Skin nontumor

Dermal collagen and elastic tissue alterations, and cutaneous deposits

Atrophic papulosis


Editor-in-Chief: Debra L. Zynger, M.D.
Maryam Aghighi, M.D.
Kiran Motaparthi, M.D.

Topic Completed: 12 March 2021

Minor changes: 22 March 2021

Copyright: 2002-2021, PathologyOutlines.com, Inc.

PubMed Search: Atrophic papulosis [TI] pathology

Maryam Aghighi, M.D.
Kiran Motaparthi, M.D.
Page views in 2021 to date: 890
Cite this page: Aghighi M, Motaparthi K. Atrophic papulosis. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/skinnontumoratrophicpapulosis.html. Accessed December 2nd, 2021.
Definition / general
  • Rare vasculopathy with benign skin limited form and frequently lethal systemic variant with intestinal involvement
Essential features
  • All ages, mostly in young adults
  • Typically affects proximal extremities and trunk
  • Papules with porcelain white centers and erythematous peripheral rims
  • Wedge shaped dermal necrosis with mucin deposition
  • Vessels with endothelial swelling, luminal obliteration, thrombosis
Terminology
  • Köhlmeier-Degos disease
  • Degos disease
  • Malignant atrophic papulosis
  • Lethal intestinocutaneous syndrome
ICD coding
  • ICD-10: L90.9 - atrophic disorders of skin
Epidemiology
Sites
  • More commonly affects proximal extremities and trunk (Clin Exp Gastroenterol 2015;141:147)
  • Less commonly affects genital, buccal and conjunctival sites
  • GI tract involvement, specifically small intestine
  • CNS, hepatorenal and cardiopulmonary system involvement
Pathophysiology
  • High interferon alpha expression and accumulation of late stage complements (C5b9) in diseased vessels (Pediatrics 2018;S481:S484)
Clinical features
  • Early disease: up to 5 mm in diameter, skin colored papules on proximal extremities and trunk
  • Established disease: atrophic papules with porcelain white centers and erythematous peripheral rims, surrounded by telangiectasia
  • Late disease: atrophic scars
  • May be associated with factor V Leiden or parvovirus B19 infection or autoimmune connective tissue diseases, including systemic lupus erythematosus, antiphospholipid syndrome, dermatomyositis or systemic sclerosis (JAMA Dermatol 2014;150:96)
Diagnosis
  • Biopsy from skin, intestines or other involved organs
  • Complete blood count
  • Stool guaiac test
  • Antinuclear antibody titer
  • Factor V Leiden, protein C and S, antithrombin III and homocysteine levels
  • Anticardiolipin antibody titer
  • Antiphospholipid antibody titer
  • Endoscopy
  • Laparotomy (Orphanet J Rare Dis 2013;8:10)
Laboratory
  • In association with systemic lupus erythematosus: antinuclear antibody titer, anti-dsDNA, Coombs test, complement protein C3, C4, CH50 or immune complex assays (C1q or C3d)
  • May be differentiated from other associated autoimmune disorders by antiphospholipid and anticardiolipin antibodies, rheumatoid factor, anti-Mi2, antitopoisomerase I and anti-Saccharomyces cerevisiae antibodies (Ann Dermatol 2017;29:215)
Prognostic factors
  • Benign atrophic papulosis
    • Normal life expectancy
    • No systemic involvement
    • Represents 70% of cases of atrophic papulosis
    • Retrospective diagnosis that requires longterm followup
  • Malignant atrophic papulosis
    • Onset of systemic disease approximately 1 year after cutaneous findings
    • Average 2 year survival in untreated patients with systemic disease
    • Peritonitis secondary to intestinal perforation is the leading cause of death (Orphanet J Rare Dis 2013;8:10)
    • Cerebral infarction is a less common cause of death
  • Patients with underlying autoimmune connective tissue diseases have a better prognosis
Case reports
Treatment
  • Monotherapy or combination therapy
Clinical images

Contributed by Kiran Motaparthi, M.D.
Malignant atrophic papulosis

Malignant atrophic papulosis

Microscopic (histologic) description
  • Early disease:
    • Superficial and deep perivascular and periadnexal lymphocytic infiltrate
  • Established disease:
    • Features of lichen sclerosus: epidermal atrophy, hyperkeratosis and edema with homogenization of the superficial dermis
    • Lymphocytic vasculitis
  • Late disease (classic features):
    • Thrombotic vasculopathy
    • Wedge shaped dermal necrosis with mucin deposition (Cutis 1985;35:131)
Microscopic (histologic) images

Contributed by Kiran Motaparthi, M.D.
Atrophic papulosis in the context of systemic lupus erythematosus Atrophic papulosis in the context of systemic lupus erythematosus Atrophic papulosis in the context of systemic lupus erythematosus Atrophic papulosis in the context of systemic lupus erythematosus

Atrophic papulosis in the context of systemic lupus erythematosus


Benign atrophic papulosis Benign atrophic papulosis Benign atrophic papulosis

Benign atrophic papulosis

Immunofluorescence description
Positive stains
Electron microscopy description
Sample pathology report
  • Right upper arm, biopsy:
    • Atrophic papulosis (Degos disease) (see comment)
    • Comment: There is a wedge shaped scar, along with a perivascular lymphocytic infiltrate. Dermal vessels have hyaline walls and contain fibrin thrombi. There is also overlying epidermal atrophy and hyperkeratosis.
Differential diagnosis
Additional references
Board review style question #1

A 35 year old woman presents with white, atrophic papules on her proximal extremities and trunk, malar rash, Raynaud phenomenon, malaise, arthritis and anemia. Laboratory evaluation is significant for thrombocytopenia, leukopenia and high titer ANA and anti-dsDNA. Skin biopsy was performed, shown above. Which of the following statements is correct?

  1. A poor prognosis is anticipated, with significant neurologic or gastrointestinal involvement
  2. Late histopathologic findings include dermal necrosis and scar
  3. The cutaneous findings are most likely idiopathic
  4. The cutaneous findings represent extragenital lichen sclerosus
  5. The cutaneous findings represent lupus erythematosus
Board review style answer #1
B. Late histopathologic findings include dermal necrosis and scar. The diagnosis is malignant atrophic papulosis.

Comment Here

Reference: Atrophic papulosis
Board review style question #2
A 30 year old man presents with porcelain white papules on his left arm and abdominal discomfort. Skin biopsy showed vacuolar interface dermatitis, hyperkeratosis, perivascular lymphocytic infiltrates, thrombi, mucin and wedge shaped necrosis. Which of the following is the best diagnosis?

  1. Dermatomyositis
  2. Leukocytoclastic vasculitis
  3. Livedoid vasculopathy
  4. Malignant atrophic papulosis
  5. Systemic lupus erythematosus
Board review style answer #2
D. Malignant atrophic papulosis

Comment Here

Reference: Atrophic papulosis
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