Severe clinical presentations of diseases usually highlight important opportunities for aggressivediagnostic testing and treatment
Severe clinical presentations of diseases usually highlight important opportunities for aggressivediagnostic testing and treatment: Excerpt from Avoiding Common Pediatric Errors
Author:
Anjali Subbaswamy, MD
What to Do - Gather Appropriate Data
Consider a diagnosis of Langerhans cell histiocytosis (LCH) as a possibility
in cases of severe seborrheic dermatitis, especially if atrophy, ulceration, or
purpura is present.
Seborrheic dermatitis, commonly known as cradle cap, is frequently
seen in infants and children. Typical lesions are scaly, oily, and located on the
scalp and sometimes behind the ears. A papulosquamous disorder patterned
on the sebum-rich areas of the scalp, face, and trunk, it is commonly aggravated by changes in humidity or seasons, scratching, or emotional stress. It
can present in combination with atopic dermatitis. Seborrheic dermatitis is
uncommon in children after infancy and before puberty. In this older age
group, scalp scaling is likely to be due to other causes such as tinea capitis,
atopicdermatitis,orpsoriasis.Forseverelesions,refractorytotheusualtherapies, an expanded differential diagnosis must be entertained. This includes
disorders with potential for organ involvement, such as LCH.
LCHisagroupofidiopathicdisorderscharacterizedbytheproliferation
of specialized bone marrow-derived Langerhans cells (LCs) and mature
eosinophils.TheBirbeckgranuleisthehistopathologicalhallmark.Theterm
LCH is generally preferred to the older term, histiocytosis X. The Histiocyte Society has divided histocytic disorders into three different groups:
(a) dendritic cell histiocytosis, (b) erythrophagocytic macrophage disorders,
(c) malignant histiocytosis. LCH belongs in group 1. The clinical spectrum
ranges from an acute fulminant, disseminated disease (Letterer Siwe) to a
few indolent, chronic lesions of bone or other organs called eosinophilic
granulomas. Hand-Schüller-Christian disease is an intermediate form that
classicallypresentsasthetriadofdiabetesinsipidus,proptosis,andlyticbone
lesions. Disseminated disease appears more commonly in children younger
than 2 years of age, whereas the chronic and acute disseminated forms are
most common in children ages 2 to 15 years.
An ongoing debate exists over whether this is a reactive or neoplastic
process. The exact pathogenesis is still unclear. Although the disease is rare,
withanannualincidenceof0.5to5casespermillionperyear,itcanbeserious.
More than one half of patients younger than 2 years with disseminated
LCH and organ dysfunction die of the disease, whereas unifocal LCH and
most cases of congenital self-healing histiocytosis are self-limited. Patients
of all ages are affected. Establishing the diagnosis and extent of disease will
facilitate the prompt initiation of appropriate therapy, which ranges from
excision of solitary lesions to steroids or chemotherapy for disseminated
disease.
Signs of LCH depend on the localization and extent of the disease.
The clinical spectrum of LCH is broad. Cutaneous manifestations occur
in up to 80% of patients with disseminated disease. These lesions may be
noduloulcerative in the oral, axillary, perineal, perivulvar, or retroauricular
regions. Extensive coalescence, scaling, or crusting may occur. Lesions consist of closely set petechiae and yellow-brown papules topped with scales and
crust.Thepapulesmaycoalescetoformanerythematous,weepingeruption,
mimicking seborrheic dermatitis. Intertriginous lesions are often exudative,
and secondary infection and ulceration may occur.
The differential diagnosis includes seborrheic dermatitis, Wiskott-
Aldrich syndrome, acrodermatitis enteropathica, erythema toxicum, neonatorum, incontinentia pigmenti, mastocytosis, and acropustulosis of infancy.
Thediagnosticworkupshouldincludehistologicexaminationofskinlesions,
appropriateradiographs,hematologicandchemistryprofiles,andlookingfor
bone and organ involvement when indicated.
Suggested Readings
Braier J, Chantada G, Rosso D, et al. Langerhans cell histiocytosis: retrospective evaluation of
123 patients at a single institution. Pediatr Hematol Oncol. 1999;16(5):377–385.
McDonald LL, Smith ML. Diagnostic dilemmas in pediatric/adolescent dermatology: scaly
scalp. J Pediatr Health Care. 1998;12(2):80–84.
Williams M. Differential diagnosis of seborrheic dermatitis. Pediatr Rev. 1986;7:204–211.
Book Source Details
- Book Title: Avoiding Common Pediatric Errors
- Author(s): Anthony D Slonim MD, DrPH; Lisa Marcucci MD
- Year of Publication: 2008
- Copyright Details: Avoiding Common Pediatric Errors, Copyright © 2008 Lippincott Williams & Wilkins.
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Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.
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More About This Book:
Title: Avoiding Common Pediatric Errors
Authors: Anthony D Slonim MD, DrPH; Lisa Marcucci MD
Publisher: Lippincott Williams & Wilkins
Copyright: 2008
ISBN: 0-7817-7489-6
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