Gloves and socks syndrome caused by parvovirus B19 infection
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https://doi.org/10.5070/D341s0b467Main Content
Gloves and socks syndrome caused by parvovirus B19 infection
M Carlesimo1, E Palese1, E Mari1, V Panasiti1, A Picarelli2, A Rossi1, G Camplone1
Dermatology Online Journal 12 (6): 19
1. Department of Dermatology, University of Rome "La Sapienza" 2. Department of Clinical Sciences, University of Rome "La
Sapienza"Abstract
The papular purpuric gloves and socks syndrome (PPGSS) is an uncommon dermatosis with a typical purpuric exanthem limited to hands and feet; it occurs mainly in young adults. We report a case of a 19-year-old man with an acute febrile illness accompanied by purpuric and papular lesions located mostly on the dorsal areas of his hands and feet. Serologic analysis for parvovirus B19 yielded positive results. The diagnosis of PPGSS was made. The eruption cleared without therapy in 12 days with plantar and palmar desquamation. Parvovirus B19 and some other viral infections have been proven to be causative agents of this syndrome.
Introduction
The papular purpuric gloves and socks syndrome (PPGSS) is an uncommon dermatosis described for the first time by Harms et al. in 1990 [1]. They named this entity gloves and socks syndrome for the typical disposition of the exanthem.
In 1991 Bagot and Revuz observed the association between PPGSS and infection by human parvovirus B19 [2], supporting the hypothesis of a viral etiology. Papular purpuric gloves and socks syndrome is clinically characterized by a monomorphic eruption of purpuric macules and papules that is accompanied by pruritic and painful edema involving the distal extremities in a peculiar gloves and socks distribution.
Some authors report symptoms and signs that occur during the course of this disease such as fever, asthenia, anorexia, arthralgia, lymphadenopathy, mucosal erosions, and perineuritis [3, 4, 5]. The exanthem is self-limited and clears within a period of 1 or 2 weeks [6].
Clinical synopsis
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| Figure 1 |
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| Figure 1: Papular purpuric lesions presented exclusively on the hands with a sharp demarcation at the wrists |
In March 2004 a 19-year-old man was seen in our department for a 6-day history of papular purpuric lesions located exclusively on the hands (Fig. 1) and feet with a sharp demarcation at the wrists and the ankles. Edema, itching, and pain were also present. The eruption was preceded by fever, abdominal pain, anorexia, and asthenia. Neither oral lesions nor lymphadenopathy were present. Laboratory studies were within normal limits with the exception of a mildly elevated white blood cell count, erythrocyte sedimentation rate at 22 mm/h, and protein C reactive at 2 mg/dl.
Serologic markers for Epstein Barr virus, parvovirus, measles virus, cytomegalovirus, hepatitis B and C virus, Rickettsia, HIV, herpes simplex virus 1 and 2 were tested with an indirect enzyme linked immunosorbent assay on the sixth day and on the eleventh day after onset of the clinical eruption. The results were within normal limits with the exception of assays for parvovirus B19 IgG and IgM confirming acute infection in the second week. The diagnosis of acute parvovirus B19 infection was made on the basis of the presence of specific IgM antibodies in serum samples assayed at two different times during the patient's illness. Based on the clinical picture and the laboratory data the diagnosis of PPGSS was made. The eruption cleared after palmoplantar desquamation in 12 days.
Discussion
PPGSS is an acute self-limited acral dermatosis that affects children or young adults. Edema and erythema appear on the hands and feet in a "gloves and socks" distribution, followed by the appearance of pruritic or painful purpuric macules and papules on the same sites [3, 4]. Sometimes similar lesions may also be observed on other sites and a variety of oral or genital manifestation have been described [6, 7].
Systemic involvement that may precede or accompany skin manifestations include flu-like symptoms such as fever, asthenia, anorexia, arthralgia, lymphadenopathy, myalgia, respiratory or gastrointestinal symptoms [3, 7, 8].
Laboratory data are nonspecific with occasional leukopenia, neutropenia, eosinophilia, and mild elevation of transaminase levels and erythrocyte sedimentation rate [4, 8].
Histologic findings are non-specific and include necrotic keratinocytes, erythrocyte extravasion, and a superficial and mild-dermal perivascular lymphocytic infiltrate.
The illness is self limited, lasting 1-2 weeks; usually the eruption clears with palmar and plantar desquamation.
The viral etiology is well documented in the literature [9,10]. B19 is the most frequently reported etiologic agent of PPGSS, whereas occasional cases are associated with Coxsackie B virus, Epstein Barr virus, Measles virus, Cytomegalovirus, Hepatitis B virus, and Herpes virus 6 infection. These data suggest that PPGSS could be a non- specific manifestation of a viral infection.
The pathogenesis of this syndrome is still not completely understood. Parvovirus B19 is a member of the erythrovirus family and is the only parvovirus known to cause disease in humans. Infection with parvovirus B19 during pregnancy can cause several serious complications in the fetus, such as fetal anemia, neurological anomalies, hydrops fetalis, and fetal death because of viral inhibition of erythropoiesis and cytolysis of erythroid progenitor cells [11].
A receptor for Parvovirus B19 is present on bone marrow erythroid precursor cells, endothelial cells of dermal vessels, and keratinocytes. An immunohistochemical study with specific anti-B19V antibodies showed the presence of viral antigens on both epidermal and endothelial cells [12]. Therefore, the mucocutaneous lesions could be virus-mediated, through cytotoxicity, and not antibody-mediated. Cutaneous lesions generally develop on days 10 to 13 after infection [13-15].
In the literature there are some sero-negative cases, perhaps because testing for IgM antibodies was done during the first week of clinic eruption when sometimes antibodies are not yet detectable. Indeed, we perform this test during the second week of clinical eruption.
Many clinical entities have been strongly associated with B19 infection such as erythema infectiosum, transient aplastic crisis, arthralgias, rheumatoid-like arthritis, spontaneous abortions, hydrops fetalis, and PPGSS.
In a recent paper, however, the sera of 110 children with cutaneous eruptions and an acute systemic illness were analyzed to evaluate the presence of antibodies against parvovirus B19. Specific IgM antibodies to Parvovirus B19 were found in only 3 cases. This data indicates that Parvovirus B19 is not one of the most common causes of acute illness and rash in children [16].
We report this case because of the importance of recognizing Parvovirus B19 illness due to the occasional serious sequelae and because of the increased frequency of this presentation in young adults in recent years [17].
References
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