Bullous Mastocytosis

Paediatric mastocytosis

“…31 The prognosis for infants with cutaneous mastocytosis in part appears to depend on whether they exhibit bullae early in the neonatal period or if bullae are delayed relative to the appearance of their skin lesions. 70 Children who manifest diffuse cutaneous mastocytosis prior to bullous eruptions appear to have a better chance at gradual improvement in their disease. Several reports of the occurrence of acute lymphocytic leukaemia in rapidly progressive and late onset paediatric onset mastocytosis suggest the possibility of a predilection for the development of a haematological malignancy in these patients.…”

The Familial Occurrence of Bullous Mastocytosis (Diffuse Cutaneous Mastocytosis)

“…However, no macular, papular, or nodular cutaneous lesions were present on her skin. The findings from laboratory studies were as follows: white blood cell count, 6.2 X lO'/L, with 0.36 neutrophils, 0.50 lymphocytes, and 0.14 monocytes; red blood cell count, 4.55 X 10'VL; hemoglobin level, 94 g/L; hematocrit, 0.31; platelet count, 681 X 10VL; aspartate aminotransferase level, 30 U/L; alanine aminotransferase level, 12 U/L; alka¬ line phosphatase level, 636 U/L; urea nitrogen level, 4.6 mmol/L of urea; creatinine level, 61.9 /umol/L; total protein level, 70 g/L; albumin level, 48.23 g/L; «.-globulin level, 1.68 g/L; a2-globulin level, 6.23 g/L; ß-globulin level, 5.18 g/L; yglobulin level, 8.75 g/L; IgA level, 1.30 g/L; IgG level, 10.30 g/L; IgM level, 1.07 g/L; blood histamine level, 228 ng/dL (normal, 15 to 80 ng/mL); and blood serotonin level, 256 ng/mL (normal, <230 ng/mL). Roentgenograms of the chest and abdomen were normal.…”

“…The results of laboratory studies included the following values: red blood cells, 4.92 X 10'VL; hemoglobin, 124 g/L; hematocrit, 0.37; white blood cells, 13.3 X 10VL, with 0.36 neutrophils, 0.62 lymphocytes, and 0.02 monocytes; plate¬ lets, 530 X 10VL; and blood histamine, 72 ng/mL. Aspira¬ tion of his bone marrow disclosed no atypical cells, and there was no hepatomegaly, splenomegaly, or lymphadenopathy.…”

“…Laboratory findings included the following values: white blood cells, 6.4 X 10VL, with 0.70 neutrophils, 0.25 lympho¬ cytes, and 0.05 monocytes; red blood cells, 4.43 X 10'*/L; he¬ moglobin, 112 g/L; hematocrit, 0.37; platelets, 226 X 10VL; blood histamine, 444 ng/mL; and blood serotonin, 160 ng/ mL. Hepatosplenomegaly and lymphadenopathy were not detected.…”

Bull�se Mastozytose bei einem Kind

“…Die bullöse Mastozytose stellt eine seltene Erkrankung des frühen Kindesalters mit zahlreichen urtikariellen Läsionen, Blasenbildung und positivem Darier-Zeichen infolge Proliferation von dermalen Mastzellen dar [9,15,18]. Atypische Verläufe erschweren oder verzögern die korrekte Diagnose.…”