Mastocytosis

Mastocytosis
Mastocytosis - cropped - very high mag.jpg
Micrograph of mastocytosis. Skin biopsy. H&E stain.
SpecialtyOncology, allergology, hematology

Mastocytosis, a type of mast cell disease, is a rare disorder affecting both children and adults caused by the accumulation of functionally defective mast cells (also called mastocytes) and CD34+ mast cell precursors.[1]

People affected by mastocytosis are susceptible to a variety of symptoms including itching, hives, and anaphylactic shock, caused by the release of histamine and other pro-inflammatory substances from mast cells.[2]

Classification

Mastocytosis can occur in a variety of forms:

Cutaneous mastocytosis (CM)

  • The most common cutaneous mastocytosis is maculopapular cutaneous mastocytosis, previously named papular urticaria pigmentosa (UP), more common in children, although also seen in adults. Telangiectasia macularis eruptiva perstans (TMEP) is a much rarer form of cutaneous mastocytosis that affects adults.[2] MPCM and TMEP can be a part of indolent systemic mastocytosis. This should be considered if patients develop any systemic symptoms[3]
  • Generalized eruption of cutaneous mastocytosis (adult type) is the most common pattern of mastocytosis presenting to the dermatologist, with the most common lesions being macules, papules, or nodules that are disseminated over most of the body but especially on the upper arms, legs, and trunk[4]
  • Diffuse cutaneous mastocytosis' has diffuse involvement in which the entire integument may be thickened and infiltrated with mast cells to produce a peculiar orange color, giving rise to the term "homme orange."[5]

Cutaneous mastocytosis in children usually presents in the first year after birth and in most cases vanishes during adolescence.

Systemic mastocytosis (SM)

Systemic mastocytosis involves the bone marrow in the majority of cases and in some cases other internal organs, usually in addition to involving the skin. Mast cells collect in various tissues and can affect organs where mast cells do not normally inhabit such as the liver, spleen and lymph nodes, and organs which have normal populations but where numbers are increased. In the bowel, it may manifest as mastocytic enterocolitis.[6]

There are five types of systemic mastocytosis:[7]

  • Indolent systemic mastocytosis (ISM). The most common SM (>90%)
  • Smouldering systemic mastocytosis (SSM)
  • Systemic mastocytosis with associated haematological neoplasm (SM-AHN)
  • Aggressive systemic mastocytosis (ASM)
  • Mast cell leukemia (MCL)
Other Languages
čeština: Mastocytóza
Deutsch: Mastozytose
español: Mastocitosis
français: Mastocytose
Bahasa Indonesia: Mastositosis
italiano: Mastocitosi
Nederlands: Mastocytose
polski: Mastocytoza
português: Mastocitose
русский: Мастоцитоз
српски / srpski: Мастоцитоза
svenska: Mastocytos
Türkçe: Mastositoz
українська: Мастоцитоз