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Indian Journal of Dermatology
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Year : 2021  |  Volume : 66  |  Issue : 3  |  Page : 326-328
An Elderly Female with Progressive Inflammatory Scar-Like Papules


1 From the Department of Wayne, State University School of Medicine, Detroit, MI, USA
2 Department of Dermatology, Henry Ford Health System, Detroit, MI, USA
3 Department of Dermatology, Henry Ford Health System; Department of Pathology and Laboratory Medicine, Henry Ford Health System, Detroit, MI, USA

Date of Web Publication13-Jul-2021

Correspondence Address:
Ben J Friedman
3031 W. Grand Blvd Ste. 800, Detroit, MI 48202
USA
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijd.IJD_214_20

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How to cite this article:
Adelman M, Gordon Spratt EA, Friedman BJ. An Elderly Female with Progressive Inflammatory Scar-Like Papules. Indian J Dermatol 2021;66:326-8

How to cite this URL:
Adelman M, Gordon Spratt EA, Friedman BJ. An Elderly Female with Progressive Inflammatory Scar-Like Papules. Indian J Dermatol [serial online] 2021 [cited 2021 Aug 2];66:326-8. Available from: https://www.e-ijd.org/text.asp?2021/66/3/326/321313





   Case Report Top


A 71-year-old woman presented with a 10-month history of increasing, non-pruritic coin-shaped skin lesions on her neck, trunk, and extremities. Past medical history included a recent diagnosis of 'irritable bowel syndrome', congestive heart failure, and a pulmonary embolus. On physical examination, she was found to have widespread papules distributed on her trunk and extremities with peripheral erythema, telangiectasias, and central white scar-like areas [Figure 1]. There was no associated palpable lymphadenopathy or organomegaly. Two 4-mm punch biopsies were taken from the right chest, with both specimens showing similar overlapping histopathological findings.
Figure 1: Clinical appearance of lesions on the chest

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   Histopathological Findings Top


The constellation of features included a patchy lichenoid infiltrate of lymphocytes with epidermal atrophy, apoptotic keratinocytes, and an underlying wedge-shaped area of hypovascular hyalinizing fibrosis [Figure 2]a and [Figure 2]b. Below the altered dermis, small vessels occluded with thrombus were observed [Figure 2]c. An Alcian blue stain demonstrated a striking increase in dermal mucin deposition [Figure 2]d.
Figure 2: (a) Histopathology. Low-power view that shows an attenuated epidermis and upper dermal pallor (H&E, original magnification, 40×). (b) Histopathology. High-power view demonstrating an atrophic epidermis with colloid bodies and overlying hyperkeratosis. In the underlying dermis, there is a patchy infiltrate of lymphocytes, melanophages, and hypovascular mucinous fibrosis (H&E, original magnification, 200×). (c) Histopathology. Below the altered wedge-shaped dermal zone, there is a small damaged vessel occluded with thrombus and surrounded by lymphocytes and extravasated erythrocytes. There is basement membrane zone reduplication (H&E, original magnification, 400×). (d) There is marked mucin deposition throughout the entirety of the biopsy specimen (Alcian blue, original magnification, 100×)

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   What is Your Diagnosis? Top


Köhlmeier-Degos disease (KDD), also known as Degos disease.


   Discussion Top


The patient underwent an extensive laboratory workup. In particular, creatine kinase, ANA, anti-histone, anti-Ro, anti-La, and anti-Jo antibodies were all normal. Elevated IgM cardiolipin antibodies to 102.7 MPL/μg (normal: <12.5) was detected. Immunohistochemical staining for MXA, a type I interferon marker, and staining for C5b-9 on the patient's biopsy specimens were both extensively positive.

KDD is a rare vasculopathy characterized by widespread cutaneous erythematous papules, which develop central white atrophy and hyperkeratotic cores along with telangiectatic rims.[1] Conditions that can resemble KDD (and should be excluded prior to diagnosis) include extragenital lichen sclerosis, Buerger's disease, lupus erythematosus, dermatomyositis, and some perforating disorders.[1],[2] Its two variants, benign atrophic papulosis (BAP) and malignant atrophic papulosis (MAP), differ by the presence of internal organ involvement.[3] The most commonly affected organs in MAP include the gastrointestinal tract and central nervous system; however, infarctions of many other organs have been described.[3]

The etiology of KDD remains largely unknown.[4] KDD may occur de novo in patients with no prior medical history or the setting of other autoimmune conditions, most frequently connective tissue diseases such as systemic lupus erythematosus.[4] Other disease associations include some inherited/acquired coagulopathies and viral infections. This particular case is an example of KDD occurring in the setting of antiphospholipid syndrome as evidenced by the patient's history of venous thromboembolism and elevated cardiolipin antibodies.

KDD may present with a wide spectrum of histologic findings depending on the age and location of the lesion biopsied. Dermatologists and dermatopathologists should be aware of this phasic variability and the need for clinical-pathologic correlation to arrive at the correct diagnosis. In early-stage flesh-colored lesions, histopathology will show a lymphohistiocytic infiltrate within a mucinous background, without overt vascular changes.[1] The lesions subsequently progress to become more umbilicated, with porcelain-white centers and telangiectatic rims. At this more developed stage, histopathology reveals a prominent lymphocytic vasculitis, melanin incontinence, and dermal sclerosis.[1] Late-stage, indurated-atrophic papules reveal the pathognomonic histology of KDD: wedge-shaped dermal necrosis and fibrosis with occlusive vasculopathy and overlying epidermal atrophy.[1] It is crucial to sample the central portion of these more developed lesions to more readily facilitate the correct diagnosis.

Magro et al. proposed that a critical effector in this microvascular thrombotic process is the membrane lytic attack complex C5b-9 and that fibrointimal expansion is a consequence of type I interferon dysregulation.[5] Many connective tissue diseases and vascular injury syndromes exhibit overlap in histomorphology. Unique to KDD is the wedge-shaped mucinous fibrosis and occlusive arteriopathy of small- and medium-sized vessels in conjunction with capillary and venule injury.[5]

The optimal management for KDD is a matter of debate and often requires an interprofessional collaborative approach. Theodoridis et al. proposed a standardized management algorithm, which includes fecal occult blood testing and an ocular fundus exam in all suspected cases.[3] Additional workup should be prompted by any signs of organ-specific symptomatology. In our case, this included referral for laparoscopy to exclude peritoneal involvement given the patient's abdominal complaints. Additionally, over the ensuing few months she began experiencing worsening anxiety and an episode of psychosis (in the setting of no prior psychiatric history) prompting referral to neurology to exclude central nervous system involvement. Regarding treatment, there have been reports of the efficacy of eculizumab (monoclonal antibody targeted against complement C5) and treprostinil (synthetic prostaglandin agonist), though additional work in this area is greatly needed.[6],[7]

Learning points

- Degos disease is a rare vasculopathy characterized by erythematous papules with central white atrophy and hyperkeratotic cores along with telangiectatic rims.

- Histopathologic findings are variable depending on the age of the lesion biopsied, and intense clinical-pathologic correlation is often required to arrive at the correct diagnosis.

- The combination of interface changes, occlusive vasculopathy, and increased mucin deposition should prompt consideration of the diagnosis.

- Screening for gastrointestinal involvement requires exploratory laparoscopy to assess for peritoneal adhesions, as an endoscopic examination is usually normal.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Kim E, Motaparthi K. Benign atrophic papulosis (Degos disease) with lymphocytic vasculitis and lichen sclerosus-like features. Am J Dermatopathol 2018;40:272-4.  Back to cited text no. 1
    
2.
Choo SZ, Simpson I, Finlay MJ, Mulley WR. Multi-organ vaso-occlusive disease: Buerger's or Kohlmeier-Degos disease? Pathology 2017;49:798-801.  Back to cited text no. 2
    
3.
Theodoridis A, Konstantinidou A, Makrantonaki E, Zouboulis CC. Malignant and benign forms of atrophic papulosis (Köhlmeier–Degos disease): Systemic involvement determines the prognosis.Br J Dermatol 2014;170:110-5.  Back to cited text no. 3
    
4.
Stavorn T, Chanprapaph K. Degos-like lesions in association with connective tissue diseases: A report of three cases and literature review. ClinCosmetInvestigDermatol 2019;12:815-22.  Back to cited text no. 4
    
5.
Magro CM, Poe JC, Kim C, Shapiro L, Nuovo G, Crow MK, et al. Degos disease: A C5b-9/interferon-alpha-mediated endotheliopathy syndrome. Am J ClinPathol 2011;135:599-610.  Back to cited text no. 5
    
6.
Magro CM, Wang X, Garrett-Bakelman F, Laurence J, Shapiro LS, DeSancho MT. The effects of Eculizumab on the pathology of malignant atrophic papulosis. Orphanet J Rare Dis 2013;8:185.  Back to cited text no. 6
    
7.
Razanamahery J, Payet-Revest C, Mareschal A, Saizonou I, Bonnet L, Gil H, et al. Early failure of eculizumab in a patient with malignant atrophic papulosis: Is it time for initial combination therapy of eculizumab and treprostinil?J Dermatol 2020;47:e22-3.  Back to cited text no. 7
    


    Figures

  [Figure 1], [Figure 2]



 

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