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Features Departments Information |
 Annette M. Wagner, MD
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Dermatology Quiz ANNETTE M. WAGNER, MD aFall 1996 A TWO-YEAR-OLD boy is referred for evaluation of a new rash that has been present for two weeks. The rash began on the cheeks and spread to involve both upper and lower extremities (See figures below).   The trunk is spared. There is mild pruritis. No other family members have symptoms of pruritis or a rash. The child is otherwise well with no systemic symptoms. He is taking no medications. Past history reveals a short-lived upper respiratory infection several weeks before the onset of the rash. The most likely diagnosis is: A. Scabies Important features to look for on physical exam are: A. Lesions in the mouth
Answers D and B, respectively. Gianotti-Crosti syndrome or papular acrodermatitis of childhood is a virally mediated symmetric, acral eruption with a distinctive clinical appearance. First described by Gianotti in 1955, the disease was associated with hepatitis B surface antigen, hepatomegaly and anicteric hepatitis.1 Since that time, many other viruses have been implicated in producing a Gianotti-Crosti-like clinical picture2 including Coxsackie virus, Epstein-Barr virus3,4 enterovirus, respiratory syncytial virus, parainfluenza virus and group A beta hemolytic streptococci. Hepatitis B, as a cause of Gianotti-Crosti syndrome, has never been reported in North America. The eruption should be viewed as a common final pathway of multiple viral infections although the mechanism responsible for the eruption is not known. Children typically present with Gianotti-Crosti between one and six years of age. Most (92%) are under four at presentation. Males are affected twice as frequently as females in North America. All races are affected equally, and there is no hereditary component.1,5 Gianotti-Crosti syndrome occurs several weeks after resolution of the viral infection responsible for producing the rash. Papules are localized to the acral extremities, face and buttocks. They are symmetrically located. Individual papules are red, 2–5 mm edematous "bug bite-like' vesicopapules. Some are violaceous and pruritic. Mucous membranes, palms and soles are spared. With hepatitis B association, lymphadenopathy, hepatomegaly and serologic evidence of hepatitis may be present. These findings are absent when the eruption is caused by other viruses and agents.4 Routine blood work is not indicated in the absence of physical findings or historical evidence of hepatitis. Lesions of Gianotti-Crosti typically persist for three to six weeks. Resolution occurs spontaneously and does not produce scarring. No specific treatment is indicated. Topical steroids are not helpful and may exacerbate the eruption.1 Antihistamines may be useful in controlling pruritus. Topical distractants such as pramasone or cool emollients can also reduce pruritis. The differential diagnosis of Gianotti-Crosti syndrome includes papular urticaria, frictional lichenoid dermatitis, erythema multiforme and other viral exanthems. Papular urticaria is characterized by papules similar in appearance to those seen in Gianotti-Crosti but in a non-symmetric distribution. Most are bug-bite induced and resolve spontaneously within a week. Frictional lichenoid dermatitis is a seasonal eruption of localized lichenoid papules seen over the elbows and knees in childhood. Lesions coalesce into pruritic plaques that resolve spontaneously and recur in spring and summer of subsequent years. The etiology of this eruption is unknown but felt to occur at sites of frictional trauma in children with atopic diathesis. Erythema multiforme produces target lesions on the trunk and extremities, often with involvement of the palms, soles and mucous membranes. Drugs and viral and bacterial infections have all been implicated in causing this eruption. Typical viral exanthems produce small erythematous papules that merge to confluent plaques with a predilection for the trunk and face. The distribution, lesion size and appearance of Gianotti-Crosti are distinctive. Morbidity, with the exception of pruritis, is rare in this syndrome. In hepatitis B-induced Gianotti-Crosti, chronic active hepatitis has been reported as a rare complication.6 The course is nonrelapsing, although one case of recurrent disease recently appeared in the literature.7 REFERENCES 1. Caputo R, Gelmetti C, Ermacora E, et al: Gianotti-Crosti syndrome: A retrospective analysis of 308 cases. J Am Acad Dermatol 1992;26:207–210. 2. Draelos ZK, Hansen RC, James WD: Gianotti-Crosti syndrome associated with infections other than hepatitis B. JAMA 1986;256:2386–88. 3. Baldari U, Monti A, Righine MG: An epidemic of infantile papular acrodermatitis (Gianotti-Crosti syndrome) due to Epstein-Barr virus. Dermatol 1994;188:203–204. 4. Lacour M, Harms M: Gianotti-Crosti syndrome as a result of vaccination and Epstein-Barr virus infection. Eur J Pediatr 1995;154:688–689. 5. Magyarlaki M, Drobnitsch I, Schneider I: Papular acrodermatitis of childhood (Gianotti-Crosti disease). Pediatr Dermatol 1991;8:224–227. 6. Colombo M, Gerber MA, Ivernace SJ, et al: Immune response to hepatitis B virus in children with papular acrodermatitis. Gastroenterol 1977;73:1103. 7. Patrize A. DiLernia V, Neri I, et al: An unusual case of recurrent Gianotti-Crosti syndrome. Pediatr Dermatol 1994;11:283–284. |