Recurrent papular-purpuric gloves and socks syndrome

ARTICLE

Auteur(s) : Heike Andrea Lörcher, Nina Booken, Astrid Schmieder, Sergij Goerdt, Matthias Goebeler

Department of Dermatology, University Hospital Mannheim, University of Heidelberg, Theodor-Kutzer-Ufer 1-3, 68135 Mannheim, Germany

In spring 2007, a 47-year-old woman presented at our Department with a 4-day history of a painful papular-purpuric erythema of palmae, plantae and dorsal surfaces of the hands and feet (figure 1). Five days before, she had experienced oropharyngeal pain and odynophagia. She reported the occurrence of similar skin lesions 2 years before. Apart from her acral purpuric-petechial rash, the patient presented in a good general condition. Mucosal membranes were not affected. A punch biopsy obtained from the left palm showed a superficial dermal lymphocytic infiltrate (figure 1D). Direct immunofluorescence was unremarkable. Laboratory tests revealed lymphopenia and a moderate elevation of alkaline phosphatase, gamma-glutamyl transpeptidase, lactate dehydrogenase and C-reactive protein. Serology showed IgG antibodies to CMV, EBV, HHV6, enteroviruses and Parvovirus B19, while IgM antibodies were not detectable. Blood tests for viral hepatitis A, B and C were negative. The diagnosis of papular-purpuric gloves and socks syndrome (PPGSS) was made and symptomatic treatment initiated. Skin lesions completely resolved within a few weeks.

One year later, in spring 2008, the patient presented again with purpuric lesions in a “gloves and socks” distribution. Additionally, petechial lesions on the palate were visible. There was no history of preceding infection. Repetition of serological tests revealed, as compared to the episode before, considerably increased levels of IgG antibodies to Parvovirus B19, while all other above mentioned viral antibody titres remained unchanged. IgM antibodies against Parvovirus B19 or other viruses were not detectable. The patient’s skin lesions completely resolved upon symptomatic treatment within two weeks.

PPGSS is a rare, self-limited exanthema characterized by sharply margined erythematopapular and purpuric lesions of the hands and feet in a “gloves and socks” distribution that are often pruritic and sometimes painful [1-3]. In about half of cases similar lesions occur at other sites, such as the cheeks, elbows, buttocks and thighs. Oral mucosal involvement is frequently observed. General symptoms such as fever, asthenia, anorexia, arthralgia, myalgia and lymphadenopathy may occur simultaneously with the skin lesions and are usually mild and transient. Histopathological findings in fully evolved PPGSS include vacuolar interface changes associated with necrotic keratinocytes and dermal haemorrhage [4]. Early histological changes are, as in our patient, non-specific, showing a superficial perivascular lymphocytic infiltrate. Laboratory findings are variable and may include leukopenia, neutropenia, monocytosis and mild elevation of transaminases and C-reactive protein.

Since its first description in 1990 by Harms et al. [1], only about 80 cases of PPGSS have been reported, which mainly occurred in young adults during spring and summer months [2]. In 50% of these, PPGSS was attributed to infection with Parvovirus B19 [5, 6]. In most of these cases clinical manifestations were due to primary infection, while reinfection or reactivation were rare [6]. Relapsing PPGSS, as reported here, has not been communicated before. Besides Parvovirus B19, other agents such as CMV, measles and rubella viruses, Coxsackie virus B6, EBV, HHV6, HHV7 and hepatitis B virus, bacteria (Arcanobacterium haemolyticum) and drugs (trimethoprim sulfamethoxazole) have been associated with PPGSS.

While the trigger of our patient’s PPGSS could not definitely be proven and other etiological agents were not identified, we assume that it might most likely be due to Parvovirus B19. Detection of IgG antibodies at the initial presentation may refer to a primary infection possibly two years before, when PPGSS-like skin lesions occurred for the first time. It remained unclear, however, whether persisting latent infection of intracellular compartments that are not accessible for protecting antibodies was responsible for the recurrence of PPGSS. Finally, we can not exclude that another agent elicited one of the PPGSS episodes. In conclusion, our case report illustrates that PPGSS may, on rare occasions, episodically recur.

Acknowledgements

References

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4 Smith SB, Libow LF, Elston DM, Bernert RA, Warschaw KE. Gloves and socks syndrome: Early and late histopathologic features. J Am Acad Dermatol 2002; 47: 749-54.

5 Bagot M, Revuz J. Papular-purpuric ‘gloves and socks’ syndrome: primary infection with parvovirus B19? J Am Acad Dermatol 1991; 25: 341-2.

6 Feldmann R, Harms M, Saurat JH. Papular-purpuric ‘gloves and socks’ syndrome: not only parvovirus B19. Dermatology 1994; 188: 85-7.