The spelling of Lupus Erythematosus Profundus, a rare form of lupus, can be a bit tricky. The word uses Latin root words, which can cause some confusion. The pronunciation is (ˈluːpʊs ɛrəˌθiːməˈtoʊsəs prəʊˈfʊndəs), and knowing the International Phonetic Alphabet (IPA) can be very helpful. The "u" in "lupus" is pronounced like "oo" in "soon," while "erythematosus" has the "th" sound as in "thin." Similarly, "profundus" is pronounced with the "u" as in "put" and the "nd" as in "end."
Lupus erythematosus profundus (LEP), also known as lupus panniculitis, is a rare subtype of cutaneous lupus erythematosus (CLE) characterized by inflammation and tissue damage deep within the subcutaneous fat layer of the skin.
LEP is an autoimmune disorder, meaning it occurs when the immune system mistakenly attacks healthy cells and tissues. In this case, the immune system targets the fat cells beneath the skin, leading to the formation of nodules or plaques that can be painful and disfiguring. These lesions typically appear as firm, deep-seated masses that are often red or violaceous in color.
The exact cause of LEP is still unknown, but it is believed to involve a combination of genetic, environmental, and hormonal factors. It is more commonly observed in women during their reproductive years.
The diagnosis of LEP is usually made based on clinical presentation, physical examination, and sometimes confirmed through skin biopsy. Treatment options for LEP aim to control symptoms and prevent disease progression. This may involve the use of anti-inflammatory medications such as nonsteroidal anti-inflammatory drugs (NSAIDs) and corticosteroids. In severe cases, immunosuppressive drugs or antimalarials may be prescribed.
While LEP can cause significant discomfort and cosmetic concerns, it is not typically life-threatening. However, it may be associated with systemic lupus erythematosus (SLE), a more widespread autoimmune disease that affects other organs. Therefore, close monitoring and periodic evaluation by a dermatologist and rheumatologist are often recommended for individuals with LEP to assess for any signs of systemic involvement.