In the practice of any doctor, the issue of timely diagnosis of any disease is acute. Although some neutrophilic dermatoses can resolve spontaneously, in most cases treatment is required to achieve remission. Delays in diagnosis, due to the heterogeneity of morphological elements in patients with neutrophilic dermatosis, lead to an increase in morbidity among the population and even to deaths. In addition, differential diagnosis is also difficult due to the large number of infectious and inflammatory diseases that mimic neutrophilic dermatoses. At estet-portal.com you will learn more about the diagnosis and treatment of neutrophilic dermatoses.
- Neutrophilic dermatoses, pathogenesis
- Sweet's syndrome - acute febrile neutrophilic dermatosis, clinic, diagnosis, treatment
- Behçet's disease, characteristic features and assessment criteria
- Doctor's tactics when making a diagnosis of neutrophilic dermatosis
Neutrophilic dermatoses, pathogenesis
Neutrophilic dermatoses are a heterogeneous group of cutaneous inflammatory disorders characterized by a sterile, predominantly neutrophilic infiltrate on histopathology. The term "neutrophilic dermatosis" used as an umbrella term to describe primarily epidermal or dermal processes, with various features of primary or secondary vasculitic lesions.
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The study of the pathogenesis of neutrophilic dermatoses has been carried out since the 1930s, so far 3 contributing factors in the pathogenesis of these diseases have been proven:
- altered expression of inflammatory effector molecules;
- abnormal functioning of neutrophils;
- genetic predisposition.
Granulocyte colony stimulating factor (G-CSF) and hematopoietic growth factor, which regulates neutrophil production and differentiation, have been shown to be elevated in the active phase of neutrophilic dermatoses and correlate with disease activity.
In addition to colony-stimulating factors, studies have revealed altered expression of other cytokines such as interleukin 1a, interleukin 1b, interleukin 2, and interferon g.
In the absence of infection, the trigger for the altered expression of inflammatory effector molecules in neutrophilic dermatoses remains not fully understood.
Sweet's syndrome - acute febrile neutrophilic dermatosis, clinic, diagnosis, treatment
In 1964, Dr. Robert Douglas Sweet coined the term "acute febrile neutrophilic dermatosis"; to describe 8 women aged 32 to 55 who presented with fever, leukocytosis, painful plaques, and a dense cutaneous neutrophilic infiltrate on histopathology. All of them lacked an infectious agent, but all patients responded to systemic corticosteroid therapy.
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Research on the comorbidities and medications associated with Sweet's syndrome has led to its classification, which includes:
- Classic type,
- associated with malignant neoplasms
- drug-induced
Classic Sweet's syndrome is an idiopathic dermatosis associated with infection, vaccination, inflammatory disorders, or pregnancy.
Sudden appearance of painful erythematous plaques or nodules with predominantly neutrophilic infiltrates in the dermis.
Age, vesiculobullous morphology, leukopenia, anemia, thrombocytopenia, elevated erythrocyte sedimentation rate, absence of arthralgia, and subcutaneous histopathology are the main clinical and pathological characteristics of the disease. One study reported that Sweet's syndrome is a prognostic marker for myelodysplasia. Several studies have also confirmed the relationship between this dermatosis and hematological disorders.
Sweet's syndrome is an exception, patients with suspected Sweet's syndrome should undergo a thorough history and physical examination, tissue samples for sterile skin biopsy are a mandatory criterion for diagnosis. Further evaluation should also be done to rule out alternative diagnoses and identify any comorbidities or drug reactions.
Clinical, histological and laboratory findings are non-specific and cannot be considered in isolation to make a definitive diagnosis.
Treatment of Sweet's syndrome
There are currently no generally accepted and approved recommendations for the treatment of Sweet's syndrome. The disease may resolve spontaneously or with treatment of the comorbid condition, however targeted therapy is often necessary. Retrospective studies have shown a recurrence rate of 15% to 45% at intervals of up to 10 years. Systemic corticosteroids are considered the standard of care for Sweet's syndrome. For patients in whom corticosteroids are contraindicated, the administration of such active substances as
colchicine, potassium iodide, dapsone is justified. Behçet's disease, characteristic features and assessment criteria
Behçet's disease is a chronic, systemic, inflammatory disease that manifests itself in the skin, mucous membranes, cardiovascular system, gastrointestinal tract, central nervous system, and urogenital system.
This pathology is most common among the inhabitants of the "Great Silk Road".
Strong regional variability in the expression of Behçet's disease is closely correlated with the geographical distribution of the HLA-B51 gene, which along the Great Silk Road ranges from 20% to 25% in the general population and from 50% to 80% among patients with this pathology.
Clinical signs
Behcet's syndrome is characterized by the following triad of recurring symptoms:
recurrent oral mucosal ulcers,
- genital ulcers,
- uveitis.
- The primary elements are erythematous papules or vesiculopustules, which develop into oval ulcers with twisted wounds and a greyish-yellow necrotic base surrounded by an erythematous halo.
How drugs affect impaired vision The diagnosis of Behçet's syndrome is based on the following criteria:
Recurrent oral ulceration: minor aphthous, large aphthous or herpetiform ulceration observed by a clinician or patient that recurred 3 times within a single 12-month period
- Recurrent genital ulceration: aphthous ulceration
- or scarring observed by a physician or patient
- Eye lesions: anterior uveitis, posterior uveitis, or cells in the vitreous on examination with a lamp;
- retinal vasculitis
- Skin lesions: erythema nodosum observed by the physician or patient, pseudofolliculitis or papulopustular lesions; acne nodules observed by a physician in post-adolescent patients
- Positive pathergy test
- Genital aphthae(2 points)
- Oral aphthous stomatitis (2 points)
- Skin lesions (1 point)
- Neurological manifestations (1 point)
- Vascular manifestations (1 point)
- Positive pathergy test
- One major and 2 minor criteria are required to confirm the diagnosis, in the absence of other clinical explanations.
Scoring system :
A score of 4 or higher indicates confirmation of the diagnosis of Behçet's disease.
Read also:Behçet's disease, the importance of timely diagnosis Treatment of Behçet's disease
In the case of oral and genital ulcers, the use of topical corticosteroids, topical anesthetics (gel, aerosol) and antiseptic agents such as chlorhexidine is recommended. Recent studies have shown the effectiveness of carbon laser and pimecrolimus cream in the treatment of Behçet's disease. Systemic therapy is prescribed in severe cases, colchicine is the first line drug in the treatment of this pathology. Other systemic agents are TNF antagonists, thalidomide,
interferon, minocycline. Doctor's tactics when making a diagnosis of neutrophilic dermatosis
It doesn't matter if you work in a dermatovenereological dispensary or in a cosmetology office - the responsibility for the patient's health lies with you. Neutrophilic dermatoses are not so common, but they definitely require careful diagnosis and additional studies, since the generalization of the process can threaten the patient's life. For prevention of complications from other organs and systems, it is required to recognize neutrophilic dermatosis as soon as possible, because the correct diagnosis is already a huge success for further therapy.
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