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ICD-11

ICD-11 Codes
for EA11 - Papular purpuric gloves and socks syndrome
Teodor Jurukovski January 1, 1970
Fact checked by: Teodor Jurukovski
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Clinical Overview of Papular Purpuric Gloves and Socks Syndrome

Papular purpuric gloves and socks syndrome (PPGSS) is a distinct viral exanthem, recognised as a self-limiting dermatosis. It is characterised by painful redness and swelling primarily affecting the hands and feet. This condition typically occurs in young adults, though it can also affect children and older individuals. PPGSS is often observed during the spring and summer months. The official ICD-11 code for this condition is EA11.

Synonyms and Alternative Terms

This condition is commonly referred to by its abbreviation, PPGSS. It is also widely known by the descriptive term "gloves and socks" syndrome, reflecting its characteristic distribution.

Causes and Risk Factors

The primary cause of Papular purpuric gloves and socks syndrome is infection with the erythrovirus Parvovirus B19 (EVB19). This single-stranded DNA virus spreads through respiratory droplets and has an incubation period of approximately 7 to 10 days. Other viral infections have also been associated with PPGSS, including Hepatitis B, Cytomegalovirus (CMV), Epstein-Barr virus, Human herpesvirus 6, Measles, and Coxsackievirus B. Additionally, drug reactions have been identified as a potential trigger for the syndrome.

Common Symptoms and Presentation

The hallmark presentation of PPGSS is a rapidly progressive, symmetrical eruption on the hands and feet, featuring painful erythema and oedema. This is often accompanied by intense pruritus. Petechiae and purpura typically develop on the palms and soles, with the rash exhibiting a sharp demarcation at the wrists and ankles. Oral inflammation and ulceration can also occur. Systemic symptoms may include mild fever, headache, malaise, myalgia, and arthralgias. In some cases, vesicles and bullae may form, potentially leading to skin sloughing. The rash may extend to other areas such as the cheeks, elbows, knees, inner thighs, buttocks, and glans penis. Enlarged lymph nodes are also a common finding.

Diagnosis of PPGSS

Papular purpuric gloves and socks syndrome is primarily diagnosed based on its characteristic clinical features, including the distinctive distribution and rapid progression of the rash. Laboratory investigations can help confirm the viral cause, with serological tests (IgG, IgM) and PCR being useful methods. While skin biopsies may be performed, the histopathological findings are generally nonspecific.

Treatment and Management Considerations

Management of PPGSS is typically symptomatic, as the condition is self-limiting. The rash usually resolves within one to three weeks without scarring. Patients can often continue their normal activities if they feel well enough, as the infectious period often precedes the appearance of the rash. In cases of chronic parvovirus infection or aplastic crisis, treatments such as red blood cell transfusions and immunoglobulin therapy may be administered. For hydrops fetalis caused by parvovirus infection, intrauterine transfusion is a treatment option.

Documentation and Coding Considerations

The official ICD-11 code for Papular purpuric gloves and socks syndrome is EA11. This code is essential for accurate medical record documentation and billing purposes. Common clinical synonyms include PPGSS and "gloves and socks" syndrome. When documenting, it is important to note the characteristic clinical presentation. If a specific viral or drug-related trigger is identified, this may also be relevant for comprehensive coding and patient management.

Frequently asked questions

What is the ICD-11 code for Papular purpuric gloves and socks syndrome?
The ICD-11 code for Papular purpuric gloves and socks syndrome is EA11.
How is Papular purpuric gloves and socks syndrome classified within the ICD-11 system?
Papular purpuric gloves and socks syndrome (EA11) is classified under 'Dermatoses from distant or systemic viral infection' within the ICD-11 system.
What is the broader ICD-11 chapter that includes Papular purpuric gloves and socks syndrome?
Papular purpuric gloves and socks syndrome falls under the ICD-11 chapter 'Diseases of the skin' (EA00–EM0Z).
What does the ICD-11 classification EA11 represent?
The ICD-11 classification EA11 represents Papular purpuric gloves and socks syndrome.
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