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Cutaneous mastocytosis. Getting beneath the skin of the issue: a case report.

Tamhankar PM, Suvarna J, Deshmukh CT - Cases J (2009)

Bottom Line: Automated epinephrine self-injectors usually prescribed in this condition for self-management of anaphylactic episodes were not available.Intramuscular administration of (1:1000) diluted adrenaline via a disposable tuberculin syringe was taught to the mother.A medical bracelet containing her diagnosis and instructions in emergency was custom-made for her.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Medical Genetics, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India. paragt_md@rediffmail.com.

ABSTRACT
An eleven month old girl presented with chronic urticaria since three months of age. There was a generalised hyperpigmented maculo-papular rash. Darier sign was positive. The skin biopsy showed plenty of spindle shaped mast cells with eosinophilic cytoplasm infiltrating the dermis and the appendiceal structures. The diagnosis of cutaneous mastocytosis (urticaria pigmentosa) was made. The child received symptomatic relief with chronic oral hydroxyzine and ranitidine therapy. Automated epinephrine self-injectors usually prescribed in this condition for self-management of anaphylactic episodes were not available. Intramuscular administration of (1:1000) diluted adrenaline via a disposable tuberculin syringe was taught to the mother. A medical bracelet containing her diagnosis and instructions in emergency was custom-made for her.

No MeSH data available.


Related in: MedlinePlus

(A) Generalised hyperpigmented maculopapular rash. Palms, soles and face are also involved. (B) Darier sign: stroking the papular lesion linearly with a blunt instrument gives rise to linear wheal in (C).
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Figure 1: (A) Generalised hyperpigmented maculopapular rash. Palms, soles and face are also involved. (B) Darier sign: stroking the papular lesion linearly with a blunt instrument gives rise to linear wheal in (C).

Mentions: An eleven months old girl presented with a generalized maculo-papular hyperpigmented pruritic rash since 3 months of age. The lesions became more prominent and itchy after a warm bath and in states of excitement (playing, crying etc.). Examination revealed multiple oval/round hyperpigmented papules and plaques distributed all over the body (face, trunk, extremities and acral areas too)(Fig. 1-A). Similar lesions were present on oral mucosa (cheeks and lips). Darier's sign (papular lesion becomes a palpable wheal after being vigorously rubbed) was positive.(Fig. 1-B &1-C) Pruritus was exaggerated after emotional excitement.** Patchy non-scarring scalp alopecia was present. There was no pallor, edema, lymphadenopathy or hepatosplenomegaly. The child's growth and development parameters were normal for her age. Skin biopsy showed plenty of spindle shaped mast cells with eosinophilic cytoplasm infiltrating the dermis and the appendiceal structures (black arrows). The basal cells showed more pigmentation (blue arrows).(Fig. 2) Complete hemogram, skeletal survey, liver function tests, blood coagulation profiles were normal. Bone marrow was normocellular.


Cutaneous mastocytosis. Getting beneath the skin of the issue: a case report.

Tamhankar PM, Suvarna J, Deshmukh CT - Cases J (2009)

(A) Generalised hyperpigmented maculopapular rash. Palms, soles and face are also involved. (B) Darier sign: stroking the papular lesion linearly with a blunt instrument gives rise to linear wheal in (C).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2651115&req=5

Figure 1: (A) Generalised hyperpigmented maculopapular rash. Palms, soles and face are also involved. (B) Darier sign: stroking the papular lesion linearly with a blunt instrument gives rise to linear wheal in (C).
Mentions: An eleven months old girl presented with a generalized maculo-papular hyperpigmented pruritic rash since 3 months of age. The lesions became more prominent and itchy after a warm bath and in states of excitement (playing, crying etc.). Examination revealed multiple oval/round hyperpigmented papules and plaques distributed all over the body (face, trunk, extremities and acral areas too)(Fig. 1-A). Similar lesions were present on oral mucosa (cheeks and lips). Darier's sign (papular lesion becomes a palpable wheal after being vigorously rubbed) was positive.(Fig. 1-B &1-C) Pruritus was exaggerated after emotional excitement.** Patchy non-scarring scalp alopecia was present. There was no pallor, edema, lymphadenopathy or hepatosplenomegaly. The child's growth and development parameters were normal for her age. Skin biopsy showed plenty of spindle shaped mast cells with eosinophilic cytoplasm infiltrating the dermis and the appendiceal structures (black arrows). The basal cells showed more pigmentation (blue arrows).(Fig. 2) Complete hemogram, skeletal survey, liver function tests, blood coagulation profiles were normal. Bone marrow was normocellular.

Bottom Line: Automated epinephrine self-injectors usually prescribed in this condition for self-management of anaphylactic episodes were not available.Intramuscular administration of (1:1000) diluted adrenaline via a disposable tuberculin syringe was taught to the mother.A medical bracelet containing her diagnosis and instructions in emergency was custom-made for her.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Medical Genetics, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India. paragt_md@rediffmail.com.

ABSTRACT
An eleven month old girl presented with chronic urticaria since three months of age. There was a generalised hyperpigmented maculo-papular rash. Darier sign was positive. The skin biopsy showed plenty of spindle shaped mast cells with eosinophilic cytoplasm infiltrating the dermis and the appendiceal structures. The diagnosis of cutaneous mastocytosis (urticaria pigmentosa) was made. The child received symptomatic relief with chronic oral hydroxyzine and ranitidine therapy. Automated epinephrine self-injectors usually prescribed in this condition for self-management of anaphylactic episodes were not available. Intramuscular administration of (1:1000) diluted adrenaline via a disposable tuberculin syringe was taught to the mother. A medical bracelet containing her diagnosis and instructions in emergency was custom-made for her.

No MeSH data available.


Related in: MedlinePlus