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Year : 2017  |  Volume : 18  |  Issue : 2  |  Page : 122-124

An infant with bullous mastocytosis: A rare form of bullous disorder

1 Department of Pediatric Medicine, Institute of Child Health, Kolkata, West Bengal, India
2 Department of Pediatric Dermatology, Institute of Child Health, Kolkata, West Bengal, India

Date of Web Publication27-Mar-2017

Correspondence Address:
Aniruddha Ghosh
11, Dr. Biresh Guha Street, Kolkata - 700 017, West Bengal
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2319-7250.202996

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Bullous mastocytosis (BM) is a rare variant of diffuse cutaneous mastocytosis where there is disorderly infiltration of the skin by mast cells. We report a 4-month-old male infant with this rare disease who presented with wrinkled tough skin and generalized multiple bullous lesions. Diagnosis was done by serum tryptase level and histopathological examination. The patient responded well to oral corticosteroid and antihistaminic therapy. Although a congenital disorder, timely diagnosis and treatment can be potentially rewarding in patients with BM.

Keywords: Bullous mastocytosis, diffuse cutaneous mastocytosis, mast cell

How to cite this article:
Ghosh A, Choudhury J, Dhar S. An infant with bullous mastocytosis: A rare form of bullous disorder. Indian J Paediatr Dermatol 2017;18:122-4

How to cite this URL:
Ghosh A, Choudhury J, Dhar S. An infant with bullous mastocytosis: A rare form of bullous disorder. Indian J Paediatr Dermatol [serial online] 2017 [cited 2020 Sep 18];18:122-4. Available from: http://www.ijpd.in/text.asp?2017/18/2/122/202996

  Introduction Top

Cutaneous mastocytosis is a rare disorder. The variants of this entity are urticaria pigmentosa, mastocytoma, diffuse cutaneous mastocytosis (DCM), and telangiectasia macularis eruptiva perstans (in decreasing order of frequency).[1] Diffuse cutaneous disease is of two types - pseudoxanthomatous or xanthelasmoid and bullous. Cutaneous mastocytosis is otherwise benign disease as it undergoes pubertal involution [2] exception being bullous mastocytosis (BM) having a more guarded prognosis.[3] It is very important to differentiate DCM from other forms of infantile bullous disorders.

  Case Report Top

A 4-month-old, well-nourished, appropriate for age, male infant presented with bullous eruptions over the scalp, face, all four limbs, and front of the trunk. Back was spared. He was a single child born by normal vaginal delivery with an uneventful perinatal history. He started developing the lesions from day-7 of neonatal life. Local physicians treated the patient with systemic and topical antibiotics but without any success. On examination, he was observed to have multiple tense bullae over the scalp, face, [Figure 1] front of the trunk, and all the limbs. There were multiple excoriated plaques indicating ruptured bullae [Figure 2]. There was the presence of typically thickened erythematous skin with enhancement of cutaneous folds and a peau d'orange appearance of entire surface of the skin [Figure 3]. The palms, soles, and mucous membranes were spared. Darier's sign was positive. Systemic examination did not reveal any abnormality, and the vital parameters were normal.
Figure 1: Tense bullae involving face and scalp regions

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Figure 2: Several bullae with hemorrhagic content and excoriated plaques indicating ruptured bullae

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Figure 3: Thickened skin, enhancement of skin folds and peau d'orange appearance of skin

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Routine hematological investigations, sepsis screen, and urine analysis were normal. Serum tryptase level was found to be as high as 88.7 μg/L (normal: <11.4 μg/L) by fluoroenzyme immunoassay method. Skin biopsy was done, and histopathological examination showed the dermis laden with sheets of mononuclear cells in the interstitium as well as around appendages. Giemsa stain showed positively stained metachromatic granules in the mononuclear cells, and the diagnosis was confirmed [Figure 4].
Figure 4: Histopathological examination of skin biopsy specimen (Giemsa stain) showing marked infiltration of dermis by mast cells

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The patient was treated with oral hydroxyzine (2 mg/kg/day), oral prednisolone (started with 2 mg/kg/day for first 7 days and then gradually tapered), and oral ranitidine (2 mg/kg/day), and for ruptured bullae, topical mupirocin was prescribed to prevent secondary infections. Bullae started to disappear after 4–5 days leaving hypopigmented spots behind [Figure 5]. The parents were counseled regarding the prognosis and course of the disease. The patient is in follow-up for the last 1 year and doing fine except for a few relapses during common febrile illnesses which subsided with short course of oral corticosteroids.
Figure 5: Disappearance of lesions following successful treatment

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  Discussion Top

BM is a rare and severe variant of cutaneous mastocytosis. Other forms of cutaneous mastocytosis usually present in infancy with a slightly higher male preponderance. Although usually sporadic in incidence, many familial cases have also been reported.[4] Glu-839-Lyc c-kit mutation has been linked with the typical childhood disease.[5] Mostly, it follows an indolent course, and the skin lesions resolve by adolescence. However, unfortunately, the subtype of mastocytosis reported by us, i.e., BM in pediatric patients has guarded prognosis with the potential risk of experiencing shock and sudden death.[5]

Cutaneous mastocytosis is associated with both local and systemic symptoms that are caused by the excessive production of mast cell-secreted mediators such as histamine, leukotrienes, proteases, and/or heparin. Although skin is the most common organ involved, other organs, i.e., liver, spleen, bones (lytic lesions), gastrointestinal tract, etc., might also be involved in this disorder giving rise to varied clinical presentation.[6] The symptoms may include cutaneous flushing, blisters, pruritus, respiratory distress, syncope, bone pain, epigastric pain, vomiting, diarrhea, etc.[7] Darier's sign is typically present.[7]

At times, the clinical picture of BM is misdiagnosed as case of staphylococcal scalded skin syndrome, erythema multiforme, or epidermolysis bullosa due to the presence of bullous lesions resembling those found commonly in the aforesaid conditions.

The diagnosis of BM is based on the clinical features and histopathology examination. Demonstration of abnormal proliferation of dermal mast cells clinches the diagnosis. The appropriate stains used to detect mast cells in the tissues include Giemsa, toluidine blue, Leder, and monoclonal antibodies that recognize tryptase or CD117.[7] Serum tryptase level is usually raised.

The main goal of treatment is to control signs and symptoms caused by release of mediators from mast cells.

Combinations of H1 and H2 blocking agents have been the mainstay of treatment for most of the uncomplicated BM cases. However, these drugs provide only symptomatic relief and do not alter disease course.[8] Other drugs mentioned in various studies include mast cell stabilizers such as ketotifen, psoralen and ultraviolet A, and alpha interferon. Systemic and topical corticosteroids show good result (as in our case) as reported by several authors.[6],[9]

Physicians should warn the parents regarding avoidance of conditions that are associated with excessive degranulation of mast cells such as drugs (e.g., aspirin, codeine, morphine, quinine, radiographic contrasts, thiamine, etc.), bacterial toxins, physical stimuli (sunlight, heat, cold, etc.), poisons (e.g., snake and hymenoptera venoms), polymers such as dextrans, and biological peptides (e.g., wasp, bees, lobster, and ascaris).

Declaration of Patient Consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of Interest

There are no conflicts of interest.

  References Top

Prendiville JS, Krol AL. Diseases of the dermis and subcutaneous tissues. In: Schachner LA, Hansen RC, editors. Pediatr Dermatol. 3rd ed. Edinburgh: Mosby; 2003. p. 713-67.  Back to cited text no. 1
Golkar L, Bernhard JD. Mastocytosis. Lancet 1997;349:1379-85.  Back to cited text no. 2
Hartmann K, Metcalfe DD. Pediatric mastocytosis. Hematol Oncol Clin North Am 2000;14:625-40.  Back to cited text no. 3
Shaw JM. Genetic aspects of urticaria pigmentosa. Arch Dermatol 1968;97:137-8.  Back to cited text no. 4
Heide R, Tank B, Oranje AP. Mastocytosis in childhood. Pediatr Dermatol 2002;19:375-81.  Back to cited text no. 5
Hartmann K, Henz BM. Mastocytosis: Recent advances in defining the disease. Br J Dermatol 2001;144:682-95.  Back to cited text no. 6
Tharp MD. Mastocytosis. In: Bolognia JL, Jorizzo JL, Rapini RP, editors. Dermatology. 2nd ed. New York: Mosby; 2008. p. 1845-53.  Back to cited text no. 7
Gerrard JW, Ko C. Urticaria pigmentosa: Treatment with cimetidine and chlorpheniramine. J Pediatr 1979;94:843-4.  Back to cited text no. 8
Verma KK, Bhat R, Singh MK. Bullous mastocytosis treated with oral betamethasone therapy. Indian J Pediatr 2004;71:261-3.  Back to cited text no. 9


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]


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