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Year : 2017  |  Volume : 18  |  Issue : 2  |  Page : 128-131

Bullous mastocytosis in an infant treated with the help of narrowband ultraviolet B

1 Department of Dermatology, Shri Ram Murti Smarak Institute of Medical Sciences, Bareilly, Uttar Pradesh, India
2 Department of Pediatrics, Shri Ram Murti Smarak Institute of Medical Sciences, Bareilly, Uttar Pradesh, India
3 Department of Pathology, Shri Ram Murti Smarak Institute of Medical Sciences, Bareilly, Uttar Pradesh, India

Date of Web Publication27-Mar-2017

Correspondence Address:
Parineeta Maria
442-D, Ashadeep, Near DAV School, Sector 4, New Shimla, Shimla - 171 009, Himachal Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2319-7250.188452

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Mastocytosis represents a group of disorders characterized by abnormal accumulation of mast cells in the bone marrow, liver, spleen, lymph nodes, gastrointestinal tract, and skin. Skin involvement is seen in almost all the cases. A 1-year-old male child presented with complaints of multiple fluid-filled lesions all over the body since 2 months. Examination revealed multiple vesicles and tense bullae present on the face, neck and trunk along with surrounding erythema and multiple excoriation marks. Darier's sign was positive. Histopathology showed dense infiltrates of mast cells in the papillary dermis. Bone marrow aspiration showed no mast cells infiltrate. The patient was started on oral antihistamines, prednisolone, and narrowband ultraviolet B (NBUVB). The skin lesions showed dramatic improvement within a week and patient is on follow-up with NBUVB. Bullous mastocytosis (BM) is a rare variant of cutaneous mastocytosis manifested by diffuse infiltration of skin by mast cells, where cutaneous bullae are the predominant feature. This case is being presented for its rarity and highlights the effectivity of NBUVB for treating BM.

Keywords: Mast cells, phototherapy, toluidine blue staining

How to cite this article:
Maria P, Chauhan S, Agrawal T, Gahalaut P. Bullous mastocytosis in an infant treated with the help of narrowband ultraviolet B. Indian J Paediatr Dermatol 2017;18:128-31

How to cite this URL:
Maria P, Chauhan S, Agrawal T, Gahalaut P. Bullous mastocytosis in an infant treated with the help of narrowband ultraviolet B. Indian J Paediatr Dermatol [serial online] 2017 [cited 2020 Oct 31];18:128-31. Available from: https://www.ijpd.in/text.asp?2017/18/2/128/188452

  Introduction Top

Mastocytosis represents a group of disorders characterized by abnormal accumulation of mast cells in the bone marrow, liver, spleen, lymph nodes, gastrointestinal tract, and skin.[1],[2] Cutaneous mastocytosis (CM) is commonly seen in children and/or has a good prognosis.[3] In children, the prevalence is estimated at approximately 5.4 cases per 1000 children treated in dermatological clinics.[4] The various categories of CM are urticaria pigmentosa, mastocytoma, diffuse CM (DCM), and telangiectasia macularis eruptiva perstans (TMEP) in order of frequency of occurrence.[3],[5] DCM disease has two clinical variants, pseudoxanthomatous or xanthelasmoid and bullous mastocytosis (BM).[3],[5] CM generally has a benign course and spontaneous resolution in puberty, but BM has a reserved prognosis.[6]

  Case Report Top

A 1-year-old male child presented to our emergency room with multiple bullous eruption and pruritus all over the body. He was a single child born by normal vaginal delivery with uneventful birth and neonatal period. He was apparently alright till 2 months of age when he started to develop recurrent episodes of itching all over the body along with wheals which usually resolved after taking medicines from a local physician. The patient started to develop coalescing vesicles and bullae along with itching from the last 1 week on the trunk and back.

Cutaneous examination revealed multiple tense bullae and vesicles filled with clear fluid over the lateral aspect of the neck, chest, abdomen [Figure 1] and back [Figure 2]. The underlying skin was thickened with peau d'orange like appearance. The palms, soles, and mucous membranes were free. Darier's sign was positive.
Figure 1: Multiple bullae and grouped vesicles with clear fluid on the chest and abdomen

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Figure 2: Multiple coalesced vesicles and bullae and erosion present on the back

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Routine hematological investigations, urine analysis, and vital parameters were within normal limits. Skin biopsy obtained from the back, showed mast cell infiltrates in the papillary dermis extending deep down [Figure 3]. Toluidine blue staining showed the presence of metachromatic granules in the cells [Figure 4] and a diagnosis of BM was confirmed. Bone marrow aspiration showed no abnormality.
Figure 3: Histopathological examination of a skin fragment showing mast cell infiltrate in the papillary dermis extending deep down (H and E, ×40)

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Figure 4: Histopathological examination of a skin fragment with dense inflammatory infiltrate and predominance of mast cells(arrow), stained by toluidin blue (40×)

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The patient was treated with fexofenadine (30 mg/5 ml) 2.5 ml twice daily, topical mupirocin for erosions and narrowband ultraviolet B (NBUVB) starting with 0.5 J/cm 2 and slowly escalated to 1.2 J/cm 2. We also added prednisolone in a dose of 15 mg and improvement was seen in 5–6 days subsequently the steroid was tapered slowly over the next 2–3 weeks. He continued on NBUVB, and his lesions subsided almost completely in 3 months and then he was lost to follow-up. He again presented to us with the complaints of increased pruritus after 1 month and was again started on NBUVB 0.5 J/cm 2 twice a week with improvement but was again lost to follow-up after 2 months.

  Discussion Top

Mast cells arise from the bone marrow as agranular, undifferentiated, CD34+, KIT+ (CD117) pluripotent progenitor cells. After migrating into the tissues, immature mast cells assume their typical granular morphology. DCM is a rare variant and accounts to 1–3% cases of CM. There is diffuse infiltration of MCs in the skin.[7] DCM commonly begin in early infancy with a diffuse leathery (pachydermatous) thickening of the skin that may be erythematous or yellow-brown (“peau d'orange” appearance).[8] The entity associated with generalized blistering (BM) is a rare subtype of CM that is characterized by partly hemorrhagic bullae, erosions, and crusts. In BM, blisters are often the first manifestation of the disease and are much more prominent and persistent than in other CM types such as urticaria pigmentosa, mastocytoma, or TMEP, where vesiculation is a common yet transient (incidental) phenomenon in infancy and childhood.[8]

The demonstration of increased mast cell in mid and superficial dermis of characteristic cutaneous lesions is the golden standard for the diagnosis of mastocytosis.[6] Although bullous lesions can be observed in the early phase of all the subtypes of mastocytosis, it is a predominant and persistent feature of BM with frequent systemic involvement.[3]

Although pediatric onset of cutaneous disease has a favorable prognosis with at least 50% cases resolving by adulthood,[1] BM in childhood has a reserved prognosis with the risk of experiencing shock and sudden death.[3]

The management of patients includes counseling the patient and caretakers about the features, avoidance of factors that provoke mast cell mediator release and their symptoms. The patient should be cautioned to avoid the mast cell degranulating agents and early treatment of any infectious condition.[1],[3],[7] The combination of H1 and H2 blocking agents are helpful in reducing pruritus and flushing associated with histamine release.[1],[3] Disodium cromoglycate a mast cell stabilizer is effective in controlling cutaneous and systemic symptoms.[3] Oral glucocorticoids are also efficacious in patients and should be tapered to lowest effective dose after attaining control.[1],[3] Oral psoralen ultraviolet A therapy has been used effectively for bullous DCM in children as young as 4 months.[7],[9],[10] NBUVB has been used in treating indolent systemic mastocytosis (ISM).[11]

NBUVB decreases the production of pro-inflammatory cytokines released by degranulation or probably also induces apoptosis of dermal mast cells. However, there are controversial results regarding mast cell depletion. UVB phototherapy is also known to induce the production of the anti-inflammatory cytokine interleukin-10, which may be responsible for the immunosuppressive and anti-inflammatory effects of UVB. We know that exposure of human skin to suberythemogenic doses of UVB can result in immunological effects including alterations in circulating CD4/CD8 T-lymphocyte ratio. Under certain circumstances, T cells can induce activation of mast cells, as well as histamine release. It was proposed that UVB primarily affects the T cells in lesional skin.[12]

In our case, the patient was started on antihistaminics, NBUVB, short course oral steroids which were tapered. The patient was maintained on alternate day NBUVB with almost the complete resolution of the bullous lesions and the other symptoms. On resuming treatment again, NBUVB twice a week and antihistaminics showed good response, even without glucocorticoids with the resolution of the lesions and pruritus.

NBVUB has been effectively used earlier to treat ISM in older age group.[11] Our patient had no systemic complaints and considering the inconsistent and irregular follow-up by parents immunosuppressants were not considered. Financial constraints and age made biologicals an unviable option. Hence, it was decided to try NBUVB phototherapy in this child. In the past NBUVB, phototherapy has been used in a 10-month-old infant [13] and 15-month-old child,[14] safely, and effectively for other diseases.

Hence, NBUVB was tried in the infant because it is considered the safest of all the UV therapies for children.

Besides the rarity of BM in infancy, the present case adds upon the growing evidence for the efficacy of NBUVB in treating CM since older case reports of BM from India have not used phototherapy for treating mastocytosis.[2],[3]


Authors would like to thank Dr. Nitin Mishra and Dr. Madhur Kant Rastogi for their guidance and help in writing the case report. We would also like to thank the patient and his parents for their cooperation.

Financial Support and Sponsorship


Conflicts of Interest

There are no conflicts of interest.

  References Top

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Asati DP, Tiwari A. Bullous mastocytosis in a 3-month-old infant. Indian Dermatol Online J 2014;5:497-500.  Back to cited text no. 2
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Fernandes EI, de Faria BC, Cartell A, dos Santos BA, Cestari TF. Systemic mastocytosis in childhood: Report of 3 cases. J Pediatr (Rio J) 2002;78:176-80.  Back to cited text no. 4
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Kleewein K, Lang R, Diem A, Vogel T, Pohla-Gubo G, Bauer JW, et al. Diffuse cutaneous mastocytosis masquerading as epidermolysis bullosa. Pediatr dermatol 2011;28:720-5.  Back to cited text no. 8
Castells M, Metcalfe DD, Escribano L. Diagnosis and treatment of cutaneous mastocytosis in children: Practical recommendations. Am J Clin Dermatol 2011;12:259-70.  Back to cited text no. 9
Mackey S, Pride HB, Tyler WB. Diffuse Cutaneous MastocytosisTreatment With Oral Psoralen Plus UV-A. Arch Dermatol 1996;132:1429-30.  Back to cited text no. 10
Brazzelli.V, Grasso.V, Manna.G, Barbaccia.V, Merante S, Boveri E, et al. Indolent systemic mastocytosis treated with narrow-band UVB phototherapy: study of five cases. J Eur Acad Dermatol Venereol 2012;26:465-9.  Back to cited text no. 11
Engin B, Özdemir M, Balev A, Mevlitoğlu I. Treatment of Chronic Urticaria with Narrowband Ultraviolet B Phototherapy: a Randomized Controlled Trial. Acta Derm Venereol 2008;88:247-51.  Back to cited text no. 12
Do JE, Lee JY, Kim YC. Successful treatment of cutaneous Langerhans' cell histiocytosis with targeted narrowband ultraviolet B phototherapy in an infant. Clin Exp Dermatol 2009;34:e280-1.  Back to cited text no. 13
Ness MJ, Lowe GC, Davis DMR, Hand JL. Narrowband Ultraviolet B Light in Langerhans Cell Histiocytosis: A Case Report. Pediatric Dermatol 2014;31:e10-2.  Back to cited text no. 14


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


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