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80]

REVIEW ARTICLE

Mastocytosis in children
Sahana M Srinivas, Sandipan Dhar1, Deepak Parikh2
Department of Pediatric Dermatology, Indira Gandhi Institute of Child Health, Bengaluru, Karnataka, 1Department of
Pediatric Dermatology, Institute of Child Health, Kolkata, West Bengal, 2Department of Pediatric Dermatology,
B.J Wadia Hospital for Children, Parel, Mumbai, Maharashtra, India

ABSTRACT

Mastocytosis is a heterogeneous group of disorders with increase in mast cells in the skin and other extracutaneous organs.
Mastocytosis is seen in children and adults. Cutaneous mastocytosis is more common in children than systemic forms.
Among the cutaneous form urticaria pigmentosa is the most common type of mastocytosis seen in children. There is a
tendency of regression with age. Childhood onset mastocytosis has a good prognosis. Counseling forms a major role in the
management of pediatric‑onset mastocytosis.

Key words: Cutaneous, mastocytosis, pediatric age group

INTRODUCTION doing an autopsy in a child with fatal UP documented

the presence of MC infiltration in skin, liver, spleen,

M astocytosis is a clonal disease of hematopoietic

stem cell characterized by local or diffuse
increased growth and accumulation of mast
lymph nodes, and bone marrow.[4]

cells (MCs) in skin, bone marrow, liver, spleen, CLASSIFICATION

and lymph nodes.[1] The disorder occurs in two
The classification of mastocytosis has evolved
spectrums: Pediatric onset mastocytosis and adult
over the years. Children present with cutaneous or
onset mastocytosis. The clinical manifestations of
systemic mastocytosis (SM) or overlap of these 2
mastocytosis are not directly due to MCs but due
conditions. Degos described many variants of UP
the functional effects of MC degranulation enzymes.
in 1955; later many changes were incorporated in
The presentation of pediatric mastocytosis is varied
the classification over the years. In 2001, WHO
and differs clinically from adult onset. Pediatric
adopted the classification of mastocytosis and in
mastocytosis has a benign nature and usually resolves
2005 working conference on the mastocytosis put
spontaneously by puberty. This article will review the
forward the criteria to diagnose and revised the
characteristic features of pediatric‑onset mastocytosis,
classification of mastocytosis [Table 1].[5,6] Cutaneous
approach and the guidelines for their management.
mastocytosis (CM) is diagnosed on the clinical and
histopathological basis with absence of criteria for SM.
HISTORY SM is based on major and minor criteria [Table 2].[5]
Diagnosis is based on fulfillment of one major and one
Mastocytosis was first described by Nettleship and minor criteria or three minor criteria.
Tay in 1869 and later due to the appearance like
urticaria, the term urticaria pigmentosa (UP) was
coined by Sangster in 1878.[2,3] Ellis in 1949 while EPIDEMIOLOGY
Access this article online Epidemiological data in Indian population are exactly
Quick Response Code not known. The incidence of mastocytosis in western
Website: literature varies from 1 in every 1000 to 1 in 8000 new
[Link]

ADDRESS FOR CORRESPONDENCE

DOI: Dr. Sahana M Srinivas
Department of Pediatric Dermatology, Indira Gandhi Institute of
10.4103/2319-7250.154801 Child Health, Bengaluru, Karnataka, India.
E‑mail: drsahanasrinivas@[Link]

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Srinivas, et al.: Mastocytosis

Table 1: Classification of mastocytosis type 1 hypersensitivity reactions. The growth and

(modified from Horny et al. 2008) development of MCs is influenced by stem cell
CM factor (SCF, also called MC growth factor or kit ligand)
Mastocytoma
which binds to the human kit protein, the receptor
Maculopapular CM
Diffuse CM for SCF on the surface of human MCs.[11] Apart from
ISM MC and their progenitors, kit is also expressed on
Smouldering SM hematopoietic and nonhematopoietic cells.
Isolated BM mastocytosis
SM‑AHNMD
ASM The etiopathogenesis is multifactoriol. There is
MC leukemia increased expression of soluble SCF in lesions of CM.
MC sarcoma There are a lot of studies documenting mutations in
Extracutaneous mastocytoma
the c‑kit gene in adult onset mastocytosis and patients
CM - Cutaneous mastocytosis; ISM - Indolent systemic mastocytosis;
SM‑AHNMD - Systemic mastocytosis with an associated clonal hematological associated with hematological malignancy. The most
non‑MC lineage disease; ASM - Aggressive systemic mastocytosis; MC - Mast cell common mutation is found on the Asp to Val at codon
816 (c‑kitD816V).[12] However, children with typical
Table 2: Proposed criteria for SM mastocytosis lack codon 816 mutation. In a recent
Major criteria study on Japanese patients, found that 12 out of 14
Multifocal dense infiltrates of MC (>15 MC aggregating) detected in children with mastocytosis had D816 substitutions.[13]
sections of bone marrow and/or of other extracutaneous organs by
Patients with Asp 816Phe mutation appear to acquire
tryptase immunohistochemistry or other stains
Minor criteria the disease earlier than those presenting with Asp 816
(a) >25% of MC in MC infiltrates detected in sections of bone Val mutations.
marrow or other extracutaneous organs are spindle‑shaped,
or presence of >25% atypical MC (type 1 plus type II) in bone
Dysregulation of MC apoptosis is another pathogenesis
marrow aspirates
(b) Detection of c‑kit point mutation at codon 816 in bone marrow
occurring in mastocytosis, and this is seen in SM.
or blood or other extracutaneous organs There is upregulation of antiapoptotic protein Bcl‑2
(c) Kit + MCs in bone marrow or blood or other extracutaneous and antiapoptotic protein Bcl‑X in bone marrow of
organs co‑express CD2 and/or CD25
patients with indolent mastocytosis.[14] Chromosomal
(d) Serum total tryptase concentration persistently >20 ng/ml
(in case of an associated clonal hematological non‑MC lineage abnormalities may be increased in patients with
disease, d. is not valid) mastocytosis associated with hematological
Diagnosis: One major and one minor criteria or three minor criteria. MC - Mast disorder.[15] Bone marrow analysis has shown that
cell; SM - Systemic mastocytosis
MCs express CD2 and CD25. CD25 is a reliable
immunohistochemical marker to differentiate
patients. An annual incidence of 5–10 new cases per neoplastic from normal MCs.
million population has been estimated.[7,8] Mastocytosis
occurs at any age. It can present during the neonatal The clinical symptoms of mastocytosis are due
period, in infancy or childhood. In approximately 15% to the release of MC mediators. MCs produce
of children, the disease is congenital and in 50% of mediators histamine, heparin, tryptase, proteases,
cases the onset is between birth and 2 years.[8] 10% of chymase, carboxypeptidase A, chondroitin sulfate
children develop mastocytosis between 2 and 15 years. glycosaminoglycans, leukotrienes, prostaglandin
There is no sex predominance and is seen in all races. D2, vascular endothelial growth factor, platelet
Few studies have documented a male preponderance. activating factor, cytokines (tumor necrosis factor
Familial cases have been reported, and mastocytosis and interleukins), and chemokines. These mediators
in identical twins and triplets have been described.[9] related symptoms are flushing, itching, blistering,
diarrhea, abdominal pain, vomiting, hypotension,
headache, and bone pain.[16]
ETIOPATHOGENESIS
MCs are connective tissue cells that play a role in CLINICAL FEATURES
immunologic and inflammatory reactions. They
originate from pluripotent progenitor cells in the The most common form of mastocytosis is the cutaneous
bone marrow that express CD34, CD17 (kit), and form. Approximately, two‑thirds of children present
CD13.[10] These MCs migrate in the blood and invade with CM. In 90% cases, only skin is involved. CM can
the tissues where they proliferate and differentiate be classified into UP (maculopapular form), solitary
into mature MCs. MCs play a role host defense mastocytoma, diffuse CM (DCM), and telangiectasia
by releasing mediators of inflammation leading to macularis eruptiva perstans (TMEP). TMEP is very
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Srinivas, et al.: Mastocytosis

rare in children and seen mostly in adults and is mastocytosis is a variant of DCM characterized by
not recognized in WHO classification.[17] Nodular multiple, flat, yellowish papules, and nodules from
and plaque type of CM have also been reported. In birth resembling xanthomatosis.[26,27]
children, CM typically presents as UP or solitary
mastocytoma.[18] Diffuse form is rare in children. Systemic symptoms are often associated with DCM
There is mild to moderate pruritus in all cutaneous and include flushing, hemorrhage, nausea, vomiting,
forms. Stroking the skin lesions produce erythema, diarrhea, hypotension, dyspnea, headache, dizziness,
swelling, and blister formation, and this is seen after irritability, tachycardia, hypotension, abdominal pain,
5–10 min. This reaction pattern is called Darier’s sign melena, sepsis, and overt shock leading to death.[28]
and is pathognomonic of all forms of CM.[19] These symptoms are more pronounced and are episodic
and occur during the release of MC mediators.
Urticaria Pigmentosa
UP is the most common type of mastocytosis seen Other Variants of Mastocytosis
in children. It is seen in 70–90% of cases. Lesions Atypical variants of mastocytosis include bullous,
can present at birth or usually within 2 years of age. anetoderma, giant inguinal and suprapubic masses.[29]
Familial cases of UP have been reported. They occur Rarely, two variants of mastocytosis can be present in
as multiple, monomorphic red to brown papules or the same child. Recently, a new variant of mastocytosis
ill‑defined gray‑brown macules distributed on the called blaschkoid pattern of mastocytosis has been
face, trunk, and extremities [Figure 1]. Lesions can described in the literature in a child where the lesions
be generalized but tend to be concentrated more on occur along the lines of blaschko.[30]
the trunk. Scalp, palms, soles, and sun exposed areas
are less commonly affected. The lesions heal without Systemic Mastocytosis
scarring. UP lesions usually resolve by the age of Systemic mastocytosis is more common in adults and
10 years, if they persist they tend to be symptomatic is rarely seen in children. It is defined as mastocytosis
and involves systemic features.[20] Darier’s sign is occurring in at least 2 organs. In a study involving
positive in one‑third of cases [Figure 2]. Diarrhea, 173 children with mastocytosis, only 2 children had
wheezing, and syncope may be associated with systemic involvement.[31] SM is diagnosed based on
UP. Flushing is seen in 36% cases of UP. Rarely, organ involvement and histopathological examination
hepatosplenomegaly and skeletal lesions may occur. of bone marrow [Table 2]. Gastrointestinal and
skeletal system is more commonly involved. Other
Mastocytoma organs involved are lungs, kidney, liver, spleen,
Mastocytoma is seen in 10–30% cases. It can occur lymph nodes, and bone marrow. The incidence
at birth or in infancy. They present as asymptomatic of the skeletal system involved in children is 15%.
solitary or up to 5 lesions, round to oval, reddish‑brown, Radiographic features include sclerotic, lytic or
pink or yellow nodule on neck, arms or trunk. mixed lesions, osteoporosis or osteosclerosis.
[21]
The surface of the skin is smooth or resembles Gastrointestinal involvement is seen in 4% of
pitted skin (‘peau d’orange’ appearance) [Figure 3]. children. Symptoms include nausea, vomiting,
[22]
Urticaria or bullous lesions do occur. There is a abdominal pain, gastrointestinal hemorrhage,
classical history of intermittent bullae occurring at diarrhea, steatorrhea, malabsorption, perforation,
the same region. Systemic involvement like flushing, and peptic ulceration.[32] The hematopoietic and
abdominal colic is rarely seen. Persistence of solitary reticuloendothelial system is rarely seen in children.
mastocytoma for many years may develop into UP. Mastocytosis syndrome results from the massive
release of mediators resulting in sudden shock and
Diffuse Cutaneous Mastocytosis may lead to death.
Diffuse CM is a rare variant and accounts to 1–3%
cases of CM. There is diffuse infiltration of MCs in HISTOPATHOLOGY
the skin. DCM presents at birth or during infancy
during the neonatal period with diffuse blisters or All types of mastocytosis show increased number of
hemorrhagic bullae. The first sign is the presence of MCs in the dermis. In UP and mastocytoma, there
extensive bullae which ruptures to form crusting and is diffuse infiltration of MCs in upper one‑third
erosions. Diffuse papules and nodules occur making of dermis along with perivascular aggregates.[31]
the skin thickened, lichenified, and doughy.[23,24] DCM There is increased melanin in the basal layer and
can present with minimal blistering with erythema melanophages in the upper dermis which explains
evolving into erythroderma.[25] Pseudoxanthomatous the pigmentation in UP [Figure 4a and b]. It is
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Srinivas, et al.: Mastocytosis

Figure 1: Urticaria pigmentosa showing multiple well-defined Figure 2: Darier’s sign in urticaria pigmentosa
hyperpigmented plaques on the trunk

a b
Figure 4: (a) Epidermis showing increased basal layer pigmentation
with perivascular lymphohistiocytic infiltrate in the dermis
(H and E, ×10), (b) Giemsa stain showing multiple perivascular mast
cells in the dermis

DIAGNOSIS
The diagnosis of mastocytosis is mainly clinical.
Figure 3: Solitary mastocytoma showing ill-defined hypopigmented plaque The diagnostic work up in a child with mastocytosis
with peau d’orange appearance is outlined in Table 3. A high index of suspicion is
necessary to recognize the skin lesions of mastocytosis,
important to choose a biopsy site that has not and this should be corroborated with other features like
been traumatized as there will be degranulation positive Darier’s sign and investigations. All children
of MCs and histopathology may not be evident with mastocytosis baseline investigations should
of mastocytosis. Precautions must be taken while be performed as shown in Table 3, only if systemic
doing skin biopsy in diffuse involvement to prevent involvement is suspected further investigations must
mediator related symptoms, and one should have an be done Table 4. Serum tryptase levels correlate
emergency kit before starting the procedure. Special with the MC numbers in the skin, and increased
stains are required to demonstrate MCs. Giemsa levels are seen in severe disease.[34] There is a positive
stain, toluidine blue or leder’s stain are special correlation between the extent of skin involved,
stains that identify metachromatic cytoplasmic physical appearance of the lesions, associated
granules in MCs. Geimsa and toluidine blue stain symptoms such as pruritus, flushing, positive Darier’s
metachromatic granules purple and leders stain sign, bone pain, diarrhea, and tryptase levels. Serum
used stain red color. levels > 20 ng/ml are seen in 80% of patients with SM.
Urinary histamine metabolite N‑methylhistamine and
Bone marrow involvement present as accumulation N‑methylimidazylacetic acid have been correlated
of dense aggregates of spindle‑shaped or round MCs with increased MC numbers in the skin.[35] Skeletal
adjacent to paratrabecular and perivascular areas.[33] radiographic survey and bone scan are done if there
At least 15 MC aggregates should be present to fulfill is bone pain, hepatosplenomegaly, anemia or failure
the criteria for SM. to thrive.

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Srinivas, et al.: Mastocytosis

DIFFERENTIAL DIAGNOSIS Table 3: Diagnostic algorithm in children with

mastocytosis
Mastocytoma should be differentiated from juvenile Complete history
xanthogranuloma, naevocellular nevus, connective (Onset, duration, progression, triggering factors, familial history)
tissue nevi, spitz nevus, angioma, histiocytoma, ↓
Cutaneous examination
bullous insect bites, bullous urticaria or nodular (Morphology of skin lesions, Darier’s sign)
scabies. Differential diagnosis of UP includes ↓
lentigines, pigmented nevi, eruptive xanthomas, Systemic examination
↓
neurofibromatosis, histiocytosis, and fixed drug
Baseline investigations
eruption. Bullous forms of UP are differentiated from (Skin biopsy, complete blood counts, serum tryptase levels, urine or
chronic bullous dermatoses of childhood, bullous plasma histamine levels, chest X‑ray, liver function test, ultrasound)
pemphigoid, and epidermolysis bullosa.[36] Diffuse ↓
If any systemic involvement is suspected further investigations
erythrodermic forms can mimic staphylococcal scalded
performed
skin syndrome. Further investigations
(Bone densitometry if bone involvement, bone marrow biopsy, flow
cytometry for bone marrow MCs for the presence of CD2 and CD25,
TREATMENT determination of c‑kit mutation (D816V) for SM)
MC - Mast cell; SM - Systemic mastocytosis
Treatment of UP is aimed at three aspects: Counseling,
avoidance of triggers known to stimulate MC
Table 4: When to suspect SM in a child
degranulation and suppressing skin and MC mediator
Severe cutaneous involvement
related symptoms. Diffuse cutaneous mastocytosis
Persistence of skin lesions after puberty
Counseling Organ involvement
Education of parents about the disorder and the natural Peripheral smear showing eosinophilia
Increased serum tryptase levels (>100 ng/ml)
history is important. As most the CM has a benign Increased urinary N‑methylhistamine (>30 umol)
course, only symptomatic treatment is necessary. SM - Systemic mastocytosis
There is a tendency for spontaneous resolution
before puberty. Caregivers must be informed about
Table 5: Trigger factors in pediatric mastocytosis
the trigger factors that can cause a sudden release of
Physical stimuli
MC mediators and induce symptoms. Information Heat, cold, sudden change of temperature
brochures regarding trigger factors and frequently Rubbing of skin lesions
asked questions about the disorder should be given Pressure
Sunlight
to parents that will help to prevent life‑threatening
Emotional factors
complications. Stress
Anxiety
Avoidance of Triggers Disturbances in sleep
MCs can be activated due to trigger factors that are Infections
Viral/bacterial toxins
listed in Table 5.[37] Children should avoid bathing in Medications
extreme temperature, prevent insect bites, and any Nonsteroidal anti‑inflammatory drugs, morphine, codeine, alcohol,
physical trauma. Over the counter cough medications procaine, polymyxin B, amphotericin B, atropine, thiamine, quinine,
dextromethorphan, radiographic contrast media containing iodine,
should be avoided as they contain dextromethorphan
gallamine, reserpine
and codeine which can activate MCs. All precautions Dental procedures
must be taken while doing surgical procedures and Vaccination
giving general anesthesia. Literature review regarding Surgery
Snake venoms
pediatric anesthesia in mastocytosis has revealed that
Insect bites
anesthetic medications can be given with precaution
and meticulous preparation to treat complications.
Propofol, vecuronium, and fentanyl are safe to use in mastocytosis can be treated with intralesional
mastocytosis patients undergoing surgery.[38] steroids (5–10 mg/ml). H1‑receptor antagonist like
diphenhydramine, hydroxyzine, loratadine, cetirizine,
Treatment of Symptoms desloratadine, levocetirizine can control pruritus
Solitary mastocytoma can be treated with emollients, and flushing. H2‑receptor antagonist (cimetidine,
moderate potent topical corticosteroids or topical ranitidine) can be given if gastrointestinal symptoms
calcineurin inhibitors.[37,39,40] Single lesions of nodular are present. MC stabilizers disodium cromoglycate

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Srinivas, et al.: Mastocytosis

(200–800 mg/day) and ketotifen (1–2 mg twice 3. Sangster A. An anomalous mottled rash, accompanied by
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mastocytosis: Correlation with mast cell burden and
Source of Support: Nil, Conflict of Interest: Nil
implication for defining the category of disease. Int Arch

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