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Differential Diagnosis

  • Drug eruption
  • Viral exanthem
  • Acute graft-versus-host disease
  • Radiation dermatitis
  • Erythema multiforme
  • Toxic epidermal necrolysis
Acute graft-versus-host disease

Diagnosis

The correct diagnosis is acute cutaneous graft-versus-host disease (GVHD), an immune-mediated condition resulting from donor lymphocytes attacking host tissues after a hematopoietic cell transplant (HCT).

The combined stages of skin, liver, and gastrointestinal disease are used to determine the overall severity (grade) of acute GVHD. A staging system is used to describe the extent of cutaneous involvement:  

  • Stage 1 – Less than 25 % body surface area (BSA) involvement
  • Stage 2 – 25 to 50 % BSA involvement
  • Stage 3 – Greater than 50 % BSA involvement
  • Stage 4 – Erythroderma with bullae or skin sloughing

In the absence of extracutaneous manifestations, making a definitive diagnosis of acute GVHD may be challenging; the rash can be difficult to distinguish clinically from other skin eruptions that occur in post-HCT patients. Biopsy may provide information that can be used in conjunction with other clinical findings to support a diagnosis of acute GVHD. A 4 mm punch biopsy sent for hematoxylin and eosin staining is generally sufficient to evaluate for acute cutaneous GVHD.

What To Look For

  • Symptoms begin with erythematous, blanchable macules on the ears, lateral neck, cheek, upper back, palms, or soles. Some lesions may follow a follicular distribution.
  • Lesions progress to a generalized, morbilliform (measles-like) appearance. Smooth or hyperkeratotic papules and postinflammatory hyperpigmentation may occur.
  • Lesions may be pruritic or accompanied by a burning sensation, sometimes these symptoms precede the onset of visible lesions.
  • Erythema, ulcers, lichenoid lesions, xerostomia, and pain involving the oral cavity may be present.

Pearls for Urgent Care Management

  • Topical corticosteroids may provide improvement in patients with limited skin disease. High-potency corticosteroid solutions or gels and topical anesthetic agents are also used for oral symptoms.
  • Patients with xerostomia should be encouraged to maintain good oral hygiene and may benefit from salivary stimulants such as sugarless gum or candy.
  • Encourage prompt follow up with patient’s transplant providers for ongoing evaluation and management of cutaneous GVHD. Phototherapy and systemic therapies are the mainstays of treatment for patients with sclerotic lesions.
A Rash and Fever Following a Bone Marrow Transplant
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