Introduction:
Pigmented patches in the skin are common. What makes them distinguishable is their color and appearance! One of the various patches on the skin is a reddish-brown patch responsible for a disease called Schamberg's. It comes under a group of conditions called progressive pigmented purpuric dermatoses. It is a form of capillaritis that mainly affects the lower limbs.
What Is Capillaritis?
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Capillaritis is caused by the leakage of blood from the capillaries (the tiny blood vessels that are present superficially in the skin).
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When the capillaries get inflamed, the red blood cells tend to break, leading to the deposition of hemosiderin (a protein compound that stores iron in the tissues), which causes pigmentation in the skin.
What Are the Causes of Schamberg's Disease?
Though the apparent cause for this disease remains unknown, it could be due to any one of the following reasons;
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Specific allergic reactions to various allergens like food or certain substances might cause this disease.
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The pigmentations associated with this disease might be a reaction to any medications taken for the underlying conditions.
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Schamberg's disease could be caused by active viral infections.
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Hereditary reasons, this disease shows a genetic predisposition, which means that it runs in families.
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Alcohol intake could also be a causative factor for this disease.
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Exercising can be the cause, too.
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Low immune responses can trigger such lesions.
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There is no such evidence that shows a correlation between Schamberg’s disease and hematological reasons. Although, the features are similar in both.
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Certain underlying diseases can aggravate this disease, like diabetes, hypertension, capillary, fragility, etc.
Who Is Prone to Schamberg's Disease?
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Otherwise, healthy people are usually affected.
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It is stated that males are more susceptible to this disease, though it affects females.
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Schamberg's disease is more common in middle-aged to older people than in children.
What Are the Characteristic Features of Schamberg's Disease?
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It is an entirely benign condition, which means it is not life-threatening.
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It is chronic (present for a more extended period) and usually is present for a lifetime with an intermittent flare-up. The spots keep fading away with time, and the intensity of the color varies based on their time of onset.
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Usually, it appears as regular or irregular reddish-brown patches with Cayenne pepper-like spots along its borders, which is a prominent feature of this condition. They form papules or macules-like lesions; they keep progressing with time.
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Predominantly they are localized to the lower extremities and spread to the thighs and buttocks, but can also appear in other body parts such as the upper extremities and trunk, or they can be generalized, which is relatively uncommon. It usually does not affect the face.
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It affects the body parts bilaterally.
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They may present as a single patch or can be multiple.
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These are not palpable, and the size typically of each macule is not more than 1.18 inches.
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These patches tend to recur.
Why Are the Spots Reddish-Brown in Color?
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Hemosiderin staining is the reason for this condition's reddish, orangish, brownish pigmentations.
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In the case of inflamed capillaries, blood seeps out, causing breakage of the red blood cells into the skin and releasing iron from the hemoglobin present in the red blood cells that get deposited as hemosiderin under the skin, causing pigmentations that are reddish-brown.
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The white blood cells, called the immune cells, can usually remove the deposited hemosiderin to an extent, but in conditions like Schamberg's, they fail to remove it.
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In some cases, the intensity of the pigment varies with time. The pigmentations that have been present for years show pale pigments due to the reabsorption of hemosiderin.
What Are the Symptoms of Schamberg’s Disease?
Usually, this condition is entirely asymptomatic; very rarely can show pruritus (itchy skin) or pain.
How Is Schamberg's Disease Diagnosed?
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This disease is diagnosed by closely examining the lesion clinically and correlating the features present.
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Dermoscopy can be used as a diagnostic tool as it uses a unique lens to view the lesion.
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This disease does not show any abnormalities in the blood test; blood tests like complete blood count peripheral smear are done to rule out other conditions associated with blood presenting similar features.
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A biopsy is not necessary to diagnose this disease but is performed to differentiate it from other similar conditions and confirm the diagnosis.
How Is Schamberg's Disease Treated?
As the reason for this disease still stands a query, there is no specific treatment for this condition. Various treatments have been tried, and few have shown significant results.
A few treatment options include the following;
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Discontinuing the drugs that occurred as the trigger factor, one should avoid any allergen or food that caused the lesion.
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It is initially treated using topical steroid ointments.
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Wearing compression stockings to compensate for the abnormalities in the capillaries.
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Phototherapy is seen to provide promising results.
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The antioxidant properties of Rutoside and Ascorbic acid have shown proven results.
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Narrowband UV light has also been used as a treatment option.
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In superimposed infection (lesions that get infected), antibiotics are given to minimize the infection.
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Advanced fluorescent technology is also a mode of treatment for this disease.
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Few lasers used for aesthetic and cosmetological purposes have also shown results.
What Are the Differential Diagnosis of Schamberg’s Disease?
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Majocci disease(Rare fungal infection of the hair follicles and surrounding skin, often caused by Trichophyton rubrum or Trichophyton mentagrophytes).
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Touraine(Also known as pachydermoperiostosis, is a rare genetic disorder characterized by thickened facial skin, clubbing of the fingers and toes, and excessive sweating).
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Gougerot and Blum's pigmented purpuric dermatosis (rare skin condition characterized by the appearance of reddish-brown spots on the skin, typically on the legs, often accompanied by itching).
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Purpuric eczematoid-like lesions by Doucas and Kapetanakis(rare skin condition characterized by eczema-like lesions with purpuric features, typically affecting the lower limbs).
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Stasis dermatitis (a skin condition that occurs when there is poor circulation in the legs, leading to swelling, redness, itching, and sometimes ulceration, often as a result of chronic venous insufficiency).
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Mycosis fungoides (the most common type of cutaneous T-cell lymphoma, characterized by skin lesions, such as patches, plaques, and tumors, that may resemble eczema or psoriasis).
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Scurvy purpuric lesions(Symptom of scurvy, presenting as purple or red spots on the skin due to bleeding underneath the skin's surface as a result of vitamin C deficiency).
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Leukocytoclastic vasculitis hemorrhagic bullae(Type of blood vessel inflammation characterized by the formation of small blood-filled blisters on the skin).
What Are the Complications of Schamberg’s Disease?
In rare cases, SD may precede mycosis fungoides, a type of skin lymphoma, sharing some clinical and histopathological features. Prolonged SD (>1 year) warrants regular annual follow-ups as it could potentially develop into mycosis fungoides. Furthermore, long-lasting pigmentation associated with SD may cause emotional distress, leading to depression and anxiety in some patients.
How Is the Prognosis of Schamberg's Disease After Treatment?
This disease usually shows spontaneous healing but has remained persistent without recovery and subsequent worsening in a few cases. Due to its chronic nature, it stays for an extended period. It has also shown recurrence in some cases even after the treatment.
Conclusion:
This disease does not necessarily have to provoke your fear factor as it is danger-free and has shown spontaneous healing in most cases. Most people get treated for this condition owing to its displeasing appearance aesthetically. Though the disease does not have a specific cause or a proper treatment protocol, it has responded to various treatment modalities, excluding a few.