Papular purpuric gloves and socks syndrome

Papular-purpuric gloves and socks syndrome (PPGSS) is a distinctive viral rash characterized by painful redness and swelling of the feet and hands ¹. Papulo-purpuric gloves and socks syndrome is characterized by the presence of palpable purpuric and petechial lesions over the hands and feet. There is often a sharp demarcation at the wrists and ankles, describing a gloves and socks distribution. The rash is typically painful and pruritic and there is usually edema in the same distribution as the rash (Figures 1 and 2). The rash may begin with an erythematous exanthem on the hands and feet and progresses into a petechial or purpuric exanthem.  Papular-purpuric gloves and socks syndrome typically occurs in young adults but can occur at any age. It also sometimes affects older adults and children. Making the correct diagnosis is important because it is sometimes misdiagnosed and treated inappropriately ².

On examination, one will find petechiae and purpura with a sharp demarcation at the wrists and ankles (Figure 1). There also will be symmetric edema and erythema of the palms and soles that may extend onto the dorsal surfaces and patients may complain of burning or pruritus ³. Some sources have documented cases involving intertriginous areas of the body as well. Unlike erythema infectiosum, patients are contagious when they have the rash. Erosions and petechiae may occur in the mouth involving the palate, pharynx, and tongue.

Figure 1. Papular-purpuric gloves and socks syndrome

[Source ⁴ ]

Figure 2. Purpuric gloves and socks syndrome

Footnote: A 72-year-old woman presented with a 5-day history of fever, rash, and limb pain. She had received clarithromycin from another clinic 3 days previously, with no improvement. She had discontinued the clarithromycin because of the rash which had appeared the next day. Her vital signs were normal other than a body temperature of 37.6 °C. Physical examination revealed systemic non-pitting edema, and painful edematous erythema on both forearms and thighs, and non-palpable petechia on her legs. These manifestations were suggestive of papular purpuric gloves and socks syndrome. Her serum anti-parvovirus B19 immunoglobulin M (IgM) was positive, confirming the diagnosis. She recovered with symptomatic treatment.

(Panel A) Bilateral non-palpable petechia on the patient’s legs. (Panel B) Painful and edematous erythema on the patient’s right forearm and back of the hand. There’s a similar findings on the left side.

[Source ² ]

Papular purpuric gloves and socks syndrome causes

Papular-purpuric gloves and socks syndrome is usually caused by an erythrovirus B19 (B19V) or Parvovirus B19. Parvovirus B19 infection is well recognized in children as Fifth disease, slapped cheek syndrome or erythema infectiosum ³. In adults, parvovirus B19 infection has various manifestations and may lead to complications including arthropathy, transient aplastic crisis, myocarditis, and acute glomerulonephritis ⁵. Parvovirus B19 is a single-stranded DNA virus that targets red cells in the bone marrow. It spreads via respiratory droplets, blood products and from a pregnant woman to her fetus. Parvovirus B19 has an incubation period of 7–10 days. If a woman becomes pregnant and happens to get infected, the virus can be passed to her baby ⁶.

Papular-purpuric gloves and socks syndrome has also 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 (EBV)
• Human herpesvirus 6, the cause of roseola
• Measles
• Coxsackievirus B, the cause of hand, foot and mouth disease
• Drug reactions

Papular purpuric gloves and socks syndrome symptoms

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 redness and swelling of the hands and feet.

• 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 complications

Although the rash itself does not have long-term complications, 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

Papular purpuric gloves and socks syndrome diagnosis

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 seven days.
• Parvovirus PCR is more sensitive. This test is reported in about three days.
• In situ hybridization or immunohistochemistry on biopsy specimens

If the patient is unwell or has hemolytic anemia, a complete blood count should be performed. Ultrasound examination and Doppler examination of at-risk pregnancies can detect hydrops fetalis.

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

Papular purpuric gloves and socks syndrome treatment

Treatment of papular purpuric gloves and socks syndrome is generally symptomatic. Affected children/adults may remain at school or work if they feel well enough, as the infectious stage of viremia 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.

References

1. Katta R. Parvovirus B19: a review. Dermatol Clin. 2002;20(2):333-342. doi:10.1016/s0733-8635(01)00013-4
2. Nishimura Y, Ueda T. Papular purpuric gloves and socks syndrome in a 72-year old woman. IDCases. 2020;20:e00752. Published 2020 Apr 25. doi:10.1016/j.idcr.2020.e00752 https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7210422
3. Macri A, Crane JS. Parvoviruses. [Updated 2020 May 4]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2020 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK482245
4. Papular-purpuric gloves and socks syndrome. The Lancet Volume 378, ISSUE 9786, P198, July 09, 2011 https://doi.org/10.1016/S0140-6736(11)60554-0
5. Young NS, Brown KE. Parvovirus B19. N Engl J Med. 2004;350(6):586-597. doi:10.1056/NEJMra030840
6. de Los Ángeles Ribas M, Tejero Y, Cordero Y, Pérez D, Sausy A, Muller CP, Hübschen JM. Identification of human parvovirus B19 among measles and rubella suspected patients from Cuba. J. Med. Virol. 2019 Jul;91(7):1351-1354.