Mastositosis Kutan Difus Tipe Bulosa: Diagnosis Dan Penatalaksanaan
Penulis/Author
dr. Istiana Fiatiningsih, Sp.KK (1); Paranita Ferronika, PhD., MD-Pathologist (Consultant) (2); dr. Dyah Ayu Mira Oktarina, Ph.D., Sp.KK (3); Dr. dr. Sunardi Radiono, Sp.KK.(K). (4); Prof. Dr. dr. Hardyanto, Sp.KK(K). (5); Prof. Dr. Med. dr. Retno Danarti, Sp.KK(K). (6)
Tanggal/Date
2015
Kata Kunci/Keyword
Abstrak/Abstract
Cutaneous mastocytosis (CM), mostly found in children, is a disease with an accumulation
of mast cells in the skin and associated with systemic symptoms caused by release of mast cell
mediators. Histopatological examination shows an increase of mast cell infiltration and negative
results of direct imuno flourescence (DIF). Bullous type, a rare variant, is most often found in
diffuse cutaneous mastocytosis (DCM) and associated with systemic involvements.
A 5-month-old boy came to outpatient with blisters and black spots all over body. Physical
examination showed multiple tense bullae in a variety of sizes on normal or hyperpigmented
patches. Darier’s sign was positive. Routine blood examination, liver and renal function tests, and
abdomen ultrasonography were within normal limit. Histopathology showed subepidermal blister
with diffuse infiltration of dominant mononuclear cells in the dermis. Giemsa and toluidin blue
stains showed positive increasing of mast cells and DIF showed linear deposition of IgG and IgM
in the basal membrane. Based on clinical, physical and histopathological findings, the diagnosis
was bullous type DCM.
The clinical manifestations of bullous diseases in children are almost the same, making it
difficult to diagnose clinically. Histopathologic and immunofluorescent examinations are needed
in establishing a diagnosis. In CM, the DIF should be negative, but in this case showed a linear
deposition of IgG and IgM in the basal membrane of the epidermis whereas C3 deposition could
not be evaluated. We conclude this DIF findings was a non-specific results. However, it could not
be concluded whether this case had a coincidence with BP or EBA. The prognosis evaluation of
children with CM is important in order to know whether it is necessary to track signs of systemic
involvement.
Rumpun Ilmu
Penyakit Kulit dan Kelamin
Bahasa Asli/Original Language
Bahasa Indonesia
Level
Nasional
Status
Dokumen Karya
No
Judul
Tipe Dokumen
Aksi
1
Mastositosis Kutan Difus Tipe Bulosa.pdf
[PAK] Full Dokumen
2
SK Akreditasi DIKTI Periode 2 Tahun 2013 No_ 040_MDVI.pdf
Dokumen Pendukung Karya Ilmiah (Hibah, Publikasi, Penelitian, Pengabdian)
3
Surat Keterangan MDVI Mastositosis Kutan Difus Tipe Bulosa.pdf
Dokumen Pendukung Karya Ilmiah (Hibah, Publikasi, Penelitian, Pengabdian)