ORF virus infection in children: clinical characteristics, transmission, diagnostic methods, and future therapeutics

Orf virus leads to self-limited, subacute cutaneous infections in children who have occupational or recreational contact with infected small ruminants. Breaches in the integument and contact with animals recently vaccinated for orf may be important risk factors in transmission. Common childhood behaviors are likely important factors in the provocation of significant contact (ie, bites) or in unusual lesion location (eg, facial lesions). Clinician recognition is important in distinguishing orf infection from life-threatening cutaneous zoonoses. Recently developed molecular techniques provide diagnostic precision and newer topical therapeutics may hasten healing.     

Neonatal cutaneous fungal infections

PURPOSE OF REVIEW: Cutaneous fungal infections are not uncommon in newborns and are seen in premature or otherwise immunocompromised neonates as well as in healthy full-term neonates. Healthy newborns can develop clinical manifestations as a result of infection with Candida species or as a result of skin colonization with Malassezia species; cutaneous infection with other fungal pathogens is rare. Immunocompromised and premature neonates, however, are susceptible to infection with opportunistic pathogens and are also at higher risk for invasive infection with common pathogens such as Candida. This review discusses the fungal species associated with cutaneous fungal infection in neonates, emphasizes the relevant clinical features, and also reviews the use of newer antifungal agents, including lipid-associated amphotericin B, voriconazole, and caspofungin. RECENT FINDINGS: Neonatal cutaneous infections with opportunistic fungal pathogens, including Aspergillus and the Zygomycetes, have been reported with increasing frequency as advances in neonatal care have improved the survival rate in very low birthweight neonates. Although these infections are frequently fatal, survival in some neonates has been reported with the use of aggressive surgical debridement and systemic antifungal therapy. Newer antifungal agents, including voriconazole and caspofungin, show promise in the treatment of potentially fatal fungal infections in neonates. SUMMARY: Cutaneous fungal infections in neonates range from generally benign conditions such as congenital candidiasis and neonatal cephalic pustulosis to potentially fatal infections with opportunistic pathogens in very low birthweight or immunocompromised neonates. The prompt recognition and appropriate treatment of cutaneous fungal disease in neonates is critical to the prevention of adverse outcomes.     

Granuloma annulare in the child]

Granuloma annulare is a benign, common, inflammatory skin lesion of unknown etiology that is seen in both adults and children. The typical lesions are single or multiple small cutaneous papules with an annular distribution. The histology is consistent with an area of fibrinoid degeneration of collagen, surrounded by palisading histiocytes and inflammatory cells. There are four clinically distinct subtypes: localized, generalized, subcutaneous and perforating. Usually a spontaneous resolution is expected. Many medical treatments have been proposed but without evidence of efficacy. The association with insulin dependent diabetes is still being discussed.     

Cutaneous Ciliated Cyst with Interspersed Apocrine Features Presenting as a Pilonidal Cyst in a Child

We present a case of a cutaneous ciliated cyst that presented in a 13-year-old female. The lesion was felt to be a pilonidal cyst, based on its location and clinical appearance. This case is unusual because it is the youngest reported example of this very rare lesion. In addition, the admixture of apocrine and ciliated cells is extremely unusual. The histogenesis of cutaneous ciliated cysts is considered. Received April 13, 2000; accepted July 21, 2000.     

Solitary ecthyma gangrenosum (EG) — like lesion consequent to <Emphasis Type="Italic">Candida albicans</Emphasis> in a neonate

Ecthyma gangrenosum (EG) is a well recognized cutaneous lesion most commonly associated with Pseudomonas aeruginosa bacteremia. It usually occurs in immunocompromised and critically ill patients. Uncommonly, EG like lesions have also been observed in patients with fungal infections, like Fusarium Candida, Mucor and Aspergillus. We present a case of EG-Like lesion due to Candida albicans, in a 12 day old neonate.     

Langerhans cell histiocytosis presenting in the neonatal period: a retrospective case series.

OBJECTIVES: To describe the morphologic characteristics of skin lesions, extent of extracutaneous disease, and outcomes in patients with neonatal presentation of Langerhans cell histiocytosis (LCH), and to examine clinical predictors of disease prognosis. DESIGN: Retrospective validation cohort study. Maximum duration of follow-up was 10 years. SETTING: A tertiary care children's hospital in Chicago, Ill. PATIENTS: Nineteen children with cutaneous findings in the first 4 weeks of life and subsequently diagnosed with LCH based on compatible tissue histologic analysis, confirmed by electron microscopy and/or immunohistochemical analysis. MAIN OUTCOME MEASURE: Cutaneous lesion morphologic characteristics, extracutaneous manifestations, treatments, and outcomes were tabulated and compared. RESULTS: The most common initial skin lesion was erythematous, often crusted, vesiculopustules. Skin lesion morphologic traits did not correlate with extent of extracutaneous disease. One third of patients had disease limited to the skin and/or mucous membranes. All of these patients are alive and well, and 1 has developed diabetes insipidus. Twelve of the 19 patients had multisystem disease, and 2 died of disease. The results of a multiorgan workup performed at the time of diagnosis were predictive of which patients in this cohort manifested multisystem disease. The overall incidence of diabetes insipidus was 21%. CONCLUSIONS: Vesiculopustular lesions are common in congenital/neonatal LCH, but the morphologic characteristics of lesions are not helpful in predicting the extent of disease. A multiorgan evaluation at the time of diagnosis may be predictive of the probability of multisystem involvement with LCH.     

Intracardiac Juvenile Xanthogranuloma in a Newborn

Juvenile xanthogranuloma (JXG) presents a normolipemic non-Langerhans cells histiocytosis. JXG usually presents with cutaneous lesions. Visceral involvement is rare but may affect various organs. Deep JXG differs histologically from the cutaneous form by its tendency to consist solely of homogeneous proliferation of histiocytes without any xanthomatous or Touton giant cells. Awareness of the possibility of this atypical presentation of JXG helps in making the correct histologic diagnosis, which is supported by proving adequate immunomarkers on histiocytes (mainly PG-M1, an antibody against the CD68 antigen). JXG may present with intramuscular lesions only; however, rarely JXG has been reported to affect the heart but not without the typical cutaneous manifestations. We present an unusual case of deep JXG without systemic disease or metabolic abnormalities. To our knowledge, this is a first reported case of intracavitar JXG without skin lesions.     

Azathioprine hypersensitivity presenting as sweet syndrome in a child with ulcerative colitis

Sweet syndrome is a cutaneous lesion characterized by tender, red inflammatory nodules or papules. We describe a pediatric case of Sweet syndrome presenting 10 days after treatment with azathioprine. As azathioprine is widely used in children with inflammatory bowel disease, clinicians should be aware of this unusual adverse reaction.     

Pediatric gastrointestinal basidiobolomycosis

Basidiobolomycosisis an unusual fungal infection that manifests in the skin and rarely involves other systems including the gastrointestinal tract. We retrospectively reviewed records of six pediatric patients (< or =14 years of age) diagnosed with gastrointestinal basidiobolomycosis from March 2000 to March 2002. Four patients came from the same region, suggesting environmental exposure. Basidiobolomycosis should be considered in the differential diagnosis in pediatric patients presenting with abdominal mass and eosinophilia.     

Congenital cutaneous candidiasis: a case presentation

When an infant presents in the delivery room with macular and papular skin lesions covering the trunk, extremities, and/or skin folds, the neonatal nurse practitioner covering deliveries must be aware of possible skin lesion differential diagnoses. Among these is congenital cutaneous candidiasis, a rare, usually benign skin infection. If this condition is recognized early, unnecessary testing and treatment of newborns who present with these symptoms might be avoided.     

Congenital self-healing histiocytosis-X

Histiocytosis-X (H-X) is heterogeneous clinically, varying from localized benign forms to disseminated fatal forms, but has a relatively uniform and specific pathologic appearance, both by light and electron microscopy. H-X localized to bone or lung has long been recognized. Much less frequent are patients with H-X localized to skin. We report two infants with congenital self-healing H-X, clinically confined to the skin. Prediction of the clinical course from the histology of a H-X lesion is unreliable, especially for cutaneous lesions. We feel that patients with localized forms of H-X should be followed closely for progression of disease but should not be treated aggressively until time is allowed for spontaneous resolution.     

A Male Patient with Humoral Hypercalcemia of Malignancy (HHM) with Leukocytosis Caused by Cutaneous Squamous Cell Carcinoma Resulting from Recessive Dystrophic Epidermolysis Bullosa

Recessive dystrophic epidermolysis bullosa (RDEB) is a severe skin disorder. Although the patients are at risk for cutaneous squamous cell carcinoma (SCC), no case of cutaneous SCC derived from RDEB with humoral hypercalcemia of malignancy (HHM) has been reported. We present the first case report of a male patient with HHM with leukocytosis caused by cutaneous SCC resulting from RDEB. A 20-yr-old Japanese male patient with RDEB; the diagnosis was confirmed by electron microscopic examination, suffered an intractable skin ulcer and hypercalcemia and leukocytosis. PTH-rP, SCC antigen and Granulocyte colony-stimulating factor (G-CSF) levels were elevated. The histological diagnosis of the skin lesion was made well-differentiated SCC. Immunohistochemical staining showed the expression of PTH-rP in atypical tumor cells. For the control of hypercalcemia before an amputation, we used zoledronate safely and could control the serum Ca concentration in the normal range. After the amputation of his right leg including SCC, leukocytosis improved immediately and PTH-rP in blood decreased to the normal range. One month after the amputation, local recurrence of cutaneous SCC and multiple lung metastases were observed. PTH-rP increased gradually associated with hypercalcemia. Although the patient reached an unfortunate turning point about 4 mo after the amputation, we propose that zoledronate is an effective and safe treatment for HHM with cardiorenal complications.     

LYMPHANGIOMATOSIS OF THE BODY WALL: A Report of Two Cases Associated with Chylothorax and Fatal Outcome

We report on two cases of an unusual but distinctive variant of lymphangiomatosis, presenting at birth with predominantly cutaneous involvement of the body wall and complicated by chylothorax. The lesion manifested clinically as a slowly progressive, diffuse, and fluctuant skin swelling. Eventually, almost the entire trunk became affected. There were no bone lesions. Histologically, the soft tissues were diffusely infiltrated by interconnecting mazelike lymphatic vessels. The two infants died from infection at the age of 23 days and 10 months, respectively. Our experience confirms that premortem histologic diagnosis of lymphangiomatosis is difficult to establish. Awareness of the condition and knowledge of its various clinical presentation forms are essential for proper recognition, assessment of the outcome, and evaluation of new therapeutic measures such as interferon.     

Type 1-primary cutaneous meningioma of the scalp.

Type 1-primary cutaneous meningioma is a rare and often clinically unsuspected lesion of the scalp, forehead or paravertebral region which occurs at birth or during childhood. The pathogenesis of these lesions still remains uncertain. Several authors have emphasized that type 1-cutaneous meningiomas are not real tumors but sequestrated meningoceles or heterotopic meningeal nodules of the skin. Nevertheless, the search for an intracranial or intravertebral connection should be carried out. We describe the clinical and pathological features of a congenital type 1-meningioma of the vertex. No cranial defect or intracranial tumor was found. However, the cutaneous lesion was directly linked to a large cranial vein. The purpose of this study was to illustrate this rare lesion and to indicate the possible surgical risks and the pathological characteristics.     

Congenital solid neck mass: a unique presentation of Langerhans cell histiocytosis

Langerhans cell histiocytosis (LCH) presenting in the neonatal period is very rare. In most cases, a self-limited cutaneous disease is the exclusive manifestation. We report an unusual case of neonatal LCH presenting with a large congenital solid neck mass without skin lesions. LCH should be considered in the differential diagnosis of solid masses in neonates and prompt physicians to search for visceral organ involvement.     

A boy born with multiple lesions of atrophoderma

Aims: The Atrophodermas include a large group of disorders appearing as localized or widespread depressed skin areas and characterized by underlying dermal atrophy. The present study aims to report a peculiar form of previously unreported focal dermal atrophy. Methods: We studied over a period of 5 years a boy who manifested, since birth, multiple hypopigmented cutaneous atrophic lesions of the atrophoderma type in a mosaic distribution over the body and the legs. Results: This boy did no develop other cutaneous or systemic stigmata except for an idiopathic thrombocytopenic purpura (ITP) manifested at age 2 years. Full serum, metabolic and infective analyses; full ophthalmological examination; ultrasound examination of the heart and internal organs; skeletal x‐rays; brain magnetic resonance imaging; and DNA analysis of the PORCN (Focal Dermal Hypoplasia ‐ FDH) gene in this boy yielded normal results. Pathological analysis of multiple skin specimens from an affected area revealed slightly reduced dermal thickness; hyperpigmentation of the basal layer; homogenized and disarrayed collagen bundles; perivascular chronic infiltrates of lymphocytes and histiocytes; and normal skin appendages. Currently, the child is healthy; he has mildly improved skin status with less‐evident skin depression throughout the lesion areas and no further complication has been recorded. The histological and clinical appearance of the skin lesions and the course were against any known disorder in the group of the atrophodermas. Conclusions: The cutaneous lesions seen in this boy represent a possibly new congenital skin disorder characterized by multiple, benign areas of focal dermal atrophy in a mosaic distribution.     

Cutaneous lymphohistiocytic infiltrates in patients with hypogammaglobulinemia

Six patients (5 to 15 years of age) with hypogammaglobulinemia had scattered papular lesions in association with widespread dermatitis. These disfiguring lesions were a portal of entry for secondary infections and led to social withdrawal. Biopsy specimens from the papules demonstrated dense lymphohistiocytic infiltrate limited to the dermis. Infectious causes (bacterial, fungal, and viral) were excluded. The skin lesions failed to respond to conventional treatment, including topical corticosteroids. Replacement therapy with monthly infusions of high doses of immune serum globulin resulted in gradual improvement and ultimate clearing of the lesions. This unusual skin abnormality, which is unresponsive to traditional treatment, may provide the earliest clue to the presence of hypogammaglobulinemia.     

Cutaneous polyarteritis nodosa in a patient with ulcerative colitis

Skin disease is common in patients with inflammatory bowel disease. Described herein is a child with ulcerative colitis and cutaneous polyarteritis nodosa. Review of the literature suggests cutaneous polyarteritis must be considered as another skin lesion associated with inflammatory bowel disease. Cutaneous polyarteritis tends to run a chronic relapsing course independent of bowel disease, however.