Persistent edema of rosacea | |
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Other names | Persistent oedema of rosacea [1] |
Specialty | Dermatology |
Persistent edema of rosacea (also known as chronic upper facial erythematous edema, Morbihan's disease or rosaceous lymphedema) is a hard, nonpitting edema found on the areas involved, those mainly being the forehead, glabella, upper eyelids, nose, and/or cheeks. [2]
Persistent edema of rosacea is an uncommon cutaneous condition characterized by a hard, nonpitting edema restricted to the forehead, glabella, upper eyelids, nose, and cheeks. This condition is also known as chronic upper facial erythematous edema, Morbihan's disease, morbus Morbihan, and rosaceous lymphedema. Despite the name, it is unclear whether this condition is a distinct disease or a rare complication of rosacea. Generally, there are few symptomatic complaints aside from redness and facial contour changes. The edema typically worsens slowly over months to years and is often on a background of chronic inflammation.
There are no specific laboratory findings associated with this condition, and the histology tends to be similar to that seen in rosacea. The differential diagnosis includes acne vulgaris, streptococcal cellulitis, and Melkersson–Rosenthal syndrome, as these conditions can also rarely manifest with a similar edema, as well as other forms of rosacea,' lupus erythematosus , and sarcoidosis.
Most cases of this condition tend to be recalcitrant to treatment, with topical and oral antibiotic regimens commonly used for rosacea generally being ineffective. For the severe forms of this disease, oral isotretinoin therapy has been used, and the condition responds well to doses of 0.5 to 1 mg/kg/day. However, unlike in the treatment of acne vulgaris, lasting responses to isotretinoin generally do not occur, and long-term maintenance therapy with oral tetracyclines is usually necessary. Eyelid reduction surgery has also been reported to help with the cosmetic appearance of the condition, but does not alter the disease progression. [3] [4] [5] [6] [7] [8]
Acne, also known as acne vulgaris, is a long-term skin condition that occurs when dead skin cells and oil from the skin clog hair follicles. Typical features of the condition include blackheads or whiteheads, pimples, oily skin, and possible scarring. It primarily affects skin with a relatively high number of oil glands, including the face, upper part of the chest, and back. The resulting appearance can lead to lack of confidence, anxiety, reduced self-esteem, and, in extreme cases, depression or thoughts of suicide.
Isotretinoin, also known as 13-cis-retinoic acid and sold under the brand name Accutane among others, is a medication primarily used to treat severe acne. It is also used to prevent certain skin cancers, and in the treatment of other cancers. It is used to treat harlequin-type ichthyosis, a usually lethal skin disease, and lamellar ichthyosis. It is a retinoid, meaning it is related to vitamin A, and is found in small quantities naturally in the body. Its isomer, tretinoin, is also an acne drug.
Rosacea is a long-term skin condition that typically affects the face. It results in redness, pimples, swelling, and small and superficial dilated blood vessels. Often, the nose, cheeks, forehead, and chin are most involved. A red, enlarged nose may occur in severe disease, a condition known as rhinophyma.
Perioral dermatitis, also known as periorificial dermatitis, is a common type of skin rash. Symptoms include multiple small (1–2 mm) bumps and blisters sometimes with background redness and scale, localized to the skin around the mouth and nostrils. Less commonly the eyes and genitalia may be involved. It can be persistent or recurring and resembles particularly rosacea and to some extent acne and allergic dermatitis. The term "dermatitis" is a misnomer because this is not an eczematous process.
Lupus vulgaris are painful cutaneous tuberculosis skin lesions with nodular appearance, most often on the face around the nose, eyelids, lips, cheeks, ears and neck. It is the most common Mycobacterium tuberculosis skin infection. The lesions may ultimately develop into disfiguring skin ulcers if left untreated.
Stewart–Treves syndrome refers to a lymphangiosarcoma, a rare disorder marked by the presence of an angiosarcoma in a person with chronic (long-term) lymphedema. Although it most commonly refers to malignancies associated with chronic lymphedema resulting from mastectomy and/or radiotherapy for breast cancer, it may also describe lymphangiosarcomas that result from congenital and other causes of chronic secondary lymphedema. Lymphangiosarcoma arising from cancer-related lymphedema has become much less common with better surgical techniques, radiation therapy, and conservative treatment. The prognosis, even with wide surgical excision and subsequent radiotherapy, is poor.
Acne fulminans is a severe form of the skin disease, acne, which can occur after unsuccessful treatment for another form of acne, acne conglobata. The condition is thought to be an immunologically induced disease in which elevated level of testosterone causes a rise in sebum and population of Cutibacterium acnes bacteria. The increase in the amount of C acnes or related antigens may trigger the immunologic reaction in some individuals and lead to an occurrence of acne fulminans. In addition to testosterone, isotretinoin may also precipitate acne fulminans, possibly related to highly increased levels of C acnes antigens in the patient's immune system. Acne fulminans is a rare disease. Over the past several years, fewer cases of this disease have occurred, possibly because of earlier and better treatment of acne. Approximately 100 patients with acne fulminans have been described.
Papular mucinosis is a rare skin disease. Localized and disseminated cases are called papular mucinosis or lichen myxedematosus while generalized, confluent papular forms with sclerosis are called scleromyxedema. Frequently, all three forms are regarded as papular mucinosis. However, some authors restrict it to only mild cases. Another form, acral persistent papular mucinosis is regarded as a separate entity.
Hidradenitis is any disease in which the histologic abnormality is primarily an inflammatory infiltrate around the eccrine glands. This group includes neutrophilic eccrine hidradenitis and recurrent palmoplantar hidradenitis.
Lichen nitidus is a chronic inflammatory disease of unknown cause characterized by 1–2 mm, discrete and uniform, shiny, flat-topped, pale flesh-colored or reddish-brown papules that may appear as hypopigmented against dark skin. Occasionally, minimal scaling is present or can be induced by rubbing the surface of the papules. The disease usually affects children and young adults and is painless and usually nonpruritic, although protracted itching may occur in some cases. It is sometimes referred to by dermatologists as "mini lichen planus".
Steatocystoma multiplex, is a benign, autosomal dominant congenital condition resulting in multiple cysts on a person's body. Steatocystoma simplex is the solitary counterpart to steatocystoma multiplex.
Acne conglobata is a highly inflammatory disease presenting with comedones, nodules, abscesses, and draining sinus tracts.
Dissecting cellulitis of the scalp, also known as dissecting folliculitis, perifolliculitis capitis abscedens et suffodiens of Hoffman, perifolliculitis abscedens et suffodiens, or folliculitis abscedens et suffodiens, is an inflammatory condition of the scalp that can lead to scarring alopecia, which begins with deep inflammatory nodules, primarily over occiput, that progresses to coalescing regions of boggy scalp. Boggy tissue has a high fluid level that results in a spongy feeling. Isotretinoin proves to be the medicine of choice for the treatment of the disease.
Lupus miliaris disseminatus faciei, also known as acne agminata, is a disease with a similar appearance to acne vulgaris. The cause of LMDF is unknown.
Gnathophyma involves swelling of the chin. It is a type of lesion associated with rosacea, a common chronic inflammatory skin disorder of the sebaceous glands characterized by redness, swelling, and acne-like pustules.
Blepharophyma is chronic swelling of eyelids, mainly due to sebaceous gland hyperplasia.
Camisa disease is the variant form of Vohwinkel syndrome, characterized by ichthyosis and normal hearing.
Frontal fibrosing alopecia is the frontotemporal hairline recession and eyebrow loss in postmenopausal women that is associated with perifollicular erythema, especially along the hairline. It is considered to be a clinical variant of lichen planopilaris.
Stasis papillomatosis is a disease characterized by chronic congestion of the extremities, with blood circulation interrupted in a specific area of the body. A consequence of this congestion and inflammation is long-term lymphatic obstruction. It is also typically characterized by the appearance of numerous papules. Injuries can range from small to large plates composed of brown or pink, smooth or hyperkeratotic papules. The most typical areas where injuries occur are the back of the feet, the toes, the legs, and the area around a venous ulcer formed in the extremities, although the latter is the rarest of all. These injuries include pachydermia, lymphedema, lymphomastic verrucosis and elephantosis verrucosa. The disease can be either localized or generalized; the localized form makes up 78% of cases. Treatment includes surgical and pharmaceutical intervention; indications for partial removal include advanced fibrotic lymphedema and elephantiasis. Despite the existence of these treatments, chronic venous edema, which is a derivation of stasis papillomatosis, is only partially reversible. The skin is also affected and its partial removal may mean that the skin and the subcutaneous tissue are excised. A side effect of the procedure is the destruction of existing cutaneous lymphatic vessels. It also risks papillomatosis, skin necrosis and edema exacerbation.