ABSTRACT
BACKGROUND/OBJECTIVES: Complications of hematopoietic stem cell transplant (HSCT) include acute graft-versus-host disease (aGVHD). Severe cutaneous aGVHD can present with generalized erythroderma, desquamation, and bullae which can mimic toxic epidermal necrolysis (TEN). TEN occurs in response to a culprit medication. Transplant patients are often on many medications, making it difficult to distinguish between the two conditions. Given that TEN-like aGVHD is rare, we describe a case series of pediatric patients and review the literature. METHODS: This is a multi-institutional case series of children who developed TEN-like aGVHD following bone marrow transplantation. Demographic, clinical, and treatment information was collected. RESULTS: Ten patients were identified. Median age at transplantation was 8.5 years (range 0.12-17 years). Median time from transplant to first skin symptoms was 35 days (range 6-110 days) and to first TEN-like symptoms was 40 days (range 16-116 days). 7/10 had other organ GVHD involvement. All patients were on concurrent medications at time of first skin symptoms including immunosuppression for GVHD prophylaxis, infection prophylaxis or treatment, and pain medication. Treatments for TEN-like aGVHD included immunosuppression. CONCLUSIONS: We observe that patients with > or equal to 50% BSA involvement of their skin with TEN-like aGVHD, extracutaneous GVHD, and lack of reepithelization tend to have poor outcomes. Given the rarity of this condition, multidisciplinary care of these patients is important for accurate and timely diagnosis and treatment.
Subject(s)
Graft vs Host Disease , Hematopoietic Stem Cell Transplantation , Stevens-Johnson Syndrome , Humans , Child , Infant , Child, Preschool , Adolescent , Graft vs Host Disease/diagnosis , Graft vs Host Disease/etiology , Graft vs Host Disease/drug therapy , Bone Marrow Transplantation/adverse effects , Stevens-Johnson Syndrome/diagnosis , Stevens-Johnson Syndrome/etiology , Stevens-Johnson Syndrome/therapy , Hematopoietic Stem Cell Transplantation/adverse effects , Bone Marrow , Acute DiseaseABSTRACT
Striated muscle hamartoma (SMH) is a rare, congenital or acquired, benign tumor that predominantly affects children. Therapeutic management has classically been surgical intervention. We present a pediatric case of a facial plaque-type SMH with spontaneous regression that highlights the importance of clinical observation for a conservative approach.
Subject(s)
Hamartoma/pathology , Muscle, Striated/pathology , Muscular Diseases/pathology , Child , Face , Female , Humans , Infant, Newborn , Remission, SpontaneousABSTRACT
Striated muscle hamartoma (SMH) is an uncommon benign lesion, that is usually congenital, polypoid, and primarily located on the head and neck. The key histopathologic sign is the existence of individualized fascicles of striated muscle affecting the dermis and subcutaneous fat tissue. Here we report the case of a newborn girl with an SMH, who presented with a congenital infiltrated plaque on her chin without any other associations.
Subject(s)
Hamartoma/pathology , Muscle, Striated/pathology , Skin Diseases/pathology , Skin/pathology , Female , Humans , Infant, NewbornABSTRACT
Tularemia is a worldwide zoonosis caused by Francisella tularensis. The most frequent forms of tularemia are ulceroglandular, followed by typhoidal forms, glandular, and oculoglandular. Respiratory involvement is an uncommon presentation. Cutaneous lesions secondary to respiratory infections occur in 30% of cases. We present a case of tularemia with cavitary pneumonia and skin lesions.
