Abstract

A 2-year-old female, with a history of global developmental delay and milestone regression, presented with recurrent bullae, ulcers and dusky color changes of the distal fingers and toes, as well as dusky and erythematous nodules on her upper and lower extremities. This had been ongoing for over a year and had been worsening during this time.  An initial biopsy showed mild vacuolar interface changes with underlying perivascular lymphohistiocytic inflammation and increased interstitial dermal mucin suggestive of a connective tissue disease. As laboratory workup for an autoimmune condition was unremarkable, a deeper biopsy of one of the nodules on her shin was performed. Pathology revealed superficial and deep perivascular and periadnexal lymphohistiocytic inflammation within the dermis with significantly increased interstitial mucin deposition. The inflammation extended into the subcutaneous fat, which also exhibited hyaline necrosis and numerous plasma cells. Immunohistochemical staining with CD123 highlighted clusters of prominent plasmacytoid dendrocytes. These findings were consistent with Lupus Panniculitis (LP). LP is an uncommon form of chronic cutaneous lupus, that can affect both adult and pediatric patients. Childhood-onset LP is rare and tends to affect the face. LP may occur alone or in association with Systemic Lupus Erythematosus, however the risk of progression in children is unknown. Skin biopsy is required for confirmation, particularly since autoimmune lab serologies can be negative. An early diagnosis and prompt treatment are important to avoid complications. We share this case of pediatric LP with an unusual presentation, as it is important to be aware of this challenging diagnosis.