Pigmented Purpuric Dermatoses (PPD) - Causes, Symptoms, and Treatment

Introduction:

Pigmented purpuric dermatoses are a rare chronic skin condition. It is characterized by small red, purple, or brown spots or patches on the skin. These spots or patches occur due to leakage of red blood cells from the small blood vessels into the skin. These red blood cells then break down, and pigment remains called hemosiderin. This pigment provides red to purple coloration to the skin. Generally, the condition is harmless and does not spread from one person to another. However, a persistent condition causes cosmetic concerns to the affected people. Treatment focuses on managing symptoms and improving the skin's appearance. The article discusses pigmented purpuric dermatoses, their causes, types, symptoms, diagnosis, and treatment.

What Are Pigmented Purpuric Dermatoses?

Pigmented purpuric dermatoses are chronic skin conditions that have a characteristic skin appearance. It causes red, brown, or purple stains on the skin, such as lower legs. It is also a pigmented purpura called capillaritis because of leaky blood vessels or capillaries. It is the inflammation of the capillaries, the body's smallest blood vessels. The tiny blood vessels exchange oxygen and nutrients between the blood and surrounding tissues. When these tiny blood vessels and capillaries become inflamed, they may leak into the surrounding tissues, causing the symptoms to develop. The examples of pigmented purpuric dermatoses are as follows:

• Schamberg disease (progressive pigmented purpura) is the most common type of pigmented purpuric dermatosis. It is characterized by brownish-red patches with a pepper-like appearance. Schamberg disease can affect people at any age, but it is commonly seen in the late teens and early 20s. The condition is mainly diagnosed in children between the ages of one and nine. It is more common in males than females.

• Majocchi’s disease (purpura annularis telangiectodes) is characterized by purple or bluish patches with petechiae (tiny spots of bleeding under the skin). It mostly affects children and young adults. Girls are more commonly affected than boys. The symmetrical distribution of patches or plaques occurs on the lower limbs but may occasionally occur on the trunk and upper limbs. It is a chronic relapsing condition that may last for several years.

• Gougerot-blum syndrome (pigmented purpuric lichenoid dermatitis) is a rust-colored lichenoid papules skin condition. It usually affects middle-aged men and is not seen in children.

• Lichen aureus (lichen purpuric) is a skin condition characterized by rust-colored macules. It mostly affects young adults and rarely affects children. The lesion may occur bilaterally on the lower limbs and unilaterally on the trunk and upper extremities. The lesions in this type follow the distribution of veins and arteries. The condition is associated with trauma and not associated with drug ingestion. Oral corticosteroids may clear the lesion.

• Ducas and Kapetanakis pigmented purpura (eczematoid or itching purpura) is a skin condition characterized by scaly patches. It predominantly occurs in adult males. The lesions associated with the condition start on the lower legs and progress to the thighs, trunk, and upper extremities. It is a chronic relapsing disorder that remits spontaneously.

What Are the Causes of Pigmented Purpuric Dermatoses?

The exact cause of pigmented purpuric dermatoses is unknown. The condition can affect anyone; males are more commonly affected than females, and it rarely affects children. It can also affect healthy people. The causes of pigmented purpuric dermatoses are as follows:

• Venous Stasis: The condition is associated with poor blood flow in the veins, mainly in the lower legs. It is caused due to other medical conditions, such as varicose veins (enlarged veins) or prolonged standing.

• Injury: Due to physical trauma, inflammatory reactions to the skin can trigger the condition. This can occur due to allergies or vigorous exercise.

• Medications: Certain medications, such as corticosteroids, non-steroidal anti-inflammatory agents, sedatives, antihistaminic drugs, and antihypertensive drugs, can cause skin changes.

• Chemicals: Certain chemicals or food additives are responsible for causing the condition.

What Are the Signs and Symptoms of Pigmented Purpuric Dermatoses?

Pigmented purpuric dermatoses are characterized by small red or brown spots called cayenne pepper spots. These spots can be scattered all over the body or in clusters to form a flat red patch. This red patch changes into a brown color with time, fading away over weeks and months. The signs and symptoms of pigmented purpuric dermatoses are as follows:

• Schamberg Disease: It is characterized by regular or irregular red-brown flat patches. The cayenne pepper spots are present on the borders. It is usually present on the lower legs but can appear anywhere on the body. The condition is usually bilateral, and there are no associated symptoms.

• Gougerot-Blum Purpura: It is a less common type of pigmented purpuric dermatoses. It is characterized by thick and itchy patches similar to eczema (itchy patches on the skin).

• Itching Purpura: The clinical features are similar to those of Schamberg disease. In addition, there is itchiness over the patches, followed by scratching. Reddish-purple macules will appear around the ankles, which may involve the legs. The commonly affected locations include the trunk and upper extremities.

• Majocchi Purpura: The blood vessels or capillaries are dilated with brown patches or cayenne spots in this condition. The patches are annular, gradually coming outwards and forming concentric circles.

• Lichen Aureus: The patch is persistent and single brown-yellow colored. It often lies on the top of varicose veins.

• Exercise-Induced PPD: Vigorous exercise can cause spots on the ankles to form that fade to brown within a few days. After some years, these spots disappear. The affected person may experience a burning sensation. The lesion recurs regularly after exercise.

How Are Pigmented Purpuric Dermatoses Diagnosed?

There is no specific diagnostic test for the condition. Routine laboratory tests, such as clotting studies and erythrocyte sedimentation rate (ESR), can show some abnormalities, which will help exclude other medical conditions. In addition, skin biopsies are performed, which will differentiate the condition from other cancerous or non-cancerous skin lesions.

How Are Pigmented Purpuric Dermatoses Treated?

The treatment strategies for pigmented purpuric dermatoses are as follows:

Non-Pharmacological Treatment:

• The person should avoid prolonged standing or work requiring prolonged leg dependency. The person can wear a compression elastic hose to reduce exercise-induced pigmented purpuric dermatoses.

• In the case of venous stasis, compression is beneficial.

• Counseling may benefit adolescents and young adults.

• Treatment with Psoralen combined with ultraviolet A (PUVA) may benefit the affected individuals.

• The affected person should avoid food preservatives and artificial coloring agents.

Pharmacological Treatment:

• The healthcare provider may prescribe steroids to clear the lesions.

• In addition, antihistaminic drugs may provide relief from itching pruritus.

• Furthermore, topical steroid creams can provide symptomatic relief.

• In the case of lichen aureus, the healthcare provider may prescribe topical one percent Pimecrolimus cream.

Conclusion:

All forms of pigmented purpuric dermatoses are chronic and relapsing. They are not associated with any physical deformity. Generally, PPD can disappear within a few weeks, recur from time to time, or persist for several years. Pharmacological and non-pharmacological treatment strategies can manage the condition. The person with red or brown spots should consult a dermatologist to differentiate the condition from other medical conditions.