Pustular Melanosis Research Articles

Erythematous Maculopapular Rash Covering Entire Body

Erythematous maculopapular rash covering entire body.Physical examination shows an erythematous maculopapular rash covering the infant’s entire body, including the face, trunk, and extremities, but no rash on the palms and soles. Lesions are in various stages of healing, with some appearing vesicular. Mucous membranes are spared. The baby is in no distress and has no other significant findings on examination.The baby undergoes a full sepsis evaluation, including lumbar puncture. Intravenous ampicillin, gentamicin, and acyclovir therapy is started. An umbilical venous line is placed due to difficulty in obtaining percutaneous venous access.The penile foreskin that was removed during circumcision contains a lesion and is sent to pathology in lieu of a skin biopsy.Erythematous maculopapular rash over entire bodyCongenital self-healing Langerhans cell histiocytosisCongenital self-healing Langerhans cell histiocytosis (CSHLCH) is a rare, usually benign, form of Langerhans cell histiocytosis. It was first reported by Hashimoto and Pritzker, who described multiple reddish-brown papulonodules in a newborn girl that showed infiltrating reticulohistiocytes on the skin biopsy and resolved without treatment within a few months. (1) The condition is now alternatively known as Hashimoto-Pritzker disease.The presentation of CSHLCH may mimic other disorders, requiring a thorough evaluation. The other conditions include neonatal hemangiomatosis, (2) congenital infections, pustular melanosis, erythema toxicum, erythropoiesis dermica, and congenital leukemia. (3)In addition to its clinical presentation of a spontaneous eruption of crusted reddish-brown nodules and papules all over the body, the diagnosis is confirmed by pathologic examination of skin containing the papular lesion. Characteristic histologic findings are positive staining for S-100 protein and membranous staining for CD1a antigen in lesional cells. Birbeck granules may also be seen by electron microscopy. Low-density microscopy may show eosinophils or multinucleated giant cells.Kapur and associates(4) published a case series from a 10-year period at Dallas Children’s Medical Center that consisted of 11 cases. All but one of the cases presented at birth; the single other case was diagnosed at 1 month of age. All of the affected infants had only skin involvement at presentation, and all were alive and without evidence of disease at follow-up evaluation.The prognosis for infants who have cutaneous CSHLCH without any other organ involvement is excellent. However, Larralde and associates(5) and others suggest an initial thorough screening evaluation. Such a screening should include a full physical examination; complete blood count; serum chemistry; liver function tests; skull, chest, and long bone radiographs; and abdominal ultrasonography. Bone marrow examination should be performed based on the degree of suspicion of progressive disease. Skin biopsy confirms the diagnosis and is helpful in diagnosing future relapses. Children who have CSHLCH require close follow-up for signs of disease progression, which underscores the importance of making an early diagnosis.JoDee M. Anderson, MD, MEd, Division of Neonatal Medicine, Oregon Health & Science University, Portland, OR.

Necrotic, ulcerated papules on a newborn male

A 1-day-old Hispanic boy presented to our department with truncal lesions that had been present since birth. The infant was born at term without prenatal or perinatal complications and was otherwise healthy. The physical examination revealed an afebrile patient with three 5-mm eroded papules with black eschars—one each on the right posterior axilla, left posterior axilla, and right buttock (Fig 3). Two 4-mm punch biopsy specimens were taken from two of the lesions. Histologic examinations of the ulcerated skin revealed an infiltrate of large, pleomorphic cells with oval to indented nuclei admixed with some neutrophils, eosinophils, and lymphocytes. Mitoses were readily identified (Fig 4). The infiltrate was heaviest in the papillary dermis, with focal epidermotropism and extension into the reticular dermis. Immunohistochemical stains were strongly positive for S-100 protein (Fig 5) and CD1a in the papillary dermal proliferation. 6.What is the most likely diagnosis? a.Congenital candidiasis b.Neonatal disseminated hemangiomatosis c.Langerhans cell histiocytosis d.Congenital leukemia e.Neonatal pustular melanosis 7.Immunohistochemical stains of this condition show that the cells demonstrate which of the following? a.S-100 b.CD1a c.Interferon-γ receptor d.Placental alkaline phosphatase e.All of the above 8.A higher degree of which of the following findings suggests a self-healing process as opposed to a multisystem disease? a.Necrosis b.Ulceration c.Dense infiltrate of eosinophils d.All of the above e.None of the above 9.Treatment of this condition when limited to the skin includes which of the following? a.Systemic chemotherapy b.Immediate excision of the lesions c.Long-term follow-up d.All of the above e.None of the above View Large Image Figure Viewer Download Hi-res image View Large Image Figure Viewer Download Hi-res image

The dermatologist in the newborn nursery: Approach to the neonate with blisters, pustules, erosions, and ulcerations

More than 30 conditions may result in blistering, pustules, erosions, or ulcerations in the newborn. Some of these, such as erythema toxicum neonatorum, pustular melanosis, and miliaria, are very common, innocuous, and self-limited. Others, such as herpes simplex infection, are life-threatening and require immediate intervention. Still others, such as epidermolysis bullosa or epidermolytic hyperkeratosis, herald a life-long chronic illness. The wide array of potential causes of these findings makes a systematic approach to differential diagnosis imperative. A careful prenatal and perinatal history, as well as a history and complete physical examination of the infant, is the basis for further evaluation. The differential diagnosis can be divided into infectious causes, transient skin lesions, most of which are common, and uncommon and rare causes. The possibility of an infectious cause should always be considered first, because most neonatal infections are treatable, and many, if left untreated, can be fatal. The differential diagnosis may also be narrowed by the specific clinical morphology. In some conditions, vesiculopustular lesions predominate; in others, blistering predominates. In yet other conditions, erosions and/or ulcerations predominate. Laboratory tests, including Tzanck and potassium hydroxide (KOH preparations, and Gram stain and Wright's stain of lesional skin, are often helpful. Bacterial, viral, or bacterial cultures from multiple sites including the skin should be performed when infection is a possible cause. Skin biopsy may be necessary, particularly if the diagnosis is not apparent from direct smears or from the clinical morphology. The many causes of blisters, pustules, erosions, and ulcerations are discussed. Emphasis is placed on their incidence in the newborn period, key points of the history and physical examination, confirmatory laboratory tests, and approaches to management.