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Papular-purpuric gloves and socks syndrome - Skin
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Papular-purpuric gloves and socks syndrome - Skin

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Contributors: Laurie Good MD, Jeffrey D. Bernhard MD, Noah Craft MD, PhD, Lindy P. Fox MD, Lowell A. Goldsmith MD, MPH, Michael D. Tharp MD
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Synopsis

Papular-purpuric gloves and stocking syndrome (PPGSS) is a rare, idiopathic, acral dermatosis related to infection with human parvovirus B19 in almost all cases or, less commonly, HHV-6, measles, or coxsackie B6. It is most commonly seen in young adults, although juvenile PPGSS has been reported in children and adolescents, with a higher incidence in spring and summer. Parvovirus B19 is spread via respiratory droplets and blood products, and from mother to fetus. A flu-like prodrome of fever, myalgia, lymphadenopathy, and malaise can precede or occur simultaneously with the distinctive cutaneous manifestations, which include rapidly progressive, extremely pruritic erythema and swelling of the hands and feet. Papular purpura then develops, covering the palmo-plantar as well as dorsal aspects of the hands and feet. Sharp margins exist at the wrists and ankles, giving this syndrome its name. Most patients also develop painful oral lesions as well. PPGSS is a self-limited disease, and therapy consists of supportive care and maintenance of adequate fluid intake.

Codes

ICD10CM:
L98.8 – Other specified disorders of the skin and subcutaneous tissue

SNOMEDCT:
402920006 – Papular purpuric gloves and socks syndrome

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Whenever petechiae or purpura are present, it is wise to rule out vasculitis, especially infectious. Included in the differential of PPGSS are the following conditions:
  • Meningococcal infection – Petechiae may be the earliest sign. Purpuric lesions usually become larger and necrotic in center. CSF and blood cultures will help differentiate in challenging cases.
  • Erythema multiforme – Lesions are more targetoid.
  • Gianotti-Crosti syndrome – Lesions are more widespread rather than acral; usually follows EBV or acute hepatitis B infection.
  • Immunoglobulin A vasculitis (formerly Henoch-Schönlein purpura) – Often has acral distribution; patient may have history of medication. Lesions are usually larger and blotchier than in PPGSS.
  • Hand-foot-and-mouth disease – Lesions are usually more vesicular in appearance and either asymptomatic or mildly tender rather than pruritic. Adults tend to look and feel more ill than children do.
  • Chemotherapy-related painful acral erythema (Burgdorf syndrome)
Pediatric Patient Considerations:
PPGSS mainly affects young adults, but it has been reported in children and adolescents and should be included in the differential diagnosis of childhood exanthems.

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Therapy

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References

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Last Updated: 01/16/2019
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Papular-purpuric gloves and socks syndrome - Skin
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Papular-purpuric gloves and socks syndrome : Acral, Erythema, Hand edema, Palms and soles, Symmetric extremities, Foot edema
Clinical image of Papular-purpuric gloves and socks syndrome
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