and Subcutaneous Tumors皮肤及皮下肿瘤（PPT76）课件.ppt

Dermal and Subcutaneous Tumors Mastocytosisurticaria pigmentosa Local and systemic accumulations of mast cells Persistent pigmented itchy skin lesions Urticate on mechanical or chemical irritation c-KIT mutation Birth to middle age, ? 6 mo Macules, papules, nodules, plaques, vesicles Lesions persist and gradually become chamois- or slate-colored Darier’s sign, pruritis Severe symptoms may result from massive liberation of histamine from mast cells after ingestion of known mast cell degranulators Spontaneous resolution is likely in those pts whose disease began in childhood Urticaria pigmentosa Solitary mastocytoma May be present at birth, may develop during the first weeks of life Brown macule that urticates upon stroking Smooth or peau d’ orange Dorsum of the hand near the wrist Edema, urtication, vesiculation may be observed Generalized eruption, childhood type Pseudoxanthomatous mastocytosis Diffuse cutaneous mastocytosis Generalized eruption, adult type Erythrodermic mastocytosis Telangiectasia macularis erupta perstans Systemic mastocytosis Familial urticaria pigmentosa Giemsa, azure A, or polychrome toluidine blue Local anesthetic adjacent to the lesion, without epi Dx is bx confirmed Histamine metabolites methylhistamine and methylimidazole acetic acid Prognosis and treatment In all forms without systemic involvement the prognosis is good Solitary lesions usually involute within 3 years H1 and H2 blockers PUVA Intralesional and topical steroids Avoid physical stimuli Abnormalities of neural tissue Solitary neurofibroma Soft, flaccid, pinkish white, 2-20 mm Invaginates on pressure, “buttonholing” Solitary or multiple Distinctive histopathologic findings, fibrils, cellular proliferation, and degenerative changes Sx excision Granular cell tumor Well-circumscribed, solitary firm nodule, with a brownish red or flesh tint Usually solitary, 10-15 % multiple 1/3 of cases have occurred on the tongue May occur anywhere on the body Grows slowly Cells stain positiv