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Pigmented Purpuric Dermatosis

Updated : September 4, 2023





Background

The term pigmented purpuric dermatosis (PPD) is used to refer to a variety of diverse subtypes of persistent, benign purpuric inflamed skin. Clinically, they are identified by petechiae accompanied by erythrocyte extravasation & hemosiderin depositing in the skin, which can cause a red brown to golden-brown color as the hemosiderin is resorbed.

They are further distinguished by red to purple pustules & patches. Although they can occasionally affect the arms here, too, these lesions more frequently affect the lower appendages. Even while pigmentation purpuric dermatitis frequently has no symptoms, it can occasionally be linked to minor itching. Treatment can be difficult, but it’s important to reassure patients that these illnesses are benign.

 

Epidemiology

Rare cases of pigment purpuric dermatitis. There isn’t any racial preference, despite the fact that males experience them slightly more frequently. Children may be impacted.

 

Anatomy

 

 

Pathophysiology

Capillaritis in the epidermis, coupled with probable concurrent venous hypertension, causes endothelium malfunction & RBC extravasation in pigment purpuric dermatitis.

The clinical manifestation of these red blood cell deposits, which take the form of purpuric patches & macules with various configurations, occurs in the dermis.

As the hemosiderin is reabsorbed over time, the skin develops a golden-brown coloring that eventually disappears. However, the illness might frequently flare up and last for a long time.

 

Etiology

Idiopathic pigment purpuric dermatosis is the most common kind. It is crucial to understand that coagulopathies and thrombocytopenia are not linked to these illnesses. Vein obstruction, exercising, & capillaries fragility are significant underlying variables, though, since the majority of cases affect the lower body. These conditions cause red blood cell extravasation into the skin, which results in a distinctive purpuric color.

Cell-mediated response is also involved in the onset of these illnesses, and it may lead to vascular instability because of the inflammatory process of macrophages, Langerhans, and lymphocyte cells that are frequently shown on biopsy specimens as capillaritis. Perivascular infiltration of lymphocytes and dendritic cells has been identified in immunohistochemical analyses of Schamberg syndrome, a form of pigment purpuric dermatitis that interacts with vascular endothelium & affects the permeation of the microvasculature.

Immunoglobulin & complement accumulation around skin vessels have been seen in some instances, suggesting that humoral defense may be implicated in the etiology. Additionally, a large number of drugs have been implicated in the development of pigment purpuric dermatoses, such as chlordiazepoxide, aspirin, acetaminophen, diltiazem, carbamazepine, furosemide, dipyridamole, glipizide, infliximab, hydralazine, interferon-alpha, and medroxyprogesterone.

 

Genetics

Prognostic Factors

The course of pigment purpuric dermatitis is frequently chronic and relapsing-remitting. The condition is benign and frequently asymptomatic, nevertheless.

Even if the capillaritis gets better and the inflammation stops being active, the dermal hemosiderin accumulation that results can take months or years to gradually go away.

 

Clinical History

Patients express dissatisfaction with the state of their skin. Uneven plaques and patches of orange-brown pigmentation appear on the lower limbs in Schamberg disease. The lesions are persistent and chronic.

Some of the lesions may spontaneously clear up over time, but many of them tend to spread and possibly turn a darker shade of brown. Lesions with itchy purpura are much larger, and patients frequently express extreme pruritis.

Physical Examination

A pigmented purpuric dermatosis is recognized by its distinctive orange-brown, specked coloring that resembles cayenne pepper. Schamberg disease affects the lower limbs, whereas itchy purpura has more widespread skin involvement. The leg is the most frequently affected portion of the body when lichen aureus is present. The eruption typically appears as a single golden-brown lesion or as a small cluster of lesions.

Lichen aureus has been observed in linear or segmental forms. Small purpuric plaques with significant telangiectasias, called annular plaques, are the hallmark of Majocchi disease. Yet another clinical variation of pigmented purpura with lichenoid-type skin changes was initially described by Gougerot and Blum. When red-brown lichenoid papules are present, the lesions resemble those of Schamberg disease.

Age group

Associated comorbidity

Associated activity

Acuity of presentation

Differential Diagnoses

  • Vasculitis
  • Scurvy
  • Drug Eruptions
  • Urticarial Vasculitis

Laboratory Studies

 

 

Imaging Studies

 

 

Procedures

 

 

Histologic Findings

 

 

Staging

 

 

Treatment Paradigm

Treatment of pigment purpuric dermatitis might be difficult. If a pharmaceutical reaction is to blame, stopping the medicine should cause the eruption to go away. Compression stockings are advised due to the potential causes of venous stasis & vascular hypertension in the extremities. In a study conducted on three individuals with pigment purpuric dermatitis, the bioflavonoid rutoside 50 mg twice a day and ascorbic acid 500 mg twice daily cleared up the outbreak after four weeks of treatment.

It seems sensible to use this as the first line of treatment, given the effectiveness of these supplements. Topical corticosteroids of medium to high potencies, such as triamcinolone, can also be utilized and may help with the pruritus & inflammatory aspect of this illness. The adverse reactions profile of these drugs, as well as the benign nature of pigment purpuric dermatitis, may not support their usage, despite the fact that systemic immunosuppressive with cyclosporine or corticosteroids can be beneficial.

However, when immunosuppression is stopped, the disease frequently returns. Topical pimecrolimus and tacrolimus can be helpful for long-term use because they reduce the chance that topical corticosteroids will cause cutaneous atrophy. Six patients with pigment purpuric dermatitis responded better to griseofulvin 500–750 mg daily. With colchicine 0.5 mg twice daily, minocycline, pentoxifylline, and methotrexate 400 mg three times daily, patients with pigment purpuric dermatitis improved in other case series.

Narrowband UVB or PUVA ultraviolet phototherapy has also been shown to have advantages. Withholding treatment in some cases caused the condition to flare up, but in other cases, a lasting remission was attained. Improved pigment purpuric dermatitis has also been reported following topical photodynamic treatment utilizing methyl aminolevulinic acid and 5-aminolevulinic acid as photosensitizing medications.

 

by Stage

 

 

by Modality

 

 

Chemotherapy

 

 

Radiation Therapy

 

 

Surgical Interventions

 

 

Hormone Therapy

 

 

Immunotherapy

 

 

Hyperthermia

 

 

 

 

Photodynamic Therapy

 

 

Stem Cell Transplant

 

 

Targeted Therapy

 

 

Palliative Care

 

 

Medication

 

 

Media Gallary

References

https://www.ncbi.nlm.nih.gov/books/NBK519562/

 

Pigmented Purpuric Dermatosis

Updated : September 4, 2023




The term pigmented purpuric dermatosis (PPD) is used to refer to a variety of diverse subtypes of persistent, benign purpuric inflamed skin. Clinically, they are identified by petechiae accompanied by erythrocyte extravasation & hemosiderin depositing in the skin, which can cause a red brown to golden-brown color as the hemosiderin is resorbed.

They are further distinguished by red to purple pustules & patches. Although they can occasionally affect the arms here, too, these lesions more frequently affect the lower appendages. Even while pigmentation purpuric dermatitis frequently has no symptoms, it can occasionally be linked to minor itching. Treatment can be difficult, but it’s important to reassure patients that these illnesses are benign.

 

Rare cases of pigment purpuric dermatitis. There isn’t any racial preference, despite the fact that males experience them slightly more frequently. Children may be impacted.

 

 

 

Capillaritis in the epidermis, coupled with probable concurrent venous hypertension, causes endothelium malfunction & RBC extravasation in pigment purpuric dermatitis.

The clinical manifestation of these red blood cell deposits, which take the form of purpuric patches & macules with various configurations, occurs in the dermis.

As the hemosiderin is reabsorbed over time, the skin develops a golden-brown coloring that eventually disappears. However, the illness might frequently flare up and last for a long time.

 

Idiopathic pigment purpuric dermatosis is the most common kind. It is crucial to understand that coagulopathies and thrombocytopenia are not linked to these illnesses. Vein obstruction, exercising, & capillaries fragility are significant underlying variables, though, since the majority of cases affect the lower body. These conditions cause red blood cell extravasation into the skin, which results in a distinctive purpuric color.

Cell-mediated response is also involved in the onset of these illnesses, and it may lead to vascular instability because of the inflammatory process of macrophages, Langerhans, and lymphocyte cells that are frequently shown on biopsy specimens as capillaritis. Perivascular infiltration of lymphocytes and dendritic cells has been identified in immunohistochemical analyses of Schamberg syndrome, a form of pigment purpuric dermatitis that interacts with vascular endothelium & affects the permeation of the microvasculature.

Immunoglobulin & complement accumulation around skin vessels have been seen in some instances, suggesting that humoral defense may be implicated in the etiology. Additionally, a large number of drugs have been implicated in the development of pigment purpuric dermatoses, such as chlordiazepoxide, aspirin, acetaminophen, diltiazem, carbamazepine, furosemide, dipyridamole, glipizide, infliximab, hydralazine, interferon-alpha, and medroxyprogesterone.

 

The course of pigment purpuric dermatitis is frequently chronic and relapsing-remitting. The condition is benign and frequently asymptomatic, nevertheless.

Even if the capillaritis gets better and the inflammation stops being active, the dermal hemosiderin accumulation that results can take months or years to gradually go away.

 

Patients express dissatisfaction with the state of their skin. Uneven plaques and patches of orange-brown pigmentation appear on the lower limbs in Schamberg disease. The lesions are persistent and chronic.

Some of the lesions may spontaneously clear up over time, but many of them tend to spread and possibly turn a darker shade of brown. Lesions with itchy purpura are much larger, and patients frequently express extreme pruritis.

A pigmented purpuric dermatosis is recognized by its distinctive orange-brown, specked coloring that resembles cayenne pepper. Schamberg disease affects the lower limbs, whereas itchy purpura has more widespread skin involvement. The leg is the most frequently affected portion of the body when lichen aureus is present. The eruption typically appears as a single golden-brown lesion or as a small cluster of lesions.

Lichen aureus has been observed in linear or segmental forms. Small purpuric plaques with significant telangiectasias, called annular plaques, are the hallmark of Majocchi disease. Yet another clinical variation of pigmented purpura with lichenoid-type skin changes was initially described by Gougerot and Blum. When red-brown lichenoid papules are present, the lesions resemble those of Schamberg disease.

  • Vasculitis
  • Scurvy
  • Drug Eruptions
  • Urticarial Vasculitis

 

 

 

 

 

 

 

 

 

 

Treatment of pigment purpuric dermatitis might be difficult. If a pharmaceutical reaction is to blame, stopping the medicine should cause the eruption to go away. Compression stockings are advised due to the potential causes of venous stasis & vascular hypertension in the extremities. In a study conducted on three individuals with pigment purpuric dermatitis, the bioflavonoid rutoside 50 mg twice a day and ascorbic acid 500 mg twice daily cleared up the outbreak after four weeks of treatment.

It seems sensible to use this as the first line of treatment, given the effectiveness of these supplements. Topical corticosteroids of medium to high potencies, such as triamcinolone, can also be utilized and may help with the pruritus & inflammatory aspect of this illness. The adverse reactions profile of these drugs, as well as the benign nature of pigment purpuric dermatitis, may not support their usage, despite the fact that systemic immunosuppressive with cyclosporine or corticosteroids can be beneficial.

However, when immunosuppression is stopped, the disease frequently returns. Topical pimecrolimus and tacrolimus can be helpful for long-term use because they reduce the chance that topical corticosteroids will cause cutaneous atrophy. Six patients with pigment purpuric dermatitis responded better to griseofulvin 500–750 mg daily. With colchicine 0.5 mg twice daily, minocycline, pentoxifylline, and methotrexate 400 mg three times daily, patients with pigment purpuric dermatitis improved in other case series.

Narrowband UVB or PUVA ultraviolet phototherapy has also been shown to have advantages. Withholding treatment in some cases caused the condition to flare up, but in other cases, a lasting remission was attained. Improved pigment purpuric dermatitis has also been reported following topical photodynamic treatment utilizing methyl aminolevulinic acid and 5-aminolevulinic acid as photosensitizing medications.

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

https://www.ncbi.nlm.nih.gov/books/NBK519562/