P KZN PLEVA

 A 7 year-old previously healthy boy with 3 weeks of progressive painful rash and fevers presented to the Emergency Department. One month ago he was also ill and positive for influenza A by rapid antigen – he recovered, then approximately a week later developed the rash and fever. His primary care doctor saw him early in the course of the rash a prescribed a 10-day course of cephalexin, which the patient completed, and the symptoms seemed to improve (fevers resolved). The symptoms soon returned with a vengeance and the rash dramatically worsened. The rash itself was initially sparse with reddish-brown papules across the body, face, extremities, and external genitals. Mucosal surfaces were spared. Eventually the papules became too numerous to count and formed necrotic ulcerative confluences as shown in the image below.

In the Emergency Department he was febrile to 38.1 degrees Celsius, tachycardic to 128 beats/minute, and ill- appearing. Initial labs were notable only for CRP of 6.83 mg/dl. CBC, CMP, & urinalysis were within normal limits. Here is the aforementioned rash.

What is the diagnosis?

A. Stevens-Johnson Syndrome

B. Serum sickness like reaction

C. Pityriasis lichenoides et varioliformis acuta (PLEVA)

D. Meningococcemia

E. Rocky Mountain Spotted Fever

Click here to reveal the correct answer

C. Pityriasis lichenoides et varioliformis acuta (PLEVA)

 

Of course it is the one with the long name… This is a severe and threatening variant of PLEVA known as Febrile Ulceronecrotic Mucha-Habermann disease (FUMHD). Though the pathogenesis is poorly understood, it is thought to be due to an aberrant t-cell immune response. Diagnosis is confirmed by skin biopsy. The prevalence is unknown; only 42 cases have been reported in the medical literature. Associated symptoms include high fevers, diarrhea, myalgias, arthritis, CNS symptoms, megaloblastic anemia, interstitial pneumonitis, lymphocytic myocarditis and sepsis.  No consensus treatment exists. Case reports describe treatments with systemic steroids, methotrexate, dapsone, cyclosporine, psoralen and ultraviolet A (PUVA), ultraviolet B (UVB), acyclovir, immunoglobulins, and 4,4-diaminodiphenylsulphone (DDS). The other options can also make you look very sick, but do not have the ulcerative appearance as seen in the image.

 

References

Sotirious E., Patsatsi A., Tsorova C., LazaridouE., Sotiriadis D. Febrile ulceronecrotic mucha-Haberman disease: a case report and review of the literature. Acta Derm Venerology, 2008.