Papular purpuric gloves and socks syndrome

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.

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

Papular purpuric gloves and socks syndrome (PPGSS) is usually caused by an erythrovirus called Parvovirus B19 (EVB19). 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 been noted to have a seasonal variation, often occurring during spring and summer. Papular purpuric gloves and socks syndrome has also been associated with:

• Hepatitis B
• Cytomegalovirus (CMV)
• Epstein-Barr virus
• Human herpesvirus 6, the cause of roseola
• Measles
• Coxsackievirus B, the cause of hand, foot and mouth disease
• Drug reactions.

Who gets papular purpuric gloves and socks syndrome?

Papular purpuric gloves and socks syndrome typically occurs in young adults; sometimes older adults and children.

What are the clinical features 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 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 affected including cheeks, elbows, knees, inner thighs, glans penis, buttocks, and vulva.

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

Papular purpuric gloves and socks syndrome in an adult due to Epstein-Barr virus

Papular purpuric gloves and socks syndrome: EBV 

Papular purpuric gloves and socks syndrome: EBV 

Papular purpuric gloves and socks syndrome: EBV 

Images from: Rosales Santillan M, Dietert JB, Jahan-Tigh R. Adult-onset papular purpuric gloves and socks syndrome. Dermatol Online J. 2018;24(4):13030/qt02x2h6sd.

What are the complications of papular purpuric gloves and socks syndrome

Parvovirus B19 infection can result in complications including:

• 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?

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 progressive course. The viral cause can be confirmed on investigations. [see Laboratory tests for viral infections]

• Serology: IgG, IgM. This test is reported in about seven days.
• PCR is more sensitive. This test is reported in about three days.
• In situ hybridisation or immunohistochemistry on biopsy specimens

If the patient is unwell, a full blood count should be performed. Routine laboratory blood tests are usually normal. Some patients may have lymphopenia, neutropenia, or thrombocytopenia.

A skin biopsy is not usually indicated for the diagnosis of papular purpuric gloves and socks syndrome, as histopathologic findings are nonspecific.

Ultrasound examination and Doppler examination of at-risk pregnancies can detect hydrops fetalis.

What is the 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.

• 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.

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

Resolution of the rash usually occurs within one to three weeks without scarring.