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An 18-month-old boy with erythematous eruption
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An 18-month-old healthy black boy presented to your office with a three-week history of an erythematous, edematous eruption, which began around his elbows and knees and spread to involve his cheeks, the extensor aspects of his upper and lower extremities, and his buttocks. His mother reported that he developed an upper respiratory infection two weeks prior that had since resolved. The lesions were asymptomatic, and the child was otherwise acting well. He had no prior history of similar lesions. On exam, involving his cheeks, forehead, nose, and extensor aspects of his extremities – with remarkable sparing of the trunk – are innumerable discrete and coalescing 2 – 10 millimeter erythematous, edematous monomorphic papules and papulovesicles. A few are centrally crusted. They tend to line up in areas of trauma, such a scratches.
What’s your diagnosis?
Case Discussion
Diagnosis: The answer is Gianotti-Crosti syndrome, or papular acrodermatitis.
First described in 1955 by Ferdinando Gianotti, this syndrome is a benign, self-limited eruption composed of erythematous, edematous papules involving the face, extremities and buttocks. The incidence is unknown, likely due to underreporting of the disease. Children between the ages of 1 and 6 are usually affected, and the pathogenesis has not been clearly established. Viral infections or immunizations are believed to play an important role in the etiology.
The exanthem of GCS is composed of numerous 1 mm to 10 mm erythematous monomorphic papules, or less commonly, papulovesicles that may coalesce and are symmetrically distributed on the cheeks, extensor surfaces of the upper and lower extremities, and buttocks, usually with distinct sparing of the trunk (see figure C). However, a few lesions on the trunk do not rule out GCS. The lesions may develop a hemorrhagic crust or scale and may be seen at sites of trauma (Koebner phenomenon). Patients are usually asymptomatic or may complain of mild pruritus. The lesions can last up to eight to 12 weeks. Patients with GCS may have several systemic findings prior or in addition to the skin lesions, including fever, malaise, upper respiratory symptoms, diarrhea, lymphadenopathy, lymphocytosis or lymphopenia, and rarely hepatosplenomegaly.
Since the initial description by Gianotti, a viral etiology is believed to play a role in the development of GCS. Hepatitis B virus (subtypes adr, ayr, adw, and ayw) was previously thought to be the most common viral infection related to GCS, especially in Europe and Asia. However, more recently, Epstein-Barr virus has been shown to be the most common cause. Several other viral infections found in patients with GCS include cytomegalovirus, human herpesvirus 6, coxsackie virus, molluscum contagiosum, rotavirus, parvovirus B19, respiratory syncytial virus, varicella zoster virus, and HIV. Bacterial infections have also been described as a possible trigger.
The pathogenesis of GCS remains unknown. Patients with atopic dermatitis appear to be more likely to develop GCS, suggesting a possible genetic predisposition or relationship with immune dysregulation. Other theories for the pathogenesis include a viral-mediated delayed hypersensitivity reaction or immune complex deposition leading to the skin lesions. The histopathology is nondiagnostic and varies between the vesicular and nonvesicular type of GCS. The vesicular form demonstrates mild acanthosis, spongiosis and vesiculation containing lymphocytes and Langerhans cells, whereas the non-vesicular form shows more parakeratosis, acanthosis, and some spongiosis. Both types have dermal edema and a mixed perivascular dermal infiltrate.
The differential diagnosis in early or atypical GCS commonly includes papular urticaria, scabies, erythema multiforme (early), lichen planus or lichenoid drug eruption (more common in adults). Atopic dermatitis, pityriasis lichenoides, other viral exanthems, infectious mononucleosis, Henoch-Schonlein purpura, and langerhans cell histiocytosis are also included but less likely. Vaccines reported to be associated with GCS include Haemophilus, oral polio, measles-mumps-rubella, diphtheria- pertussis-tetanus, hepatitis B and Japanese B encephalitis vaccines.
Further workup in most patients, including laboratory studies, is usually unnecessary. If there is evidence of hepatitis or concern for exposure to hepatitis B, hepatitis serologies should be obtained. Due to the prolonged course and striking presentation of GCS, reassurance is an important aspect of therapy. Most patients are asymptomatic and require only supportive care. Moderate potency topical steroids may help; however, worsening of lesions has also been reported. Topical preparations with menthol, camphor or praxomine or the administration of oral anti-histamines can be used in patients with pruritus.
Post-inflammatory hypo- or hyperpigmentation may develop and persist for several months after the lesions have resolved. The ability of the physician to recognize this common childhood exanthem may help to reassure families of its benign nature in the great majority of those affected.
For more information:
- Brandt O, Abeck D, Gianotti R, Burgdorf W. Gianotti-Crosti syndrome. J Am Acad Dermatol. 2006 Jan;54(1):136-45. Epub 2005 Dec 2.
- Fastenberg M, Morrell DS. Acral papules: Gianotti-Crosti syndrome. Pediatr Ann. 2007 Dec;36(12):800-4.
- Paller, Amy S, and Mancini, Anthony J. Hurwitz Clinical Pediatric Dermatology. A Textbook of Skin Disorders of Childhood and Adolescence. Philadelphia: Elsevier, 2006; pp 434-36.
Marissa L. Perman is a PL-III resident at Cincinnati Children’s Hospital.
Spot the Rash is a monthly case study featured in Infectious Diseases in Children designed to test your skills in pediatric dermatology issues.