Pediatric treatment strategies of Pityriasis lichenoides et varioliformis acuta Mucha Habermann–effects and side effects

Aims:

Febrile ulceronecrotic pityriasis lichenoides et varioliformis acuta Mucha Habermann is a potentially lethal autoimmune disorder characterized by polymorphous generalized skin lesions that rapidely deteriorate associated with a severe disease pattern.

Case report:

We report on a 22 months old boy, to our knowledge the youngest out of 42 cases reviewed in the literature since its first description in 1966. He presented with bug-bite-like lesions that rapidely progressed into erythematous ulcers with hemorrhagic crusts and necroses including most of the integument. The diagnosis was confirmed by skin biopsy. Despite a timely started immunosuppressive treatment with topical and systemic corticosteroids and in the course cyclosporine A and methotrexate the clinical condition aggravated including a SIRS with need of multiple blood transfusions, vasoactive agents, long term artificial nutrition and in the end artificial ventilation. Pseudomonas aeruginosa was substantiated in several skin swabs. Wound care was undertaken in permanent analgosedation consisting of desinfectious agents and silver-nitrate layers. These caused an additional silver toxicopathy (serum levels seriously elevated). The complexity of the clinical and dermatological state improved after months of long term systemic immunosuppression leaving discoloration and residual hypertrophic cicatrices. Problems in intensive care management could be reduced, in the end a good clinical and neurological state was found.

Discussion:

The probable cause of febrile ulceronecrotic Mucha Habermann is an inflammatory reaction triggered by infectious agents. Although no randomised studies validate a sufficient therapy, reviews suggest intensive local and systemic immunosuppressive and -modulating treatment strategies in childhood. Early use of methotrexate and careful adapted wound care might help to alleviate and shorten the pattern of this rare disorder. These intensified treatment strategies should be reserved to special pediatric intesive care units.

Pityriasis lichenoides et varioliformis acuta Mucha Habermann - immunosuppression - specialized intensive care treatment - silver toxicopathy