Pityriasis rubra pilaris (PRP) is an uncommon dermatosis occasionally reported in association with an underlying malignancy. We report a 61-year-old woman in whom PRP preceded the diagnosis of bronchogenic carcinoma by approximately 11 months. The clinical picture in this case included atypical cutaneous signs and mucous membrane involvement. Curative treatment of the malignancy with radiotherapy led to resolution of the mucocutaneous changes within months. A presentation of PRP, particularly if atypical, or in an older patient, should prompt consideration of an associated internal malignancy.
A 53-year-old woman with necrobiotic xanthogranuloma presented as infiltrated eyelid plaques, with later development of arm and thigh lesions. Clinical features, including association with immunoglobulin G-kappa paraprotein, and pathological findings were typical of this disorder. Treatment for 15 months with varying combinations of prednisone and multiple chemotherapeutic agents (melphalan, cyclophosphamide and chlorambucil) has led to minimal or no improvement in the clinical lesions or paraprotein. The case demonstrates some of the difficulties in managing this unusual disorder.
@r1.7, a highly purified proteolytic eilzyrne fr-on1 ~l s p e r g i l l~~v orysae, acts 011 a 11nrnbea of the c~:~yulatiori iactors. I n low c-oncentrrstio~~s, inlrncclinte action is to decrease the rec.alcifleti clottirrg tirne. 'The c\idcc~ce, as furrlish~d by exg~erirnientl-011 a prothroinbin fractio~i, is t h a t it C~) C S thi:, tiy co~iv~'rhi~lg pruthronltin to thronr~bii~. In this regard its actio11 is similar to that of thr-onil~r~plastii~. Ilurialg inc-uI,ation, espec*irnl%y mith ialcreasing concentraticirrii, it destroys prothrombi11 and fibrinogen, dud plasirla beco~i~es inc-ongul,ible. It aicstroys ilc~l-~nal pl,sm'~ ailtithronlbirr hut, paradoxically, generates ;t. thrortibiii-fibrinogen inhibiting dctivity by its proteoiytic action on f~briiiogcii in plasma. 111 :t search for better tllerapeaatic-agents to aid the treatraaent of thro~l-nbotic disorders, Stefai-aiali and illari~a (li described the preparation of a crrlde proteolytic extract frorn i1,sf)ergiii'us oryznc. Connaught hIedicaI Research 1,aboratories iinprovecl the t.xtr:~ction armd obtained a Inore l~urified product (lot 142). Tising this anaterial, Stefanini and his colleregucs studied its action on blood coagulation in vitro (2) and in rnlan (3).More recently Tosol-ai and his colleagues a t the Connaught Lal~sratorics have produced a still more acaive inaterial (Ci1.7). 'I'lais r~matcrial has the characteristics of a single enz3me. Roschlau has recently (4) evaluated the use of this eilzynle in the renlov:\l of experirncntal arterial arscl venous tlmrombi and devised : I technique for evaluatiisg indiviclual variations in protcase resistance. Our paper descritxs the action of Ci1.7 CHI different con~ponents of the blood clottirlg system.
Methods and MaterialsRecnlci,fied clolting tis7zes were carried out at 37' ' C. 'To 0.1 1111 aliquots of citrated or oxalated plasrrla 0.1 rlnl of 0.0:3 , I f 'I'ris buffer and 0.1 ml of calcium chloride were added. The buffer was used as a mlet1iur-n ira which to add the proteolytic enzyrile to the test system.
Idiopathic dermatitis over the site of previous trauma is a recognized but infrequently reported phenomenon that provides a diagnostic dilemma. Careful investigation is needed in order to exclude other rare and/or treatable causes.
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