Purpura chronica progressiva is a group of diseases that causes punctiform skin lesions due to bleeding and hemosiderin deposits. Food additives, textile additives, medicines and various primary diseases have been identified as the cause. The skin disease is treated depending on the cause.
What is purpura chronica progressivea?
Iron is an essential trace element that is essential for the blood, skin and nervous system. Hemosiderin is an iron storage complex. This storage iron is considered a protein complex made up of iron, ferritin and other proteins. According to current knowledge, hemosiderin is a phagocytic degradation product of erythrocytes and hemoglobin. In the case of hemosiderosis, there is an increased deposit of the iron-containing compound hemosiderin as a result of an increased iron blood level. See topbbacolleges for Definitions of Cat Eye Syndrome.
Purpura chronica progressiva is a group of diseases associated with small punctiform bleeding and secondary hemosiderosis. The condition is also known as Schamberg’s disease, Schamberg’s syndrome or Schamberg’s dermatitis. Equally common is the term purpura pubis. Other subtypes of the disease are:
- Dermatosis pigmentaria progressive
- Progressive pigmentary purpura
- Progressive pigmentary dermatosis
- Capillaritis haemorrhagica maculosa
- adaline purpura.
The same applies to Majocchi disease or Majocchi syndrome and purpura annularis telangiectodes. Punctual hemorrhages with iron deposits may correspond equally well to eczematid-like purpura, carbromal rash, carbamide purpura, or essential familial telangiectasia.
Causes
The etiology of purpura chronica progressiva has not been finally elucidated. In the past, the disease was often associated with adaline and was therefore referred to as adaline purpura. In addition to adaline, various other substances have been discussed as triggers for the inflammatory and relapsing, haemorrhagic-pigmentary skin diseases.
Drugs such as benzodiazepines or meprobamate, for example, were often taken as part of the illness. Food additives also seem to be associated with skin reactions. The same applies to house dust and the dyes and bleaches in cotton textiles. Clothing that is too tight can trigger a reaction in this context. Symptoms from the group of purpura chronica progressiva have also been observed in the context of various primary diseases.
For example, the skin reactions were associated with liver diseases or as a symptom of cryoglobulinemia type III. Chronic venous insufficiency has also been causally linked to purpura chronica progressiva. Presumably, depending on the respective sub-form of the disease, various of the causes mentioned are involved.
Symptoms, Ailments & Signs
Purpura chronica progressiva usually progresses in phases and is clinically extremely variable. Depending on the acuteness and the frequency of relapses, the diseases manifest themselves as 0.3 to 20 centimeters in size, diffusely limited and asymptomatic yellow, yellow-brown or red-brown spots. The distal lower leg is usually affected in particular. In addition to the lower leg, however, the abdomen, thigh or arm of the patient can also be affected by the lesions.
In most cases, the skin lesions are smooth and atrophic. In some cases, however, they also have fine lamellar scales and thus give an eczematous impression. Immediately after bleeding, the skin lesions are usually light red in color. Only after hemosiderin storage do the lesions change color and become ocher. Depending on the particular primary cause of the skin reactions, numerous accompanying symptoms can be present.
Since the punctiform spots are usually reactions caused by inflammation, the affected skin areas are sometimes warmer than the surrounding area. Under certain circumstances, there may be a slight fever accompanying the inflammation.
Diagnosis & course of disease
The visual diagnosis can arouse the first suspicion of purpura chronica progressiva in a doctor. The differential diagnosis turns out to be difficult. Other purpuras should be considered in the differential diagnosis. All multiple, small-spotted capillary hemorrhages, which are either punctiform or strip-shaped, are to be understood as purpura. There are numerous small-area and extensive purpuras, which under certain circumstances can hardly or not at all be distinguished from purpura chronica progressivea.
To determine the cause of the skin lesions, the medical history is the most important tool. From the medical history, the doctor could discover, for example, indications of a dietary supplement, medication or certain clothing additives as the cause of the punctate skin changes. In the histology, the changes in purpura chronica progressiva correspond to a sparse, band-shaped and mostly subepidermal, lymphohistiocytic infiltrate.
In some forms, hemosiderin deposits are also present in the dermis. In the Gougerot-Blum type, degeneration of the basal epithelial cells is evident. The course of the disease depends on the cause and subtype. The Majocchi form, for example, often heals spontaneously, but relapses that last for years are also conceivable.
Complications
Due to purpura chronica progressiva, the patients primarily suffer from various skin complaints and, above all, from bleeding. However, the further course of this disease depends very much on its cause, so that a general prognosis cannot be given. The patient’s skin is affected by local spots that can spread to different regions of the body.
The spots themselves reduce the aesthetics and can thus lead to inferiority complexes or reduced self-esteem in the patient. The skin can also flake or be affected by itching. However, the symptoms do not occur permanently, but in phases. Purpura chronica progressiva can also lead to fever and continue to restrict and complicate the patient’s everyday life.
The treatment of purpura chronica progressiva primarily depends on the cause of the disease. In the case of intolerance or side effects of medication, these must be discontinued or changed. Treatment with the help of care products is also possible and significantly reduces the symptoms. As a rule, purpura chronica progressiva leads to a positive course of the disease without complications.
When should you go to the doctor?
Purpura chronica progressiva should always be treated by a doctor. This disease does not heal itself and usually also leads to a deterioration in the general condition. For this reason, medical treatment of purpura chronica progressiva is essential. A doctor should be consulted for purpura chronica progressiva if the person concerned suffers from spots on the body. The spots themselves can cover the entire body but are relatively small.
Usually they are brown or yellow. Furthermore, very scaly skin can indicate the disease and should be examined by a doctor if the complaint occurs permanently and does not go away on its own. It is not uncommon for the symptoms of purpura chronica progressiva to be accompanied by fever.
The disease is primarily treated by a dermatologist. The course of the disease is usually positive and there are no further complications. The patient’s life expectancy is also not negatively influenced by purpura chronica progressiva.
Treatment & Therapy
The causal therapy of purpura chronica progressiva depends on the respective trigger. Therefore, determining the trigger is already the most important step in therapy. In the case of medication-related symptoms, the medication is discontinued. However, it may take several months for the lesions to subside after discontinuation. If the lesions are itchy or burning, symptomatic therapy is given with external therapeutics and compression treatments.
A cooling Lotio alba or an ethanolic zinc oxide shaking mixture may be indicated. If the lesions are asymptomatic, external agents containing glucocorticoids are usually given at intervals. Hydrocortisone and prednicarbate come into consideration as such. In extreme cases, internal therapy takes place. Systemic glucocorticoids are usually administered for this purpose. Depending on the clinical findings, the dose is gradually reduced again until the medication is completely discontinued.
In order to stop the bleeding, vaso-tightening medication can also be given as a test. In most cases, this step of the inner therapy corresponds to the administration of vitamin or rutoside. If the lesions spread to the trunk, the disease is treated with PUVA and thus long-wave UV light and psoralens.
Prevention
If necessary, purpura chronica progressiva can be prevented by avoiding the causative medication, food additives and textile additives. Purpura chronica progressiva in the context of certain primary diseases can be prevented by taking preventive measures for the corresponding diseases.
Aftercare
Promising follow-up care for purpura chronica progressiva depends on the cause. If it is a drug-induced form, the skin will return to its healthy normal state once the drug in question is discontinued. The same applies to food intolerance and food additives.
In the future, the use of the triggering ingredient must be avoided here. If it is a chronic, recurrent form without a clearly identifiable cause, treatment with UV light can be promising in follow-up care. A sufficient supply of the antioxidant vitamin C is also an option, and the flavonoid rutin should also be mentioned here, both of which are very well tolerated.
An ointment or lotion containing zinc oxide can help to relieve any itching that may occur. The patient often suffers enormously because it is also an aesthetic problem. A medication containing corticosteroids is then often used as therapy and also to counteract a recurrence, whereby the side effects of a corticosteroid must be weighed against the effects of the dermatosis.
In the aftercare of such a treatment, it is important to counteract these side effects with appropriate care. This can be done using an ointment or lotion containing urea. In principle, tight-fitting or compressive clothing should be avoided.
