Diagnosis: Atrophic papulosis
Pathology suggested a diagnosis of atrophic papulosis. A consultation with a dermatologist and additional biopsies confirmed the diagnosis. The biopsies showed wedge-shaped areas of superficial dermal sclerosis with thinning of the overlying epidermis. The superficial dermal vessels contained scattered, small thrombi at the periphery of the areas of sclerosis.
Atrophic papulosis (also known as Degos disease or Kölmeier-Degos disease) is a vasculopathy characterized by thrombotic occlusion of small arteries.1 Although rare—with fewer than 200 published case reports in the literature—it is likely underdiagnosed.1 Atrophic papulosis can be distinguished by hallmark skin findings, including 0.5- to 1-cm papular skin lesions with central porcelain-white atrophy and an erythematous, telangiectatic rim.1 It usually manifests between ages 20 to 50 but can occur in infants and children.1 The etiology is unknown, but case evidence suggests the condition is sometimes familial.1,2
Easy to confuse with common conditions
Clinical presentation of atrophic papulosis can vary, but evaluation should rule out systemic lupus erythematosus and other connective tissue diseases.1 In addition, the lesions can easily be confused with other common conditions such as molluscum contagiosum or insect bites.
The hallmark finding of molluscum contagiosum is raised papules with central umbilication, whereas atrophic papulosis lesions are characterized by white centers. While insect bites typically disappear within weeks, atrophic papulosis lesions persist for years or are even lifelong.1
Is it benign or malignant?
Benign atrophic papulosis is limited to the skin.1 The probability of a patient having benign atrophic papulosis is about 70% at the onset of skin lesions and 97% after 7 years without other symptoms.2
Malignant atrophic papulosis—although less common—is systemic and life-threatening. About 30% of patients with atrophic papulosis develop lesions manifesting both on the skin and in internal organs.1,2 Systemic involvement can develop at any time, sometimes years after the appearance of skin lesions, but the risk declines over time.2 In a case series, systemic signs were shown to develop, on average, 1 year after skin lesions.2 Some evidence suggests a mortality rate of 50% within 2 to 3 years of the onset of systemic involvement, making regular follow-up necessary.1
Continue to: Patients with malignant atrophic papulosis...