Mastocytosis in the skin accompanied by pseudo-Kaposi's sarcoma.
Kaposi’s sarcoma
acroangiodermatitis
cutaneous mastocytosis
mast cell
mastocytosis
Journal
The Journal of dermatology
ISSN: 1346-8138
Titre abrégé: J Dermatol
Pays: England
ID NLM: 7600545
Informations de publication
Date de publication:
May 2021
May 2021
Historique:
revised:
01
12
2020
received:
21
07
2020
accepted:
03
12
2020
pubmed:
9
3
2021
medline:
15
5
2021
entrez:
8
3
2021
Statut:
ppublish
Résumé
Mastocytosis is a heterogeneous group of diseases characterized by abnormal proliferation of neoplastic mast cells in the skin and/or other extracutaneous tissues. Most patients with skin involvement can be subclassified into one of the three subtypes of cutaneous mastocytosis currently recognized by the World Health Organization (i.e., mastocytoma, maculopapular cutaneous mastocytosis and diffuse cutaneous mastocytosis); however, some patients may occasionally present with atypical skin lesions that cannot be ascribed to any of these disease subtypes. Here, we report three patients diagnosed with mastocytosis and an unusual cutaneous involvement mimicking Kaposi's sarcoma. Skin biopsies showed neoplastic mast cell infiltrates together with features commonly seen in acroangiodermatitis, and immunohistochemistry for human herpesvirus 8 was negative. One patient fulfilled the criteria for aggressive systemic mastocytosis, showed no response to cytoreductive therapy, and died because of disease progression. The remaining two patients had indolent and smoldering systemic mastocytosis, respectively, but they showed several features associated with an unfavorable prognosis such as extensive involvement of the hematopoiesis by the KIT D816V mutation, increased serum β2-microglobulin, and decreased serum lactate dehydrogenase. The presence of pseudo-Kaposi's sarcoma skin lesions is an uncommon finding in mastocytosis which may alert physicians to the possible existence of underlying features indicative of a poor prognosis.
Identifiants
pubmed: 33684229
doi: 10.1111/1346-8138.15734
doi:
Substances chimiques
Proto-Oncogene Proteins c-kit
EC 2.7.10.1
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
657-660Subventions
Organisme : Hospital Virgen de la Salud Biobank (BioB-HVS), supported by grant PT13/0010/0007 from the Instituto de Salud Carlos III, Madrid, Spain.
ID : PT13/0010/0007
Organisme : Asociación Española de Mastocitosis y enfermedades relacionadas
Informations de copyright
© 2021 Japanese Dermatological Association.
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