Gougerot–Blum syndrome
Editor-In-Chief: Prab R Tumpati, MD
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Gougerot–Blum syndrome | |
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Synonyms | Pigmented purpuric lichenoid dermatitis |
Pronounce | N/A |
Specialty | Dermatology |
Symptoms | Purpura, lichenification, pigmentation |
Complications | N/A |
Onset | Typically in adulthood |
Duration | Chronic |
Types | N/A |
Causes | Unknown |
Risks | Possible genetic predisposition |
Diagnosis | Clinical diagnosis, skin biopsy |
Differential diagnosis | Schamberg's disease, lichen planus |
Prevention | N/A |
Treatment | Topical corticosteroids, phototherapy |
Medication | N/A |
Prognosis | Generally good with treatment |
Frequency | Rare |
Deaths | N/A |
Gougerot–Blum syndrome is a rare dermatological condition characterized by the presence of lichenoid and pigmented purpuric dermatosis. It is named after the French dermatologists Henri Gougerot and Paul Blum, who first described the syndrome.
Clinical Features
Gougerot–Blum syndrome typically presents with small, reddish-brown papules that may coalesce into larger plaques. These lesions are often found on the lower extremities but can also appear on the trunk and upper limbs. The condition is chronic and may persist for many years.
Pathophysiology
The exact cause of Gougerot–Blum syndrome is unknown. It is believed to be an autoimmune disorder, where the body's immune system mistakenly attacks its own skin cells. Histopathological examination usually reveals a lichenoid tissue reaction with a band-like infiltrate of lymphocytes at the dermoepidermal junction.
Diagnosis
Diagnosis is primarily clinical, supported by skin biopsy findings. The biopsy typically shows features of both lichenoid dermatitis and pigmented purpuric dermatosis. Differential diagnosis includes other lichenoid disorders such as lichen planus and other pigmented purpuric dermatoses like Schamberg's disease.
Treatment
There is no definitive cure for Gougerot–Blum syndrome. Treatment is symptomatic and may include topical corticosteroids, calcineurin inhibitors, and phototherapy. In some cases, systemic treatments such as immunosuppressive drugs may be considered.
Prognosis
The prognosis for Gougerot–Blum syndrome is generally good, although the condition can be persistent and may require long-term management. It is not associated with any significant morbidity or mortality.
See Also
References
External Links
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Contributors: Prab R. Tumpati, MD