1-year-old boy with cluster of red papules on arm
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You are called to see a 1-year-old boy admitted to the hospital for treatment of an atypical teratoid rhabdoid tumor of the brainstem. He was transferred to your hospital 2 weeks ago for diagnosis and management. The oncology team plans to start IV and intrathecal chemotherapy, but calls you first to evaluate a red plaque on the patient’s left arm that has been present and stable since his transfer. It does not appear to be symptomatic, but the patient’s mental status is altered from his tumor. He is afebrile. The plaque is composed of four coalescing, smooth, red papules (see figure). Upon further questioning, you learn that IV placement was attempted in that location at the outside hospital. In addition, he has had therapy with prednisone before your exam.
Surface swabs initially were performed and showed negative herpes simplex virus and varicella zoster virus via PCR, negative candida screen, and no growth from a surface wound culture.
Can you spot the rash?
Diagnosis: Cutaneous Aspergillus fumigatus
Case discussion
Aspergillus fumigatus is found in the environment and may cause opportunistic fungal infections. It is an angioinvasive fungus that rarely affects immunocompetent patients. This patient was at risk for two reasons: possible occlusion of the area with tape while IV placement was attempted at the outside hospital, and immunosuppression from prednisone in a patient with a tumor.
Cutaneous infection with Aspergillus can present in many ways, including as eschar, hemorrhage, ulcerative nodules, papules, cellulitis and sporotrichoid nodules. Presentation with eschar and/or hemorrhage can occur because it is an angioinvasive pathogen. Sporotrichoid spread would point to lymphatic involvement. Infection of an IV puncture site or of skin occluded with a dressing around an IV is typical of this uncommon infection. Extremities, as well as the head and neck, are more often affected than other parts of the body.
The differential diagnosis includes bacterial infection, Staphylococcus aureus being the most likely bacteria, or viral infection with HSV or VZV due to the grouped nature of the papules. Candidal infection can present with red papules, but the distribution of few fixed papules on one extremity is uncommon; more typically you would see scattered red papules or pustules that would spread and have satellite lesions without treatment. The lack of scale, lack of small surrounding papules, and lack of current contactant made irritant or allergic contact dermatitis less likely in this particular case.
If there is suspicion for infection with Aspergillus, skin biopsy is imperative and should include tissue for both routine microscopy and fungal culture. Hematoxylin and eosin stains often will show necrotic tissue with inflammation due to the angioinvasive nature of the fungus, and occasionally may highlight organisms. Fungal stains typically will show the organisms that appear as hyphae with acute-angle branching and frequent septations, usually highlighted with Gomori methenamine silver staining. Tissue culture is key in confirming the diagnosis and identifying the exact species of Aspergillus.
Aspergillus infection in the skin can be primary (direct inoculation) or secondary (hematogenous spread, likely from primary lung or sinus infection). Treatment often depends on the underlying immune status and stability of the patient. When possible, urgent excision of a single lesion or few localized foci is recommended, as excision can prevent distant spread if performed quickly. Systemic antifungal treatment is advisable as an adjunctive treatment for localized disease, and as primary treatment for systemic disease or in patients with localized disease not amenable to primary excision.
Early identification of cutaneous fungal infections in immunocompromised patients is critical. Given the myriad presentations of cutaneous Aspergillus, the index of suspicion should be high when examining immunocompromised patients at risk for developing such infections.
References:
Galimberti R. Br J Dermatol. 1998;139:522-526.
Mays SR. Am J Clin Dermatol. 2006;7:31-43.
Palmero ML. Pediatr Dermatol. 2009;26:592-596.
For more information:
Carrie C. Coughlin, MD, is a pediatric dermatology fellow at The Children’s Hospital of Philadelphia. She can be reached at coughlincc@email.chop.edu.
Marissa J. Perman, MD, is an attending physician at The Children’s Hospital of Philadelphia.
Disclosure: Coughlin and Perman report no relevant financial disclosures.