dermatology quiz and case discussion gloves and socks synd

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Dermatology Quiz and Case Discussion

From The Child's Doctor, Fall 2004

Anthony J. Mancini, MD

Head, Dermatology, Ann & Robert H. Lurie Children's Hospital of Chicago; Professor of

Pediatrics and Dermatology, Northwestern University Feinberg School of MedicineDisclosure: Dr. Mancini has no industry relationships to disclose and does not refer to products that are still

investigational or not labeled for the use in discussion.

Daniela Russi, MD

Fellow, Pediatric Dermatology, Children's Memorial HospitalDisclosure: Dr. Russi has no industry relationships to disclose and does not refer to products that are still

investigational or not labeled for the use in discussion.

Other Disclosure Information

Educational objectives

At the conclusion of this activity, participants will be able to:

Recognize the exanthematous disorder presented in the photographs

Describe the epidemiologic concerns associated with the causative agent

Evaluate the associated findings and relate the course of the disease

CME credit

Credit statement

A 22-month-old male presented to the dermatology clinic for evaluation of a rash involving both

hands. The rash was pruritic and gradually progressing. He was originally treated with

hydrocortisone 1% cream and oral diphenhydramine, without improvement. Review of systems wasnotable for upper respiratory symptoms 3 weeks prior, and negative for fever or gastrointestinal

symptoms. Physical examination revealed partially blanchable, erythematous-to-purpuric patches

without any truly palpable purpura, involving both palms (Figure 1). His oral mucosa revealed asingle superficial ulceration of his right posterior palate. His feet were clear, as was the remainder of

his cutaneous examination, and he had no lymphadenopathy.

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One week later, a 9-month-old male was referred to the dermatology clinic for a rash involving his

hands and feet. His skin eruption was associated with pruritus, and he had had low grade fevers andupper respiratory symptoms for 2 days. Physical examination revealed petechial, erythematous patches of the palms and soles (Figure 2). The remainder of his physical examination wasunremarkable, including a normal oral mucosal examination and the absence of lymphadenopathy.

What is the most likely diagnosis in both patients?

a. Palmoplantar hidradenitis

b. Hand-foot-and-mouth disease

c. Papular-purpuric gloves and socks syndrome

d. Rocky Mountain spotted fever

Answer: C

Discussion:

Papular-purpuric gloves and socks syndrome (PPGSS) is an acute, self-limited condition initially

described in 1990. The characteristic features include purpuric erythema involving the hands and feet

(especially palms and soles) in a “glove and stocking” distribution. It is occasionally associated with



fever and oral lesions. Serological studies have revealed that the vast majority of patients withPPGSS have IgM antibodies to parvovirus B19 (B19). Other infectious agents that have been

proposed in association with PPGSS include human herpesvirus 6 (HHV6), human herpesvirus 7

(HHV7), measles virus and cytomegalovirus. PPGSS occurs most often during the spring andsummer months.

The incubation period in PPGSS is around 10 days. Skin manifestations are quite characteristic, and

begin with edema and erythema symmetrically localized to the hands and feet. Subsequently, petechial and purpuric changes appear, and vary from a few millimeters in diameter to larger,

confluent patches. Pruritus, tingling, and pain may be reported by the patient. Polymorphous lesions(usually small erosions) of the oral cavity may be present, affecting the hard and soft palate, pharynx,

tongue, and inner aspects of the lips. Other occasionally reported signs and symptoms include fatigue, headache, anorexia, arthralgias and lymphadenopathy. Low grade fever is common, often

developing 2 to 4 days following the onset of the rash, and usually not exceeding 38.5 degreesCelsius.

Laboratory findings in patients with PPGSS are variable, and depend upon the specific etiologic

agent. Hematologic findings may include mild and transient abnormalities, including anemia,neutropenia, eosinophilia, monocytosis and thrombocytopenia. In addition, elevation of hepatic

transaminases, C-reactive protein, and erythrocyte sedimentation rate have been observed. Skin biopsies, which are rarely necessary, have revealed nonspecific histopathologic features, includinglymphocytic perivascular infiltrates, edema, and erythrocyte extravasation within the papillarydermis. Direct immunofluorescence studies have shown deposits of immunoglobulin and C3 in a

granular pattern within papillary dermal vessel walls, and B19 DNA has been demonstrated in skin biopsy specimens by polymerase chain reaction (PCR) analysis. These findings suggest that PPGSSmay result from a direct viral effect, as well as an immune-mediated vascular response to infection.

The timing of the antibody response to B19 in PPGSS appears to differ from that seen in erythema

infectiosum (EI). Patients with PPGSS may develop mucocutaneous lesions during the period of

viremia (and hence while still considered infectious), and subsequently develop a humoral immuneresponse. In contrast, in patients with EI, development of the skin exanthem coincides with the

appearance of antibody and disappearance of viremia.

The course of PPGSS is one of spontaneous involution. Resolution of the exanthem occurs over 1 to

2 weeks, during which time desquamation may occur. No residual sequelae are usually found.Treatment is generally supportive, and includes cool fluids and analgesics for oral mucosal

discomfort, if necessary. The affected skin surfaces can be treated with cool compresses and oralantihistamines in symptomatic patients.

For Further Reading:

[1.] Aractingi S, Bakhos D, Flageul B, et al. Immunohistochemical and virological study of skin in the papular-purpuric gloves and socks

syndrome. British Journal of Dermatology 1996;135:599-602.

[2.] Larralde M, Enz PA, Sanchez Gomes A, Corbella MC. Papular-purpuric “gloves and socks” syndrome due to parvovirus B19 infection in

childhood. Pediatr Dermatol 1998 Sep-Oct;15(5):413-414.

[3.] Messina MF, Ruggeri C, Rosano M, et al. Purpuric gloves and socks syndrome caused by parvovirus B19 infection. Pediatr Infect Dis J 2003

Aug;22(8):755-756.



[4.] Pavlovic MD. Papular-purpuric “gloves and socks” syndrome caused by parvovirus B19. Vojnosanit Pregl 2003 Mar-Apr;60(2):223-225.

[5.] Smith PT, Landry ML, Carey H, et al. Papular-purpuric gloves and socks syndrome associated with acute parvovirus B19 infection: case

report and review. Clin Infect Dis 1998;27:164-168.

[6.] Vargas-Diez E, Buezo GF, Aragues M, et al. Papular-purpuric gloves-and-socks syndrome. Int J Dermatol 1996;35:626-632.

[7.] Veraldi S, Rizzitelli G, Scarabelli G, et al. Papular-purpuric “gloves and socks” syndrome. Arch Dermatol 1996;132:975-977.

Accreditation Statement

The Northwestern University Feinberg School of Medicine is accredited by the Accreditation

Council for Continuing Medical Education to provide continuing medical education for physicians.

Credit Designation Statement

The Northwestern University Feinberg School of Medicine designates this enduring material for amaximum of 2 AMA PRA Category 1 Credit(s)™ . Physicians should claim only the creditcommensurate with the extent of their participation in the activity.

Gloves and Socks Syndrome

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copyright 1996 - 2013 DermIS

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diagnosis localization lesions additional descriptions

Gloves and Socks Syndrome back of the hands erythema

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Gloves and socks syndrome caused by parvovirus B19 infection

M Carlesimo1, E Palese1, E Mari 1, V Panasiti 1, A Picarelli 2 , A Rossi 1, 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 typicalpurpuric 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 onthe dorsal areas of his hands and feet. Serologic analysis for parvovirus B19 yielded positive results. Thediagnosis of PPGSS was made. The eruption cleared without therapy in 12 days with plantar and palmardesquamation. Parvovirus B19 and some other viral infections have been proven to be causative agentsof this syndrome.

Introduction

The papular purpuric gloves and socks syndrome (PPGSS) is an uncommon dermatosis described for thefirst time by Harms et al. in 1990 [1]. They named this entity gloves and socks syndrome for the typicaldisposition of the exanthem.

In 1991 Bagot and Revuz observed the association between PPGSS and infection by human parvovirusB19 [2], supporting the hypothesis of a viral etiology. Papular purpuric gloves and socks syndrome isclinically characterized by a monomorphic eruption of purpuric macules and papules that is accompaniedby pruritic and painful edema involving the distal extremities in a peculiar gloves and socks distribution.

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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]. Theexanthem is self-limited and clears within a period of 1 or 2 weeks [6].

Clinical synopsis

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 waspreceded by fever, abdominal pain, anorexia, and asthenia. Neitheroral lesions nor lymphadenopathy were present. Laboratory studieswere within normal limits with the exception of a mildly elevated whiteblood cell count, erythrocyte sedimentation rate at 22 mm/h, andprotein C reactive at 2 mg/dl.

Serologic markers for Epstein Barr virus, parvovirus, measles virus,cytomegalovirus, hepatitis B and C virus, Rickettsia, HIV, herpessimplex virus 1 and 2 were tested with an indirect enzyme linkedimmunosorbent assay on the sixth day and on the eleventh day afteronset of the clinical eruption. The results were within normal limits withthe exception of assays for parvovirus B19 IgG and IgM confirmingacute infection in the second week. The diagnosis of acute parvovirusB19 infection was made on the basis of the presence of specific IgM antibodies in serum samplesassayed at two different times during the patient's illness. Based on the clinical picture and the laboratorydata the diagnosis of PPGSS was made. The eruption cleared after palmoplantar desquamation in 12days.

Discussion

PPGSS is an acute self-limited acral dermatosis that affects children or young adults. Edema anderythema appear on the hands and feet in a "gloves and socks" distribution, followed by the appearanceof pruritic or painful purpuric macules and papules on the same sites [3, 4]. Sometimes similar lesionsmay 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 symptomssuch as fever, asthenia, anorexia, arthralgia, lymphadenopathy, myalgia, respiratory or gastrointestinalsymptoms [3, 7, 8].

Laboratory data are nonspecific with occasional leukopenia, neutropenia, eosinophilia, and mild elevationof transaminase levels and erythrocyte sedimentation rate [4, 8].

Histologic findings are non-specific and include necrotic keratinocytes, erythrocyte extravasion, and asuperficial and mild-dermal perivascular lymphocytic infiltrate.

The illness is self limited, lasting 1-2 weeks; usually the eruption clears with palmar and plantardesquamation.

The viral etiology is well documented in the literature [9,10]. B19 is the most frequently reported etiologicagent of PPGSS, whereas occasional cases are associated with Coxsackie B virus, Epstein Barr virus,

Figure 1

Figure 1: Papular purpuric

lesions presented exclusively

on the hands with a sharp

demarcation at the wrists












































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Measles virus, Cytomegalovirus, Hepatitis B virus, and Herpes virus 6 infection. These data suggest thatPPGSS could be a nonspecific manifestation of a viral infection.

The pathogenesis of this syndrome is still not completely understood. Parvovirus B19 is a member of theerythrovirus family and is the only parvovirus known to cause disease in humans. Infection withparvovirus 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 oferythropoiesis and cytolysis of erythroid progenitor cells [11].

A receptor for Parvovirus B19 is present on bone marrow erythroid precursor cells, endothelial cells ofdermal vessels, and keratinocytes. An immunohistochemical study with specific anti-B19V antibodiesshowed the presence of viral antigens on both epidermal and endothelial cells [12]. Therefore, themucocutaneous 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 wasdone 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, andPPGSS.

In a recent paper, however, the sera of 110 children with cutaneous eruptions and an acute systemicillness were analyzed to evaluate the presence of antibodies against parvovirus B19. Specific IgMantibodies to Parvovirus B19 were found in only 3 cases. This data indicates that Parvovirus B19 is notone 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 theoccasional serious sequelae and because of the increased frequency of this presentation in young adultsin recent years [17].

References

1. Harms M, Feldmann R, Saurat JH . Papular-purpuric "gloves and socks" syndrome. J Am Acad

Dermatol 1990 Nov;23: 850-854.

2. Bagot M, Revuz J. Papular-purpuric "gloves and socks" syndrome: primary infection with parvovirus

B19? J Am Acad Dermatol. 1991 Aug;25(2 Pt 1):341-2.

3. Borradori L, Cassinotti P, Perrenoud D, Frenk E. Papular-purpuric "gloves and socks" sindrome. Int J

Dermatol. 1994 Mar;33(3):196-7.

4. Halasz CL, Cormier D, Den M. Petechial glove and sock sindrome caused by parvovirus B19. J Am

Acad Dermatol. 1992 Nov;27(5 Pt 2):835-8.

5. Aguilar-Bernier M, Bassas-Vila J, Torne-Gutierrez J.I, Martinez-Garcia G, Aragoneses-Fraile H,

Miranda-Romero A. Presence of perineuritis in a case of papuar purpuric gloves and socks syndrome

associated with mononeuritis multiplex attributable to B19 parvovirus. J Am Acad Dermatol. 2006;

54:896-899.

6. Smith SB, Libow LF, Elston DM, Bernert RA, Warschaw KE Gloves and socks syndrome: early and late

histopathologic features. J Am Acad Dermatol. 2002 Nov; 47(5):749-54.



















7. Grilli R, Izquierdo MJ, Farina MC, Kutzner H, Gadea I, Martin L, Requena L. Papular-purpuric "gloves

and socks" sindrome: polymerase chain react demonstration of parvovirus B19 DNA in cutaneous lesions

and sera. J Am Acad Dermatol. 1999 Nov; 41(5 Pt):793-6.

8. Smith Pt, Landry ML, Carey H, Krasnoff J, Cooney E. Papular-purpuric gloves and socks syndrome

associated with acute parvovirus B19 infection: case report and review. Clin Infect Dis. 1998Jul;27(1):164-8.

9. Vargas-Diez E, Buezo GF, Arageus M, Dauden E, De Ory F. Papular-purpuric gloves-and-socks

syndrome. Int J Dermatol. 1996 Aug;132(8):975-7.

10. Stone MS, Murph JR. Papular-purpuric gloves and socks syndrome: a characteristic viral exanthema.

Pediatrics. 1993 Dec;92(6):864-5.

11. de Jong EP, de Haan TR, Kroes AC, Beersma MF, Oepkes D, Walther FJ. Parvovirus B19 infection in

pregnancy. J Clin Virol. 2006 May;36(1):1-7.

12. Alfadey A, Alijubran A, Hainau B, Alhokail A. Papular-purpuric"gloves and socks" syndrome in amother and daughter. J Am Acad Dermatol. 2003 Jun; 48(6):941-4.

13. Feldmann R, Harms M, Borradori L, Saurat JH. "Gloves and socks" papulo-purpuric syndrome. Ann

Dermatol Venereol. 1993;120(11):810-2.

14. Aractingi S, Bakhos D, Flageul B, Verola O, Brunet M, Dubertret L, Morinet F. Immunohistochemical

and virological study of skin in the popular-purpuric gloves and socks syndrome. Br J Dermatol. 1996

Oct;135(4):599-602.

15. Nelson JS, Stone MS. Update on selected viral exanthems. Curr Opin Pediatr. 2000 Aug;12(4):359-

64.

16. Pruksachatkunakorn C, Apichartpiyakul N, Kanjanaratanakorn K. Parvovirus B19 infection in children

with acute illness and rash. Pediatric Dermatology. 2006; 23:216-218.

17. Hsieh MY, Huang PH. The juvenile variant of papular-purpuric gloves and socks syndrome and its

association with viral infections. Br J Dermatol. 2004 Jul;151(1):201-6.

© 2006 Dermatology Online Journal

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Gloves and Socks Syndrome feet petechiae

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Gloves and Socks Syndrome feet macula / patch petechiae









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Gloves and Socks Syndrome palms erythema


more information about this diagnose

Gloves and Socks Syndrome (7)

differential diagnoses

Measles (7)Erythema Infectiosum (10)Chemotherapy (0)

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Stevens-Johnson Syndrome


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Stevens-Johnson Syndrome palms plaque

Stevens-Johnson Syndrome palms vesicle










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Stevens-Johnson Syndrome palms plaque

Stevens-Johnson Syndrome palms vesicle










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Stevens-Johnson Syndrome lower arms plaque

Stevens-Johnson Syndrome lower arms vesicle










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Stevens-Johnson Syndrome lower arms erythema












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Stevens-Johnson Syndrome lips (skin) erosion

Stevens-Johnson Syndrome lips (skin) crust










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more information about this diagnose

Stevens-Johnson Syndrome (55)

differential diagnoses

Gingivostomatitis Herpetica (2)Gingivostomatitis Herpetica Gravis (0)Pemphigus, Paraneoplastic (0)Drug

Eruption (43)Lyell Syndrome (20)Pemphigus Vulgaris (43)Pemphigus Mucosae (44)Bullous Pemphigoid

(71)Linear IgA Dermatosis (3)Erythema Exsudativum Multiforme, Minor Form (12)Acute Cutaneous

Graft-versus-Host Reaction (0)Lichen Planus of the Mucosa, Erosive (30)Lupus Erythematosus Mucosae

Oris (8)Epidermolysis Bullosa Hereditaria (36)

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