Gianotti Crosti syndrome or papular acrodermatitis in children. Papular acrodermatitis of children, or Gianotti-Crosti syndrome Children's papular acrodermatitis

Symptoms of psoriasis are inflamed, scaly red patches accompanied by intense itching. Such spots (plaques) are more often located on the skin of the scalp, knees and elbow joints, in the lower back and in places of skin folds. In about a quarter of patients, the nails are affected. Depending on the seasonality of relapses (exacerbation of the disease), there are three types of psoriasis: winter, summer, indefinite. The most common type of winter psoriasis. During the period of exacerbation, the manifestations of psoriasis on the hands, on the knees, on the head, as well as in the lower back and in the places of skin folds appear in the form of reddish plaques. Their sizes vary from the head of a pin to vast areas the size of a palm or more. Rashes are usually accompanied by peeling and excruciating itching. In the process of peeling, superficial scales are easily peeled off, remaining denser, located in depth (hence the second name of psoriasis - psoriasis). Sometimes cracks and suppurations appear in the area of ​​the affected areas of the skin. For progressive psoriasis, the so-called Koebner phenomenon is characteristic: the development of psoriatic plaques in places of injuries or skin scratches. In about a quarter of patients, the nails are affected. In this case, there are point depressions and spotting of the nail plates. In addition, nails can thicken and crumble. In summer, under the influence of sunlight, in patients with the winter form of psoriasis, the symptoms weaken, and sometimes completely disappear. Patients with the summer form of psoriasis, on the contrary, are advised to avoid exposure to the sun, as it worsens the course of the disease.

Papular acrodermatitis or Gianotti-Crosti syndrome is a reaction to the introduction of a viral infection. The disease became known in 1955, and the infectious etiology of the disease was confirmed in 1970. The average age of the diseased is 2 years, but cases of adult infection are known. In this case, the disease does not require any special treatment, since the symptoms usually go away on their own after a while.

The reasons

Papular acrodermatitis occurs in response to the re-introduction of viral particles into the body. Symptoms occur more frequently when infected with hepatitis B or Epstein-Barr, infection with other viruses may develop. At the first contact of the body with the virus, antibodies are produced, which, when the agent enters again, begin to attack the cells of its own body.

The disease often occurs in winter or autumn, when the activity of viruses is especially high.

Symptoms

Gianotti-Crosti syndrome includes several main symptoms: papular rash with rare vesicular elements, lymphadenopathy and hepatosplenomegaly. Papules are pink or reddish, up to 5 mm in diameter, appear symmetrically on the face, extensor surfaces of the joints, skin of the extremities and buttocks. The skin of the body is rarely affected. The papules are painless and do not itch. The elements of the rash appear and spread within seven days, gradually disappearing over 2-8 weeks.

The appearance of a rash is accompanied by an increase in peripheral lymph nodes, less often - the liver and spleen. Perhaps an increase in body temperature, an increase in general weakness.

Diagnostics

Diagnosis is based on an assessment of the clinical picture and blood test data. In the blood, leukopenia or lymphocytosis is detected - nonspecific signs of viral infections. Specific tests are effective only in the search for hepatitis B, usually infection with this virus is excluded in the first place.

Treatment

Treatment of the disease is symptomatic and includes the use of antihistamines, antipyretics, vitamin and mineral complexes. Rarely resort to the use of corticosteroid hormones, instead of it, ointments with an antibacterial effect are prescribed.

To alleviate the condition, bed rest and plenty of fluids are indicated.

A photo


Gianotti-Crosti Syndrome



Papular dermatitis is not an independent disease, but only skin manifestations of various diseases. To get rid of rashes, it is necessary to cure the disease that provoked the development of the rash. But the need for proper care of inflamed skin should not be forgotten. Let's figure out what types of dermatitis with papular rashes exist.

Papules are small lesions on the skin that look like nodules or bumps. A papular rash forms for various reasons. It could be an infectious disease. For example, measles. Or non-infectious diseases. Let's figure out in which cases rashes appear in the form of papules and how to treat the affected skin.

Description of the rash

Papules are small neoplasms on the skin in the form of a nodule that rises above the surface of healthy skin. The lesions may be soft or firm to the touch. And their upper part can have a domed or flattened shape.

Papules are asexual formations, that is, they do not have an internal cavity filled with a clear liquid or pus. The size of the formations can be different, individual elements can have a diameter of 1 to 20 mm.

Varieties of papules

Depending on the size and shape, the following forms of papules are distinguished:

  • miliary. These are very small nodules, their diameter does not exceed 2 mm. They, most often, have a conical shape and are located above the hair follicle.
  • Lenticular. The shape of this variety of papules can be any - cone-shaped, dome-shaped, with a flat surface, the diameter of the formations is up to 5 mm.
  • Numular. These are the largest formations, they are usually formed by the merger of several small elements. Most often, they have a flat upper surface, the diameter of the formation can reach 2 cm.


According to the location of the papules, they are divided into external and internal, located in the thickness of the skin. The first version of the rash can be seen visually, the second, most often, is detected only by palpation of the affected area.

A papular rash may appear on intact skin, but often before the appearance of rashes, redness of the affected area and the formation of edema are noted. After the formations heal, hyperpigmented areas may remain in their place for some time. Scars and scars, as a rule, do not form.

Rash classification

A papular rash can be located on different parts of the body. To determine the type of rash, the following factors are evaluated:

  • localization;
  • location - symmetrical or not;
  • the color of the elements and skin on the affected area;
  • a tendency to merge individual elements with the appearance of large formations;
  • the presence of additional symptoms - itching, burning, etc.

There are three types of rash, we will talk about them in more detail.

Macro-papular

With dermatitis with macro-papular rashes, small dense formations up to 1 cm in diameter are formed. The color of the rash can be unchanged (tone of healthy skin), or dark red with a bluish tint.


This type of dermatitis can affect the skin on any part of the body, depending on the nature of the disease that caused the appearance of skin lesions. Diseases in which macro-papular rashes appear:

  • Measles. Rashes with measles are formed first on the oral mucosa, and then on the skin. Places of localization of rashes are the lateral surfaces of the neck, the area behind the auricles, along the hairline. Then the formations cover the skin of the face and neck, arms and chest.
  • Rubella. With this disease, rashes appear two weeks after infection. First, spots appear in the oral cavity, then on the face and neck, within a few hours, red spots cover the entire body. The size of the papules does not exceed 5 mm, on the first day they are flat, then they become dome-shaped. Individual elements are not prone to merging, and this is the main difference from a measles rash.
  • enteroviral infections. The rash is small, on the skin it is represented by papules, on the mucous membranes of the mouth - by vesicles (vesicles). There may be a rash on the palms, which is not observed with measles and rubella.
  • adenovirus infections. With this disease, papular rashes are small, reddish in color, they form against the background of edematous reddened skin. The appearance of rashes is accompanied by itching.
  • Allergic reactions. With allergies, papular rashes are called urticaria. Rashes can appear after eating allergenic foods or after insect bites. Rash with small pinkish elements, its appearance is accompanied by severe itching.


In addition, the formation of a macro-papular rash may be associated with diseases such as helmitosis, fungal or bacterial infection, autoimmune processes.

Erythematous papular

This type of rash appears on the skin of the face, limbs, lower back. Especially often formations appear under the knees and on the elbow bends. The pattern of formations is symmetrical, the boundaries of the foci are clearly defined.

The pattern of rashes in this type of papular dermatitis is specific:

  • the size of the papules is large;
  • formations appear against the background of inflamed, flaky skin;


  • after the disappearance of the formations, pigmented areas form in their place;
  • most often, rashes appear first on the face, then they spread to the body, and lastly to the limbs;
  • papules are pink, bright red or brown.

This kind of rash appears with scarlet fever, drug allergies, infectious mononucleosis.

Treatment

By itself, the treatment of papular rash is meaningless. It is necessary to identify the cause of its appearance, and then proceed to the treatment of the underlying ailment. After all, a rash is just an external manifestation of the negative processes occurring in the body.

For diagnosis, it is necessary to carry out a number of activities, including examination, assessment of the objective condition of the patient and the implementation of a number of necessary tests. After the diagnosis is made, a treatment plan is prescribed.


In case of a bacterial infection, antibiotics are prescribed. With the viral nature of the disease, antiviral drugs are needed. Allergies are treated with antihistamines. In the course of treatment, it is important to exclude secondary infection of the skin. Therefore, the elements of the rash are recommended to be wiped with antiseptic solutions.


For citation: Grigoriev D.V. Papular acrodermatitis in children, or Gianotti-Crosti syndrome // RMJ. 2014. №3. S. 214

In 1955, Gianotti described a disease characterized by lymphadenopathy, anicteric hepatitis, and a red papular rash symmetrically distributed over the face, buttocks, and extremities. He believed that this disease was caused by a virus, and called it "papular acrodermatitis of children." In subsequent reports in the late 1950s. the eponym "Gianotti-Crosti syndrome" was used. In 1970, Gianotti and an independent group of pediatricians confirmed the infectious origin of the condition by detecting the Australian antigen (hepatitis B surface antigen) in their patients. They believed that papular acrodermatitis in children was a specific manifestation of a viral infection - hepatitis B. In later reports from various countries, a similar disease, not associated with a viral infection, hepatitis B, came to the fore. Gianotti believed that these cases were a different disease that clinically different from papular acrodermatitis in children. He coined the term papulovesicular limb-localized syndrome for cases not associated with hepatitis B.

Caputo et al. performed a critical review of 69 cases of papular acrodermatitis in children and 239 cases of papulovesicular limb-localized syndrome to determine whether the two syndromes could be distinguished clinically, as suggested by Gianotti. These authors were unable to clinically distinguish between cases caused by the hepatitis B virus and those caused by other viruses. They concluded that clinical differences were due to individual response to the virus, not virus type. They suggested that the terms "papular acrodermatitis of children" and "papulovesicular limb-localized syndrome" should be replaced by the term "Gianotti-Crosti syndrome", covering all virus-induced papular and papulovesicular lesions that are symmetrically distributed in acral areas (face, buttocks, outer surface of the limbs).

Epidemiology

There is no data on the involvement of genetic factors in the development of papular acrodermatitis. Caputo et al. in a review of 308 cases of the disease, a slight predominance of males was found and the average age of onset of the disease was determined to be 2 years (6 months - 14 years). Most manifestations of acrodermatitis were observed in the autumn and winter months. It has been reported in adults. Most cases associated with hepatitis B have been described in Italy and Japan. Other countries report the involvement of other viruses in the development of the disease, especially the Epstein-Barr virus in North America.

Medical history

Patients develop a developing rash on the face, buttocks, and extremities. This rash may be itchy and not preceded or accompanied by symptoms and signs of a viral illness.

Physical examination

Gianotti-Crosti syndrome is characterized by a monomorphic papular or papulovesicular rash dispersed on the face (Fig. 1), buttocks, and extremities (Fig. 2, 3). The trunk is often (but not always) clean. There have been reports of Gianotti-Crosti syndrome associated with an infection caused by the Epstein-Barr virus, the manifestations of which were noted only on the face. Individual papules or papulovesicles are dense, dome-shaped, and range in size from 1 to 5 mm in diameter. Papules can “Koebnerize” (i.e., with papular acrodermatitis in children, the Koebner phenomenon is observed) at the sites of injuries and merge over the elbows and knees, forming plaques of various sizes. Although the papules are typically pink or red, in some cases they may be flesh-colored or purple. The rash may begin on the face, buttocks, or extremities and spread over a 7-day period, affecting additional areas. Partial manifestation of the disease without involvement of the face and/or buttocks is common. The rash is usually asymptomatic, but may be accompanied by itching in some cases. The resolution of rashes can take from 2 to 8 weeks.

General examination may reveal signs of a causative viral infection: fever, lymphadenopathy, hepatosplenomegaly, oral mucosal ulcers, pharyngitis, and signs of respiratory tract involvement. Lymphadenopathy and splenomegaly are not specific to cases associated with hepatitis B.

Data from laboratory studies

Cases not associated with hepatitis B virus infection usually have no specific laboratory findings other than virus identification by culture, immunofluorescence, polymerase chain reaction, and/or serology. Lymphopenia or lymphocytosis is often observed as a nonspecific response to a viral infection. Although abnormal liver function tests are a constant feature of cases associated with hepatitis B, they can be seen in cases not associated with hepatitis B (eg, Epstein-Barr virus infection). Most cases of hepatitis B-associated Gianotti-Crosti syndrome in Southern Europe and Japan are caused by a subtype of hepatitis B, referred to as "ayw"; in Korea - subtype "adr". Patients with risk factors for hepatitis B should be screened for this virus at initial presentation.

Pathophysiology and histogenesis

Gianotti-Crosti syndrome is considered as a self-limiting skin response to various infections. It can develop due to the following infections: hepatitis B virus, Epstein-Barr virus, cytomegalovirus, rotavirus, parvovirus B19, Coxsackie A-16 virus, respiratory syncytial virus, enterovirus, echovirus, adenovirus, poliovirus, rubella virus, parainfluenza virus, herpes -human virus types 1 and 6 and human immunodeficiency virus. In addition, Gianotti-Crosti syndrome has been reported to result from immunizations including diphtheria-pertussis, measles-mumps-rubella, influenza, poliomyelitis, diphtheria-whooping-cough-tetanus-polio-Haemophilus influenzae B, hepatitis B, measles-hepatitis B and BCG. Recent reports have described an eruption similar to Gianotti-Crosti syndrome due to infection by Mycoplasma pneumonia, Bartonella henselae, group A β-hemolytic streptococcus, and Neisseria meningitidis. Like other viral exanthems, the initial stage of the syndrome is hematogenous dissemination of the causative virus into the skin. The subsequent immune response to the virus causes an inflammatory reaction in the skin structures (epidermis, skin vessels) that gives rise to a rash. It has been suggested that this is a virus-induced type IV hypersensitivity reaction.

It is possible that the deposition of immune complexes in the blood vessels of the skin may be responsible for the development of rash in hepatitis B-induced cases.

Histological data

The histological picture of the Gianotti-Crosti syndrome is nonspecific. The epidermis shows slight acanthosis, focal parakeratosis, and focal spongiosis. The papillary dermis is moderately edematous, with a superficial lymphohistiocytic infiltrate that is usually perivascular but may be streak-like. Occasionally, overt lymphocytic vasculitis with red blood cell extravasation may be seen.

Immunochemical dyes revealed that CD4+ T-lymphocytes predominate in the inflammatory infiltrate, about 20% - CD8+ T-lymphocytes. Also in the epidermis, the number of Langerhans cells is increased.

Diagnosis

Chuh proposed diagnostic criteria for Gianotti-Crosti syndrome. Positive clinical signs include:

1) monomorphic, dome-shaped, pink-brown papules or papulovesicles from 1 to 10 mm in diameter;

2) any 3 or all 4 affected areas: face, buttocks, forearms and extensor surfaces of the legs;

3) symmetry;

4) Duration at least 10 days.

Negative clinical signs:

1) widespread lesions on the trunk;

2) peeling of lesions.

Differential Diagnosis

The classic Gianotti-Crosti syndrome is rarely confused with other skin diseases, but the diagnosis is possible if the doctor is aware of the clinical picture of such a pathology. If purple lesions are present, they must be distinguished from septicemia, lichenoid parapsoriasis, Henoch-Schonlein purpura, and, in the presence of lymphadenopathy and hepatosplenomegaly, from Langerhans cell histiocytosis. Skin biopsy and appropriate microbiological studies can distinguish between these diseases.

is a combination of dermatological and concomitant reactions that occur in response to a viral infection in children. There is a syndrome of papular or papulo-vesicular rash on the skin of the face, buttocks, upper and lower extremities. Other symptoms of a viral infection may occur - lymphadenopathy, hepatosplenomegaly, etc. Diagnosis involves a comparison of physical examination data and the results of laboratory diagnostic methods. PCR and RIF allow you to identify the pathogen virus. No specific treatment is required, Gianotti-Crosti syndrome tends to regress within up to 8 weeks.

General information

Papular acrodermatitis in children, or Gianotti-Crosti syndrome, is a parainfectious disease that combines a nodular rash of typical localization, an increase in regional lymph nodes, liver and spleen. For the first time, papular acrodermatitis was described in 1955 by Italian dermatologists F. Gianotti and A. Crosti. In 1970, together with a group of pediatricians, Gianotti confirmed the infectious etiology of the disease, while considering it to be a characteristic manifestation of hepatitis B in children. Some time later, Caputo and co-authors proved that papular acrodermatitis is a specific reaction of the child's body to infection, regardless of the type of virus. Thus, the concept of "Gianotti-Crosti syndrome" was introduced, which includes all papular and papulo-vesicular rashes provoked by a viral infection.

No genetic predisposition to this syndrome has been identified. It can occur in children from 6 months to 14 years of age, with an average age of 2 years. Rarely occurs in adults. The male sex is more prone to the disease. The syndrome has a seasonal pattern that falls on the autumn-winter period. In Italy and Japan, the main cause of Gianotti-Crosti syndrome is the hepatitis B virus, in North America, the Epstein-Barr virus. In other countries, there is a mixed etiology of the disease.

Causes of Gianotti-Crosti Syndrome

Gianotti-Crosti syndrome is an immune response of a child's skin to a viral infection. The first stage of development is the dissemination of the virus in the child's body at the first contact and its entry into the skin. When the infectious agent re-enters, inflammation of the epidermis and blood capillaries occurs according to type IV hypersensitivity reaction according to Gell and Coombs. Gianotti-Crosti syndrome can cause hepatitis B virus, Epstein-Barr virus, cytomegalovirus, Coxsackie A-16 virus, enterovirus, adenovirus, influenza and parainfluenza viruses, rubella virus, type I and VI herpes virus, HIV, parvovirus B19. Also, this syndrome can be provoked by immunization of a child with a vaccine against influenza, polio, MMR vaccine, BCG, etc. According to recent studies, β-hemolytic streptococcus, M.pneumoniae, N.meningitidis can cause the disease.

Histologically specific changes in the skin in Gianotti-Crosti syndrome do not occur in a child. The epidermis may have mild acanthosis, parakeratosis, and spongiosis. Rarely, vasculitis and erythrocyte outflow into surrounding areas occur. In the immunochemical study of tissues, it is possible to establish the presence of CD4 and CD8 T-lymphocytes.

Symptoms of Gianotti-Crosti Syndrome

Gianotti-Crosti syndrome is manifested by a symmetrical, monomorphic and monochrome skin rash. Its main elements are papules or papulo-vesicles of dense consistency. The average diameter is 1-5 mm. More often they have a pink, pale red or "copper" color, less often - flesh or purple. In places of frequent trauma, the Koebner phenomenon may occur. On the elbows and knees, groups of papules may merge and form large plaques. Primary localization of the rash: face, buttocks, forearm and extensor surfaces of the lower extremities, rarely - the trunk. An ascending sequence of the appearance of elements on the body is characteristic: from the lower extremities to the face.

Often the rash is preceded by a low-grade fever. After 5-7 days from the onset of the disease, the rash spreads to the adjacent areas. There are options without rashes on the face or buttocks. As a rule, the elements are not accompanied by any somatic sensations, only in some cases itching occurs. Skin manifestations of the infection disappear on their own after 14-60 days.

Depending on the etiological factor, other symptoms of a viral infection may develop: lymphadenopathy, hyperthermia, hepatomegaly, splenomegaly, erosion of the mucous membranes, rhinitis, pharyngitis and other diseases of the upper respiratory tract. The most common is the enlargement of the lymph nodes. At the same time, they are painless, elastic, have a dense texture, are not soldered to each other and to the surrounding tissues.

Diagnosis of the Gianotti-Crosti syndrome

Diagnosis of Gianotti-Crosti syndrome consists in comparing anamnestic, clinical and laboratory data. When collecting an anamnesis, the pediatrician manages to establish the characteristic primary localization of skin rashes, possible causes of infection. The physical examination may reveal symptoms specific to a particular viral infection. Laboratory diagnostic methods can detect monocytosis, lymphocytosis or lymphopenia in the KLA; in a biochemical blood test, an increase in alkaline phosphatase, ALT, AST is determined, rarely - an increase in total bilirubin due to the direct fraction. To exclude viral hepatitis B, ultrasound and liver biopsy, determination of anti-HBs, HBc, HBe markers in the blood can be performed. PCR and RIF make it possible to identify with high accuracy the virus that provoked the development of the Gianotti-Crosti syndrome.

In practical pediatrics, criteria are used that indicate the development of the Gianotti-Crosti syndrome: characteristic elements of epidermal rashes; damage to 3 or 4 parts of the body: face, buttocks, forearms or extensor surfaces of the thigh and lower leg; symmetry of the lesion; duration not less than 10 days. In the presence of papules or papulo-vesicles on the body or their peeling, this syndrome is excluded. Differential diagnosis of Gianotti-Crosti syndrome is carried out with infectious mononucleosis, lichenioid parapsoriasis, hemorrhagic vasculitis, lichen planus and septicemia. For this purpose, the child is consulted by a pediatric dermatologist and an infectious disease specialist.

Treatment of the Gianotti-Crosti syndrome

Gianotti-Crosti syndrome does not require specific treatment. After some time (from 14 days to 2 months), all manifestations disappear on their own, without the use of medications. The prognosis for recovery is favorable. Complications and relapses are not typical. Symptomatic therapy may include topical steroids that do not contain fluorine (mometasone furoate, methylprednisolone aceponate) to prevent pustular complications and antihistamines (suprastin) to relieve itching. Constant monitoring by a pediatrician or family doctor is recommended. When hepatitis B virus infection is confirmed, hepatoprotectors (Essentiale) can be used. Rarely used immunomodulators.

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