Dermatology

Papular-purpuric Gloves and Socks Syndrome

Papulo-purpuric Gloves and Socks Syndrome ICD-9-CM 079.83

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Characteristic findings on physical examination

Papulo-purpuric gloves and socks syndrome (PPGSS) 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 (Figure 1). The rash may begin with an erythematous exanthem on the hands and feet and progresses into a petechial or purpuric exanthem.

Figure 1.

Erythematous papular lesions and edema of the distal extermities typical of papulo-purpuric gloves and socks syndrome.

Mucosal involvement is common, and may include oral petechial lesions, pharyngeal erythema, swollen lips, and painful oral erosions. Palpable lymphadenopathy may also be present.

Low-grade fever, anorexia, myalgias, arthralgias, and fatigue are common.

The illness resolves spontaneously over several days, usually without sequelae. The exanthem may rarely spread to involve non-acral sites, such as the face, trunk, and groin. Younger children may present with atypical findings, including less frequent occurence of fever, lymphadenopathy and oral erosions.

Expected results of diagnostic studies

Common laboratory findings, when sought, include mild anemia, reticulocytosis, leukopenia, and thrombocytopenia. Histopathologic findings are nonspecific and show a lymphocytic, perivascular infiltrate of the papillary dermis associated with extravasation of erythrocytes in the dermis, without evidence of vasculitis. When the entity is caused by parvovirus B19 (about two-thirds of cases), immunofluorescent testing with antibodies against this virus has revealed the presence of the virus in the endothelial cells of the dermal vessel walls. Direct immunofluorescence analysis reveals deposition of IgM antibody and C3 in the walls of the papillary vessels.

Diagnosis confirmation

Vasculitis is in the differential diagnosis of PPGSS given the appearance of the lesions. There are usually other systemic features of vasculitis that are present, which help to differentiate these from PPGSS. An additional point of differentiating vasculitis from PPGSS is that PPGSS is self-limited and usually resolves within 1-2 weeks.

Patients with acute disseminated meningococcal infection may exhibit a similar rash to patients with PPGSS.Patients with meningococcemia are toxic appearing and have a rapidly deteriorating course, unlike the course of PPGSS.

Henoch-Schönlein purpura (HSP) is heralded by palpable purpuric and petechial lesions on dependent areas of the body. This disorder is most common in school-aged children and usually is accompanied by arthritis, abdominal pain, and nephropathy. Histopathogy with HSP shows leukocytoclastic vasculitis with deposition of IgA antibodies on direct immunofluorescence.

Who is at Risk for Developing this Disease?

PPGSS usually affects young adults during the spring or summer, although cases of PPGSS have been reported in children as well.

What is the Cause of the Disease?

Etiology

Pathophysiology

The etiology and pathophsiology of PPGSS is not entirely clear, although about two-thirds of cases have been linked to human parvovirus B19 infection. The lack of evidence of the presence of this virus in about one-third of patients with PPGSS however, has caused speculation that the clinical manifestations of this entity are nonspecific features of infection with a variety of different viruses.

PPGSS occurs mostly in the spring and summer, which is different from erythema infectiosum, the most common exanthem caused by parvovirus B19, which occurs most commonly in winter and spring. When PPGSS is associated with parvovirus B19, it appears that the immune response to the virus occurs after the appearance of the rash. This also is in contradistinction to erythema infectiosum, which occurs after the appearance of parvovirus-specfic IgM and IgG antibodies.

Thus, it appears that patients with PPGSS, unlike those with erythema infectiosum, are contagious at the time of the appearance of the rash. It is important to remember that patients with PPGSS may be contagious, and caution should be exercised when these patients are admitted to the hospital, or are in contact with individuals who have hemoglobinopathies or who are pregnant.

Systemic Implications and Complications

The lesions of PPGSS are self-limited and resolve spontaneously over days to several weeks in most cases. PPGSS has not been described in patients who have hemoglobinopathies. Thus, it is not clear whether there are any complications related to this entity in patients with underlying conditions. Likewise, this syndrome has not been reported in pregnant patients.

Treatment Options

There is no effective therapy for PPGSS.

Optimal Therapeutic Approach for this Disease

The course of PPGSS is self-limited. Symptomatic relief of any systemic symptoms that may be present, such as fever and pain, may be provided with anti-pyretics and analgesics. The use and safety of nonsteroidal antiinflammatory agents for this entity has not been established.

Patient Management

The rash is self-limited and the lesions resolve spontaneously over days to several weeks.

Unusual Clinical Scenarios to Consider in Patient Management

Unlike the rash of erythema infectiousum, patients with PPGSS may be contagious and caution is warranted when these patients are hospitalized or are in contact with patients with hemoglobinopathies or pregnant women. Strict hand hygiene procedures and droplet isolation should be instituted if these patients are hospitalized.

What is the Evidence?

Harel, L, Straussberg, I, Zeharia, A, Praiss, D, Amir, J. " Papular purpuric rash due to parvovirus B19 with distribution on the distal extremities and the face". CID . vol. 35. 2002. pp. 1558-61.

(This is the first description of three patients (two young adults and one preadolescent patient) with PPGSS who had the classic acral purpura in the extremities accompanied by the perioral rash, which has since been described in other patients. Nice review of the clinical features and pathology of PPGSS.)

Edmondson, MB, Riedesel, EL, Williams, GP, DeMuri, GP. " Generalized petechial rashes in children during a parvovirus B19 outbreak". Pediatrics. vol. 125. 2010. pp. e787-92.

(A systematic evaluation performed by general pediatricians of children with petechial rashes for evidence of parvovirus infection, during a parvovirus outbreak. The authors found that parvovirus is a common cause of petechial rashes in children, with the surprising finding that the rash attributed to the virus was often more generalized than erythema infectiousum and PPGGS. Thus, parvovirus B19 may be the cause of generalized rashes in children distinct from erythema infectiousum and PPGGS.)

Harms, M, Feldman, R, Suarat, JH. " Papular-purpuric ’gloves and socks’ syndrome". J Am Acad Dermatol . vol. 23. 1990. pp. 850-54.

(This was the first description of the clinical entity “papular-purpuric gloves and socks syndrome,” in which five patients are described with an acute, self-limited eruption characterized by petechial purpura, erythema and edema occuring in an acral distribution.)

McNeely, M, Friedman, J, Pope, E. " Generalized petechial eruption induced by parvovirus B19 infection". J Am Acad Dermatol. vol. 52. 2005. pp. S109-113.

(A report of a seven-year boy with high fever, generalized macular rash on his entire body, and petechial rash on his arms and legs, attributed to parvovirus infection. The authors provide a very nice literature review of all cases reported to date of patients with petechial and purpuric rashes attributed to parvovirus infection, as well as a summary of the clinical manifestations and systemic diseases associated with parvovirus infection.)

Fretzayas, A, Douros, K, Moustaki, M, Nicolaidou, P. " Papular-purpuric gloves and socks syndrome in children and adolescents". Pediatr Infect Dis J . vol. 28. 2009. pp. 250-52.

(A case report of a child with PPGSS associated with coinfection with parvovirus and human herpes virus 6. This provides a literature review of all cases of PPGSS occuring in children and adolescents that have been reported.)

Hsieh, MY, Huang, PH. " The juvenile variant of papular purpuric gloves and socks syndrome and its association with viral infections". Br J Dermatol . vol. 151. 2004. pp. 201-6.

(This descriptive report of PPGSS occurring in a pediatric population highlighted the presence of viruses other than parvovirus B19, such as Epstein-Barr virus and cytomegalovirus, as etiologic agents. This also highlighted the difference in clinical presentations in children with PPGSS as opposed to adult patients.)

Grilli, R, Izquierdo, MJ, Farina, MC. " Papular-purpuric “gloves and socks” syndrome: polymerase chain reaction demonstration of parvovirus B19 DNA in cutaneous lesions and sera". J Am Acad Dermatol . vol. 41. 1999. pp. 793-6.

(One of the first clinical descriptions of PPGSS in which parvovirus B19 DNA was recovered from the skin lesions by polymerase chain reaction. The diagnosis of parvovirus infection was further supported by the fact that the patient serovonverted to the virus and had evidence of parvovirus B19 DNA in the sera as well as the cutaneous lesions.)
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