FEBRILE ULCERONECROTIC MUCHA-HABERMANN DISEASE

By Gary M. White, MD


In this more severe form of PLEVA, diffuse ulceronecrotic lesions develop along with fever, malaise, myalgia, and arthralgia. Other potential complications include gastrointestinal and central nervous system involvement, interstitial pneumonitis, lymphocytic myocarditis, and death.

Clinical

Diffuse ulceronecrotic lesions are seen.

Treatment

Patients are often admitted to the hospital. Good skin care for denuded areas is important. Systemic steroids are usually prescribed initially. They may be given alone or combined with an antibiotic, e.g., erythromycin, tetracycline, or doxycycline. If the condition is unresponsive, methotrexate may be added with excellent results [ Indian J Dermatol 2014;59:631]. A 20-month-old boy was treated successfully with prednisolone combined with low-dose cyclosporine [Infection 2015 Jan 28]. Cyclosporine has been used successfully in many other cases as well. Other reported treatment options include prednisone combined with nbUVB; and IVIG [Dermatology 2012:225;334].

References

A previously healthy 27-year-old woman. Dermatology Online Journal 11(3)

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