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Diffuse cutaneous mastocytosis with bullous lesions and pulmonary involvement: a rare case.

Dhar S, Maji B, Roy S, Dhar S - Indian J Dermatol (2015 Mar-Apr)

Bottom Line: Mastocytosis is defined as a heterogeneous group of disorders characterized by an accumulation of mast cells in one or more organs, particularly in the skin, bone marrow, liver, spleen and lymph nodes.However here we describe an 11-month-old girl child presented with diffuse cutaneous mastocytosis with bullous lesion, having pulmonary involvement, which is very rare and there is no available case report from India.

View Article: PubMed Central - PubMed

Affiliation: Department of Paediatrics, Institute of Child Health, Kolkata Reference Lab, SRL Ltd., Kolkata, West Bengal, India.

ABSTRACT
Mastocytosis is defined as a heterogeneous group of disorders characterized by an accumulation of mast cells in one or more organs, particularly in the skin, bone marrow, liver, spleen and lymph nodes. However here we describe an 11-month-old girl child presented with diffuse cutaneous mastocytosis with bullous lesion, having pulmonary involvement, which is very rare and there is no available case report from India.

No MeSH data available.


Related in: MedlinePlus

(a) Sub-epidermal bullae with a dense cellular infiltration in the upper dermis (H and E, ×200). (b) Dense mast cell infiltration with some eosinophils in the upper dermis (H and E, ×400)
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Figure 2: (a) Sub-epidermal bullae with a dense cellular infiltration in the upper dermis (H and E, ×200). (b) Dense mast cell infiltration with some eosinophils in the upper dermis (H and E, ×400)

Mentions: The histological examination of a biopsy taken from the thigh revealed sub-epidermal bullae with a dense infiltration of mast cells and in the upper dermis [Figure 2]. The toluidine blue and Giemsa stains showed that almost all of the infiltrating cells in the dermis were mast cells [Figure 3]. The direct immunofluorescence was negative. The diagnosis of dilated cardiomyopathy (DCM) was made based on these clinical and histopathological findings. The patient was treated with oral betamethasone and oral hydroxyzine. On follow-up after 1 month, the skin lesions were improved, pulmonary infiltration were regressed and there was no such sudden attacks of the bouts of coughs or respiratory distress after starting steroid and antihistaminic.


Diffuse cutaneous mastocytosis with bullous lesions and pulmonary involvement: a rare case.

Dhar S, Maji B, Roy S, Dhar S - Indian J Dermatol (2015 Mar-Apr)

(a) Sub-epidermal bullae with a dense cellular infiltration in the upper dermis (H and E, ×200). (b) Dense mast cell infiltration with some eosinophils in the upper dermis (H and E, ×400)
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4372912&req=5

Figure 2: (a) Sub-epidermal bullae with a dense cellular infiltration in the upper dermis (H and E, ×200). (b) Dense mast cell infiltration with some eosinophils in the upper dermis (H and E, ×400)
Mentions: The histological examination of a biopsy taken from the thigh revealed sub-epidermal bullae with a dense infiltration of mast cells and in the upper dermis [Figure 2]. The toluidine blue and Giemsa stains showed that almost all of the infiltrating cells in the dermis were mast cells [Figure 3]. The direct immunofluorescence was negative. The diagnosis of dilated cardiomyopathy (DCM) was made based on these clinical and histopathological findings. The patient was treated with oral betamethasone and oral hydroxyzine. On follow-up after 1 month, the skin lesions were improved, pulmonary infiltration were regressed and there was no such sudden attacks of the bouts of coughs or respiratory distress after starting steroid and antihistaminic.

Bottom Line: Mastocytosis is defined as a heterogeneous group of disorders characterized by an accumulation of mast cells in one or more organs, particularly in the skin, bone marrow, liver, spleen and lymph nodes.However here we describe an 11-month-old girl child presented with diffuse cutaneous mastocytosis with bullous lesion, having pulmonary involvement, which is very rare and there is no available case report from India.

View Article: PubMed Central - PubMed

Affiliation: Department of Paediatrics, Institute of Child Health, Kolkata Reference Lab, SRL Ltd., Kolkata, West Bengal, India.

ABSTRACT
Mastocytosis is defined as a heterogeneous group of disorders characterized by an accumulation of mast cells in one or more organs, particularly in the skin, bone marrow, liver, spleen and lymph nodes. However here we describe an 11-month-old girl child presented with diffuse cutaneous mastocytosis with bullous lesion, having pulmonary involvement, which is very rare and there is no available case report from India.

No MeSH data available.


Related in: MedlinePlus