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Update of New Infectious Viral Exanthems in Children
BULLETIN FOR MEDICAL PRACTITIONERS
Assoc Prof. Giam Yoke Chin, Senior Consultant Dermatologist and Deputy Medical Director, Natonal Skin Centre
Dr Swe Swe Win, Visiting Fellow, Natonal Skin Centre
Introduction
Infectious exanthems are common problems in children, particularly during the preschool years. A number of infections have cutaneous manifestations that provide a clue to the diagnosis. Early recognition of distinct exanthems also helps to differentiate viral infections from drug reactions, bacterial and rickettsial rashes and reactive erythemas. New infectious exanthems are recently described. This paper highlights the epidemiology, clinical features and diagnosis of these exanthems:
I. Gianotti-Crosti Syndrome
II. Parvovirus B19
III. Human herpes virus 6,7,8
IV. Unilateral laterothoracic exanthem
V. Eruptive pseudoangiomatosis
VI. STAR complex.
I. Gianotti-Crosti Syndrome (GCS)
In 1965, Gianotti and Crosti first described a distinctive exanthem associated with hepatitis and lymphadenopathy. Several viral infections have been implicated with this syndrome, including Ebstein Barr virus (EBV), Coxsackie, Echo, Hepatitis B, human immunodeficiency virus (HIV), and Cytomegalovirus (CMV).
This is an exanthem presenting in children between 8 to 46 months of age. There are characteristic symmetrical, flat-topped, edematous and tiny hemispherical rose-coloured papules. There is a characteristic acral (Figs. 1,2) and facial distribution, with trunk and palmoplantar distribution in some cases. It may be accompanied by lymphadenopathy and hepatosplenomegaly.[1] Laboratory findings may include lymphocytosis and mild transaminitis. Most children are asymptomatic and do not require treatment. The eruption may persist for several months. Symptomatic treatment with mid-potency topical steroids usually suffices.
II. Parvovirus B19 (PVB19)
Parvovirus B19 is a small DNA virus. About half of the adult population have demonstrable antibodies to PVB19. Most infections (70% of cases) occur in children between 5-15 years of age and 20-50% of patients are asymptomatic.[2] The symptomatic conditions caused by parvovirus include erythema infectiosum, papular-purpuric glove and stockings syndrome, hydrops fetalis, aplastic crisis, as well as possible neurological and rheumatological manifestations.
a. Erythema infectiosum (Fifth disease)
Erythema infectiosum is common in girls of between 1 to 17 years of age. The characteristic features are that of slapped cheeks' and a reticulate rash on the limbs. (Figs. 3,4) Facial erythema appears in about 1-4 days. A discrete transient maculopapular eruption appears on extensors and buttocks with or without involvement of the palms and soles. A lacy reticulate erythema appears within 1 to 3 weeks and heat and exercise enhance this rash. Associated symptoms include fever and mild arthralgia in young children. In adult, rheumatological complaints like arthritis and fibromyalgia may appear. PVB1 9 can interrupt the normal production of red blood cells and result in transient aplastic crisis for people who have blood related problems such as hereditary spherocytosis or hemolytic anemia.[2] Parvovirus infection in pregnant women can be transmitted to the developing fetus, resulting in liver problems, spontaneous abortion, or death in utero. Congenital infections produce chronic anemia.
b. Papular-purpuric gloves-and-socks syndrome
This is a self-limiting dermatosis associated with primary infection by PVB19. In 1990, Harmes et al.[3] described a novel and distinctive acute acral dermatitis in five adults. They named this dermatosis the papular-purpuric glove-and-socks syndrome. The characteristic features consist of pruritic edema and erythema of the hands and feet in a sharply demarcated gloves-and-socks distribution. Oral lesions and fever are followed by the rapid development of petechial purpura. Bagot et a14 described a patient who had the same clinical pattern with sero-conversion to PVB19. These cases involved the cheeks, inner aspect of thighs, buttocks, and penis. This acute exanthem clears in 1-2 weeks.
III. Human herpes virus 6,7,8 (HHV 6,7,8)
HHV 6 and 7 are newly discovered viruses that belong to the genus Roseola virus within the subfamily Betaherpesvirinae. HHV 6 and 7 are closely related to cytomegalovirus (CMV), and interaction between the viruses has also been suggested. HHV 6 may cause fever, neurological disorders and hepatitis. The clinical significance of HHV 7 is less clear. In the dermatological arena, a definite association has been proven only for HHV6 and exanthem subitum (roseola infantum).
Exanthem subitum is one of the commonest causes of a viral rash in children under the age of two. The rash is preceded by a high fever, but children remain well. After 3-5 days, the fever resolves and this is followed by appearance of a disseminated pink papular rash on the trunk, which lasts for 1-2 days and then disappears. Serologic studies show almost universal exposure of most populations to HHV 6. Although only a third of infants develop clinical disease, the prevalence of antibodies against HHV 6 increases from less than 10 percent in children under 6 months age to 75 percent to 90 percent in adults. Hall et al.[5] described 1653 children with acute febrile illness in which 160 children (9.7%) were HHV 6 positive. Of these, 21(13%) required hospitalization for febrile seizures.
HHV7 infections may also present as exanthema subitum with neurological manifestations of convulsion and hemiplegia.
HHV8 is the probable course of Kaposis sarcoma (KS). However, its presence in children suggests a non-sexual route of transmission. The risk of parenteral transmission is low. Lennette et al.[6] reported antibodies to human herpes virus 8 in the general population and in KS patients. HHV 8 has been found in 100% of African endemic KS, 96% of American AIDS-associated KS, and in 25% of the general population and 2.8% of children.
IV. Unilateral laterothoracic exanthem (ULE)
This is pruritic and erythematous morbilliform eruption usually characterized by its asymmetric localization and unilateral onset. It begins close to the axilla, lateral trunk, large flexures, upper inner arm or groin. The exanthem then extends centrifugally to become bilateral, frequently pruritus. Reticulated annular patches, excoriations, vesicles and purpura may be seen. Individual lesions are micropapules surrounded by a halo. Eczematization, coalescence and the formation of dusky centers, and finally desquamation occur. Spontaneous resolution occurs after 5 weeks.
In a clinicopathologic study of 48 patients, a mean age of 24.3 months was reported.[7] Signs of infection were present in 77% of children, the majority occurring before the eruption. Older children and adults are not generally affected by ULE. Lack of recurrence in the same patient suggests that immunity develops. Although signs of infection were reported by most of the patients, no infectious agent was identified.[7]
V. Eruptive pseudoangiomatosis
An acute onset of angioma-like papules followed by spontaneous resolution, occurring during an apparent viral illness was first described by Prose et al.[8] Three children aged 6 months to 6 years developed 2 to 10 angiomatous lesions following a probable viral illness, resolving spontaneously within one week. It may be associated with Echovirus infection. A biopsy specimen from one patient revealed dilated dermal blood vessels with plump hobnail-shaped endothelial cells. The natural history and histopathologic feature of this exanthem suggest the name eruptive pseudoangiomatosis.
VI. STAR Complex
Star is characterized by Sore throat, elevated Temperature, and migratory Arthritis, pruritic Rash.[9]
Jundt et al.[9] described 20 patients aged 3.5 to 48 years with the STAR complex. The duration of illness ranged between 2 weeks to 1 year. Laboratory findings included leucocytosis, thrombocytopenia and anemia. PVB19 was isolated in 8 patients and rubella virus was isolated in 6 patients.
References
- Badari U, Monti A, Righini MG. An epidemic of infantile papular acrodermatitis (Gianotti-Crosti Syndrome) due to EBV Dermatology 1994; 188:203-4.
- Heegaard ED, Hornsieth A. Parvovirus: The expanding spectrum of disease. Acta Pediatr 1995; 84:109-117.
- Harmes M, Feldmann, Saurat JH. Papular- purpuric glove and socks syndrome. JAm Acad Dermatol 1990; 23:850-4.
- Bogot M, Revuz J. Papular - purpuric glove and socks syndrome: primary infection with parvovirus B19? J Am Acad Dermatol 1991; 25:34 1-2.
- Hall ET, Long CE, Schnabel KC, Caserta MT, McIntyre KM, Costanzo MA, et al. Human herpes virus 6 infection in children: A prospective study of complications and reactivation. N Engl J Med 1994; 33 1:432-8
- Lennette EL, Blackbourn DJ, Levy JA. Antibodies to human herpesvirus type 8 in the general population and in kaposi's sarcoma patients. Lancet 1996; 348:858-6 1.
- McCuaig CC, Russo P, Power J, Pedneauit, Lebei L, Marcoux D. Unilateral laterothoracic exanthem: a clinico-pathologic study of 48 patients. JAm Acad Dermatol 1996; 34:979-84.
- Prose NS, Tope W, Miller SE, Kamino H. Eruptive pseudoangiomatosis: a unique childhood exanthema? JAm Acad Dermatol 1993; 29:857-9.
- Jundt JW, Creager AH. STAR complexes. South Med J 1993; 86:52 1-8.
DEDICATED TO EXCELLENCE IN DERMATOLOGY
By National Skin Centre (Singapore)
Copyright (C) 1995 - National Skin Centre (Singapore)
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