Malignant Atrophic Papulosis

Skin Diseases, Papulosquamous

Lymphomatoid Papulosis

Degos disease

Treprostinil

List of skin conditions

Papules5

• Both the benign and malignant forms have development of the characteristic papules. (wikipedia.org)
• If an individual develops the malignant form, it means that not only are the papules present, but inner organs are involved. (wikipedia.org)
• These papules heal to leave scars with pathognomonic, central, porcelain white atrophic centers. (medscape.com)
• A skin punch biopsy taken from the porcelain white atrophic papules which revealed features of Degos disease. (biomedcentral.com)
• On examination, the lesions were porcelain white atrophic papules with surrounding erythema present over the trunk, upper limbs, thighs, and chest (Figure 1 a-d). (biomedcentral.com)

Degos7

• Degos disease, also known as Köhlmeier-Degos disease or malignant atrophic papulosis, is an extremely rare condition caused by blockage of arteries and veins. (wikipedia.org)
• In 1941, in an article entitled "Multiple Hautrekrosen bei Thromboangiitis obliterans," Kohlmeier described a case of a disease that has now been termed malignant atrophic papulosis (MAP) or Degos disease. (medscape.com)
• Degos' disease (malignant atrophic papulosis) as a fatal cause of acute abdomen: report of a case. (degosdisease.org)
• Degos' disease, otherwise known as "malignant atrophic papulosis," is a rare condition characterized by typical cutaneous lesions. (degosdisease.org)
• Diagnosis: malignant atrophic papulosis (Degos' disease). (degosdisease.org)
• Degos disease is a rare disorder, characterized by the development of typical atrophic porcelain-white macules on the trunk and extremities. (degosdisease.org)
• [ 7 ] reiterated that malignant atrophic papulosis/Degos disease has both a (1) limited, cutaneous type and a (2) systemic, fatal variant. (medscape.com)

Lymphomatoid papulosis1

• 2] Other cases have given rise to lymphomatoid papulosis. (medscape.com)

Disease7

• Malignant atrophic papulosis is different from other vasculitides in that inflammation is not a prominent component of the disease. (medscape.com)
• It has also been theorized that malignant atrophic papulosis may not be a distinct disease but rather several processes that converge to produce characteristic clinical and histologic findings. (medscape.com)
• Malignant atrophic papulosis is a rare disease, with approximately 200 cases reported to date. (medscape.com)
• The morbidity and mortality of malignant atrophic papulosis depend upon the extent of disease involvement. (medscape.com)
• Symptoms vary, depending on whether an individual has the benign variant or malignant variant of the disease. (wikipedia.org)
• Malignant atrophic papulosis or Dego's disease is a type of cutaneous disease with involvement of the gastrointestinal and central nervous system. (degosdisease.org)
• Malignant Atrophic Papulosis (Dego&sgrave;Disease). (booksdo.com)

Porcelain white1

• 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. (nih.gov)

Lesions2

• Most malignant cases involve problems of the gastrointestinal tract leading to small intestine lesions, abdominal pain, diarrhea, and bowel perforation. (wikipedia.org)
• The diagnosis is difficult because of the slow course of the lesions, which present initially as apparently benign atrophic morphoeaor keloid -like plaques. (lookfordiagnosis.com)

Cutaneous1

• Cutaneous T-cell lymphomas (CTCLs) are a group of disorders characterized by abnormal accumulation of malignant T-cells in the skin, which may result in the development of rashes, plaques, and tumors. (patientworthy.com)

Plaques1

• We report six childhood cases of DFSP which presented initially with the misleading clinical appearance of atrophic plaques, and we review over 140 cases of DFSP in childhood. (lookfordiagnosis.com)

Variant2

• A malignant variant affects multiple organ systems and results in death (most commonly from intestinal perforation) within a 2-year period. (medscape.com)
• El-Darouti et al reported on a 7-year study of a hypopigmented disorder that the researchers believe should be classified as a new variant of parapsoriasis en plaque. (medscape.com)

Histologic1

• Lymphocytes do not show histologic atypia to suggest malignant transformation. (medscape.com)

Blood vessels1

• Malignant atrophic papulosis is a multisystem disorder involving small-caliber blood vessels. (medscape.com)

Initially1

• [ 9 , 10 ] The benign and malignant variants are clinically indistinguishable initially but become distinct once systemic complications arise. (medscape.com)

Cases1

• Most cases of malignant atrophic papulosis reported from Europe and North America have been in white individuals. (medscape.com)

Case1

• Kohlmeier described a case of malignant atrophic papulosis (MAP) as a form of thromboangiitis obliterans in 1941. (medscape.com)

Sections1

• Histopathological examination of the sections showed atrophic epidermis with mild hyperkeratosis and flattened rete ridges focally. (biomedcentral.com)

Exists1

• A source of confusion exists in understanding atrophic papulosis. (medscape.com)

Form1

• Someone with the benign form may suddenly develop symptoms of the malignant form. (wikipedia.org)

Presenting as gastroparesis2

• Malignant atrophic papulosis presenting as gastroparesis. (medscape.com)
• Beales, ILP 2001, ' Malignant atrophic papulosis presenting as gastroparesis ', American Journal of Gastroenterology , vol. 96, no. 12, pp. 3462. (uea.ac.uk)

Degos Disease9

• Pirolla E, Fregni F, Miura IK, Misiara AC, Almeida F, Zanoni E. Degos disease - malignant atrophic papulosis or cutaneointestinal lethal syndrome: rarity of the disease. (medscape.com)
• Degos' disease (malignant atrophic papulosis) as a fatal cause of acute abdomen: report of a case. (medscape.com)
• Malignant atrophic papulosis (Degos' disease)--diffuse involvement of brain and bowel in an African patient. (medscape.com)
• Black MM. Malignant atrophic papulosis (Degos' disease). (medscape.com)
• Degos disease, also known as Köhlmeier-Degos disease or malignant atrophic papulosis, is an extremely rare condition caused by blockage of arteries and veins. (wikipedia.org)
• In 1941, in an article entitled "Multiple Hautrekrosen bei Thromboangiitis obliterans," Kohlmeier described a case of a disease that has now been termed malignant atrophic papulosis (MAP) or Degos disease. (medscape.com)
• Also known as atrophic malignant papulosis, Degos disease is a perplexing, ultra-rare disease affecting the skin and digestive system with approximately 300 known cases. (nih.gov)
• Degos' disease, also known as "malignant atrophic papulosis" is a rare vasculopathy characterized by typical cutaneous lesions with an unknown etiology wich was first described by Dego in 1942 [1], but another case, reported in 1941 by Köhlmeier, who interpreted it as thromboangiitis obliterans of the mesenteric vessels [2] This is an arteriopathy which involves vessels of small-caliber. (actascientific.com)
• 2- Theodoridis A, Makrantonaki E, Zouboulis CC. Malignant atrophic papulosis (Köhlmeier-Degos disease) - a review. (edu.iq)

20012

• WHO, 2001) Malignant C45929 Acidophil Stem Cell Pituitary Gland Adenoma Acidophil Stem Cell Adenoma An infrequent pituitary gland adenoma composed of cells showing immunoreactivity for prolactin and less often growth hormone. (nih.gov)
• WHO, 2001) Malignant C4673 Acute Biphenotypic Leukemia Acute Biphenotypic Leukemia An acute leukemia of ambiguous lineage characterized by blasts which coexpress myeloid and T or B lineage antigens or concurrent B and T lineage antigens. (nih.gov)

Syndrome1

• Malignant atrophic papulosis (Degos' syndrome). (medscape.com)

Vasculopathy characterized1

• 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. (beds.ac.uk)

Benign and malignant2

• [ 9 , 10 ] The benign and malignant variants are clinically indistinguishable initially but become distinct once systemic complications arise. (medscape.com)
• Both the benign and malignant forms have development of the characteristic papules. (wikipedia.org)

Antibodies1

• Absence of antiphospholipid and anti-endothelial cell antibodies in malignant atrophic papulosis: a study of 15 cases. (medscape.com)

Nonatrophic1

• Classic mycosis fungoides is divided into 3 stages: patch (atrophic or nonatrophic), plaque, and tumor. (medscape.com)

Disorder2

• Malignant atrophic papulosis is a multisystem disorder involving small-caliber blood vessels. (medscape.com)
• Also, the term "primary cutaneous CD4 + small/medium T-cell lymphoma" was changed to "primary cutaneous CD4 + small/medium T-cell lymphoproliferative disorder" because of its indolent clinical behavior and uncertain malignant potential. (medscape.com)

Organs1

• If an individual develops the malignant form, it means that not only are the papules present, but inner organs are involved. (wikipedia.org)

Neoplasm2

• Malignant C7419 Acanthoma A benign skin neoplasm composed of epithelial cells. (nih.gov)
• Acinic Cell Carcinoma A malignant glandular epithelial neoplasm consisting of secretory cells forming acinar patterns. (nih.gov)

19411

• Kohlmeier described a case of malignant atrophic papulosis (MAP) as a form of thromboangiitis obliterans in 1941. (medscape.com)

Gastrointestinal tract1

• Most malignant cases involve problems of the gastrointestinal tract leading to small intestine lesions, abdominal pain, diarrhea, and bowel perforation. (wikipedia.org)

Abdominal1

• Malignant form of atrophic papulosis with lethal abdominal involvement. (medscape.com)

Acute1

• Malignant C6923 Acute Bilineal Leukemia Acute Bilineal Leukemia An acute leukemia of ambiguous lineage in which there is a dual population of blasts with each population expressing markers of a distinct lineage (myeloid and lymphoid or B-and T-lymphocyte). (nih.gov)

Date1

• To date, no medications have proven beneficial in the treatment of malignant atrophic papulosis. (medscape.com)

Unknown1

• The etiology of malignant atrophic papulosis is unknown. (medscape.com)