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Acute Myelopathy in a Man With Cutaneous Papules

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A 46-year-old man presented with acute-onset weakness of his bilateral lower limbs for 3 days, accompanied by ascending numbness to just below his nipples. Three weeks prior, he had developed acute urinary retention. Neurological examination revealed weakness in bilateral lower limbs (Medical Research Council grade 4 on the right and grade 3 on the left), extensor plantar responses, and a sensory level at T6. Magnetic resonance imaging of his thoracic spine was performed 10 days after the onset of weakness and did not show any signal abnormalities or restricted diffusion. Cerebrospinal fluid analysis findings were remarkable for elevated protein (0.14 g/dL [to convert to grams per liter, multiply by 10]) but white cell count (5 cells/mm3) and glucose levels (54.05 mg/dL [to convert to millimoles per liter, multiply by 0.0555]) were normal. Cytology and flow cytometry were negative for malignancy. Antibodies to aquaporin 4 and myelin oligodendrocyte glycoprotein were not detected. The patient developed a sudden worsening of weakness in his legs 2 weeks after initial presentation. Repeated thoracic spine imaging showed an area of T2 hyperintensity in the left posterolateral cord at T7, which did not enhance with contrast or show restricted diffusion (Figure 1A). Notably, he had a 4-year history of an asymptomatic papular skin eruption with more lesions developing in the last 4 months. These were distributed on the neck, trunk, and limbs, sparing his face, scalp, and genitalia (Figure 1B). Findings of extensive workup for malignancy, including full-body computed tomography scan, paraneoplastic antibody panel, and systemic autoimmune diseases (antinuclear antibody, antidouble stranded DNA, and myositis antibody panel), were negative. The skin papules were biopsied.

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A. Malignant atrophic papulosis

The differential diagnoses to consider in a patient who has cutaneous papules and myelopathy can include infectious (eg, secondary syphilis, HIV), ischemic (eg, malignant atrophic papulosis), autoimmune (eg, systemic lupus erythematosus, Behçet disease), and paraneoplastic (eg, scleromyxedema with paraproteinemia) causes.

The diagnosis of malignant atrophic papulosis (MAP) or Köhlmeier-Degos disease was supported by the characteristic cutaneous eruption and histological findings of wedge-shaped dermal collagen degeneration, interstitial mucin deposition, small–vessel angiopathy with luminal thrombi, perivascular and perineural lymphocytic infiltration, and interface vacuolar alteration of the epidermis (Figure 2). These findings, indicative of microvascular injury, can also be seen in the setting of Degos-like diseases, namely dermatomyositis, lupus erythematosus, and scleroderma. Negative autoimmune markers and the absence of characteristic clinical features excluded these differential diagnoses. Scleromyxedema is an unusual condition of dermal mucin deposition and fibroblast proliferation and is typically associated with a monoclonal gammopathy and other systemic manifestations. Histopathological findings of microvascular injury in the patient are not consistent with this diagnosis. Lues maligna is a rare but severe form of secondary syphilis that has been described in patients who have HIV co-infection—cutaneous lesions start out as papules that ulcerate to become well-demarcated necrotic plaques. Syphilis was ruled out with negative findings on serum, cerebrospinal fluid treponemal, and nontreponemal antibody tests, and the absence of spirochetes on skin biopsy. The acute and stepwise deterioration of this patient’s thoracic myelopathy is consistent with a vascular event.

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Article Information

Corresponding Author: Shermyn Neo, MBBS, Department of Neurology, National Neuroscience Institute, 11 Jalan Tan Tock Seng, Singapore 308433 (shermyn.neo.x.m@singhealth.com.sg).

Published Online: November 15, 2021. doi:10.1001/jamaneurol.2021.4194

Correction: This article was corrected on January 31, 2022, to fix an arrowhead that was incorrectly located.

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient for granting permission to publish this information. We thank Lee Shapiro, MD (Albany Medical College, Albany, New York), Cynthia Magro, MD (Weill Cornell Medicine, New York, New York), Peter A. Merkel, MD (University of Pennsylvania, Philadelphia), and Robert G. Micheletti, MD (University of Pennsylvania), for providing expert advice on the investigation and management of Degos disease. They did not receive any forms of compensation for their advice.

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Credit Designation Statement: The American Medical Association designates this Journal-based CME activity activity for a maximum of 1.00  AMA PRA Category 1 Credit(s)™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Successful completion of this CME activity, which includes participation in the evaluation component, enables the participant to earn up to:

  • 1.00 Medical Knowledge MOC points in the American Board of Internal Medicine's (ABIM) Maintenance of Certification (MOC) program;;
  • 1.00 Self-Assessment points in the American Board of Otolaryngology – Head and Neck Surgery’s (ABOHNS) Continuing Certification program;
  • 1.00 MOC points in the American Board of Pediatrics’ (ABP) Maintenance of Certification (MOC) program;
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  • 1.00 CME points in the American Board of Surgery’s (ABS) Continuing Certification program

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