A 63-year-old Caucasian woman presented with a 6-month history of erythematous papules on the lower extremities. She complained of mild pruritus and a burning sensation of the lower extremities but denied flushing, gastrointestinal complaints, palpitations or syncope. Her past medical history was unremarkable.Physical examination on both lower extremities revealed numerous red-brown macules and papules varying in size from 2 mm to 4 mm. Head, neck, trunk and upper extremities were spared. Stroking of one of the papules revealed a mild urtication with surrounding erythema (Darier’s sign). Hepatomegaly, splenomegaly and lymphadenopathy were absent. A complete blood count, chemistry profile, liver function tests, rheumatoid factor, antinuclear antibody, erythrocyte sedimentation rate, and a rapid plasma reagin test were normal or negative. Serum plasma electrophoresis, serum tryptase levels, and 24-hour urine for metabolites of histamine were within normal limits. No significant abnormalities were found on the chest X-ray. Histology A biopsy taken from a representative papule on the left thigh showed an epidermis within normal limits. The dermis contained slightly dilated capillaries. At the periphery of the capillaries a mild lymphohistiocytic infiltrate with occasional mast cells was present. A Giemsa stain showed about 10 mast cells per high power field. We diagnosed telangiectasia macularis eruptiva perstans (TMEP). More Details on this Condition TMEP is rare form of cutaneous mastocytosis, mainly affecting adults. Characteristically, TMEP consists of tan-to-brown macules and patchy erythema with telangiectases that most often involve the trunk. Unilateral facial involvement has been reported.Pruritus can occur in varying degrees. Systemic involvement is rare but can include episodic headaches, flushing, gastrointestinal complaints, palpitations, syncope and splenomegaly. Patients with extensive cutaneous lesions may have elevated 24-hour urine histamine excretion level. An elevated plasma tryptase level (>20 ng/mL) is usually suggestive of the diagnosis of systemic disease. Abnormal skeletal radiographs and increased number of mast cells in bone marrow biopsy may also be present. TMEP hasn’t been associated with malignancies. A histologic examination can confirm diagnosis of TMEP. Mast cells surrounding capillary venules and the superficial venular plexus can be detected with Giemsa, toluidine blue, chloroacetate esterase and aminocaproate esterase stains. Treatment The treatment of TMEP is usually symptomatic. H1 and H2 receptor antagonists and/or mast cell stabilizers like cromolyn with or without potent topical corticosteroids can help reduce pruritus or flushing. PUVA therapy results in general and cosmetic benefits. PUVA should be reserved for severe unresponsive cases in adults. Long-term complications should be considered prior to initiating the treatment. The use of flashlamp-pumped dye laser may also be helpful. In patients with a limited number of lesions, consider administering intralesional corticosteroid injections. Our patient was treated with fluocinolone ointment and hydroxyzine and reported a slight improvement in her symptoms but no change in the lesions’ appearance. She’s taking fexofenadine HCl (Allegra) 60 mg PO q.d. and was instructed to avoid major histamine release triggers. (See “Dr. Khachemoune’s Pearl.”)
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Erythematous Papules on the Legs: What’s Your Diagnosis?
A 63-year-old Caucasian woman presented with a 6-month history of erythematous papules on the lower extremities. She complained of mild pruritus and a burning sensation of the lower extremities but denied flushing, gastrointestinal complaints, palpitations or syncope. Her past medical history was unremarkable.Physical examination on both lower extremities revealed numerous red-brown macules and papules varying in size from 2 mm to 4 mm. Head, neck, trunk and upper extremities were spared. Stroking of one of the papules revealed a mild urtication with surrounding erythema (Darier’s sign). Hepatomegaly, splenomegaly and lymphadenopathy were absent. A complete blood count, chemistry profile, liver function tests, rheumatoid factor, antinuclear antibody, erythrocyte sedimentation rate, and a rapid plasma reagin test were normal or negative. Serum plasma electrophoresis, serum tryptase levels, and 24-hour urine for metabolites of histamine were within normal limits. No significant abnormalities were found on the chest X-ray. Histology A biopsy taken from a representative papule on the left thigh showed an epidermis within normal limits. The dermis contained slightly dilated capillaries. At the periphery of the capillaries a mild lymphohistiocytic infiltrate with occasional mast cells was present. A Giemsa stain showed about 10 mast cells per high power field. We diagnosed telangiectasia macularis eruptiva perstans (TMEP). More Details on this Condition TMEP is rare form of cutaneous mastocytosis, mainly affecting adults. Characteristically, TMEP consists of tan-to-brown macules and patchy erythema with telangiectases that most often involve the trunk. Unilateral facial involvement has been reported.Pruritus can occur in varying degrees. Systemic involvement is rare but can include episodic headaches, flushing, gastrointestinal complaints, palpitations, syncope and splenomegaly. Patients with extensive cutaneous lesions may have elevated 24-hour urine histamine excretion level. An elevated plasma tryptase level (>20 ng/mL) is usually suggestive of the diagnosis of systemic disease. Abnormal skeletal radiographs and increased number of mast cells in bone marrow biopsy may also be present. TMEP hasn’t been associated with malignancies. A histologic examination can confirm diagnosis of TMEP. Mast cells surrounding capillary venules and the superficial venular plexus can be detected with Giemsa, toluidine blue, chloroacetate esterase and aminocaproate esterase stains. Treatment The treatment of TMEP is usually symptomatic. H1 and H2 receptor antagonists and/or mast cell stabilizers like cromolyn with or without potent topical corticosteroids can help reduce pruritus or flushing. PUVA therapy results in general and cosmetic benefits. PUVA should be reserved for severe unresponsive cases in adults. Long-term complications should be considered prior to initiating the treatment. The use of flashlamp-pumped dye laser may also be helpful. In patients with a limited number of lesions, consider administering intralesional corticosteroid injections. Our patient was treated with fluocinolone ointment and hydroxyzine and reported a slight improvement in her symptoms but no change in the lesions’ appearance. She’s taking fexofenadine HCl (Allegra) 60 mg PO q.d. and was instructed to avoid major histamine release triggers. (See “Dr. Khachemoune’s Pearl.”)
A 63-year-old Caucasian woman presented with a 6-month history of erythematous papules on the lower extremities. She complained of mild pruritus and a burning sensation of the lower extremities but denied flushing, gastrointestinal complaints, palpitations or syncope. Her past medical history was unremarkable.Physical examination on both lower extremities revealed numerous red-brown macules and papules varying in size from 2 mm to 4 mm. Head, neck, trunk and upper extremities were spared. Stroking of one of the papules revealed a mild urtication with surrounding erythema (Darier’s sign). Hepatomegaly, splenomegaly and lymphadenopathy were absent. A complete blood count, chemistry profile, liver function tests, rheumatoid factor, antinuclear antibody, erythrocyte sedimentation rate, and a rapid plasma reagin test were normal or negative. Serum plasma electrophoresis, serum tryptase levels, and 24-hour urine for metabolites of histamine were within normal limits. No significant abnormalities were found on the chest X-ray. Histology A biopsy taken from a representative papule on the left thigh showed an epidermis within normal limits. The dermis contained slightly dilated capillaries. At the periphery of the capillaries a mild lymphohistiocytic infiltrate with occasional mast cells was present. A Giemsa stain showed about 10 mast cells per high power field. We diagnosed telangiectasia macularis eruptiva perstans (TMEP). More Details on this Condition TMEP is rare form of cutaneous mastocytosis, mainly affecting adults. Characteristically, TMEP consists of tan-to-brown macules and patchy erythema with telangiectases that most often involve the trunk. Unilateral facial involvement has been reported.Pruritus can occur in varying degrees. Systemic involvement is rare but can include episodic headaches, flushing, gastrointestinal complaints, palpitations, syncope and splenomegaly. Patients with extensive cutaneous lesions may have elevated 24-hour urine histamine excretion level. An elevated plasma tryptase level (>20 ng/mL) is usually suggestive of the diagnosis of systemic disease. Abnormal skeletal radiographs and increased number of mast cells in bone marrow biopsy may also be present. TMEP hasn’t been associated with malignancies. A histologic examination can confirm diagnosis of TMEP. Mast cells surrounding capillary venules and the superficial venular plexus can be detected with Giemsa, toluidine blue, chloroacetate esterase and aminocaproate esterase stains. Treatment The treatment of TMEP is usually symptomatic. H1 and H2 receptor antagonists and/or mast cell stabilizers like cromolyn with or without potent topical corticosteroids can help reduce pruritus or flushing. PUVA therapy results in general and cosmetic benefits. PUVA should be reserved for severe unresponsive cases in adults. Long-term complications should be considered prior to initiating the treatment. The use of flashlamp-pumped dye laser may also be helpful. In patients with a limited number of lesions, consider administering intralesional corticosteroid injections. Our patient was treated with fluocinolone ointment and hydroxyzine and reported a slight improvement in her symptoms but no change in the lesions’ appearance. She’s taking fexofenadine HCl (Allegra) 60 mg PO q.d. and was instructed to avoid major histamine release triggers. (See “Dr. Khachemoune’s Pearl.”)
