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LETTER TO THE EDITOR
Year : 2017  |  Volume : 6  |  Issue : 1  |  Page : 69-71

Bullous urticaria pigmentosa – A rare case report


Department of DVL, Gandhi Hospital, Hyderabad, Telangana, India

Date of Web Publication20-Mar-2017

Correspondence Address:
Sudha R Chintagunta
Department of DVL, Gandhi Hospital, Hyderabad, Telangana
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/JDRNTRUHS.JDRNTRUHS_97_16

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How to cite this article:
Chintagunta SR, Srinivas M, SriShilpa P, Nagula SK, Velgam R. Bullous urticaria pigmentosa – A rare case report. J NTR Univ Health Sci 2017;6:69-71

How to cite this URL:
Chintagunta SR, Srinivas M, SriShilpa P, Nagula SK, Velgam R. Bullous urticaria pigmentosa – A rare case report. J NTR Univ Health Sci [serial online] 2017 [cited 2019 Nov 13];6:69-71. Available from: http://www.jdrntruhs.org/text.asp?2017/6/1/69/202588



Sir,

Mastocytosis is a disease associated with infiltration of tissues by mast cells. Cutaneous mastocytosis (CM) is characterized by the presence of increased dermal mast cells, and the symptoms are caused by the release of mediators and their local and/or systemic actions.[1] CM is the most common form in children and can be classified into urticaria pigmentosa, solitary mastocytoma, and diffuse CM. Urticaria pigmentosa is the most common form of pediatric onset mastocytosis accounting for 70–90% of the cases, presenting as well-demarcated red-to-brown macules, plaques, or nodules.[2] Urticaria pigmentosa presenting with vesicular and bullous lesions as the predominant feature is a rare entity. We present the case of an 8-month-old infant with bullous urticaria pigmentosa, a rare clinical variant.

An 8-month-old boy presented with multiple blisters and hyperpigmented skin lesions affecting the head, trunk, and extremities associated with intense itching. The rash started at 5 months of age which was preceded by severe itching. There was no history of flushing, diarrhea, or fainting episodes. General and systemic examination were unremarkable. Cutaneous examination revealed generalized involvement with multiple pigmented macules, erythematous, edematous plaques ranging 0.5–3 cm in size. Multiple tense vesicles, bullae, erosions, and crusted lesions were present over the face, scalp, trunk [Figure 1], and upper and lower extremities. There was diffuse hyperpigmentation all over the body without scarring. Darier's sign could be elicited on both lesional and nonlesional areas [Figure 2].
Figure 1: Multiple tense bullae and hyperpigmentation on the upper back and extremities

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Figure 2: Positive Darier sign and diffuse hyperpigmention on the trunk

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Routine investigations were within normal limits. Chest X-ray and ultrasound of the abdomen were normal. Skin biopsy from the lesional skin revealed epidermis with mild acanthosis. Dermis showed a diffuse infiltrate of mast cells with small round-to-oval nuclei, with some of them showing nuclear groove and scanty cytoplasm. Toluidine blue stain showed metachromatic granules [Figure 3] and [Figure 4]. Based on the clinical and histologic findings, positive Darier sign, and absence of systemic involvement, a diagnosis of bullous urticaria pigmentosa was made. The child was treated with oral hydroxyzine and topical 0.25% desonide ointment once daily for 3 weeks. All the cutaneous lesions healed with hyperpigmentation in 3 weeks. The parents were instructed about careful handling and avoiding exposure to specific drugs.
Figure 3: Round to oval cells with darkly stained nucleus (H and E , X40)

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Figure 4: Metachromatic granules in the cytoplasm of mast cells. (High power, Toludine blue stain)

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Skin is the most common organ involved in children and may be the only manifestation of the disease. Children with CM typically present with a spectrum of findings ranging from solitary or multiple mastocytomas to urticaria pigmentosa, or diffuse cutaneous mastocytosis (DCM).[3] Urticaria pigmentosa is the most frequent form of CM that presents as red-brown macules, papules, or plaques predominantly on the trunk. Darier's sign, i.e., development of wheal on stroking of the lesional skin is present in a majority of cases. Blistering may occur in younger individuals, particularly with urticaria pigmentosa or DCM and generally heal without scarring, except in case of secondary infection.[4]

Bullous urticaria pigmentosa is described as an extreme form of urticaria pigmentosa that is difficult to distinguish from bullous mastocytosis, which is a form of diffuse CM. Bullae were described as a predominant feature in two forms of CM by Orkin et al., i.e., the neonatal form, with a greater risk of severe complications and extracutaneous involvement, and late onset, which shows minimal extracutaneous involvement and a better prognosis. Our patient fits in to the later presentation.

CM is diagnosed on the basis of history, clinical presentation, and histopathological examination of skin biopsy. Stains such as toluidine blue or Giemsa help to identify mast cells in the tissues through demonstration of metachromatic granules. Though there is no standardized treatment till date, management is focused on counselling, trigger avoidance, and symptom control.[2] H1 antihistamines are given to control pruritus, flushing, and urticarial, and H2 antihistamines for gastrointestinal symptoms. Oral mast cell-stabilizers (cromolyn) and ketotifen also work in some cases. In general, pediatric-onset mastocytosis is found to have good prognosis, except for diffuse CM which has a reserved prognosis.

CM in children may mimic many dermatological disorders in view of its varied clinical presentation, and hence, we need to be aware of these clinical patterns and differentiate them from other common childhood dermatoses.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Castells M, Metcalfe D, 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. 1
    
2.
Srinivas SM, Dhar S, Parikh D. Mastocytosis in children. Indian J Paediatr Dermatol 2015;16:57-63.  Back to cited text no. 2
  [Full text]  
3.
Carter MC, Metcalfe DD. Paediatric mastocytosis. Arch Dis Child 2002;86:315-9.  Back to cited text no. 3
    
4.
Caplan RM. Urticaria pigmentosa and systemic mastocytosis. JAMA 1965;194:1077-80.  Back to cited text no. 4
    


    Figures

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



 

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