Pityriasis lichenoides et varioliformis acuta

Pityriasis lichenoides et varioliformis acuta
ICD-10	L41.0
ICD-9	696.2
eMedicine	derm/334

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Kiran Singh, M.D. [2]

Overview

Pityriasis Lichenoides et Varioliformis Acuta, or Mucha Habermann's Disease, short form PLEVA, is a disease of the immune system. It is the more severe version of Pityriasis lichenoides chronica. The disease is characterized by rashes and small lesions on the skin. The disease is most common in males and usually occurs in childhood, although it has been seen in every age group and every race. It is possible for the disease to go into remission for short periods of time or forever.

Historical Perspective

• [Disease name] was first discovered by [scientist name], a [nationality + occupation], in [year] during/following [event].
• In [year], [gene] mutations were first identified in the pathogenesis of [disease name].
• In [year], the first [discovery] was developed by [scientist] to treat/diagnose [disease name].

Classification

• [Disease name] may be classified according to [classification method] into [number] subtypes/groups:

• [group1]
• [group2]
• [group3]

• Other variants of [disease name] include [disease subtype 1], [disease subtype 2], and [disease subtype 3].

Pathophysiology

• The pathogenesis of [disease name] is characterized by [feature1], [feature2], and [feature3].
• The [gene name] gene/Mutation in [gene name] has been associated with the development of [disease name], involving the [molecular pathway] pathway.
• On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
• On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].

Causes

There is no known cause of this disease; however, many links from viruses, vaccines, and other illnesses have been made. The easiest way to understand this disease is to think of it like this: Everyone has T cells that fight disease; in a person with PLEVA, the T cells get mixed up and decide to form in the skin, causing the lesions. It is unknown why this happens.

The trigger which causes the dysfunctional immune system response, PLEVA, is unknown; bacterial, viral, and environmental causes are suspected, but not conclusively demonstrated.

Differentiating [disease name] from other Diseases

• [Disease name] must be differentiated from other diseases that cause [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as:

• [Differential dx1]
• [Differential dx2]
• [Differential dx3]

Epidemiology and Demographics

• The prevalence of [disease name] is approximately [number or range] per 100,000 individuals worldwide.
• In [year], the incidence of [disease name] was estimated to be [number or range] cases per 100,000 individuals in [location].

Age

• Patients of all age groups may develop [disease name].

• [Disease name] is more commonly observed among patients aged [age range] years old.
• [Disease name] is more commonly observed among [elderly patients/young patients/children].

Gender

• [Disease name] affects men and women equally.

• [Gender 1] are more commonly affected with [disease name] than [gender 2].
• The [gender 1] to [Gender 2] ratio is approximately [number > 1] to 1.

Race

• There is no racial predilection for [disease name].

• [Disease name] usually affects individuals of the [race 1] race.
• [Race 2] individuals are less likely to develop [disease name].

Risk Factors

• Common risk factors in the development of [disease name] are [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].

Natural History, Complications and Prognosis

• The majority of patients with [disease name] remain asymptomatic for [duration/years].
• Early clinical features include [manifestation 1], [manifestation 2], and [manifestation 3].
• If left untreated, [#%] of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
• Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
• Prognosis is generally [excellent/good/poor], and the [1/5/10­year mortality/survival rate] of patients with [disease name] is approximately [#%].

Diagnosis

Diagnostic criteria

It is commonly misdiagnosed as chickenpox or rosacea. The most common way to diagnose is by biopsy. This disease has not been known to be life threatening.

Physical Examination

Skin

Trunk

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  Pityriasis lichenoides et varioliformis acuta. With permission from Dermatology Atlas.
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  Pityriasis lichenoides et varioliformis acuta. With permission from Dermatology Atlas.
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  Pityriasis lichenoides et varioliformis acuta. With permission from Dermatology Atlas.
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  Pityriasis lichenoides et varioliformis acuta. With permission from Dermatology Atlas.
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  Pityriasis lichenoides et varioliformis acuta. With permission from Dermatology Atlas.
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  Pityriasis lichenoides et varioliformis acuta. With permission from Dermatology Atlas.
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  Pityriasis lichenoides et varioliformis acuta. With permission from Dermatology Atlas.
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  Pityriasis lichenoides et varioliformis acuta. With permission from Dermatology Atlas.
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  Pityriasis lichenoides et varioliformis acuta. With permission from Dermatology Atlas.
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  Pityriasis lichenoides et varioliformis acuta. With permission from Dermatology Atlas.
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  Pityriasis lichenoides et varioliformis acuta. With permission from Dermatology Atlas.
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  Pityriasis lichenoides et varioliformis acuta. With permission from Dermatology Atlas.
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  Pityriasis lichenoides et varioliformis acuta. With permission from Dermatology Atlas.
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  Pityriasis lichenoides et varioliformis acuta. With permission from Dermatology Atlas.
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  Pityriasis lichenoides et varioliformis acuta. With permission from Dermatology Atlas.
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  Pityriasis lichenoides et varioliformis acuta. With permission from Dermatology Atlas.
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  Pityriasis lichenoides et varioliformis acuta. With permission from Dermatology Atlas.
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  Pityriasis lichenoides et varioliformis acuta. With permission from Dermatology Atlas.
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  Pityriasis lichenoides et varioliformis acuta. With permission from Dermatology Atlas.
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  Pityriasis lichenoides et varioliformis acuta. With permission from Dermatology Atlas.
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  Pityriasis lichenoides et varioliformis acuta. With permission from Dermatology Atlas.
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  Pityriasis lichenoides et varioliformis acuta. With permission from Dermatology Atlas.
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  Pityriasis lichenoides et varioliformis acuta. With permission from Dermatology Atlas.
• 
  Pityriasis lichenoides et varioliformis acuta. With permission from Dermatology Atlas.
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  Pityriasis lichenoides et varioliformis acuta. With permission from Dermatology Atlas.
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  Pityriasis lichenoides et varioliformis acuta. With permission from Dermatology Atlas.
• 
  Pityriasis lichenoides et varioliformis acuta. With permission from Dermatology Atlas.
• 
  Pityriasis lichenoides et varioliformis acuta. With permission from Dermatology Atlas.

Treatment

It is not contagious and currently there is no cure for the disease, although the lesions can be treated with ultraviolet therapy as well as topical steroids and antibiotics.

Treatment often involves multiple therapies that address the immune system and bacterial, viral, or dermatological causes.

See also

• Parapsoriasis

External links

• Template:DermNet

Template:WikiDoc Sources