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Author: Stewart P Adams, MD, FRCPC, Clinical Assistant Professor, Department of Medicine, University of Calgary Faculty of Medicine

Stewart P Adams is a member of the following medical societies: American Academy of Dermatology, American Medical Association, and Canadian Medical Association

Coauthor(s): Patricia T Ting, MD, Staff Physician, Department of Medicine, University of Calgary

Editors: Timothy McCalmont, MD, Director, UCSF Dermatopathology Service, Professor of Clinical Pathology and Dermatology, Departments of Pathology and Dermatology, University of California at San Francisco; David F Butler, MD, Professor of Dermatology, Texas A&M University College of Medicine; Director, Division of Dermatology, Scott and White Clinic; Director Dermatology Residency Training Program, Scott and White Clinic; Robert A Schwartz, MD, MPH, Professor and Head of Dermatology, Professor of Medicine, Professor of Pediatrics, Professor of Pathology, Professor of Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School; Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University; Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center

Author and Editor Disclosure

Synonyms and related keywords: unilateral laterothoracic exanthem of childhood, ULE, APEC, papular erythema

Background

In 1962, Brunner et al reported a "new papular erythema" in 75 children aged 6 months to 5 years. Later, in 1992, Bodemer and de Prost published a case series of 18 children and named the condition unilateral laterothoracic exanthem (ULE). In 1993, Taieb and colleagues suggested the term asymmetric periflexural exanthem of childhood (APEC) to replace ULE, as the latter did not fully depict the morphologic distribution of the skin lesions present in this condition. APEC is classified as a rare self-limited and spontaneously resolving exanthem with unknown etiology that occurs in children. To date, only 3 case presentations in adults have been documented.

Pathophysiology

The etiology of APEC is unknown. The patient's history (eg, age at presentation, multiple affected children in a family), lack of efficacy of broad-spectrum antibiotic treatment, serologic findings, and the tendency for presentation during spring and winter raise the possibility of a viral etiology. However, the evidence has been inconclusive, and clinicians have not been able to isolate a specific virus. Therefore, this hypothesis has never been confirmed.

APEC manifests as an exanthem with stereotypical morphology and distribution. Biopsy is rarely if ever performed, as the presentation of this condition is unique and resolves spontaneously without treatment or adverse sequelae.

Frequency

United States

APEC is a relatively rare condition that often appears in spring and winter months.

International

Approximately 300 cases have been reported in the literature. Case series of affected children have been documented internationally from the United States, Canada, and Europe.

Mortality/Morbidity

None is reported.

Race

APEC predominantly affects individuals from light-skinned ethnic groups.

Sex

APEC tends to affect females more frequently than males, with an estimated female-to-male ratio of 2:1.

Age

  • The average age of presentation is 2 years, though affected children may be aged 4 months to 10 years.
  • Four cases of APEC in adults have been reported in the literature.



History

  • Most affected children are healthy and asymptomatic at presentation, with an unremarkable medical history.
  • Occasionally, patients may report a current and/or recent episode of upper respiratory tract infection, adenopathy/lymphadenopathy, fever, otitis media, or diarrhea.
  • In rare instances, other children in the family may also have APEC.
  • Mild pruritus is reported in approximately 50% of patients.

Physical

  • The primary (pathognomonic) lesion is a small erythematous papule with a surrounding pale halo. The general appearance of lesions includes a morbilliform, eczematous, and occasionally reticulated group of macules, papules, or coalescent plaques. These are occasionally accompanied with fine scaling.
  • At the initial onset, lesions are unilateral and usually begin near the axillae, lateral trunk, and upper inner arm or groin. During the course of the condition, lesions often progress bilaterally with an asymmetric predominance.
  • The 4 sequential stages of the lesions are as follows:
    • Eczematous, when initial lesions occur on the axillae and lateral chest wall
    • Coalescence, when lesions extend to the trunk and proximal extremities and are separated by areas of normal skin
    • Regression, when older lesions may develop a central dusky-gray center
    • Desquamation, when residual branlike scale appears and resolves with time
  • APEC lesions spare the face, palms, soles, and mucous membranes.
  • Lichenification is not usually observed.

Causes

The exact cause of this eruption is unknown, and no specific viral pathogens have been identified.



Contact Dermatitis, Allergic
Drug Eruptions
Gianotti-Crosti Syndrome (Papular Acrodermatitis of Childhood)
Milia
Miliaria
Pityriasis Rosea
Scabies
Scarlet Fever
Tinea Corporis

Other Problems to be Considered

Nonspecific viral exanthem



Lab Studies

  • No specific laboratory blood tests are required.
  • APEC may be accompanied by an elevated erythrocyte sedimentation rate.
  • Occasionally, serologic results are positive for respiratory pathogens such as adenovirus and parainfluenza virus.
  • APEC has been reported in a 3-year-old girl following an upper respiratory tract infection and the onset of axonal Guillain-Barré syndrome (acute motor axonal neuropathy).
  • Parvovirus B19 has been associated with APEC in several children and one adult.
  • Most studies have revealed negative serologic results for hepatitis, borreliosis, Mycoplasma organisms, Epstein-Barr virus, cytomegalovirus, parvovirus B19, HIV, coxsackievirus, toxoplasmosis, and rickettsiae.

Imaging Studies

  • No imaging studies are required.

Other Tests

  • No other tests are required.

Procedures

  • No procedures are required.

Histologic Findings

Biopsy is uncommonly performed.

When obtained, microscopic examination reveals a superficial and deep perivascular, interstitial, and periadnexal lymphohistiocytic infiltrate in the dermis. This finding may also be accompanied by epidermal spongiosis and lymphocytic infiltration of the epidermal portion of the eccrine ducts.

Staging

This is an inflammatory disorder of skin, and staging is not performed.



Medical Care

No specific medical treatment is required.

  • Low-potency topical steroids may be used, though a minimal response is expected.
  • Hydroxyzine may be used to alleviate pruritus.
  • The use of moisturizers may be recommended to manage pruritus and fine scaling.

Surgical Care

None is required.

Consultations

No consultations are required.

Diet

APEC is unrelated to diet.



The management of APEC typically does not require the use of prescription medications. Low-potency topical steroids such as hydrocortisone 0.5-1% may be used to control inflammation although it usually offers marginal benefit. Hydroxyzine may also be used if the lesions are pruritic and appear disruptive to daily functioning and interfere with normal sleep patterns.

Drug Category: H1-receptor antagonist antihistamines

These agents prevent the histamine response in sensory nerve endings and blood vessels but are not effective at reversing it. They competitively inhibit the binding of histamine at the H1 receptor. Histamine is responsible for mediating wheal and flare reactions, smooth muscle contraction, bronchial constriction, mucus secretion, edema, CNS depression, hypotension, and cardiac arrhythmias.

Drug NameHydroxyzine hydrochloride (Vistaril, Atarax, Vistazine)
DescriptionAntagonizes H1 receptors in periphery. May also suppress histamine activity in subcortical region of CNS.
Adult Dose50-100 mg PO qd/qid
Pediatric Dose<6 years: 50 mg/d PO (2 mg/kg/d) in divided doses
>6 years: 50-100 mg/d PO in divided doses
All age groups: 1 h qhs
ContraindicationsDocumented hypersensitivity
InteractionsPotentiates effect of CNS depressants and alcohol. Syrup formulation contains alcohol; do not give to patients taking medications with disulfiramlike properties.
PregnancyC - Safety for use during pregnancy has not been established.
PrecautionsCauses drowsiness; caution when operating heavy machinery or motor vehicles; may exacerbate porphyria, angle-closure glaucoma, hyperthyroidism, peptic ulcer, and anticholinergic effects (eg, dry mouth, urinary tract obstruction); ECG abnormalities (T-wave alterations) reported

Drug Category: Topical anti-inflammatory agents

These agents provide relief of inflammatory eczematous lesions.

Drug NameHydrocortisone - topical (LactiCare-HC, Cortaid, Cortate)
DescriptionLow-potency topical corticosteroid with anti-inflammatory activity, as well as mineralocorticoid and glucocorticoid properties. Decreases inflammation by suppressing the migration of polymorphonuclear leukocytes and reversing increased capillary permeability. Use 1% cream.
Adult DoseApply sparingly to affected areas bid/tid as needed
Pediatric DoseApply as in adults
ContraindicationsDocumented hypersensitivity; do not use for bacterial, fungal, or viral infections
InteractionsNone reported
PregnancyC - Safety for use during pregnancy has not been established.
PrecautionsAvoid contact with eyes (risk of cataracts); prolonged or excessive use over large surface areas and with occlusive dressings may increase systemic absorption, leading to Cushing syndrome, reversible HPA-axis suppression, hyperglycemia, and glycosuria



Further Inpatient Care

  • Inpatient care is never required for this self-limited syndrome.

Further Outpatient Care

  • None is required.

Complications

  • No complications have been documented.

Prognosis

  • The prognosis is excellent; the course of the disease is self-limited and spontaneously resolves in 4-6 weeks without medical intervention.

Patient Education

  • The unique presentation and appearance of skin lesions may be a cause of significant concern to the patient and his or her parents or caregivers.
  • The patient should be educated and reassured that APEC a benign, self-limited exanthem without sequelae (eg, systemic symptoms, post-inflammatory hyperpigmentation, scarring, other skin changes).
  • For excellent patient education resources, visit eMedicine's Skin, Hair, and Nails Center and Blood and Lymphatic System Center. Also, see eMedicine's patient education articles Skin Rashes in Children and Swollen Lymph Glands.



Media file 1:  Morbilliformlike eruption in a child with involvement of the axilla, lateral thorax, and abdomen. Used with permission from McCuaig et al (1996) from the Journal of the American Academy of Dermatology.
Click to see larger pictureClick to see detailView Full Size Image
Media type:  Image

Media file 2:  Eczematouslike eruption with a predominantly hemicorporeal distribution photographed on the eighth day after initial appearance of lesions. Used with permission from Bodemer and de Prost (1992) from the Journal of the American Academy of Dermatology.
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Media type:  Image

Media file 3:  Pattern of reticulated plaques on the posterior lower limb of a child. Used with permission from McCuaig et al (1996) from the Journal of the American Academy of Dermatology.
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Media type:  Image

Media file 4:  Histopathologic slide demonstrates perivascular, interstitial, and periadnexal infiltrate of lymphocytes and histiocytes in the deep dermis (hematoxylin-phloxine-saffron stain). Used with permission from McCuaig et al (1996) from the Journal of the American Academy of Dermatology.
Click to see larger pictureClick to see detailView Full Size Image
Media type:  Image

Media file 5:  Histopathologic slide demonstrates epidermal spongiosis and lymphocytic infiltration of the intraepidermal portion of an eccrine duct (hematoxylin-phloxine-saffron stain). Used with permission from McCuaig et al (1996) from the Journal of the American Academy of Dermatology.
Click to see larger pictureClick to see detailView Full Size Image
Media type:  Image



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Asymmetric Periflexural Exanthem of Childhood excerpt

Article Last Updated: Feb 16, 2007