ENFERMEDAD DE DEGOS PDF

Dowling-Degos disease is characterized by a lacy or net-like reticulate pattern of abnormally dark skin coloring hyperpigmentation , particularly in the body's folds and creases. These skin changes typically first appear in the armpits and groin area and can later spread to other skin folds such as the crook of the elbow and back of the knee. Less commonly, pigmentation changes can also occur on the wrist, back of the hand, face, scalp, scrotum in males , and vulva in females. Individuals with Dowling-Degos disease may also have dark lesions on the face and back that resemble blackheads, red bumps around the mouth that resemble acne, or depressed or pitted scars on the face similar to acne scars but with no history of acne.

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Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. For all other comments, please send your remarks via contact us.

Only comments written in English can be processed. Malignant atrophic papulosis MAP is a rare, chronic, thrombo-obliterative vasculopathy characterized by papular skin lesions with central porcelain-white atrophy and a surrounding teleangiectatic rim. Systemic lesions may affect the gastrointestinal tract and the central nervous system CNS and are potentially lethal. MAP onset occurs in adults aged with skin lesions that appear initially as small erythematous papules, predominantly on the trunk and the upper extremities.

Over several days, the center of the lesions sinks and develops a characteristic morphology: 0. This condition is chronic and lesions persist over years, often throughout life. Face, scalp, palms of hands and soles of feet are rarely involved. It may more rarely manifest as pericarditis or in other organs such as the lungs, presenting as pleuritis. Ocular involvement, which affects the eyelids, conjuctiva, retina, sclera and the choroid plexus, as well as the development of diplopia and ophthalmoplegia as secondary side effects of the neurologic involvement , has also been described.

The etiopathogenesis of the disease remains unknown. Hypotheses implicating vasculitis, coagulopathy or a primary dysfunction of endothelial cells have been proposed. Many patients have been reported to have defects in blood coagulation. Diagnosis is based primarily on the cutaneous clinical picture that is nearly pathognomonic.

In early stages, histology of lesions may reveal a superficial and deep perivascular lymphocytic infiltration with distinct mucin deposition. Later a wedge-shaped connective tissue necrosis in the deep dermis, due to a thrombotic occlusion of the small arteries and sparse lymphocytes, occurs.

More developed lesions show prominent changes in the dermoepidermal junction, with atrophy of the epidermis and an area of sclerosis in the papillary dermis. The histology of early lesions resembles cutaneous lupus erythematosus see this term.

More developed lesions can imitate lichen sclerosus see this term. Therapeutic efforts with anticoagulants and compounds that facilitate blood perfusion, such as acetylosalicylic acid, pentoxifylline, dipyridamole, ticlodipine and heparin have achieved a partial regression of skin lesions in some individual cases.

As all patients may potentially develop the systemic, life-threatening variant, an annual follow-up is mandatory. A clinical inspection of the skin should be combined with additional examinations including brain magnetic resonance tomography, gastroscopy, colonoscopy, X-ray of the chest and abdominal ultrasound, in order to assess the long-term prognosis.

No effective treatment for the systemic manifestations has been established, however, subcutaneous treprostinil has been tested successfully in one case with intestinal and CNS manifestations. Idiopathic, monosymptomatic, cutaneous presentations are benign, however, systemic manifestations can develop years after the occurrence of skin lesions.

Other search option s Alphabetical list. Suggest an update. Summary and related texts. Related genes. Clinical signs. Check this box if you wish to receive a copy of your message. Disease definition Malignant atrophic papulosis MAP is a rare, chronic, thrombo-obliterative vasculopathy characterized by papular skin lesions with central porcelain-white atrophy and a surrounding teleangiectatic rim.

Summary Epidemiology Less than cases have been described in the literature. Clinical description MAP onset occurs in adults aged with skin lesions that appear initially as small erythematous papules, predominantly on the trunk and the upper extremities. Etiology The etiopathogenesis of the disease remains unknown.

Diagnostic methods Diagnosis is based primarily on the cutaneous clinical picture that is nearly pathognomonic. Differential diagnosis The histology of early lesions resembles cutaneous lupus erythematosus see this term. Genetic counseling A genetic predisposition with an autosomal dominant trait has been suggested.

Management and treatment Therapeutic efforts with anticoagulants and compounds that facilitate blood perfusion, such as acetylosalicylic acid, pentoxifylline, dipyridamole, ticlodipine and heparin have achieved a partial regression of skin lesions in some individual cases. Prognosis Idiopathic, monosymptomatic, cutaneous presentations are benign, however, systemic manifestations can develop years after the occurrence of skin lesions.

Additional information Further information on this disease Classification s 3 Gene s 0 Clinical signs and symptoms Publications in PubMed Other website s 7. Health care resources for this disease Expert centres 88 Diagnostic tests 0 Patient organisations 32 Orphan designation s and orphan drug s 0.

Specialised Social Services Eurordis directory. The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.

ISOMETH APAP DICHLOR PDF

Loli from Madrid, Spain

Skin macules tend to be the earliest symptom in multisystem disease. Small bowel involvement is very common, and Degos disease can cause intestinal perforation tear. Click here to learn more about the signs and symptoms of intestinal perforation. Unfortunately intestinal disease tends to recur in these individuals. This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed.

JRC386D PDF

Dowling-Degos disease

Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. For all other comments, please send your remarks via contact us. Only comments written in English can be processed. Malignant atrophic papulosis MAP is a rare, chronic, thrombo-obliterative vasculopathy characterized by papular skin lesions with central porcelain-white atrophy and a surrounding teleangiectatic rim. Systemic lesions may affect the gastrointestinal tract and the central nervous system CNS and are potentially lethal. MAP onset occurs in adults aged with skin lesions that appear initially as small erythematous papules, predominantly on the trunk and the upper extremities.

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