A 33-year-old man with a reported history of sickle cell anemia and a penicillin allergy presented to the emergency department (ED) with an extensive ulcerative rash involving his face, extremities, trunk, genitals, and back with sparing of the mucous membranes, eyes, palms, and feet (Figure 1). The rash initially began 5 days prior to presenting at our hospital as painful, small ulcerations localized to the left arm. The patient originally sought care at an outside ED where he was diagnosed with severe dermatitis with suspected bacterial superinfection, but the patient declined admission and was subsequently discharged with a prescription of cephalexin. The rash progressively worsened and disseminated despite antibiotic treatment, prompting the patient to present to our ED. He denied a history of eczema, asthma, allergic rhinitis, recent exposure to a new environmental allergen, shortness of breath, headaches, and visual disturbances. Cardiovascular, pulmonary, and neurologic examinations were normal.
The patient's social history is remarkable for a monogamous relationship with 1 male partner. He endorsed consistent use of barrier contraception during intercourse. He denied a history of sexually transmitted infections, prior blood transfusions, and intravenous (IV) drug use.
Given the severity of the rash, recent antibiotic exposure, and history of serious penicillin allergy, initial differential diagnoses were drug allergy and a disease on the Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis (SJS/TEN) spectrum. However, the latter was ruled out by the burn team and dermatology (virtually) due to the absence of mucosal involvement, negative nikolsky sign, and absence of skin sloughing.
Laboratory studies in the ED revealed a reactive HIV antigen/antibody screen with a subsequently positive HIV-1 antibody and negative HIV-2 antibody. In addition, his labs were significant for CD4 count of 259, white blood cell count (WBC) of 7.4 k/microL with 54% neutrophils, 9% lymphocytes, 0% eosinophils, 0% basophils, and creatinine and sodium of 3.05 mg/dL and 119 mEq/L, respectively. These findings suggested an immune deficiency cause rather than an allergic reaction, and dermatology subsequently recommended starting IV acyclovir for possible disseminated HSV-1 infection. Infectious disease (ID) was consulted, and he was admitted for further workup of the skin lesions.
Screening for hepatitis, syphilis, gonorrhea and chlamydia was negative. Further tests for opportunistic infections, including coccidiomycosis antigen/antibody, cryptococcal antigen, tuberculosis, varicella zoster virus (VZV), and cytomegalovirus (CMV) were also negative. In addition, autoimmune disease screening with antinuclear antibodies (ANAs), antineutrophil cytoplasmic antibodies (ANCAs), and serum complements were unremarkable. However, HSV polymerase chain reaction (PCR) from the skin lesions was positive for HSV-1 and negative for HSV-2. A positive repeat test further suggested a diagnosis of disseminated HSV-1, and subsequent HSV antibody testing showed elevated IgM. A skin punch biopsy of the left upper extremity revealed irregular psoriasiform hyperplasia with epidermal hypergranulosis, hyperkeratosis, and superficial pigmentary incontinence. There was superficial perivascular inflammation consisting of lymphocytes and histiocytes. No spongiosis and intraepidermal vesicles were present. There was no evidence of viral morphologic changes.
The patient was continued on IV acyclovir and emollients with gradual improvement in the lesion throughout the admission (Figure 2). He was discharged with 2 weeks of oral valganciclovir followed by lifelong daily acyclovir prophylaxis. He was scheduled to follow up with ID outpatient for initiation of highly active antiretroviral therapy (HAART).
acyclovir, cutaneous, dermatology, disseminated, herpes simplex virus, human immunodeficiency virus