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 was notable 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 a single superficial ulceration of his right posterior
palate. His feet were clear, as was the remainder of his cutaneous examination,
and he had no lymphadenopathy.
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 and upper respiratory symptoms for 2
days. Physical examination revealed petechial, erythematous patches of the palms
and soles (Figure 2). The remainder of his physical examination was
unremarkable, 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 with PPGSS 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 and summer 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 degrees
Celsius.
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,
including lymphocytic perivascular infiltrates, edema, and erythrocyte
extravasation within the papillary dermis. 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 PPGSS may 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 immune response. 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 oral
antihistamines 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. | 
