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Atypical Diffuse Cutaneous Mastocytosis FREE

Taraneh Sahihi, MD; Nancy B. Esterly, MD
[+] Author Affiliations

Received for publication Nov 26, 1971; accepted March 1, 1972.

Reprint requests to College of Medicine, Department of Dermatology, University of Illinois at the Medical Center, 840 S Wood St, PO Box 6998, Chicago 60680 (Dr. Sahihi).


Am J Dis Child. 1972;124(1):133-135. doi:10.1001/archpedi.1972.02110130135022.
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A 19-month-old boy was found to have atypical diffuse cutaneous mastocytosis. Although severe dermographism and pressure urticaria were present from early infancy, his disease remained undiagnosed because he lacked the characteristic bullous and pigmented lesions of urticaria pigmentosa and the pebbly skin texture usually found in diffuse cutaneous mastocytosis. A skin biopsy from clinically normal skin disclosed a dense infiltrate of mast cells in the upper dermis. Since involvement of other organ systems is frequent in this form of mastocytosis, the patient was studied for internal lesions but none was found at this time. Parents of affected children should be informed that trauma to the skin such as brisk toweling after a bath, and drugs such as aspirin, codeine, and polymyxin B sulfate may precipitate episodes of histamine shock due to degranulation of the mast cells.

REFERENCES

Nettleship E:  Rare forms of urticaria . Brit Med J 2:323-324, 1869;.
Simone JF, Hayes W:  Bullous urticaria pigmentosa with bleeding . J Pediat 78:160-166, 1971;.
Burgoon CF Jr, Graham JH, McCaffree DL:  Mast cell disease: A cutaneous variant with multisystem involvement . Arch Derm 98:590-605, 1968;.
Asboe-Hansen G:  Bullous diffuse mastocytosis with urinary hyaluronate excretion: Treatment with cyproheptadine . Acta Dermatovener 42:211-217, 1962;.
Robinson HM Jr, Kile RL, Hitch JM, et al:  Bullous urticaria pigmentosa . Arch Derm 85:346-357, 1962;.
Robbins JG:  Bullous urticaria pigmentosa . Arch Derm Syph 70:232-235, 1954;.
Sagher F, Even-Paz Z: Mastocytosis and Mast Cells . Chicago, Year Book Medical Publishers, 1967;.
Caplan RM:  The natural course of urticaria pigmentosa: Analysis and follow-up of 112 cases . Arch Derm 87:146-157, 1963;.
Miller RC, Shapiro L:  Bullous urticaria pigmentosa in infancy . Arch Derm 91:595-598, 1965;.
Orkin M, Good RA, Clawson CC, et al:  Bullous mastocytosis . Arch Derm 101:547-564, 1970;.
Demis DJ, Walton MD, Higdon RS:  Histaminuria in urticaria pigmentosa and the mastocytosis syndrome . Arch Derm 83:127-138, 1961;.
Asboe-Hansen G, Clausen J:  Urinary excretion of acid mucopolysaccharides in 12 cases of mastocytosis . J Invest Derm 42:81-85, 1964;.
Archer RK:  Increased circulating eosinophils as a result of excess histamine release . J Path Bact 78:95-103, 1959;.
Brett EM, Ong BH, Friedman T:  Mast cell disease in children: Report of 11 cases . Brit J Derm 79:197-209, 1967;.
Asboe-Hansen G:  Urticaria pigmentosa hemorrhagica . Acta Dermatovener 30:159-162, 1959;.
Sagher F, Schorr S:  Bone lesions in urticaria pigmentosa . J Invest Derm 26:431-434, 1956;.
Demis DJ:  The mastocytosis syndrome: Clinical and biological studies . Ann Intern Med 59:194-206, 1963;.
Sutter MC, Beaulieu G, Birt AR:  Histamine liberation by codeine and polymyxin B in urticaria pigmentosa . Arch Derm 86:217-221, 1962;.

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References

Nettleship E:  Rare forms of urticaria . Brit Med J 2:323-324, 1869;.
Simone JF, Hayes W:  Bullous urticaria pigmentosa with bleeding . J Pediat 78:160-166, 1971;.
Burgoon CF Jr, Graham JH, McCaffree DL:  Mast cell disease: A cutaneous variant with multisystem involvement . Arch Derm 98:590-605, 1968;.
Asboe-Hansen G:  Bullous diffuse mastocytosis with urinary hyaluronate excretion: Treatment with cyproheptadine . Acta Dermatovener 42:211-217, 1962;.
Robinson HM Jr, Kile RL, Hitch JM, et al:  Bullous urticaria pigmentosa . Arch Derm 85:346-357, 1962;.
Robbins JG:  Bullous urticaria pigmentosa . Arch Derm Syph 70:232-235, 1954;.
Sagher F, Even-Paz Z: Mastocytosis and Mast Cells . Chicago, Year Book Medical Publishers, 1967;.
Caplan RM:  The natural course of urticaria pigmentosa: Analysis and follow-up of 112 cases . Arch Derm 87:146-157, 1963;.
Miller RC, Shapiro L:  Bullous urticaria pigmentosa in infancy . Arch Derm 91:595-598, 1965;.
Orkin M, Good RA, Clawson CC, et al:  Bullous mastocytosis . Arch Derm 101:547-564, 1970;.
Demis DJ, Walton MD, Higdon RS:  Histaminuria in urticaria pigmentosa and the mastocytosis syndrome . Arch Derm 83:127-138, 1961;.
Asboe-Hansen G, Clausen J:  Urinary excretion of acid mucopolysaccharides in 12 cases of mastocytosis . J Invest Derm 42:81-85, 1964;.
Archer RK:  Increased circulating eosinophils as a result of excess histamine release . J Path Bact 78:95-103, 1959;.
Brett EM, Ong BH, Friedman T:  Mast cell disease in children: Report of 11 cases . Brit J Derm 79:197-209, 1967;.
Asboe-Hansen G:  Urticaria pigmentosa hemorrhagica . Acta Dermatovener 30:159-162, 1959;.
Sagher F, Schorr S:  Bone lesions in urticaria pigmentosa . J Invest Derm 26:431-434, 1956;.
Demis DJ:  The mastocytosis syndrome: Clinical and biological studies . Ann Intern Med 59:194-206, 1963;.
Sutter MC, Beaulieu G, Birt AR:  Histamine liberation by codeine and polymyxin B in urticaria pigmentosa . Arch Derm 86:217-221, 1962;.

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