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Background: Infants with Down syndrome are at increased risk for hematologic abnormalities, including leukemoid reaction, transient myeloproliferative disorder, and congenital leukemia. The differential diagnosis of a vesiculopustular eruption in an infant with Down syndrome and these hematologic abnormalities is broad and includes benign, self-limited disorders as well as life-threatening infections., Observation: We describe 3 newborns with Down syndrome and vesiculopustular eruptions associated with myeloproliferative disorders during the neonatal period. These lesions differ from other neonatal vesicular eruptions in that they have a unique distribution, display pathergy, and contain immature hematopoietic cells similar to circulating blast cells. Resolution occurs without treatment as the hematologic disorder subsides., Conclusions: Infants with Down syndrome and hematologic abnormalities may have a cutaneous eruption that has characteristic clinical and histopathologic findings. It is possible that this eruption has been unrecognized in the past because of its self-limited course. Whether this eruption is a prognostic factor for the subsequent development of leukemia is uncertain.

Bullous disease in children can be confusing clinically and challenging to mange. Five groups of autoimmune bullous disease are discussed with emphasis on unique features in children. Pemphigus (vulgaris, foliaceus, neonatal), pemphigoid (bullous and cicatricial), dermatitis herpetiformis, linear IgA dermatosis, and epidermolysis bullosa acquisita are uncommon pediatric disorders. A high index of suspicion is necessary to diagnose and treat these conditions without delay.

Three children had an extensive erosive and vesicular dermatosis at birth that healed with supple reticulated scarring. This article discusses the differential diagnosis and pathogenesis of the disorder in these patients, and makes recommendations for medical evaluation and parent counseling.

The clinical features, laboratory studies, and therapeutic responses of two boys with chronic bullous dermatosis of childhood are described. Direct immunofluorescent preparations of sections from a lesion, skin adjacent to a lesion, and uninvolved skin demonstrated linear deposition of IgA at the dermoepidermal junction in all three biopsy specimens from one patient. Similar preparations from the second child were negative for staining. No circulating antibodies to skin components were detected in either child by means of multiple substrates. Neither child had clinical manifestations nor laboratory findings suggestive of an associated gastrointestinal lesion; therefore, small bowel biopsies were not performed. Immunologic studies failed to demonstrate any of the abnormalities frequently described in dermatitis herpetiformis. Both boys responded dramatically to sulfapyridine therapy.

The infant described in this report had been generalized, subepidermal bullous eruption and concurrent glomerulonephritis. Immunofluorescence studies demonstrating deposition of immunoglobulin and complement in the skin and kidney suggest a common pathogenesis for the two disorders. The elevation of this infant's serum immunoglobulins and depression of serum complement support an immunologic process as the likely mechanism. It is important for clinicians caring for children with bullous skin diseases to search for evidence of renal disease and complement abnormalities.

Cicatricial pemphigoid has been documented previously in only four patients under the age of 20 years. We report a 6-year-old male who had erosions of the oral and genital mucosa, conjunctivitis, hoarseness, dysphagia, recurrent vomiting, and weight loss. Upper airway obstruction due to a chronically inflamed and scarred epiglottis necessitated tracheostomy. Biopsy of tissue from a solitary cutaneous lesion demonstrated a subepidermal bulla. Direct and indirect immunofluorescence confirmed a diagnosis of cicatricial pemphigoid. Therapy was begun consisting of prednisone, 2 mg per kg per day, in combination with dapsone, 2 mg per kg per day. He did not improve until the prednisone dosage was increased to 4 mg per kg per day.

Palmoplantar pustules may rarely be associated with chronic recurrent multifocal osteomyelitis in children and young adults. Additional features of this disorder are recurrent low-grade fevers and bone pain. Skeletal radiographs and bone films suggest osteomyelitis, but bone cultures yield no growth of organisms. The cause of this disorder is unknown, and treatment remains unsatisfactory.