University Journal of Medicine and Medical Specialities

ISSN 2455- 2852 2018, Vol. 5(9)

CHILDHOOD ONSET CUTANEOUS MASTOCYTOSIS A CASE REPORT

DEEPA
Department of Dermatology, Venerology & Leprosy,MADRAS MEDICAL COLLEGE AND GOVERNMENT GEN-
ERAL HOSPITAL

Abstract : The term mastocytosis is referred to as an array of

uncommon, usually sporadic, heterogeneous clinical
presentation that result from abnormal accumulation and
proliferation of mast cells in one or more organs1. A 1 year
old male child presented with generalised eruption of multiple
brownish lesions with history of occasional blister formation
since 3months of ages. On examination—multiple brownish
maculopapular rashes patches of varying sizes and few
plaques showing yellowish colour are present over the entire
body except palms and soles. Histopathological examination
and special staining confirmed the diagnosis of mastocytosis.
Keyword :Cutaneous Mastocytosis, Special stain Toludine
Blue, Darriers Sign. Multiple brownish maculopapular lesions along with multiple
INTRODUCTION: plaques of yellowish pigmentation. Histopathological examination
Mastocytosis is an uncommon heterogenous group of of the lesional skin showed infiltration of numerous mast cells
disorders characterized by abnormal growth and upper dermis. Mast cell granules were demonstrated by special
accumulation of mast cells in the skin and sometimes in the staining using Toludine Blue. Overlying epidermis showed
other organs such as bones, gastrointestinal tract, liver and increased amount of melanin suggesting a diagnosis of
spleen2. Increased expression of soluble SCF (Stem Cell cutaneous
Factor) receptor which promotes mast cell development is
increased in the skin of patients with mastocytosis.3
CASE REPORT:
A 1 year old male child presented with generalised eruption of
multiple brownish lesions with history of occasional blister
formation since 3months of age. There was no family history
of similar disease and the general health, growth,
development of the child was unaffected. There was no
history of drug intake. On examination - multiple brownish
maculopapular lesions & patches of varying sizes and few
plaques showing yellowish colour are present over the entire
body except palms and soles. Few erosions were present
over the limbs and trunk however there was no bulla at the
time of examination. Darrier’s sign was positive over the Mastocytosis Multiple Mastcells with pigment incontinence in the
lesional skin. There was no hepatosplenomegaly or basal layer.
lymphadenopathy. Complete Hemogram was normal. Chest
Xray and Ultrasound Abdomen were within normal limits.

An Initiative of The Tamil Nadu Dr. M.G.R. Medical University

University Journal of Medicine and Medical Specialities
DISCUSSION:
mastocytosis is a neoplastic disease involving mast cells and
their CD34+ progenitors4
Urticaria pigmentosa is the commonest pattern of cutaneous
mastocytosis. The skin is affected in 95% -99% of cases .
Mastocytosis limited to skin is primarily a disease of childhood
wheres in adult systemic mastocytosis is more common.5 The
symptoms result from the release of pharmacologically active
substances from mast cells into the tissues4 The updated WHO
classification of cutaneous mastocytosis include : Urticaria
pigmentosa, Mastocytoma, Telangiectasia mascularis eruptiva
perstans (TMEP), Diffuse cutaneous mastocytosis. 6 Urticaria
pigmentosa develop usually in the first 2 years of life.7 The most
common age of onset of adult utricaria pigmentosa is 20-40 years.
Numerous reddish brown or pale monomorphic maculopapules,
plaques or nodules appear in a symmetric distribution anywhere on
the body,except the face head, palms and soles with highest
concentration usually in the trunk and thighs.6 The edges of the
lesion are not completely sharp.
They utricate within a few minutes of gentle rubbing (Darrier’s sign)
1. The positive Darrier’s sign rules out other differential diagnosis
like Staphylococcal scalded skin syndrome, Erythema multiforme,
Epidermolysis bullosa.8 Other symptoms include heat or cold
intolerance, recurrent diarrhea, headache.6 Histopathologically
increased mast cells in the dermis of all types of mastocytosis. The
epidermis is normal apart from increase in melanin. The mast cells
are usually oval or spindle shaped and have granules that stain
metachromatically with toluidine blue.9 They are also well
demonstrated by giemsa or choloroacetate esterase stains in
formalin fixed biopsy mast cell infiltrate are predominantly found
around blood vessels and skin appendages in papillary dermis2. In
urticaria pigmentosa, they are increased upto 15 times above
normal. When systemic disease is suspected, a full blood count,
blood tryptase should be performed at presentation.10 Children
presenting with cutaneous or indolent systemic mastocytosis will
have excellent prognosis1. It is important to provide reassurance
about the nature and outlook of the condition.6 Management
includes avoidance of triggering factors for mast cell degranulation,
symptomatic relief and detection of significant systemic
disease.5
CONCLUSION:
It is considered that cutaneous manifestation of mastocytosis
compose a clinical spectrum, thus explaining the coexistence of
different clinical lesions. This case is presented for its rarity.
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An Initiative of The Tamil Nadu Dr. M.G.R. Medical University

University Journal of Medicine and Medical Specialities
An Initiative of The Tamil Nadu Dr. M.G.R. Medical University
University Journal of Medicine and Medical Specialities
An Initiative of The Tamil Nadu Dr. M.G.R. Medical University
University Journal of Medicine and Medical Specialities
An Initiative of The Tamil Nadu Dr. M.G.R. Medical University
University Journal of Medicine and Medical Specialities