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'''''Synonyms and Keywords:''''' Epithelioid hemangioma; Histiocytoid hemangioma; Inflammatory angiomatous nodule; Intravenous atypical vascular proliferation; Papular angioplasia; Inflammatory arteriovenous hemangioma; Pseudopyogenic granuloma; ALHE
'''''Synonyms and Keywords:''''' Epithelioid hemangioma; Histiocytoid hemangioma; Inflammatory angiomatous nodule; Intravenous atypical vascular proliferation; Papular angioplasia; Inflammatory arteriovenous hemangioma; Pseudopyogenic granuloma; ALHE; EH


==Overview==
==Overview==
Angiolymphoid hyperplasia with eosinophilia <ref>Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 1-4160-2999-0.</ref> is defined as red-brown, dome-shaped, dermal [[papule]] or [[nodule]], present in the head or neck, specifically around the ears and on the scalp.<ref name="Andrews">James, William; Berger, Timothy; Elston, Dirk (2005). ''Andrews' Diseases of the Skin: Clinical Dermatology''. (10th ed.). Saunders. ISBN 0-7216-2921-0.</ref>
Angiolymphoid hyperplasia with eosinophilia was first discovered by G. C. Wells and I. W. Whimster, two British physicians, in 1969.<ref name="pmid5763634">{{cite journal| author=Wells GC, Whimster IW| title=Subcutaneous angiolymphoid hyperplasia with eosinophilia. | journal=Br J Dermatol | year= 1969 | volume= 81 | issue= 1 | pages= 1-14 | pmid=5763634 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=5763634  }} </ref>Angiolymphoid hyperplasia with eosinophilia <ref>Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 1-4160-2999-0.</ref> is defined as red-brown, dome-shaped, dermal [[papule]] or [[nodule]], present in the around the ears, scalp, neck, trunk, extremities, genitalia, lips and oral mucosa.<ref name="Andrews">James, William; Berger, Timothy; Elston, Dirk (2005). ''Andrews' Diseases of the Skin: Clinical Dermatology''. (10th ed.). Saunders. ISBN 0-7216-2921-0.</ref>On gross pathology, smooth-surface red to brown papules or nodules are characteristic findings of angiolymphoid hyperplasia with eosinophilia. On microscopic histopathological analysis, florid vascular proliferation with atypical endothelial cells surrounded by a lymphocytic and eosinophilic infiltrate are characteristic findings of angiolymphoid hyperplasia with eosinophilia. Angiolymphoid hyperplasia with eosinophilia may be caused by either allergic reactions, traumas, or autoimmune disorders. Angiolymphoid hyperplasia with eosinophilia must be differentiated from other diseases that cause swelling of face and neck, such as [[Kimura disease]], acial granuloma, insect bite reaction, [[Lymphoma|cutaneous lymphoma]], [[sarcoidosis]]. The prevalence of angiolymphoid hyperplasia with eosinophilia is unknown as it is an extremely rare disease. Angiolymphoid hyperplasia with eosinophilia is more commonly observed among young patients. Females are more commonly affected with angiolymphoid hyperplasia with eosinophilia than males. Angiolymphoid hyperplasia with eosinophilia usually affects predominently individuals of the Asian race. Common complications of angiolymphoid hyperplasia with eosinophilia include [[pruritis]], pulsation, and spontaneous bleeding. Findings on [[biopsy]] include proliferation of small blood vessels with endothelial cells having a cobblestone appearance, a perivascular and interstitial infiltrate of lymphocytes and [[eosinophils]]. [[Surgery]] is the mainstay of therapy for angiolymphoid hyperplasia with eosinophilia.
==Historical Perspective==
==Historical Perspective==
*[Disease name] was first discovered by [scientist name], a [nationality + occupation], in [year] during/following [event].
*Angiolymphoid hyperplasia with eosinophilia was first discovered by G. C. Wells and I. W. Whimster, two British physicians, in 1969.<ref name="pmid5763634">{{cite journal| author=Wells GC, Whimster IW| title=Subcutaneous angiolymphoid hyperplasia with eosinophilia. | journal=Br J Dermatol | year= 1969 | volume= 81 | issue= 1 | pages= 1-14 | pmid=5763634 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=5763634  }} </ref>
*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==
==Pathophysiology==
*Angiolymphoid hyperplasia with eosinophilia <ref>Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 1-4160-2999-0.</ref> is defined as red-brown, dome-shaped, dermal [[papule]] or [[nodule]], present in the head or neck, specifically around the ears and on the scalp.<ref name="Andrews">James, William; Berger, Timothy; Elston, Dirk (2005). ''Andrews' Diseases of the Skin: Clinical Dermatology''. (10th ed.). Saunders. ISBN 0-7216-2921-0.</ref>
*The pathogenesis of angiolymphoid hyperplasia with eosinophilia is characterized by  red to brown papules or nodules dislocated in the dermis or subcutaneous tissue.
*The pathogenesis of angiolymphoid hyperplasia with eosinophilia is characterized by  red to brown papules or nodules dislocated in the dermis or subcutaneous tissue.
* Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare and idiopathic vascular disorder.
* Angiolymphoid hyperplasia with eosinophilia is a rare and idiopathic vascular disorder.
* Angiolymphoid hyperplasia with eosinophilia (ALHE) is an uncommon benign lesion, primarily occurring in the head and neck.
*On gross pathology, smooth-surface red to brown papules or nodules on the head, neck, trunk, extremities, genitalia, lips and oral mucosa are characteristic findings of angiolymphoid hyperplasia with eosinophilia.
*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, florid vascular proliferation with atypical endothelial cells surrounded by a lymphocytic and eosinophilic infiltrate are characteristic findings of angiolymphoid hyperplasia with eosinophilia.
*On microscopic histopathological analysis, florid vascular proliferation with atypical endothelial cells surrounded by a lymphocytic and eosinophilic infiltrate are characteristic findings of angiolymphoid hyperplasia with eosinophilia.
==Causes==
==Causes==
* Angiolymphoid hyperplasia with eosinophilia may be caused by either allergic reactions, traumas, or autoimmune disorders.
* Angiolymphoid hyperplasia with eosinophilia may be caused by either allergic reactions, traumas, or autoimmune disorders.
==Differentiating Angiolymphoid hyperplasia with eosinophilia from other Diseases==
==Differentiating Angiolymphoid hyperplasia with eosinophilia from other Diseases==
*Angiolymphoid hyperplasia with eosinophilia must be differentiated from other diseases that cause swelling of face and neck, such as:
*Angiolymphoid hyperplasia with eosinophilia must be differentiated from other diseases that cause swelling of face and neck, such as:
:*Kimura’s disease
:*[[Kimura disease]]
 
:*Acial granuloma
:*Insect bite reaction
:*[[Lymphoma|Cutaneous lymphoma]]
:*[[Sarcoidosis]]
==Epidemiology and Demographics==
==Epidemiology and Demographics==
* The prevalence of [disease name] is approximately [number or range] per 100,000 individuals worldwide.
* The prevalence of angiolymphoid hyperplasia with eosinophilia is unknown as it is an extremely rare disease.
* In [year], the incidence of [disease name] was estimated to be [number or range] cases per 100,000 individuals in [location].
===Age===
===Age===
*Patients of all age groups may develop [disease name].
*Angiolymphoid hyperplasia with eosinophilia is more commonly observed among young patients.
*[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===
===Gender===
*Females are more commonly affected with angiolymphoid hyperplasia with eosinophilia than males.
*Females are more commonly affected with angiolymphoid hyperplasia with eosinophilia than males.
===Race===
===Race===
*Angiolymphoid hyperplasia with eosinophilia usually affects predominently individuals of the Asian race.
*Angiolymphoid hyperplasia with eosinophilia usually affects predominently individuals of the Asian race.
==Risk Factors==
==Risk Factors==
*Common risk factors in the development of angiolymphoid hyperplasia with eosinophilia are allergic reactions, traumas or autoimmune disorders.
*Common risk factors in the development of angiolymphoid hyperplasia with eosinophilia are allergic reactions, traumas or autoimmune disorders.
== Natural History, Complications and Prognosis==
== Natural History, Complications and Prognosis==
*The majority of patients with [disease name] remain asymptomatic for
*The majority of patients with angiolymphoid hyperplasia with eosinophilia remain asymptomatic for several years.
[duration/years].
*Common complications of angiolymphoid hyperplasia with eosinophilia include pruritis, pulsation, and spontaneous bleeding.
*Early clinical features include [manifestation 1], [manifestation 2],
* One-third of cases of angiolymphoid hyperplasia with eosinophilia recur when incompletely excised.
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 ==
== Diagnosis ==
===Diagnostic Criteria===
*The diagnosis of [disease name] is made when at least [number] of the
following [number] diagnostic criteria are met:
:*[criterion 1]
:*[criterion 2]
:*[criterion 3]
:*[criterion 4]
=== Symptoms ===
=== Symptoms ===
*[Disease name] is usually asymptomatic.
* Angiolymphoid hyperplasia with eosinophilia lesions may be asymptomatic, painful or pruriginous.
*Symptoms of [disease name] may include the following:
:*[symptom 1]
:*[symptom 2]
:*[symptom 3]
:*[symptom 4]
:*[symptom 5]
:*[symptom 6]
=== Physical Examination ===
=== Physical Examination ===
*Patients with [disease name] usually appear [general appearance].
*Physical examination may be remarkable for:
*Physical examination may be remarkable for:
:*[finding 1]
:*Dome-shaped, smooth-surfaced papules or expanding nodule or group of nodules
:*[finding 2]
:*[finding 3]
:*[finding 4]
:*[finding 5]
:*[finding 6]
=== Laboratory Findings ===
*There are no specific laboratory findings associated with [disease
name].
*A [positive/negative] [test name] is diagnostic of [disease name].
*An [elevated/reduced] concentration of
[serum/blood/urinary/CSF/other] [lab test] is diagnostic of [disease
name].
*Other laboratory findings consistent with the diagnosis of [disease
name] include [abnormal test 1], [abnormal test 2], and [abnormal test
3].
===Imaging Findings===
*There are no [imaging study] findings associated with [disease name].
*[Imaging study 1] is the imaging modality of choice for [disease
name].
*On [imaging study 1], [disease name] is characterized by [finding 1],
[finding 2], and [finding 3].
*[Imaging study 2] may demonstrate [finding 1], [finding 2], and
[finding 3].
=== Other Diagnostic Studies ===
*[Disease name] may also be diagnosed using [diagnostic study name].
*Findings on [diagnostic study name] include [finding 1], [finding 2],
and [finding 3].
== Treatment ==
=== Medical Therapy ===
*There is no treatment for [disease name]; the mainstay of therapy is
supportive care.
*The mainstay of therapy for [disease name] is [medical therapy 1] and
[medical therapy 2].
*[Medical therapy 1] acts by [mechanism of action1].
*Response to [medical therapy 1] can be monitored with [test/physical
finding/imaging] every [frequency/duration].
=== Surgery ===
*Surgery is the mainstay of therapy for angiolymphoid hyperplasia with eosinophilia.
=== Prevention ===
*There are no primary preventive measures available for [disease
name].
*Effective measures for the primary prevention of [disease name]
include [measure1], [measure2], and [measure3].
*Once diagnosed and successfully treated, patients with [disease name]
are followedup
every [duration]. Followup
testing includes [test 1],
[test 2], and [test 3].
 
==Diagnosis==
====Ear====
====Ear====
<gallery>
<gallery>
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Image:Epithelioid hemangioma09.jpg|Epithelioid hemangioma. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  Adapted from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite
Image:Epithelioid hemangioma09.jpg|Epithelioid hemangioma. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  Adapted from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite
Image:Epithelioid hemangioma10.jpg|Epithelioid hemangioma. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  Adapted from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite
Image:Epithelioid hemangioma10.jpg|Epithelioid hemangioma. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  Adapted from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite
</gallery>
</gallery>
=== Laboratory Findings ===
*A eosinophilia in [[complete blood count]] is  characteristic of angiolymphoid hyperplasia with eosinophilia.
*[[Eosinophils]] in [[urinalysis]] is also a feature.
=== Other Diagnostic Studies ===
*Angiolymphoid hyperplasia with eosinophilia may also be diagnosed using [[biopsy]].
*Findings on [[biopsy]] include proliferation of small blood vessels with endothelial cells having a cobblestone appearance, a perivascular and interstitial infiltrate of lymphocytes and [[eosinophils]].
== Treatment ==
=== Medical Therapy ===
*The mainstay of therapy for angiolymphoid hyperplasia with eosinophilia is intralesional corticoid therapy and [[imiquimod]].
*[[Imiquimod]] acts by induction of the production of interferon-α and induction or inhibition of certain cytokines, mainly interleukin-5.<ref name="EstevesBarbalho2015">{{cite journal|last1=Esteves|first1=Paola|last2=Barbalho|first2=Marcella|last3=Lima|first3=Tiago|last4=Quintella|first4=Leonardo|last5=Niemeyer-Corbellini|first5=Jo�o Paulo|last6=Ramos-e-Silva|first6=Marcia|title=Angiolymphoid Hyperplasia with Eosinophilia: A Case Report|journal=Case Reports in Dermatology|volume=7|issue=2|year=2015|pages=113–116|issn=1662-6567|doi=10.1159/000381843}}</ref>
=== Surgery ===
*[[Surgery]] is the mainstay of therapy for angiolymphoid hyperplasia with eosinophilia.
* Mohs micrographic surgery with complete margin examination is performed.
* Other treatment options include [[radiotherapy]], curettage, shave excision with electrodessication, [[cryotherapy]], [[corticosteroids]] (topical, systemic or intralesional preparation), and laser therapy using continuous wave carbon dioxide and argon lasers.<ref name="pmid21487464">{{cite journal| author=Lembo S, Balato A, Cirillo T, Balato N| title=A Long-Term Follow-Up of Angiolymphoid Hyperplasia with Eosinophilia Treated by Corticosteroids: When a Traditional Therapy is Still Up-to-Date. | journal=Case Rep Dermatol | year= 2011 | volume= 3 | issue= 1 | pages= 64-7 | pmid=21487464 | doi=10.1159/000323182 | pmc=PMC3073756 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21487464  }} </ref>
=== Prevention ===
*There are no primary preventive measures available for angiolymphoid hyperplasia with eosinophilia.


==References==
==References==

Latest revision as of 20:48, 9 May 2016

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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] Ammu Susheela, M.D. [3]

Synonyms and Keywords: Epithelioid hemangioma; Histiocytoid hemangioma; Inflammatory angiomatous nodule; Intravenous atypical vascular proliferation; Papular angioplasia; Inflammatory arteriovenous hemangioma; Pseudopyogenic granuloma; ALHE; EH

Overview

Angiolymphoid hyperplasia with eosinophilia was first discovered by G. C. Wells and I. W. Whimster, two British physicians, in 1969.[1]Angiolymphoid hyperplasia with eosinophilia [2] is defined as red-brown, dome-shaped, dermal papule or nodule, present in the around the ears, scalp, neck, trunk, extremities, genitalia, lips and oral mucosa.[3]On gross pathology, smooth-surface red to brown papules or nodules are characteristic findings of angiolymphoid hyperplasia with eosinophilia. On microscopic histopathological analysis, florid vascular proliferation with atypical endothelial cells surrounded by a lymphocytic and eosinophilic infiltrate are characteristic findings of angiolymphoid hyperplasia with eosinophilia. Angiolymphoid hyperplasia with eosinophilia may be caused by either allergic reactions, traumas, or autoimmune disorders. Angiolymphoid hyperplasia with eosinophilia must be differentiated from other diseases that cause swelling of face and neck, such as Kimura disease, acial granuloma, insect bite reaction, cutaneous lymphoma, sarcoidosis. The prevalence of angiolymphoid hyperplasia with eosinophilia is unknown as it is an extremely rare disease. Angiolymphoid hyperplasia with eosinophilia is more commonly observed among young patients. Females are more commonly affected with angiolymphoid hyperplasia with eosinophilia than males. Angiolymphoid hyperplasia with eosinophilia usually affects predominently individuals of the Asian race. Common complications of angiolymphoid hyperplasia with eosinophilia include pruritis, pulsation, and spontaneous bleeding. Findings on biopsy include proliferation of small blood vessels with endothelial cells having a cobblestone appearance, a perivascular and interstitial infiltrate of lymphocytes and eosinophils. Surgery is the mainstay of therapy for angiolymphoid hyperplasia with eosinophilia.

Historical Perspective

  • Angiolymphoid hyperplasia with eosinophilia was first discovered by G. C. Wells and I. W. Whimster, two British physicians, in 1969.[1]

Pathophysiology

  • Angiolymphoid hyperplasia with eosinophilia [4] is defined as red-brown, dome-shaped, dermal papule or nodule, present in the head or neck, specifically around the ears and on the scalp.[3]
  • The pathogenesis of angiolymphoid hyperplasia with eosinophilia is characterized by red to brown papules or nodules dislocated in the dermis or subcutaneous tissue.
  • Angiolymphoid hyperplasia with eosinophilia is a rare and idiopathic vascular disorder.
  • On gross pathology, smooth-surface red to brown papules or nodules on the head, neck, trunk, extremities, genitalia, lips and oral mucosa are characteristic findings of angiolymphoid hyperplasia with eosinophilia.
  • On microscopic histopathological analysis, florid vascular proliferation with atypical endothelial cells surrounded by a lymphocytic and eosinophilic infiltrate are characteristic findings of angiolymphoid hyperplasia with eosinophilia.

Causes

  • Angiolymphoid hyperplasia with eosinophilia may be caused by either allergic reactions, traumas, or autoimmune disorders.

Differentiating Angiolymphoid hyperplasia with eosinophilia from other Diseases

  • Angiolymphoid hyperplasia with eosinophilia must be differentiated from other diseases that cause swelling of face and neck, such as:

Epidemiology and Demographics

  • The prevalence of angiolymphoid hyperplasia with eosinophilia is unknown as it is an extremely rare disease.

Age

  • Angiolymphoid hyperplasia with eosinophilia is more commonly observed among young patients.

Gender

  • Females are more commonly affected with angiolymphoid hyperplasia with eosinophilia than males.

Race

  • Angiolymphoid hyperplasia with eosinophilia usually affects predominently individuals of the Asian race.

Risk Factors

  • Common risk factors in the development of angiolymphoid hyperplasia with eosinophilia are allergic reactions, traumas or autoimmune disorders.

Natural History, Complications and Prognosis

  • The majority of patients with angiolymphoid hyperplasia with eosinophilia remain asymptomatic for several years.
  • Common complications of angiolymphoid hyperplasia with eosinophilia include pruritis, pulsation, and spontaneous bleeding.
  • One-third of cases of angiolymphoid hyperplasia with eosinophilia recur when incompletely excised.

Diagnosis

Symptoms

  • Angiolymphoid hyperplasia with eosinophilia lesions may be asymptomatic, painful or pruriginous.

Physical Examination

  • Physical examination may be remarkable for:
  • Dome-shaped, smooth-surfaced papules or expanding nodule or group of nodules

Ear

Laboratory Findings

Other Diagnostic Studies

  • Angiolymphoid hyperplasia with eosinophilia may also be diagnosed using biopsy.
  • Findings on biopsy include proliferation of small blood vessels with endothelial cells having a cobblestone appearance, a perivascular and interstitial infiltrate of lymphocytes and eosinophils.

Treatment

Medical Therapy

  • The mainstay of therapy for angiolymphoid hyperplasia with eosinophilia is intralesional corticoid therapy and imiquimod.
  • Imiquimod acts by induction of the production of interferon-α and induction or inhibition of certain cytokines, mainly interleukin-5.[5]

Surgery

  • Surgery is the mainstay of therapy for angiolymphoid hyperplasia with eosinophilia.
  • Mohs micrographic surgery with complete margin examination is performed.
  • Other treatment options include radiotherapy, curettage, shave excision with electrodessication, cryotherapy, corticosteroids (topical, systemic or intralesional preparation), and laser therapy using continuous wave carbon dioxide and argon lasers.[6]

Prevention

  • There are no primary preventive measures available for angiolymphoid hyperplasia with eosinophilia.

References

  1. 1.0 1.1 Wells GC, Whimster IW (1969). "Subcutaneous angiolymphoid hyperplasia with eosinophilia". Br J Dermatol. 81 (1): 1–14. PMID 5763634.
  2. Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 1-4160-2999-0.
  3. 3.0 3.1 James, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Diseases of the Skin: Clinical Dermatology. (10th ed.). Saunders. ISBN 0-7216-2921-0.
  4. Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 1-4160-2999-0.
  5. Esteves, Paola; Barbalho, Marcella; Lima, Tiago; Quintella, Leonardo; Niemeyer-Corbellini, Jo�o Paulo; Ramos-e-Silva, Marcia (2015). "Angiolymphoid Hyperplasia with Eosinophilia: A Case Report". Case Reports in Dermatology. 7 (2): 113–116. doi:10.1159/000381843. ISSN 1662-6567. replacement character in |first5= at position 3 (help)
  6. Lembo S, Balato A, Cirillo T, Balato N (2011). "A Long-Term Follow-Up of Angiolymphoid Hyperplasia with Eosinophilia Treated by Corticosteroids: When a Traditional Therapy is Still Up-to-Date". Case Rep Dermatol. 3 (1): 64–7. doi:10.1159/000323182. PMC 3073756. PMID 21487464.