Pyoderma Gangrenosum Associated with an Aseptic Splenic Abscess in a Patient with Neurofibromatosis

Abstract

Pyoderma gangrenosum (PG) is a painful, ulcerating neutrophilic dermatosis commonly associated with a variety of underlying systemic conditions. We report a child with neurofibromatosis-1 (NF-1) and an aseptic splenic abscess who developed multifocal PG in areas of iatrogenic skin trauma. There is no clinical evidence or theoretical basis to suggest a causal relationship between NF-1 and PG. Systemic corticosteroid and cyclosporine therapy led to complete resolution of the lesions.

Association of pyoderma gangrenosum, erythema nodosum and aseptic liver abscess without significant underlying disease

“…Systemic conditions such as fever, pain and peripheral leucocytosis with neutrophilia are sometimes accompanied by abscess‐like lesions in the spleen, liver and lung, and termed ‘aseptic abscess syndrome’. To date, several cases of aseptic abscess occurring as extracutaneous manifestations of PG have been reported, involving the lung, liver, spleen and bone . Our patient did not develop elevated fever or abdominal pain.…”

Pyoderma gangrenosum with splenic involvement

“…Extracutaneous manifestation of PG in our patient was in the form of splenic involvement. Allen CP et al reported a child to have pyoderma gangrenosum with splenic and lung involvement [13], and Johnson JL et al reported a child with only splenic involvement [14]. There is limited data regarding extracutaneous manifestation of PG in the pediatric age group compared to adult patients, who frequently report this comorbidity.…”

Extracutaneous involvement of pyoderma gangrenosum

“…Splenic involvement in PG is extremely rare but can affect any age, including a few pediatric cases under the age of 10 [7,34] (Fig. 2).…”