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Gianotti-Crosti Syndrome

Infantile Papular Acrodermatitis [giannotti Crosti]

Gianotti-Crosti syndrome is a type of childhood exanthem that is self-limiting, has a characteristic acral distribution and is rarely accompanied by systemic findings.


Presentation

Gianotti-Crosti syndrome (GCS) is a sudden onset of rash in children aged 15 months to 2 years that may be associated with immunization or an acute infectious disease. The most common inciting factor in GCS is infection with Epstein-Barr virus (EBV). Although GCS is usually asymptomatic, it may cause mild pruritus. The rash lasts from 2 to 4 weeks but can be present for as long as 4 months. Recurrent episodes are rare [1] [2].

In general, children affected with GCS do not appear to be ill but have multiple discrete papular and papulovesicular eruptions on the cheeks, extensor surfaces of the extremities and the buttocks. The distribution is symmetrical and acral. The individual monomorphous papules range from 1 to 5 or 10 millimeters and can have a range of color from flesh-toned to erythematous to red-brown. They have a flat top and may coalesce to form larger plaques. However, development of a petechial or purpuric appearance is uncommon. The papules may acquire a lichenoid appearance over days to weeks. It usually resolves after 10 to 60 days but new lesions may appear for 8 to 11 weeks [3] [4]. One report has shown that all cases with an exclusive eruption on the face were infected with EBV [5].

The most important etiologic factor is an infection but immunomodulation can further accentuate the condition and this explains the association of GCS with vaccination [6].

Other physical findings on examination may include fever (27%), lymphadenopathy (31%), hepatosplenomegaly (4%), and oropharyngeal vesicles or ulcers, pharyngeal erythema, or tonsillar swelling. Axillary or inguinal adenopathy, hepatosplenomegaly, and an extensive involvement of the trunk are considered as inconsistent findings in GCS.

Inguinal Lymphadenopathy
  • Cervical, axillary, or inguinal lymphadenopathy may be occasionally found. Rarely, patients may develop hepatomegaly, hepatitis, or splenomegaly secondary to underlying HBV, EBV, or CMV infections.[dermatologyadvisor.com]
Constitutional Symptom
  • Prodrome might occur, suggesting upper respiratory infection, or constitutional symptoms. Diagnosis is eminently clinical, and this disease is associated with viral infections.[ncbi.nlm.nih.gov]
  • The condition was not associated with or preceded by fever or other constitutional symptoms. There was no family history of similar complaints.[mjmsr.net]
  • Constitutional symptoms are usually mild; low-grade fever and lassitude sometimes occur. Pruritus is not a prominent feature, and excoriations are never seen. Lymphadenopathy is common but does not occur in every case.[plasticsurgerykey.com]
Pediatric Disorder
  • […] unusual presenting features for over 300 pediatric disorders.[euro-libris.ro]
Burkina Faso
  • Pityriasis rosea in secondary schools in Ouagadougou, Burkina Faso . Ann Dermatol Venereol 2001; 128 :605-9. [ PubMed ] [ Google Scholar ] 32. Kyriakis KP, Palamaras I, Terzoudi S, et al.[ncbi.nlm.nih.gov]
Night Blindness
  • Acrodermatitis enteropathica is usually associated with other clinical manifestations (eg, alopecia, night blindness, diarrhea, impaired taste, growth retardation, and immune dysfunction) and has a different clinical course from GCS.[slideshare.net]
Joint Tenderness
  • The patients are generally older, and may develop joint tenderness and visceral symptoms. In erythema multiforme, which is also rare in young children, the lesions are larger and more oedematous, and are often dusky and targetoid.[plasticsurgerykey.com]
Palpable Purpura
  • Immunoglobulin A vasculitis (formerly Henoch-Schönlein purpura) presents with palpable purpura on the legs, arms, and buttocks with associated abdominal pain and arthritis.[visualdx.com]
  • It is characterized by palpable purpura in patients with neither thrombocytopenia nor coagulopathy (picture 11A-B), arthritis/arthralgia, abdominal pain, and renal disease.[slideshare.net]
Facial Papule
  • It is important to remember that some truncal lesions do not exclude a diagnosis of GCS.15 There are also reports of GCS presenting with only facial papules,20 although such cases would not fit Chun's criteria.[healio.com]
Night Sweats
  • Increased amount of night sweats along with the swelling and increased tenderness of the lymph nodes in the armpits and groin may also occur in few cases.[healthosphere.com]
Suggestibility
  • The literature of adult cases of Gianotti-Crosti syndrome is reviewed with the suggestion that the syndrome may not be as uncommon as previously supposed.[ncbi.nlm.nih.gov]
Aggressive Behavior
  • Children who are hepatitis B surface antigen positive can be admitted to child-care without restrictions provided that they have no behavioral or medical risk factors (eg, aggressive behavior, biting, bleeding disorder) [69].[slideshare.net]

Workup

The proposed diagnostic criteria for GCS is as follows [7] [8]:

  • Presence of monomorphous, pink to brown papules or papulovesicles with a flat top and 1 to 10 millimeters in diameter; at least three of these four sites have to be involved: cheeks, extensor surfaces of legs, extensor surfaces of the forearms, buttocks; symmetrical eruption; duration should be at least 10 days
  • The absence of an extensive eruption on the trunk and scaling.
  • Exclusion of other diagnoses based on their clinical features.
  • If skin biopsy is done, a compatible histological finding.

Laboratory studies are generally not needed to diagnose GCS but the presence of jaundice or hepatomegaly should raise suspicion of hepatitis B virus infection and tests may reveal elevated liver enzymes, although elevated liver enzymes are mostly related to EBV infection.

Findings on skin biopsy can help rule out other conditions since they are not specific for GCS. Focal spongiosis, mild acanthosis, parakeratosis, and psoriasiform hyperplasia can be observed in the epidermis [3]. The dermis shows moderate perivascular infiltrate of lymphocytes and histiocytes, while eosinophils are scattered occasionally [9]. The papillary dermis reveals extravasated erythrocytes [2].

Lymphocytic Infiltrate
  • Histopathological analysis of 4-mm punch biopsies from the upper extremity of both patients revealed an interstitial dermatitis with mild to moderate perivascular lymphocytic infiltrate and occasional scattered eosinophils in the superficial and mid-dermis[ncbi.nlm.nih.gov]
  • The most common findings are epidermal spongiosis and parakeratosis, with an associated superficial perivascular and interstitial lymphocytic infiltrate. Deep inflammation may also be present.[cancertherapyadvisor.com]
  • Histological findings in PAC include moderately dense lymphocytic infiltrate along with histiocytes in the perivascular area and endothelial swelling. Biopsy could not be done due to rapid and uncomplicated course of the disease.[e-ijd.org]
  • Liver biopsy is not indicated but, not surprisingly, shows acute hepatitis with periportal lymphocytic infiltrates and hepatocellular degeneration. HISTOLOGY The histologic picture of GCS may be dramatic but is not diagnostic.[dermapress.wordpress.com]

Treatment

  • Treatment *The condition has no specific treatment, as it is self-resolving *Associated conditions should be treated accordingly *Topical steroids may be prescribed for itching There is no specific treatment for Gianotti-Crosti Syndrome, as the condition[ozarkderm.com]
  • A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions.[leehealth.org]
  • There is no specific treatment for Gianotti-Crosti syndrome. Treatment can be given to alleviate the symptoms of Gianotti-Crosti syndrome such as itching. Using a moisturiser may be helpful.[dermcoll.edu.au]
  • Treatment for Gianotti–Crosti Syndrome (Acrodermatitis) The rash will go away on its own, after 2-8 weeks, so treatment is typically for the control of itching symptoms. Steroid creams, pramoxine and antihistamines can relieve itching.[firstderm.com]
  • Under the diagnosis of Gianotti-Crosti syndrome following JE vaccination, he was conservatively treated with an antihistamine agent, and his symptoms were all cleared 2 weeks after treatment.[ncbi.nlm.nih.gov]

Prognosis

  • The prognosis is favorable and successful management relies upon general supportive and symptomatic care.[ncbi.nlm.nih.gov]
  • Prognosis The majority of children suffering from Gianotti-Crosti syndrome have a favorable prognosis. They would just eventually recover from the disease without developing any complications.[syndrome.org]
  • More than 90% of patients are younger than 4 years, with a mean age of diagnosis of 15 months to 2 years. [7] Prognosis Prognosis is excellent. Lesions clear within 4-12 weeks.[emedicine.medscape.com]
  • Prognosis. Prognosis is good. Severe active liver disease in patients with hepatitis B may regress without treatment [7,15]. One case with Gianotti–Crosti syndrome secondary to EBV was associated with Bell’s palsy [59].[plasticsurgerykey.com]

Etiology

  • Gianotti-Crosti syndrome is self-limited, characterized by papular eruption with a symmetrical distribution on the limbs and face of children, and a dermatosis of unknown etiology.[ncbi.nlm.nih.gov]

Epidemiology

  • A prompt diagnosis and reassurance to patients and their families may save unnecessary procedures and medical expenditures. back to top Related Articles Skin Cancer Epidemiology and Sun Protection Behaviors Among Native American....[jddonline.com]
  • An epidemiological study of pityriasis rosea in Middle Road Hospital . Singapore Med J 1989; 30 :60-2. [ PubMed ] [ Google Scholar ] 28. Harman M, Aytekin S, Akdeniz S, et al. An epidemiological study of pityriasis rosea in the Eastern Anatolia .[ncbi.nlm.nih.gov]
  • […] be differentiated from: [7] Acrodermatitis enteropathica Erythema infectiosum Erythema multiforme Hand-foot-and-mouth disease Henoch-Schönlein purpura Kawasaki disease Lichen planus Papular urticaria Papular purpuric gloves and socks syndrome Scabies Epidemiology[wikidoc.org]
  • As mentioned under ‘‘Epidemiology,’’ a personal or family history of atopic dermatitis appears to be more common in children with GCS than in the general population.[dermapress.wordpress.com]
  • No genetic or familial predisposition is apparent. [4] An association with oral polio vaccination has been reported. [5] Epidemiology Frequency United States Because of the benign self-limited nature of Gianotti-Crosti syndrome (GCS), most cases are not[emedicine.medscape.com]
Sex distribution
Age distribution

Pathophysiology

  • Case Overview Gianotti-Crosti syndrome Member Rated 0 Patient case no. 3662 Date added 10 July 2003 Patient details Age Child Localisation Upper limbs / entire limb Primary Lesions Papule / erythematous Erythema Pathophysiology infectious diseases / viral[dermquest.com]
  • 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.[ncbi.nlm.nih.gov]
  • The pathophysiologic process underlying Gianotti-Crosti syndrome remains unknown, although it is believed to represent an immunologic response to transient viremia or bacteremia, possibly a delayed-type hypersensitivity response.[emedicine.medscape.com]

Prevention

  • You can prevent some types of eczema by avoiding Things that irritate your skin, such as certain soaps, fabrics, and lotions Stress Things you are allergic to, such as food, pollen, and animals NIH: National Institute of Arthritis and Musculoskeletal[icdlist.com]
  • Treatment is mainly focused on controlling itching and symptomatic relief and treatment of any associated conditions or to prevent any further complications.[dermatalk.com]
  • Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, Rutgers New Jersey Medical School; Visiting Professor, Rutgers University School of Public Affairs[emedicine.medscape.com]

References

Article

  1. Metelitsa AI, Fiorillo L. Recurrent Gianotti-Crosti syndrome. J Am Acad Dermatol. 2011;65(4):876–877.
  2. Iorizzo LJ 3rd, Scott G, Tausk FA. Gianotti-Crosti syndrome: a case report in an adult. Cutis. 2012;89(4):169-72.
  3. Caltabiano R, Vecchio GM, De Pasquale R, Loreto C, Leonardi R, Vasquez E. Human ß-defensin 4 expression in Gianotti-Crosti. Acta Dermatovenerol Croat. 2013;21(1):43-7.
  4. Tagawa C, Speakman M. Papular rash in a child after a fever. Gianotti-Crosti syndrome. Am Fam Physician. 2013;87(1):59-60.
  5. Yoshida M, Tsuda N, Morihata T, et al. Five patients with localized facial eruptions associated with Gianotti-Crosti syndrome caused by primary Epstein-Barr virus infection. J Pediatr. 2004;145(6):843-4.
  6. Baldari U, Monti A, Righini M. An epidemic of infantile papular acrodermatitis (Gianotti-Crosti syndrome) due to Epstein-Barr virus. Dermatology. 1994;188:203–204.
  7. Chuh A, Zawar V, Sciallis GF, Lee A. The diagnostic criteria of pityriasis rosea and Gianotti-Crosti syndrome - a protocol to establish diagnostic criteria of skin diseases. J R Coll Physicians Edinb. 2015;45(3):218-25.
  8. Chuh AA. Diagnostic criteria for Gianotti-Crosti syndrome: a prospective case-control study for validity assessment. Cutis. 2001;68(3):207-13.
  9. Retrouvey M, Koch LH, Williams JV. Gianotti-Crosti syndrome following childhood vaccinations. Pediatr Dermatol. 2013;30(1):137-8.

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Last updated: 2019-07-11 20:37