?

The prodromal phase of parvovirus B19 infection causes nonspecific viral symptoms such as mild fever, headache and arthralgia.

Papular purpuric gloves and socks syndrome is rapidly progressive, presenting as symmetrical, painful erythema and oedema of the feet and hands.

  • Petechiae and/or purpura soon follow on the palms and soles, and may spread to the dorsal surfaces of hands and feet.
  • Vesicles and bullae may develop, with subsequent skin sloughing.
  • The rash is sharply demarcated at the wrists and ankles.
  • Other areas may also be afffected, including cheeks, elbows, knees, inner thighs, glans penis, buttocks, and vulva.

Enlarged lymph nodes are common. Neurological symptoms may also occur.

Complications of papular purpuric gloves and socks syndrome

Although the rash itself does not have long-term sequelae, erythrovirus B19 infection can result in complications. These include:

  • Polyarthropathy in infected adults (painful, swollen joints)
  • Aplastic crisis or potentially dangerous low blood cell count in patients with haemolytic blood disorders such as autoimmune haemolytic anaemia and sickle cell disease
  • Spontaneous abortion, intrauterine death (9%) or hydrops fetalis in 3% of the offspring of infected pregnant women. This can occur if erythema infectiosum occurs in the first half of pregnancy. Parvovirus B19 does not cause congenital malformations. As the risk of an adverse outcome is low, the infection is not routinely screened for in pregnancy
  • Chronic parvovirus infection in immunodeficient patients, such as organ transplant recipients, causing erythropoietin-resistant anaemia, proteinuria, and glomerulosclerosis in a renal allograft
  • Rarely, encephalitis, hepatitis, non-occlusive bowel infarction, amegakaryocytic thrombocytopenia, myositis and heart disease

How is the diagnosis of purpuric gloves and socks syndrome made?

In most cases, papular purpuric gloves and socks syndrome is a clinical diagnosis based on its characteristic features of a sharp cut-off at the wrists and ankles and rapidly progressing course. Parvovirus can cause other rashes such as erythema infectiosum. The diagnosis can be confirmed by blood tests.

  • Parvovirus serology: IgG, IgM. This test is reported in about 7 days.
  • Parvovirus PCR is more sensitive. This test is reported in about 3 days.
  • In situ hybridisation or immunohistochemistry on biopsy specimens

If the patient is unwell, or has haemolytic anaemia, a full blood count should be performed. Ultrasound examination and Doppler examination of at-risk pregancies can detect hydrops fetalis.

Routine laboratory tests are usually normal. Some patients may have lymphopaenia, neutropaenia, or thrombocytopenia. A skin biopsy is not indicated for the diagnosis of papular purpuric gloves and socks syndrome, as histopathologic findings are nonspecific.

Treatment of purpuric gloves and socks syndrome

Treatment of papular purpuric gloves and socks syndrome is generally symptomatic. Affected children/adults may remain at school/work if they feel well enough, as the infectious stage of viraemia occurs before the rash is evident. Resolution of symptoms usually occurs within one to three weeks without scarring.

  • Red blood cell transfusions and immunoglobulin therapy can be successful in chronic parvovirus infection or during an aplastic crisis.
  • Hydrops fetalis due to parvovirus infection is treated by intrauterine transfusion.

 

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