Acrodermatitis Papular (Gianotti Crosti Syndrome)


Article Author:
Jessica Snowden


Article Editor:
Talel Badri


Editors In Chief:
Jon Parham
Abigail Frank
Jon Sivoravong


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Abdul Waheed
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Kristina Soman-Faulkner
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Ahmad Malik
Mark Pellegrini
James Hughes
Beata Beatty
Hajira Basit
Phillip Hynes
Kavin Sugumar


Updated:
4/16/2019 10:14:37 PM

Introduction

Papular acrodermatitis or Gianotti-Crosti syndrome is a benign rash that occurs in childhood in association with a wide variety of viral illnesses. While historically thought to be a manifestation of hepatitis B infection, it has been demonstrated to occur following many viral illnesses and vaccination, which suggests that this is an immunologic response rather than a primary manifestation of infection.[1][2][3]

Etiology

When papular acrodermatitis was initially described in the 1950s, it was proposed as a manifestation of the hepatitis B virus infection. In the post-hepatitis B vaccination era, physicians do not see this association as often. Ongoing studies have demonstrated that papular acrodermatitis can occur following a wide variety viruses. These viruses include but are not limited to Epstein-Barr virus, cytomegalovirus, coxsackievirus, adenovirus, influenza, enteroviruses, echovirus, hepatitis A virus, herpes simplex viruses, HHV-6, HIV, mumps, parainfluenza virus, parvovirus B19, poxviruses, respiratory syncytial virus, and rotavirus. Additionally, it has been reported after vaccination, including influenza, Calmette-Guerin bacillus, diphtheria-pertussis-tetanus, poliomyelitis, hepatitis B, Japanese encephalitis, and measles vaccines. The Epstein-Barr virus is the most commonly reported cause of papular acrodermatitis in the United States. In many cases, no infectious trigger is identified. The relationship between these potential triggers and the manifestation of papular acrodermatitis is not well defined but is presumed to be immunologically mediated.[4][5]

Epidemiology

Reports of papular acrodermatitis are most common in children younger than four years of age, which is consistent with its viral etiology. In children, there is no sex predilection; however, in adulthood, it is slightly more common in female patients. In papular acrodermatitis, there does not appear to be a genetic or familial predisposition. Most cases occur in spring and summer. Papular acrodermatitis occurs more often in children with atopic diseases, including atopic dermatitis. Although the disease is observed worldwide, the etiological agents exhibit geographic variations.

Pathophysiology

The precise pathophysiology of papular acrodermatitis is unknown, but it is presumed to be immunologically mediated given its association with viral infections and vaccination. Its increased prevalence in patients with a history of atopic disease, family history of atopic disease, and/or elevated immunoglobulin E also supports an immune-mediated pathology. Proposed processes include immune complexes or delayed hypersensitivity reactions, but to date, this has not been well defined.

History and Physical

Papular acrodermatitis clinically manifests as a papular rash in the acral distribution as its name implies. Fever, lymphadenitis, and other symptoms such as hepatosplenomegaly or pharyngitis may be seen in association with the rash or preceding it. This is consistent with a variety of viral triggers for this entity. There also may be a history of vaccination preceding the onset of the rash. The rash is abrupt in onset. 

Papular acrodermatitis is a symmetrical, monomorphous, papular eruption that is observed most commonly on the extensor surfaces of the extremities; however, the rash may also affect the face and buttocks. Palms and soles may also be involved. Arms are more commonly involved than legs. The trunk and scalp are relatively spared, as are the popliteal and antecubital fossae. However, the involvement of any of these areas does not preclude the diagnosis of papular acrodermatitis. The rash may be pruritic. Symptoms frequently last for 2 to 4 weeks. In some cases, new lesions may continue to appear for up to 8 to 11 weeks after onset of illness. The lesions are usually pale pink to flesh-colored and 1 mm to 10 mm in size. The lesions tend to be larger in younger children and smaller in older children and adolescents. Rarely, lesions may be vesicular or hemorrhagic. They are firm and discrete. Rarely, the lesions can become confluent over pressure points like knees and elbows. Koebner phenomena, with the accentuation of lesions at the site of trauma, has also been reported. There is not an associated mucosal enanthem. Importantly, the appearance of the rash itself may not help to distinguish the variously associated etiologies of papular acrodermatitis; however, careful assessment of associated or antecedent symptoms is essential for recognizing possible infectious triggers that may require further evaluation or isolation. Usually, the rash resolves without any residual effects or scarring. Occasionally, hyperpigmentation or hypopigmentation may persist after the rash resolves.

Evaluation

The differential diagnosis of papular acrodermatitis includes mainly other viral exanthems, insect bites, atopic dermatitis, erythema multiforme, Langerhans cell histiocytosis, and lichenoid dermatoses. In general, papular acrodermatitis is a clinical diagnosis with no laboratory testing indicated unless there are symptoms or physical exam findings, such as hepatomegaly, to suggest an underlying viral illness, such as hepatitis, that may require further management. Diagnostic criteria have been proposed and include the following symptoms:

  • Lesions lasting at least 10 days;
  • One millimeter to 10 mm papules or papulovesicular lesions with involvement of three of the four of the following sites: extensor surfaces of the forearms, extensor surfaces of the legs, cheeks, or buttocks;
  • Symmetrical distribution of lesions.

Practitioners also may need to further evaluate the causative virus in children who are immunocompromised or who reside in immunocompromised areas. If a patient presents with jaundice, hepatomegaly, or generalized lymphadenopathy, evaluation for other viral infections, including hepatitis B, may be indicated. Rarely, a skin biopsy may be performed to exclude other conditions in immunocompromised or other high-risk patients. Normally, biopsy findings are non-specific, with dermal perivascular mononuclear cell infiltrates and capillary endothelial swelling.[6][7]

Treatment / Management

Practitioners should reassure patients that papular acrodermatitis on its own is a benign and self-limited illness. Symptomatic management of itching may be helpful. Patients may use emollients or oral antihistamines. Mild to moderate potency topical steroids may be used in patients who fail to achieve relief of itching with emollients and oral antihistamines as infectious agents do not directly cause the skin lesions. However, treatment with topical steroids or oral antihistamines does not shorten the course of illness. Any underlying disorder, such as hepatitis B, that requires further management should be addressed. Lesions resolve without scarring although hyperpigmentation may persist in some patients.

Enhancing Healthcare Team Outcomes

Healthcare workers including nurse practitioners and clinicians need to be aware that papular acrodermatitis is a benign viral-induced skin disorder, which is often confused with other skin infections of childhood. If there is any doubt about the diagnosis, the patient should be referred to the dermatologist. Papular acrodermatitis is not person-to-person transmissible and does not require isolation beyond standard precautions or restriction from school or other community activities. However, the associated viral infection may be contagious, thus isolation may need to be considered based on other symptoms suggestive of infectious disease. The dermatologist and dermatologist nurse specialist should provide a team effort to educate patients and their family about the disease and the necessary precautions. [Level V]


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Acrodermatitis Papular (Gianotti Crosti Syndrome) - Questions

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A 3-year-old is brought in with sudden onset of a rash involving the face, extremities, and buttocks but sparing the trunk. The rash is papular, brownish, and non-pruritic. The child is otherwise healthy. What is the most likely diagnosis?



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Gianotti-Crosti syndrome is most often associated with which infectious agent?



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Which of the following conditions is characterized by a monomorphous papular eruption?

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A 2-year-old boy presents with a rash on his arms and legs. The rash is itchy and consists of raised bumps. His parents are concerned because this rash is very different from his usual eczema. He is otherwise doing well and has no other symptoms or physical exam abnormalities. What is the next appropriate step in management?



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Which histopathological sign usually is not seen in papular acrodermatitis?



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Acrodermatitis Papular (Gianotti Crosti Syndrome) - References

References

Robl Imoto R Dr,Uber M,de Carvalho VO, Papular crusted rash in a child after immunisation. Archives of disease in childhood. 2018 May 4     [PubMed]
Pedreira RL,Leal JM,Silvestre KJ,Lisboa AP,Gripp AC, Gianotti-Crosti syndrome: a case report of a teenager. Anais brasileiros de dermatologia. 2016 Sep-Oct     [PubMed]
Drago F,Javor S,Ciccarese G,Parodi A, Gianotti-Crosti syndrome as presenting sign of cytomegalovirus infection: A case report and a critical appraisal of its possible cytomegalovirus etiology. Journal of clinical virology : the official publication of the Pan American Society for Clinical Virology. 2016 May     [PubMed]
Gianotti F,Pesapane F,Gianotti R, Ferdinando Gianotti and the papular acrodermatitis of childhood: a scientist against all the odds. JAMA dermatology. 2014 May     [PubMed]
Llanora GV,Tay CM,van Bever HP, Gianotti-Crosti syndrome: case report of a pruritic acral exanthema in a child. Asia Pacific allergy. 2012 Jul     [PubMed]
Liaw FY,Huang CF,Wu LW,Chiang CP, Acral papular rash in a 2-year-old boy. The Journal of family practice. 2012 Mar     [PubMed]
Bilenchi R,De Paola M,Poggiali S,Acciai S,Feci L,Sansica P,Fimiani M, Papular-purpuric     [PubMed]

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