Exploring the Differential Diagnosis of Hemorrhagic Vesicopustules in a Newborn

Abstract

Hemorrhagic vesicles in a newborn present a challenging differential diagnosis including both infectious and neoplastic disorders. Patients should be evaluated in an efficient manner to arrive at the correct diagnosis as quickly as possible. We present here an interesting case that outlines the methodical workup that ultimately revealed the diagnosis of congenital Langerhans cell histiocytosis. After a diagnosis of Langerhans cell histiocytosis is made, it is important to evaluate the patient thoroughly for systemic involvement. Historically, the diagnosis of congenital self-healing Langerhans cell histiocytosis was used to delineate a benign self-limited disorder limited to the skin with spontaneous resolution during the first several months of life; this disorder may also be referred to as "self-regressive Langerhans cell histiocytosis." However, some newborns with initial skin-only Langerhans cell histiocytosis progress to have multisystem disease after spontaneous resolution has occurred. For this reason, the nomenclature is changing. We suggest using the term "skin-only Langerhans cell histiocytosis." Periodic long-term follow-up is recommended to monitor for relapse or progression to systemic disease.

A neonate with a challenging diagnosis (Discussion and Diagnosis)

“…The differential diagnosis of a newborn with vesiculopustules is broad and includes numerous infectious and noninfectious diseases. Infectious disorders to be considered should include HSV, VZV, CMV, syphilis, toxoplasmosis, rubella, candidiasis, bullous impetigo and listeriosis, additionally, noninfectious diseases such as neonatal pustular melanosis, erythema toxicum neonatorum, incontinentia pigmenti, juvenile xanthogranulomas and Langerhans cell histiocytosis (LCH), as well as neoplastic disorders including neuroblastoma and leukaemia . At initial evaluation, paediatrician or neonatologist should first rule out life‐threatening infections such as neonatal herpes and varicella infections and should promptly begin acyclovir therapy and for bacterial infections ampicillin + aminoglycoside combination, as in our case.…”

“…The disease can present at any age, although it is most often seen in infancy or early childhood, often with bone or cutaneous lesions . Cutaneous findings of LCH vary from vesiculopustules, which are the most common, to eczematous patches and plaques such as seborrheic dermatitis, mucosal erosions, erythematous papules, reddish brown nodules and generalized petechiae . Congenital LCH of the skin should be considered, especially in cases where a newborn appears nontoxic .…”

Eroded Papule in a Newborn

“…Other diagnoses that can be excluded by histopathology include Spitz nevus, erythema toxicum neonatorum, acropustulosis of infancy, incontinentia pigmenti, disseminated neonatal hemangiomatosis, and congenital leukemia cutis. LCH and infectious diseases may mimic each other, especially with widespread involvement, but may be excluded with cultures or other clinical clues; these include herpesvirus infection, bullous impetigo, syphilis, cytomegalovirus, rubella, congenital candidiasis, and toxoplasmosis . CSHLCH should also be differentiated from other self‐regressing conditions of infancy such as juvenile xanthogranuloma, generalized eruptive histiocytoma, and indeterminate cell histiocytoma ; histopathology of CSHLCH typically includes positive immunostaining for CD1a, Langerin (CD207), and S100 and negative expression of factor XIIIa, CD68, and HAM56 .…”

“…If LCH is limited to the skin, as it often is with CSHLCH, regression typically occurs without treatment, although topical corticosteroids, tacrolimus, or nitrogen mustard may be tried for persistent lesions . Because there are no definitive criteria to distinguish CSHLCH from disseminated forms of LCH , patients with an initial diagnosis of CSHLCH should have monthly physicals and screening laboratory tests for the first year of life, followed by annual screenings until the age of 5 years and then every other year . Systemic involvement examination should include physical examination, with special attention to lymph nodes, liver, and spleen, as well urine osmolality, hematologic and coagulation studies, liver function tests, abdominal ultrasound, and skeletal survey .…”

“…Because there are no definitive criteria to distinguish CSHLCH from disseminated forms of LCH , patients with an initial diagnosis of CSHLCH should have monthly physicals and screening laboratory tests for the first year of life, followed by annual screenings until the age of 5 years and then every other year . Systemic involvement examination should include physical examination, with special attention to lymph nodes, liver, and spleen, as well urine osmolality, hematologic and coagulation studies, liver function tests, abdominal ultrasound, and skeletal survey . Two years of regular physical examinations, basic laboratory screenings, and radiographs is thought to be sufficient for solitary CSHLCH .…”