Purpura chronica progressiva is a group of diseases that cause punctate skin lesions due to hemorrhage and hemosiderin deposition. Food additives, textile additives, medications and various primary diseases have been identified as causes. Therapy of the skin disease is cause-dependent.
What is purpura chronica progressiva?
Iron is an essential trace element that is indispensable for the blood, skin, and nervous system. Hemosiderin is a storage complex of iron. This storage iron is considered a protein complex of iron, ferritin and other proteins. According to current findings, hemosiderin is a phagocytic degradation product of erythrocytes and hemoglobin. In hemosiderosis, increased deposition of the iron-containing compound hemosiderin is present as a consequence of elevated iron blood levels. Purpura chronica progressiva is a group of diseases associated with punctate microhemorrhages and secondary hemosiderosis. The condition is also known as Schamberg’s disease, Schamberg’s syndrome, or Schamberg’s dermatitis. The term Purpura Schamberg is equally common. Other subtypes of the disease include:
- Dermatosis pigmentaria progressiva
- Progressive pigmentary purpura
- Progressive pigmentary dermatosis
- Capillaritis haemorrhagica maculosa
- Adalin purpura.
The same is true for Majocchi disease or Majocchi syndrome and purpura anularis teleangiectodes. Pinpoint hemorrhages with iron deposition 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 definitively determined. Often in the past, the condition was associated with adaline and therefore referred to as adaline purpura. In addition to adalin, various other substances have been discussed as triggers for the inflammatory and episodic hemorrhagic pigmentary skin disease. Drugs such as benzodiazepines or meprobamate, for example, have often been taken in the context of the disorders. Food additives also seem to be related to the skin reactions. The same applies to house dust and the dyeing and bleaching agents in cotton textiles. Especially too tight-fitting clothing can trigger a reaction in this context. Appearances from the group of purpura chronica progressiva have also been observed in the context of various primary diseases. For example, the skin reactions appeared in the context of liver diseases or as a symptom of cryoglobulinemia type III. Chronic venous insufficiency was also causally associated with purpura chronica progressiva. Presumably, several of the above causes are involved depending on the particular subtype of the disease.
Symptoms, complaints, and signs
Purpura chronica progressiva usually progresses in episodes and is extremely variable clinically. Depending on the acuity and frequency of relapses, the disease manifests as 0.3 to 20 centimeters in size, diffusely circumscribed, and asymptomatic patches of yellow, yellow-brown, or red-brown color. As a rule, the distal lower leg is mainly affected. However, in addition to the lower leg, the patient’s abdomen, thigh, or arm may also be affected by the lesions. In most cases, the skin lesions are smooth and atrophic. In some cases, however, they are finely lamellar scaled, giving an eczematous impression. Immediately after hemorrhage, the skin lesions usually have a light reddish color. Only after hemosiderin incorporation do the lesions change color and become ocher. Depending on the particular primary cause of the skin reactions, numerous accompanying symptoms may be present. Since the punctate patches are usually inflammatory reactions, the affected skin areas are sometimes warmer than the surrounding area. In some circumstances, mild fever may accompany inflammation.
Diagnosis and course of the disease
The visual diagnosis may raise a physician’s initial suspicion of purpura chronica progressiva. Differential diagnosis turns out to be difficult. Other purpura should be considered for differential diagnosis.Purpura is defined as all multiple, small-spotted capillary hemorrhages that are either punctate or striate. There are numerous small-area and areal purpura, which may be difficult or impossible to differentiate from purpura chronica progressiva. To determine the cause of the skin lesions, the history is the most important tool. Anamnetically, the physician might discover, for example, evidence of dietary supplementation, medications, or certain clothing additives as the cause of the punctate skin changes. Histologically, the changes in purpura chronica progressiva correspond to sparse, band-like and usually 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. For example, the Majocchi form often heals spontaneously, but relapses lasting for years are also possible.
Complications
Because of purpura chronica progressiva, patients primarily suffer from various skin complaints and, in particular, from hemorrhages. However, the further course of this disease depends very much on its cause, so a general prognosis cannot be given. The patient’s skin is affected by localized spots, which can spread to different regions of the body. The spots themselves reduce the aesthetics and can lead to inferiority complexes or a lowered self-esteem in the patient. The skin may also flake or be affected by itching. However, the symptoms do not occur permanently, but in episodes. Purpura chronica progressiva can also lead to fever and continue to restrict and complicate the patient’s daily life. The treatment of purpura chronica progressiva depends primarily on the cause of the disease. In the case of intolerance or side effects of medications, these must be discontinued or changed. Treatment with the help of care products is also possible and significantly limits the symptoms. As a rule, purpura chronica progressiva has a positive course of the disease without complications.
When should one go to the doctor?
Purpura chronica progressiva should always be treated by a physician. There is no self-healing in this disease, and there is usually a worsening of 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 affected person suffers from spots on the body. The spots themselves can cover the entire body, but they are relatively small. As a rule, they are brown or yellow. Furthermore, very scaly skin may also indicate the disease and should be examined by a doctor if the complaint is permanent and does not disappear on its own. It is not uncommon for the symptoms of purpura chronica progressiva to be accompanied by fever. In the first instance, the disease is treated by a dermatologist. There is usually a positive course of the disease and no further complications. The patient’s life expectancy is also not negatively affected by purpura chronica progressiva.
Treatment and therapy
The causative therapy of purpura chronica progressiva depends on the particular trigger. Therefore, determination of the trigger is already the most important therapeutic step. In the case of drug-induced manifestations, the drugs are discontinued. However, it may take several months for the lesions to subside after discontinuation. If the lesions itch or burn, symptomatic therapy with external therapeutics and compression treatments is given. Provision of a cooling lotio alba or an ethanolic zinc oxide shake mixture may be indicated. If the lesions are asymptomatic, glucocorticoid-containing externals are usually given intermittently. Hydrocortisone and prednicarbate may be considered as such. In extreme cases, internal therapy takes place. For this purpose, systemic glucocorticoids are usually administered. The dose is gradually reduced again depending on the clinical findings until the drugs are fully discontinued. In order to stop the bleeding, vasoconstrictive drugs can also be given on a trial basis.This step of internal therapy corresponds in most cases to the administration of vitamin or rutoside. If the lesions spread to the trunk of the body, the disease is treated by PUVA and thus long-wave UV light and psoralenes.
Prevention
Purpura chronica progressiva can be prevented, if necessary, by avoiding causative medications, food additives, and textile additives. Purpura chronica progressiva in the setting of certain primary diseases can be prevented by taking preventive measures for the corresponding diseases.
Aftercare
Promising aftercare of purpura chronica progressiva depends on the cause. If it is a drug-induced form, the skin will return to its normal healthy state after discontinuation of the corresponding drug. The same applies to food intolerances and food additives. Here, the use of the triggering ingredient must be avoided in the future. If it is a chronic recurrent form without a clearly identifiable cause, a treatment with UV light can be promising in the follow-up. An adequate 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. To alleviate any itching that may occur, an ointment or lotion containing zinc oxide can provide relief. Often the patient suffers enormously, since it is also an aesthetic problem. Often, a corticosteroid-containing medication is used as a 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 by means of an ointment or lotion containing urea. In principle, tight-fitting or compressive clothing should be avoided.
