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WHO Classification of Tumours
Cutaneous mastocytosis
Tumours of haematopoietic and lymphoid tissues


Definition

Mastocytosis is due to a clonal, neoplastic proliferation of mast cells that accumulate in multifocal compact clusters or cohesive aggregates or infiltrates. The diagnosis of cutaneous mastocytosis (CM) requires the demonstration of typical clinical findings and histological proof of abnormal mast cell infiltration of the dermis. In isolated CM, there is no evidence of systemic involvement using such parameters as elevated levels of total serum tryptase or organomegaly (Table 2.10).

> Variants of cutaneous mastocytosis
Recently, consensus criteria for the diagnosis of CM have been further refined, and three major variants of CM are now recognized (Table 2.11)

Click to access Pubmed
Valent P, Akin C, Escribano L, Födinger M, Hartmann K, Brockow K, Castells M, Sperr WR, Kluin-Nelemans HC, Hamdy NA, Lortholary O, Robyn J, van Doormaal J, Sotlar K, Hauswirth AW, Arock M, Hermine O, Hellmann A, Triggiani M, Niedoszytko M, Schwartz LB, Orfao A, Horny HP, Metcalfe DD (2007)
Standards and standardization in mastocytosis: consensus statements on diagnostics, treatment recommendations and response criteria.
Eur J Clin Invest 37: 435-53



:

Urticaria pigmentosa (UP) / Maculopapular cutaneous mastocytosis (MPCM)
This is the most frequent form of CM. In children, the lesions of UP tend to be larger and papular. Histopathology typically reveals aggregates of spindle-shaped mast cells filling the papillary dermis and extending as sheets and aggregates into the reticular dermis, often in perivascular and periadnexal positions

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Wolff K, Komar M, Petzelbauer P (2001)
Clinical and histopathological aspects of cutaneous mastocytosis.
Leuk Res 25: 519-28



. A subvariant, usually occurring in young children, presents as non-pigmented, plaque-forming lesions. In adults, the lesions are disseminated, and tend to be red or brown-red and macular or maculopapular. Histopathology of adult UP tends to reveal fewer mast cells than observed in children.
The number of lesional mast cells may sometimes overlap with the upper range of mast cell numbers found in normal or inflamed skin. In some cases, examination of multiple biopsies and immunohistochemical analysis may be necessary to establish the diagnosis of CM

Click to access Pubmed
Valent P, Akin C, Escribano L, Födinger M, Hartmann K, Brockow K, Castells M, Sperr WR, Kluin-Nelemans HC, Hamdy NA, Lortholary O, Robyn J, van Doormaal J, Sotlar K, Hauswirth AW, Arock M, Hermine O, Hellmann A, Triggiani M, Niedoszytko M, Schwartz LB, Orfao A, Horny HP, Metcalfe DD (2007)
Standards and standardization in mastocytosis: consensus statements on diagnostics, treatment recommendations and response criteria.
Eur J Clin Invest 37: 435-53




Click to access Pubmed
Wolff K, Komar M, Petzelbauer P (2001)
Clinical and histopathological aspects of cutaneous mastocytosis.
Leuk Res 25: 519-28



.

Diffuse cutaneous mastocytosis
This clinically remarkable subvariant of CM is much less frequent than UP and presents almost exclusively in childhood. Here the skin is diffusely thickened and may have a peau chagrine or peau d’orange (orange peel) appearance. There are no individual lesions. In patients with clinically less obvious infiltration of the skin, the biopsy usually shows a band-like infiltrate of mast cells in the papillary and upper reticular dermis. In massively infiltrated skin, the histological picture may be the same as that seen in solitary mastocytoma

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Soter NA (2000)
Mastocytosis and the skin.
Hematol Oncol Clin North Am 14: 537-55, vi




Click to access Pubmed
Wolff K, Komar M, Petzelbauer P (2001)
Clinical and histopathological aspects of cutaneous mastocytosis.
Leuk Res 25: 519-28



.

Solitary mastocytoma of skin
This occurs as a single lesion, almost exclusively in infants, without predilection of site. The histologic picture is one of sheets of mature-appearing highly metachromatic mast cells with abundant cytoplasm that densely infiltrate the papillary and reticular dermis. These mast cell infiltrates may extend into the subcutaneous tissues

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Kiszewski AE, Alvarez-Mendoza A, Ríos-Barrera VA, Hernández-Pando R, Ruiz-Maldonado R (2007)
Mastocytosis in children: clinicopathological study based on 35 cases.
Histol Histopathol 22: 535-9



. Cytological atypia is not detected. This allows separation of mastocytoma from an extremely rare mast cell sarcoma of the skin
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Scheck O, Horny HP, Ruck P, Schmelzle R, Kaiserling E (1987)
Solitary mastocytoma of the eyelid. A case report with special reference to the immunocytology of human tissue mast cells, and a review of the literature.
Virchows Arch A Pathol Anat Histopathol 412: 31-6



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