James G. H. Dinulos

Research from JAMA Dermatology — A Business Case for Shared Medical Appointments in Dermatology — Improving Access and the Bottom Line

Abstract:  Granular cell tumors are benign neurally derived neoplasms, involving cutaneous and subcutaneous tissues; and typically occur as solitary lesions. Multiple granular cell tumors occur in 10% of affected individuals, but are in children. Children with underlying somatic and genetic syndromes, including neurofibromatosis and Noonan syndrome, appear to be at higher risk of developing multiple granular cell tumors. Skin biopsy assists in diagnosis, since granular cell tumors have a similar appearance to other cutaneous nodules. Painful or rapidly growing granular cell tumors should be excised and asymptomatic non‐growing granular cell tumors may be observed. Children with multiple granular cell tumors should have a complete physical examination to rule out an underlying genetic syndrome.

Ichthyotic-appearing skin changes have been associated with autoimmune disorders, including systemic lupus erythematosus and systemic sclerosis in adults. We report three children with ichthyotic-appearing skin changes that may be an early feature reflecting underlying dermal sclerosis. Detection of these subtle skin changes may be predictive of more widespread cutaneous involvement, ultimately prompting earlier treatment.

Abstract:  Mycoplasma pneumoniae is an important and highly relevant cause of bullous erythema multiforme, isolated mucositis, and Stevens–Johnson syndrome in children. In this article, we present two children with respiratory Mycoplasma pneumoniae infection and associated cutaneous findings within the spectrum of erythema multiforme. We review the literature associating these three entities with Mycoplasma pneumoniae infection and discuss controversies regarding the classification of erythema multiforme, as well as update reported infectious causes of the bullous form. Many understand the erythema multiforme spectrum to include bullous erythema multiforme, mucositis, and Stevens–Johnson syndrome in the order of increasing severity. We feel that this relationship should be reconsidered to help better understand the prognosis and outcomes. It is our opinion that bullous erythema multiforme is a separate, yet related condition that can occur in the context of Mycoplasma pneumoniae infection. With many similarities to mucositis and Stevens–Johnson syndrome, bullous erythema multiforme can be considered part of a spectrum of disease that includes Stevens–Johnson syndrome. Unlike mucositis and Stevens–Johnson syndrome, bullous erythema multiforme caused by Mycoplasma pneumoniae infection has low morbidity for the child. Mycoplasma pneumoniae‐associated mucositis and Stevens–Johnson syndrome seem to occur along a spectrum with separate prognosis and potential pathogenesis compared with bullous erythema multiforme. Making the distinction between these conditions is valuable for predicting the child's prognosis. Patients who develop symptoms consistent with these conditions should be appropriately evaluated for Mycoplasma pneumoniae infection and closely monitored.

Abstract:  Cutaneous erosions and ulcerations in the diaper area are common in infancy and usually result from local irritation. We describe an infant with chronic diarrhea and failure to thrive who developed extensive ulcerations in the inguinal folds and perineum that were initially thought to be exclusively caused by local irritation. A cutaneous examination found signs consistent with those of pyoderma gangrenosum, leading to a diagnosis of infantile Crohn disease. Cutaneous signs can lead to the diagnosis of an underlying systemic disease in infants with chronic diarrhea and rash. Prompt diagnosis is especially important in infantile Crohn disease, since many infants require surgical resection of affected bowel, and 60% die from disease complications. This article reports a rare instance of an infant who developed pyoderma gangrenosum due to Crohn disease and reviews cutaneous signs of systemic disease in infants presenting with chronic diarrhea and rash.