Malignant Atrophic Papulosis
Skin Diseases, Papulosquamous
Lymphomatoid Papulosis
Pairing and comparing nine diseases with Degos Disease (Malignant Atrophic Papulosis): an attempt to illustrate our understanding and direct future inquiry. (1/8)
Degos disease is a poorly defined condition that encompasses cutaneous and systemic findings that overlap with a large number of other rheumatologic and coagulation disorders. Comparisons are made herein with 9 conditions that share findings and presentations. (+info)Prominent Degos-like skin lesions in a patient with chronic cutaneous lupus erythematosus. (2/8)
Malignant atrophic papulosis, commonly known as Degos disease, is a rare vasculopathy encompassing both benign, cutaneous and lethal systemic variants. We report a case of chronic cutaneous lupus erythematosus in a 41-year-old male presenting with prominent Degos-like skin lesions. Multiple atrophic, porcelain-white, scar-like papules and plaques with dusky, erythematous borders, suggestive of malignant atrophic papulosis, were noted on the patient's back. Additional cutaneous findings included photo-distributed facial erythema and discoid lupus-like plaques on the face, shoulders, and arms. Clinicopathological correlation supported a diagnosis of chronic cutaneous lupus erythematosus; hydroxychloroquine was initiated with good clinical response. No new or active lesions were observed at the sixteen-month follow-up. This case highlights a rare skin finding associated with chronic cutaneous lupus erythematosus and underscores the importance of ruling out primary autoimmune disease, particularly lupus, before a diagnosis of malignant atrophic papulosis can be made. (+info)Degos disease: a C5b-9/interferon-alpha-mediated endotheliopathy syndrome. (3/8)
(+info)Commentary on 'Degos disease: a C5b-9/interferon-alpha-mediated endotheliopathy syndrome' by Magro et al: a reconsideration of Degos disease as hematologic or endothelial genetic disease. (4/8)
Magro et al in April of 2011 published a new article in the American Journal of Clinical Pathology on the etiology and treatment of Degos Disease (DD), and importantly, its fatal variant malignant atrophic papulosis (MAP). Specifically, Magro noted that MAP is a disease involving the complement cascade that can be treated effectively with eculizumab. DD has two variants, a benign variant confined to the skin and a malignant (heretofore fatal) variant that involves the skin and systemic organs. Five aspects of DD are discussed: (1) the clinical findings of DD, (2) thrombosis and DD, (3) the histology of DD, (4) the presence of viral like inclusions in the endothelial cells of patients with DD, and (5) the lack of any apparent immune defect that relates to DD. It seems the previous criteria for Degos Disease must be amended. Paroxysmal nocturnal hemoglobinuria (PNH) is discussed and its relationship with DD explored. Eculizumab has been approved to treat paroxysmal nocturnal hemoglobinuria. A review of the data suggests that MAP is a hematological or endothelial disease like PNH. PNH, eculizumab, and data about DD is discussed to give a basis for understanding DD and speculate why eculizumab may be promising for the treatment of MAP. (+info)Malignant atrophic papulosis (Kohlmeier-Degos disease) - a review. (5/8)
(+info)Degos' syndrome complicated by bowel perforation: focus on radiological findings. (6/8)
We describe a 50-year-old man who first presented with multiple skin lesions which were characteristic of Degos' syndrome. The patient developed multiple episodes of abdominal pain. Some episodes resolved with conservative management, for others he underwent urgent operations for bowel perforations. The patient subsequently underwent extensive small bowel resection, but further systemic deterioration ensued and he died. The imaging findings of Degos' syndrome and the implications of pneumatosis intestinalis and pneumoperitoneum are discussed. (+info)Effective treatment of malignant atrophic papulosis (Kohlmeier-Degos disease) with treprostinil--early experience. (7/8)
(+info)The effects of Eculizumab on the pathology of malignant atrophic papulosis. (8/8)
(+info)Malignant atrophic papulosis (MAP), also known as Kohlmeier-Degos disease, is a rare and progressive cutaneous vasculopathy of unknown etiology. It is characterized by the development of porcelain-white atrophic macules, which evolve into papules with a central necrotic depression or ulceration, surrounded by an erythematous halo. The lesions typically appear on the trunk and extremities, but may also affect mucous membranes, gastrointestinal tract, and other organs.
MAP is considered to be a chronic inflammatory disorder that affects small-sized blood vessels, leading to tissue ischemia and necrosis. The disease can have a variable clinical course, ranging from self-limited cutaneous involvement to systemic manifestations with potentially life-threatening complications.
The diagnosis of MAP is based on the clinical presentation, histopathological findings, and exclusion of other similar conditions. Treatment options for MAP are limited, and there is no cure for this disease. The management typically involves a multidisciplinary approach to address the various organ manifestations and prevent complications.
Papulosquamous skin diseases are a group of chronic inflammatory disorders of the skin characterized by the development of papules (small, solid, often conical bump) and scales. These diseases include psoriasis, lichen planus, and seborrheic dermatitis among others. The skin lesions in these conditions are often red, scaly, and may be pruritic (itchy). They can vary in severity and distribution, and can have a significant impact on a person's quality of life. The exact cause of these diseases is not fully understood, but they are believed to involve an abnormal immune response and genetic factors. Treatment typically involves a combination of topical therapies, phototherapy, and systemic medications.
Lymphomatoid papulosis (LyP) is a rare, chronic skin disorder characterized by recurrent, self-healing papules and nodules. It is considered a low-grade T-cell lymphoma, but it is distinct from other cutaneous lymphomas due to its benign clinical course and lack of systemic involvement in most cases. The lesions typically undergo cycles of appearing, ulcerating, and then resolving over a period of several weeks to months, only to recur elsewhere on the body.
Histologically, LyP is characterized by an inflammatory infiltrate composed of small lymphocytes, histiocytes, eosinophils, and atypical large cells with Reed-Sternberg-like morphology. The condition is often associated with other lymphoproliferative disorders, such as mycosis fungoides or Hodgkin's lymphoma, and it is important to monitor patients closely for signs of progression to more aggressive lymphomas.
The exact cause of LyP remains unclear, but it is thought to involve an abnormal immune response and genetic factors. Treatment options include topical therapies, phototherapy, and systemic medications such as methotrexate or retinoids. However, the choice of treatment depends on the severity and extent of the disease, as well as the individual patient's needs and preferences.
Degos disease
Treprostinil
List of skin conditions
Malignant Atrophic Papulosis: Background, Pathophysiology, Etiology
Degos disease - Wikipedia
Malabsorption | Download book PDF
Degos Disease: Background, Pathophysiology, Etiology
Publications
Degos Disease Articles & Papers | Page 3
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NEW (2008) DeCS DESCRIPTORS WITH SCOPE NOTES (UNIT RECORD FORMAT; 21/02/2008
NEW (2008) DeCS DESCRIPTORS WITH SCOPE NOTES (UNIT RECORD FORMAT; 21/02/2008
NEW (2008) DeCS DESCRIPTORS WITH SCOPE NOTES (UNIT RECORD FORMAT; 21/02/2008
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
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)