Acrodermatitis is several nosologies different in etiopathogenesis, united by a single term based on the localization of the pathological process on the skin of the distal extremities. At the heart of each of the diseases is inflammation of the dermis. Enteropathic acrodermatitis occurs in children with zinc deficiency. Atrophic acrodermatitis is a cutaneous manifestation of borreliosis (an infectious disease after a tick bite). Pustular acrodermatitis is the result of trophoneurosis, which is commonly considered as a violation of the autonomic nervous system as a result of a malfunction of metabolic processes and tissue nutrition. Diagnosis, treatment and prognosis of any of the acrodermatitis are individual.
ICD 10
L40.2 L90.4 L44.4
General information
Acrodermatitis is a single name for several rare dermatitis with predominant rashes on the skin of the hands and feet. These include: persistent Allopo pustular acrodermatitis (Crocker dermatitis, persistent Setton dermatitis), idiopathic progressive skin atrophy (chronic atrophic Herksheimer-Hartmann acrodermatitis, diffuse idiopathic Buchwald skin atrophy, Peak erythromelia), systemic or enteropathic acrodermatitis affecting newborns and leading to death with untimely or incorrect diagnosis.
The only acrodermatitis, the etiology of which is precisely known, is atrophic acrodermatitis. Its causative agent is a bacterium from the genus of spirochetes – Borrelia Burgdorfer. Due to the fact that the epidemiological situation in the United States due to the intensification of summer attacks of ticks – carriers of borreliosis spirochetes – worsens from year to year, causing an increase in borreliosis infections exponentially, the relevance of knowledge of the symptoms of this disease is beyond doubt. The importance of getting to know other dermatitis of this group is due to the severity of the possible consequences.
Causes
The etiology and pathogenesis of enteropathic and pustular acrodermatitis are not fully known. In modern dermatology, it is believed that enteropathic acrodermatitis of newborns is an infrequent genodermatosis inherited by an autosomal recessive principle. It refers to systemic diseases, which are based on enzymopathy (failure in the normal functioning of enzymes) of the gastrointestinal tract with further disruption of metabolic processes. Against the background of enzyme deficiency, zinc deficiency and inflammation of all layers of the skin occur. The hereditary theory is confirmed by the fact that this pathology occurs mainly in children born from closely related marriages.
As for persistent pustular acrodermatitis Allopo, dermatologists tend to think that it is based on neurodystrophy – a violation of the nervous trophic system. For some reason (infection, injury), the nerve fiber is damaged, and the nerve impulse does not reach the end point. The innervation of tissues, including the skin, is disturbed. As a result, all stages of inflammation consistently develop in the dermis. The elimination of the infectious origin or injury does not change the situation, which gives the right to talk about the unclear etiology of the disease. In addition, many dermatologists consider acrodermatitis to be a type of pustular psoriasis, the cause of which is also not clear.
Atrophic chronic acrodermatitis, unlike its cohorts, has a well-known cause of occurrence. Its causative agent is borrelious spirochete, which enters the human body with a tick bite. Initially, the microbe is localized in the lymph nodes, and then, having reached the maximum concentration, enters the bloodstream, spreads with the blood flow throughout the body and causes the development of inflammation in various organs and tissues, including the skin.
Classification
According to modern ideas, there are three independent types of acrodermatitis, which have different etiopathogenetic mechanisms and clinical manifestations:
- Enteropathic acrodermatitis – the primary element of skin rashes are blisters (vesicles and pustules) of different sizes, accompanied by disruption of the gastrointestinal tract, the development of dystrophy, a violation of the infant’s psyche.
- Pustular acrodermatitis Allopo is a form of local acrodermatitis affecting mainly the nail phalanges of the fingers and feet. The primary element is small oval pustules that pour out symmetrically. Generalization of the process is very rarely possible.
- Atrophic acrodermatitis – cutaneous manifestations of borreliosis (Lyme disease), its stage III, in the form of saturated maroon erythema of different sizes.
Somewhat apart is atypical acrodermatitis – papular Gianotti-Crosti syndrome, which occurs in children. Its localization is typical for acrodermatitis, but the primary element is an atypical papule.
Symptoms
Enteropathic acrodermatitis
The clinical picture of the disease depends on its variety. Enteropathic acrodermatitis affects children from birth to 1.5 years, most often when transferred to artificial feeding. Later dates of the onset of the disease are an exception to the rule. This is a rare dermatitis with a severe progressive course. Typical is the localization of grouped blistering rash around natural openings: eyes, mouth, anus. Over time, the rash spreads to the folds of the skin: on the genitals, groin, thighs, popliteal pits, elbow bends, armpits.
The primary elements pour out against the background of hyperemia and edema, evolve into serous-purulent crusts, then into peeling plaques, which makes this pathology differentiate with psoriasis. The result of evolution is an erosive-ulcerative, poorly healing surface. In parallel, there is a lesion of nails and mucous membranes. The general well-being of the child is disturbed: due to developing dyspepsia, he loses weight; due to neuroprophic disorders, hair loss, eyelashes begins; due to eye damage (conjunctivitis, blepharitis) photophobia joins. The baby is lagging behind in development, the quality of his life is sharply deteriorating.
Pustular acrodermatitis
Pustular acrodermatitis has a different clinical picture. It develops at any age, mainly in men. Debuts with rashes on the skin of the fingers and toes, has no tendency to spread. Usually the lesion is limited to the distal parts of the extremities, sometimes – only one finger. Generalization of the process is extremely rare. As the name implies, the primary element is a pustule (a bubble with purulent contents). But there is also a vesicular variety of acrodermatitis (the primary element is a bubble with a transparent liquid inside), and an erythematous-squamous (“dry”, spotty-scaly) form.
In a typical clinic, the process begins with the fingertips, gradually moving to the back of the hands. At the same time, the nails are swollen, the periarticular bed is hyperemic, pus is released from under the nail plate when pressed. Rashes are accompanied by sharp soreness, difficulty to bend or unbend the fingers, so the brush is forced to take an unnatural half-bent shape. The fingers swell, the muscles of the hands are involved in the process, itching and burning are added. Fortunately, this form lends itself well to therapy. After the resolution of the pathological process, only slightly tense skin remains, which eventually returns to normal.
The abortive form of acrodermatitis has no tendency to aggravate the disease, is characterized exclusively by a pustular-vesicular rash often on one finger, regresses very quickly without a trace. The malignant variety of acrodermatitis Allopo is a purulent expansion of the disease with the seizure of the entire skin, accompanied by the loss of nails, their lysis, necrotizing of the surrounding tissues and, as a consequence, shortening of the phalanges. It is treated for a long time, with great difficulty. There is a risk of relapse.
Atrophic acrodermatitis
Atrophic acrodermatitis is an infectious process that occurs as a reaction of the skin to the toxins of the borreliosis spirochete that enters the human body with the saliva of a tick infected with borreliosis. Gender and age do not matter. There are two variants of the skin’s response to a tick bite: the immediate occurrence of erythema at the site of tick penetration into the dermis (sometimes this is the first and only symptom of the onset of the disease) and the development of acrodermatitis of the lower extremities after several years against the background of constant progression of the disease.
Clinically, acrodermatitis has the appearance of dark brown spots on the background of thin and atrophic skin. Over time, the spots thicken and thicken, and then atrophy with the outcome of dermatosclerosis. Over time, the malignancy of acrodermatitis is possible.
Diagnostics
Enteropathic acrodermatitis is diagnosed on the basis of clinical manifestations, biochemistry of blood, urine (zinc balance), consultation of a geneticist. It is differentiated with secondary zinc deficiency against the background of inflammatory processes in the gastrointestinal tract of the patient.
The diagnosis of pustular acrodermatitis is based on the results of histology: acanthosis with inflammatory neutrophil infiltration of the malpighian dermis layer is detected in the biopsy. Clinically differentiated with banal pyoderma, histologically – with exudative palmar-plantar psoriasis, During’s herpetiform dermatitis, impetigo Herba, Andrews’ pustulosis.
Atrophic acrodermatitis is confirmed by the release of the pathogen from the skin or serological reactions to the presence of antibodies to borrelious spirochetes in the patient’s blood.
Treatment
The timeliness and reliability of the diagnosis is very important for the effectiveness of therapy. It is necessary to correct the detected violations, eliminate the cause and strengthen the immune system. With a favorable scenario, the treatment of acrodermatitis gives good results. Radical cure is possible only at the beginning of therapy at the earliest stages of the disease and the benign course.
Enteropathic acrodermatitis is treated with zinc preparations to correct the level of this element in the child’s body (dietary supplements, vitamins, candles containing zinc) in combination with symptomatic therapy (vitamins C, E, A, probiotics, gamma or immunoglobulins, enzymes that improve the functioning of the gastrointestinal tract. Secondary infection is dealt with by the appointment of antimycotic agents, antibiotics inside and aniline paints externally. The fight against sepsis is a matter of extreme importance, since it is he who causes deaths.
Pustular acrodermatitis Allopo, depending on the severity of the course and the prevalence of the process, responds well to treatment with broad-spectrum antibiotics (fluoroquinolones) in combination with short courses of corticosteroids. Locally used aniline dyes, hormonal ointments, gels. PUVA therapy is indicated. Treatment of atrophic acrodermatitis pathogenetic – antibiotic therapy (macrolides-azalides per kilogram of weight). In parallel, borreliosis is treated with an emphasis on the therapy of the affected organ (heart, kidneys, joints).
Prognosis and prevention
Taking into account the achievements of modern dermatology, the prognosis of acrodermatitis is favorable, but depends on accurate diagnosis and timely treatment. Prevention is reduced to an attentive attitude to one’s health, regular observation by a dermatologist and maintaining a healthy lifestyle.
