Diaper Rash












CHAPTER 6
DIAPER RASH

 


Langerhans Cell Histiocytosis







































Synonyms Langerhans cell histiocytosis (LCH), Langerhans cell disease (LCD), histiocytosis X, eosinophilic granuloma of bone, Hand-Schüller-Christian disease, Letterer-Siwe disease, and Hashimoto-Pritzker disease. LCD is the suggested unifying diagnostic term and is divided into single-organ disease (eg, skin or bone) and multi-organ disease.
Inheritance Rare familial inheritance.
Prenatal Diagnosis n/a
Incidence 1 in 200,000 children annually.
Age at Presentation Depends on type; overall most commonly in children <15 years old; peak incidence is 1 to 3 years old; 3:2 female-to-male ratio; less commonly affects adults.
Pathogenesis Unknown trigger; reactive versus neoplastic process is debated; leads to the accumulation of abnormal dendritic cells (CD1a+/CD207+ histiocytes) derived from immature myeloid precursor cells causing local destruction in the affected tissue.
Key Features

Depends on the location and extent of disease; two-thirds of children present with single-system involvement; skin-only disease present in only ~2% of LCH cases. Skin and lymph node manifestations in children present as part of multisystem disease in about 90% of cases and are associated with fever, hepatosplenomegaly, bone lesions, and lung symptoms.


The most common organs of involvement are bones (80%) and skin; pituitary system (>25%); liver, spleen, hematopoietic system, lymph nodes, and lungs (<15%).



  • Neonate: Red-brown papules and nodules with or without crust; vesicles or eroded papules or nodules in a newborn; blueberry muffin rash.
  • Older children/adolescents: Pink to red scaly or crusted papules and patches on the scalp and trunk; macerated, pink to red patches in flexural areas or perineum; pustules, vesicles, or petechiae and purpura; localized yellow or bronze-colored plaques and macules; deep, painful acneiform nodules; pink- or flesh-colored, ulcerated or umbilicated papules, plaques on the genitalia; oral ulcers or gingivitis or mucosal bleeding; rare presentation of vitiligo-like patches, chalazion, subungual pustules.
  • Bone: ~80% of cases; osteolytic lesions or solid masses most commonly skull but any bone can be involved except those of the hands and the feet.
  • Mucocutaneous: Diverse presentation.
  • Endocrine: Diabetes insipidus and/or growth hormone deficiency associated with pituitary infiltration (>25% of patients).
  • Pulmonary: <15%; can be associated with difficulty breathing or cough; more common in the third to fourth decades of life.
  • Liver: Sclerosing cholangitis.
  • Eosinophilic granuloma of bone (chronic unifocal LCH): Solitary bony lesions in young adults; calvaria, mandible, vertebrae, scapula, ribs, ilium, and femur; usually asymptomatic; if lesions extend deeper, then one may see characteristic symptoms (spontaneous fractures, neurologic symptoms, proptosis, tooth loss, pituitary dysfunction).
  • Hashimoto-Pritzker disease (congenital self-healing reticulohistiocytosis): Very rare; single or multiple red-purple or brown papules and nodules at birth or soon thereafter; classic signs and symptoms include congenital or early development of painless papules, nodules, or plaques with spontaneous regression in 2 to 3 months; most patients have multiple lesions, but in about 25% of cases, there is only 1 lesion.
  • Hand-Schüller-Christian disease (classic multifocal LCH): ~25% of patients with multifocal disease; triad of exophthalmos, lytic bone lesions (most often in skull; will present with recurrent episodes of otitis media or mastoiditis), and diabetes insipidus (due to pituitary stalk infiltration; can have polyuria/polydipsia); lesions in liver (20%), spleen (30%), and lymph nodes (50%) with possible pulmonary involvement; peak age onset 2 to 10 years.
  • Letterer–Siwe disease (acute disseminated LCH): 10% of LCH disease; the most severe form; 1:500,000; almost exclusively occurs in children <2 years; skin lesions (~80%) are often the first sign and extensive, seborrheic dermatitis appearance; ear drainage, lymphadenopathy, osteolytic lesions are not common, and hepatosplenomegaly; oncogenic mutation of BRAF in 50% to 70%; often rapidly fatal, 5-year survival ~50%; thrombocytopenia is a poor prognostic sign.
Differential Diagnosis Depends on the presentation, includes seborrheic dermatitis, psoriasis, atopic dermatitis, contact/diaper dermatitis; TORCH infections, dermatophytes, varicella, herpes simplex virus (HSV), impetigo or candidiasis, purpura, congenital syphilis; hidradenitis suppurativa or other follicular disorders; malignancies; lichen aureus; erythema toxicum neonatorum, transient neonatal pustular melanosis, ulcerated hemangiomas.
Laboratory Data Wood’s lamp examination, skin scraping for potassium hydroxide (KOH), viral culture or viral polymerase chain reaction, serology for viral infection or syphilis, skin biopsy, bone marrow biopsy, lymph node biopsy, x-rays.
Management

  • Skin lesions: Usually no treatment is necessary; consider topical steroids or nitrogen mustard.
  • Referral to oncology: Solitary (noncutaneous) lesions may be treated with curettage or low-dose ionizing radiation; for multi-organ disease a 12-month course of systemic steroids and vinblastine.
Prognosis Often, LCH is a self-limited disease, but high risk forms risk morbidity and death, especially in children <2 years old; depends on the number of organs involved, degree of organ dysfunction, and response to induction chemotherapy in the first 6 weeks; BRAFV600E mutation may predict treatment resistance to induction therapy. Overall, 1-year survival is 80% and 5-year survival is 70%. Mortality is associated with CNS involvement.

image PEARL/WHAT PARENTS ASK


Risk factors include maternal perinatal as well as neonatal infections, blood transfusions during infancy, Hispanic ethnicity, socioeconomic factors and in vitro fertilization. Protective factors include black race, childhood vaccinations, and supplemental vitamins. Common long-term complications include hypothalamic-pituitary dysfunction, cognitive delay, cerebellar disease, growth retardation; less commonly, tooth loss, pulmonary fibrosis, chronic liver disease may occur; long-term risk for second malignancy; most children do well with the resolution of skin and internal lesions, but there is a long-term risk of recurrent of more disseminated disease, so affected children need to be followed carefully for a decade or longer. LCD is part of the Langerhans group of histiocytic diseases, which includes Erdheim-Chester disease (ECD), mixed LCH/ECD, extracutaneous juvenile xanthogranuloma, and indeterminate cell histiocytosis.

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6.1. Langerhans cell histiocytosis.

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6.2. Langerhans cell histiocytosis.











Skin | Associated Findings
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Candidiasis
























Synonym Diaper dermatitis, intertrigo, thrush.
Inheritance n/a
Prenatal Diagnosis n/a
Incidence Common.
Age at Presentation Neonatal candidiasis presents in the first week of life, may present at any age when the barrier function is compromised on skin, mucous membranes, and, particularly, at the mucocutaneous junction. More common at any age in immunosuppressed individuals.
Pathogenesis

Yeast from the Candida genus; normally colonize gastrointestinal tract, mucous membranes, and/or skin, including the perianal area as early as 4 weeks of age; Candida albicans is the most common disease-associated species, others include Candida tropicalis, Candida glabrata, and Candida auris; acute infection occurs commonly in areas of irritation and in intertriginous skin folds; can also be associated with diabetes, immunosuppression (eg, HIV, cancer, and medications), and prolonged antibiotic therapy. Other risk factors include infancy (especially prematurity), occlusive clothing, chronic digit sucking, and warm climate.

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Aug 17, 2025 | Posted by in PEDIATRICS | Comments Off on Diaper Rash

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