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Xanthelasmoid mastocytosis: a rare form of cutaneous mastocytosis.

Abstract Mastocytosis is a rare disease characterized by the abnormal accumulation of mast cells in the skin and possibly in other organs. It can occur in a variety of forms; xanthelasmoid mastocytosis(XM) is a very rare form classified as papulo-nodular. Clinically, it appears as buff-yellow soft papules or nodules of variable size. Triggerings are those of classic mastocytosis. Darier's sign is often absent. Histology shows dense infiltrate of mast cells in the deep dermis. This clinical form can be singled out because of the persistence of lesions beyond puberty without the additional risk of systemic involvement. We here report the case of a 18-month old female infant, with no previous medical history. Maculopapular, intensely pruritic, ovalaires, brownish lesions with a buff-yellow centre, with elastic consistency and different sizes first occurred at the age of 8 months. The initial interview of parents revealed that the infant had had episode of flush especially caused by warmth. Darrier's sign was negative. Skin biopsy showed dermal infiltrate of mast cells, allowing to retain the diagnosis of xanthelasmoid mastocytosis. Complementary examinations as well as trypsin dosage were normal. Treatment was based on the exclusion of drugs and of factors participating in mast cells degranulation and antihistamines.
PMID
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Authors

Mayor MeshTerms
Keywords

Darrier’s sign

Xanthelasmoid mastocytosis

cutaneous mastocytosis

Journal Title the pan african medical journal
Publication Year Start




PMID- 29515722
OWN - NLM
STAT- MEDLINE
DCOM- 20180313
LR  - 20180313
IS  - 1937-8688 (Electronic)
VI  - 28
DP  - 2017
TI  - [Xanthelasmoid mastocytosis: a rare form of cutaneous mastocytosis].
PG  - 104
LID - 10.11604/pamj.2017.28.104.13629 [doi]
AB  - Mastocytosis is a rare disease characterized by the abnormal accumulation of mast
      cells in the skin and possibly in other organs. It can occur in a variety of
      forms; xanthelasmoid mastocytosis(XM) is a very rare form classified as
      papulo-nodular. Clinically, it appears as buff-yellow soft papules or nodules of 
      variable size. Triggerings are those of classic mastocytosis. Darier's sign is
      often absent. Histology shows dense infiltrate of mast cells in the deep dermis. 
      This clinical form can be singled out because of the persistence of lesions
      beyond puberty without the additional risk of systemic involvement. We here
      report the case of a 18-month old female infant, with no previous medical
      history. Maculopapular, intensely pruritic, ovalaires, brownish lesions with a
      buff-yellow centre, with elastic consistency and different sizes first occurred
      at the age of 8 months. The initial interview of parents revealed that the infant
      had had episode of flush especially caused by warmth. Darrier's sign was
      negative. Skin biopsy showed dermal infiltrate of mast cells, allowing to retain 
      the diagnosis of xanthelasmoid mastocytosis. Complementary examinations as well
      as trypsin dosage were normal. Treatment was based on the exclusion of drugs and 
      of factors participating in mast cells degranulation and antihistamines.
FAU - Ramid, Hind
AU  - Ramid H
AD  - Service de Dermatologie et de Venerologie du CHU Ibn Rochd, Casablanca, Maroc.
FAU - Hali, Fouzia
AU  - Hali F
AD  - Service de Dermatologie et de Venerologie du CHU Ibn Rochd, Casablanca, Maroc.
LA  - fre
PT  - Case Reports
PT  - Journal Article
TT  - Mastocytose xanthelasmoide: entite rare de mastocytose cutanee.
DEP - 20171004
PL  - Uganda
TA  - Pan Afr Med J
JT  - The Pan African medical journal
JID - 101517926
SB  - IM
MH  - Biopsy
MH  - Female
MH  - Humans
MH  - Infant
MH  - Mast Cells/*metabolism
MH  - Mastocytosis, Cutaneous/*diagnosis/pathology
MH  - Skin/*pathology
PMC - PMC5837140
OTO - NOTNLM
OT  - Darrier's sign
OT  - Xanthelasmoid mastocytosis
OT  - cutaneous mastocytosis
EDAT- 2018/03/09 06:00
MHDA- 2018/03/14 06:00
CRDT- 2018/03/09 06:00
PHST- 2017/08/17 00:00 [received]
PHST- 2017/08/29 00:00 [accepted]
PHST- 2018/03/09 06:00 [entrez]
PHST- 2018/03/09 06:00 [pubmed]
PHST- 2018/03/14 06:00 [medline]
AID - 10.11604/pamj.2017.28.104.13629 [doi]
AID - PAMJ-28-104 [pii]
PST - epublish
SO  - Pan Afr Med J. 2017 Oct 4;28:104. doi: 10.11604/pamj.2017.28.104.13629.
      eCollection 2017.