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.
Entire Body System
- Infectious Mononucleosis
The patient had had infectious mononucleosis at the age of 3 years. [ncbi.nlm.nih.gov]
BACK TO TOPText only Gianotti-Crosti syndrome on the leg illustration Infectious mononucleosis illustration Gianotti-Crosti syndrome on the leg illustration Infectious mononucleosis illustration A Closer Look Talking to your MD Self Care Tests for Gianotti-Crosti [limamemorial.org]
Other viral diseases include infectious Mononucleosis, Cytomegalovirus, and after vaccination with a live virus serum. [firstderm.com]
The most common triggers for Gianotti-Crosti in the United States include: Coxsackievirus (hand, foot, and mouth disease) Epstein-Barr virus (infectious mononucleosis) Cytomegalovirus (CVM) Enterovirus infections (often seen as respiratory infections) [webmd.com]
Gianotti-Crosti syndrome as a presenting sign of EBV-induced acute infectious mononucleosis. Clin Exp Dermatol. 1997;22(6):301-302. 8. Hofmann B, Schuppe HC, Adams O, Lenard HG, Lehmann P, Ruzicka T. [consultant360.com]
- Inflammation
Definición: Inflammation involving the skin of the extremities, especially the hands and feet. Several forms are known, some idiopathic and some hereditary. The infantile form is called Gianotti-Crosti syndrome. [hon.ch]
Diseases such as jaundice may also accompany or lead to Gianotti Crosti syndrome along with inflammation in the liver. [healthosphere.com]
Treatment consisted of topical or oral corticoids depending on the extent and degree of inflammation of the lesions. After 7 to 15 days of treatment, the lesions disappeared, as did the MC. [analesdepediatria.org]
There were no signs of periungual inflammation. Potassium hydroxide preparationwas negative. A diagnosis of onychomadesisfollowing GCSwas made. During the follow up, two fingernails developed complete nail shedding (Figure 5). [oatext.com]
- Streptococcal Infection
If there are any associated conditions like streptococcal infections, antibiotics may be required. [11] References ↑ "Gianotti-crosti syndrome, papulovesicular acrodermatitis. DermNet NZ". ↑ 2.0 2.1 Michitaka K, Horiike N, Chen Y; et al. [wikidoc.org]
Diagnosis Based on the clinical dermatological findings associated with a viral or streptococcal infection. GCS primarily occurs in children aged 3 months to 15 years, with a peak in children aged 1 to 6 years. [accessanesthesiology.mhmedical.com]
If there are any associated conditions like streptococcal infections, antibiotics may be required. Mild fever and discomfort may be relief with some antipyretic medications (aspirin is not recommended in child below 12 years). [dermatalk.com]
Antibiotics When GCS is associated with streptococcal infection and other types of infections, antibiotics may be given alongside. Anti-pyretics For mild cases of fever, anti-pyretics may be taken. [syndrome.org]
If there are any associated conditions like streptococcal infections, antibiotics may be required. [10] [11] [12] [13] See also List of cutaneous conditions References 1 2 Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). [ipfs.io]
- Constitutional Symptom
It is a benign and self-limited disease; the symptoms disappear after two to eight weeks, without recurrences or scars. Skin lesions are usually asymptomatic. Prodrome might occur, suggesting upper respiratory infection, or constitutional symptoms. [ncbi.nlm.nih.gov]
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]
In five cases a diagnosis of Epstein--Barr virus infection was made on the basis of significant titres of anti-Epstein-Barr virus antibodies (IgM) associated with constitutional symptoms (fever, pharyngitis-tonsillitis). [medicaljournals.se]
- Streptococcal Pharyngitis
PHARYNGITIS STREPTOCOCCAL VULVOVAGINITIS STURGE- WEBER SYNDROME (SWS, ENCEPHALOFACIAL OR ENCEPHALOTRIGEMINAL ANGIOMATOSIS) SUBCUTANEOUS FAT NECROSIS (SCFN) SUCKING BLISTER – online-only SYNOPHRYS – online-only SYRINGOMA T TALIPES CAVUS AND HAMMER TOE [euro-libris.ro]
Respiratoric
- Pharyngitis
Two patients with fever and pharyngitis had group A beta-hemolytic streptococci isolated from the throat. Skin biopsies were done in three cases, and findings were consistent with GCS. [ncbi.nlm.nih.gov]
In five cases a diagnosis of Epstein--Barr virus infection was made on the basis of significant titres of anti-Epstein-Barr virus antibodies (IgM) associated with constitutional symptoms (fever, pharyngitis-tonsillitis). [medicaljournals.se]
Other physical findings on examination may include fever (27%), lymphadenopathy (31%), hepatosplenomegaly (4%), and oropharyngeal vesicles or ulcers, pharyngeal erythema, or tonsillar swelling. [symptoma.com]
Liver, Gall & Pancreas
- Hepatomegaly
[…] manifestation of hepatitis B infection occurring in young children; an exanthem comprising nonpruritic dusky papules on the legs, buttocks, and extensors of the arms; Gianotti-Crosti syndrome lasts 2-8 weeks and is associated with adenopathy, anicteric hepatomegaly [medical-dictionary.thefreedictionary.com]
Lymphadenopathy was noted in eight cases, and hepatomegaly in one case. In 12 cases, histopathology and direct immunofluorescence were non-contributory. Cytolytic hepatitis occurred in one case and was associated with HBs antigenemia. [ncbi.nlm.nih.gov]
Hepatomegaly Hepatomegaly is the medical condition of inflammation of the liver which acts as a symptom of Gianotti Crosti syndrome and may also be accompanied by jaundice infection. [healthosphere.com]
In hepatitis B-negative cases, hepatomegaly and liver function abnormalities, if present, are slight. Serum transaminases rarely exceed 100 U/mL. Prognosis. Prognosis is good. [plasticsurgerykey.com]
Skin
- Urticaria
The rash of papular urticaria resolves much sooner than that of Gianotti-Crosti syndrome. [aafp.org]
[…] of Job Syndrome Dermographism Urticaria Drug Eruptions Drug-Induced Photosensitivity Exfoliative Dermatitis Fixed Drug Eruptions Id Reaction (Autoeczematization) Irritant Contact Dermatitis Papular Urticaria Pressure Urticaria Protein Contact Dermatitis [jurnalpediatri.com]
Allergic rhinitis, atopic urticaria, and allergic conjunctivitis were diagnosed clinically. Participants were children with GCS diagnosed over the previous 5 years. [ncbi.nlm.nih.gov]
Although papular urticaria can occur in all age groups, it predominantly affects children. Intense pruritus and excoriations may be present. The rash of papular urticaria resolves much sooner than that of Gianotti-Crosti syndrome. [dermatologyadvisor.com]
In rare cases, Gianotti–Crosti syndrome must be differentiated from papular urticaria and frictional lichenoid dermatitis; in adults, lichen planus and lichenoid drug eruptions should be considered. [plasticsurgerykey.com]
- Hyperpigmentation
Occasionally, hyperpigmentation or hypopigmentation may persist after the rash resolves. [ncbi.nlm.nih.gov]
Post-inflammatory hypo- or hyperpigmentation may follow resolution of the skin lesions, but permanent scarring is uncommon. [actasdermo.org]
His parents reported a previous similar episode one year ago where the eruptions left large areas of hyperpigmentation up to 4 cm in diameter, and resolved in two weeks. [synapse.koreamed.org]
Rarely postinflammatory hypopigmentation or hyperpigmentation may evolve. Recurrences are possible but rare. The lymphadenopathy may persist for several months. Similarly, hepatosplenomegaly takes longer to resolve than the cutaneous lesions. [dermapress.wordpress.com]
There may be mild postinflammatory hyperpigmentation or hypopigmentation. However, scarring and/or pigment changes usually are not permanent. Recurrences are possible, but rare [67]. [fr.slideshare.net]
- Papular Skin Rash
The symptoms of this syndrome as visible in a patient are: The major characteristic of the Gianotti Crosti syndrome includes average to extreme papular skin rashes. [healthosphere.com]
Neurologic
- Agitation
C Calcifiere în craniu - on-line - doar Candidoză ( CUTANAT ) Candidoza, orale (vezi afte) Cataracta, CONGENITALĂ celulita cefalhematom Pustuloză cefalic, benigne ( acnee nou-născut ) șalazion POX pui ( vezi varicela ) ABUZ ( TRAUMA nonaccidental ) AGITATE [euro-libris.ro]
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].
Ultrasound
- Enlargement of the Liver
Sometimes the child may present with a fever, enlarged tender lymph nodes and an enlarged spleen or liver. The rash may become itchy over time. Once the rash appears, the patient is no longer contagious. GCS is diagnosed clinically. [aocd.org]
When associated findings include enlargement of the lymph nodes (lymphadenopathy) and/or enlargement of the liver (i.e., in association with liver inflammation [hepatitis]), such findings may persist for several months after initial symptom onset. < Previous [rarediseases.org]
Other Pathologies
- 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]
Histopathologic examination of a skin biopsy specimen from the right buttock revealed a perivascular and somewhat interstitial lymphocytic infiltrate in the superficial and mid-dermis with intraepidermal exocytosis of lymphocytes, mild spongiosis and [jaad.org]
Histopathologic findings can include mild epidermal acanthosis and spongiosis with focal paraketatosis, as well as papillary dermis edema and perivascular lymphocytic infiltrate.1 The disease has a benign course, with spontaneous resolution usually within [actasdermo.org]
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]
Treatment
treatment may be required. [11] Treatment is mainly focused on controlling itching, symptomatic relief and to avoid any further complications. [wikidoc.org]
Treatment of Skin Disease: Comprehensive Therapeutic Strategies. 5th ed. Philadelphia, PA: Elsevier; 2018:chap 91. [pennstatehershey.adam.com]
Treatment consisted of topical or oral corticoids depending on the extent and degree of inflammation of the lesions. After 7 to 15 days of treatment, the lesions disappeared, as did the MC. [analesdepediatria.org]
Symptomatic treatment with antihistamines and antipyretics was prescribed. Spontaneous regression occurred after five weeks of evolution, leaving no scars. [ncbi.nlm.nih.gov]
Prognosis
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]
The prognosis is favorable and successful management relies upon general supportive and symptomatic care. [ncbi.nlm.nih.gov]
The diagnosis is clinic and the prognosis is excellent. The treatment is supportive through the use of emollients/topical antipruritic lotions. Ethics statements Patient consent for publication Parental/guardian consent obtained. [casereports.bmj.com]
Outlook (Prognosis) The rash usually disappears on its own in about 3 to 8 weeks without treatment or complication. Associated conditions must be watched carefully. [healthing.ca]
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]
Etiology
[…] of childhood; papulovesicular acrolocated syndrome; acrodermatitis papulosa infantum Overview Infantile papular acrodermatitis is a reaction of the skin to a viral infection. [1] Hepatitis B [2] and Epstein-Barr virus are the most frequently reported etiologies [wikidoc.org]
ETIOLOGY — The epidemiologic features (eg, young age of onset, equal sex distribution in children, and occurrences of outbreaks) support an infectious etiology for GCS [19]. Viral infections are considered the most common etiologic agents. [medilib.ir]
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]
While initial reports implicated hepatitis B virus as the etiologic agent, many other bacterial, viral, and vaccine triggers have since been described., , 3 Baleviciene G. Maciuleviciene R. Schwartz R. [jaad.org]
Epidemiology
Inflammatory reactions are well-known manifestations of MC infection; however, there is a dearth of published data on their frequency, epidemiology and clinical spectrum. [analesdepediatria.org]
[…] 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]
(See "Scabies: Epidemiology, clinical features, and diagnosis".) [medilib.ir]
Owing to the relatively small number of patients, virological and epidemiology studies, and treatment trials usually recruit few study and control subjects. This leads to low statistical powers and thus results have little clinical significance. [mdpi.com]
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 most likely explanation for the exanthem is a local Type IV hypersensitivity reaction to the offending viral or bacterial antigen within the dermis. [accessanesthesiology.mhmedical.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]
Education about GCS is important to prevent over-treatment and avoidance of vaccination in the future. [ijord.com]
References
- Metelitsa AI, Fiorillo L. Recurrent Gianotti-Crosti syndrome. J Am Acad Dermatol. 2011;65(4):876–877.
- Iorizzo LJ 3rd, Scott G, Tausk FA. Gianotti-Crosti syndrome: a case report in an adult. Cutis. 2012;89(4):169-72.
- 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.
- Tagawa C, Speakman M. Papular rash in a child after a fever. Gianotti-Crosti syndrome. Am Fam Physician. 2013;87(1):59-60.
- 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.
- 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.
- 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.
- Chuh AA. Diagnostic criteria for Gianotti-Crosti syndrome: a prospective case-control study for validity assessment. Cutis. 2001;68(3):207-13.
- Retrouvey M, Koch LH, Williams JV. Gianotti-Crosti syndrome following childhood vaccinations. Pediatr Dermatol. 2013;30(1):137-8.