Lichen mixedematos

Terms of lichen mixedematos, papular or scleromixedem mucinoza are used together to describe the same condition. Exista un spectru de conditii patologice cu forme mai localizate, mai putin severe denumite general lichen mixedematos sau mucinoza papulara iar forma mai difuza, sclerotica se refera la scleromixedem. There is a spectrum of pathological conditions with more localized, less severe or mucinoza mixedematos called papular lichen general and diffuse forms, refers to scleromixedem sclera. Lichenul mixedematos este o afectiune a pielii rara caracterizata de proliferare fibroblastica si depozitare de mucina in derm, in absenta bolii tiroidiene. Mixedematos Lichen is a rare skin disorder characterized by fibroblast proliferation and storage of mucin in the dermis, in the absence of thyroid disease.
Lichenul mixedematos este o afectiune cronica de obicei. Lichen mixedematos is usually a chronic disease. Desi cei mai multi pacienti prezinta o paraproteinemie monoclonala , asociaza rar mielomul multiplu . Totusi cind mielomul multiplu este prezent pacientul are un prognostic negativ. Although most patients have a monoclonal paraproteinemias, rarely associated multiple myeloma . However, multiple myeloma is present when the patient has a negative prognosis. Pacientii cu afectare cardiaca sau pulmonara au de asemeni prognostic negativ. Patients with heart or lung disease also have negative prognostic.
Clinic pacientii descriu aparitia unor papule pruritice generalizate sau localizate, pe fata, extremitati si degete . Clinic patients describe the occurrence of generalized or localized pruritic papules on the face, extremities and fingers. Acestea sunt acompaniate de chisturi si leziuni urticariene . They are accompanied by urticarial lesions and cysts. Scleromixedemul prezinta piele indurate, eritematoasa similara scleroedemului . Scleromixedemul shows skin indurated, erythematous scleroedemului similar. Ridurile de expresie a fetei sunt diminuate. Facial expression wrinkles are diminished. Mobilitatea degetelor este diminuata. Finger mobility is diminished. Aceasta forma se asociaza si cu manifestari gastrointestinale, cardiovasculare si pulmonare. This form is associated with gastrointestinal manifestations, cardiovascular and pulmonary.
Tratamentul lichenului mixedematos este dificil si frecvent ineficient. S-au incercat terapii cu retinoizi, PUVA, plasmafereza, excizie cu laser, dermoabraziune, radioterapie, dexametazona, cu rezultate variabile. Treatment of lichen mixedematos is difficult and often ineffective. We have tried therapy with retinoids, PUVA, plasmapheresis, laser excision, dermoabraziune, radiation therapy, dexamethasone, with variable results. Evolutia bolii este cronica cu tendinte de rezolutia spontana. Evolution is chronic disease with a tendency to spontaneous resolution. Scleromixedemul prezinta un prognostic nefavorabil fata de alte forme. Scleromixedemul shows a poor prognosis than other forms. Pacientii cu implicare pulmonara si cardiaca prezinta de asemeni un prognostic negativ. Patients with pulmonary and cardiac involvement also shows a negative prognosis.

Patogenie si cauze Pathogenesis and causes
Etiologia este necunoscuta totusi boala este frecvent asociata cu discrazia celulelor plasmatice . The etiology is still unknown disease is frequently associated with plasma cell dyscrasia. Defectul de baza este considerat a fi afectarea fibroblastelor determinind cresterea depozitarii de mucina in piele. Cei mai mutli pacienti prezinta banda de paraproteine monoclonale la electroforeza, de tip Ig G. Asocierea dintre paraproteine si depozitarea de mucina nu este clara, iar paraproteinele nu stimuleaza direct fibroblastele. Basic defect is thought to be affecting the fibroblasts increased the storage of mucin in the skin. Mutli Most patients have a monoclonal band paraproteinemic electrophoresis, Ig G type paraproteinemic association between mucin and storage is not clear, and not directly stimulates paraproteinele fibroblasts.

Semne si simptome Signs and symptoms
Lichenul mixedematos sau mucinoza papulara. Mucinoza mixedematos or papular lichen.
Pacientii acuza debutul prin papule asimptomatice sau pruritice, care pot fi generalizate sau localizate . Patients began by accusing asymptomatic or pruritic papules, which may be generalized or localized. Nu sunt prezente simptome sistemice. No systemic symptoms are present. Leziunea primara este o papula in dom, de 2-4 mm, de culoarea pielii sau eritematoasa . Primary lesion is a papule in the dome, 2-4 mm, flesh-colored or erythematous. Aceste leziuni pot fuziona in papule lichenoide pe fata dorsal a miinilor, fata sau suprafetele extensoare ale bratelor si picioarelor . These lesions may coalesce in lichenoid papules on the back of the hands, face and extensor surfaces of the arms and legs. Papulele au un model cu linii paralele. Papules have a pattern of parallel lines. La pacientii cu forma lichenoida generalizata, ridurile si pliurile faciale pot fi distorsionate , aceasta conditie denumindu-se fata leonina . In patients with generalized lichenoid form, facial wrinkles and folds can be distorted, this condition is to naming leonina. Pacientii cu fata leonina au dificultati in deschiderea cavitatii bucale. Patients face leonina have difficulty opening the mouth.

Scleromixedemul Scleromixedemul
Pacientii cu aceasta forma prezinta indurare extinsa si diminuarea mobilitatii fetei, degetelor si extremitatilor . Patients with this form shows mercy and decreased mobility extended face, fingers and extremities. Pacientii prezinta chisturi si leziuni urticariene. Pot acuza simptome sistemice cum este disfagia sau slabiciunea . Patients have cysts and urticarial lesions. I blame systemic symptoms such as dysphagia or weakness. Leziunile primare constau in piele indurata, eritematoasa generalizata similara scleredemului, cu ingrosarea difuza a pielii. Primary skin lesions consist endured, generalized erythematous scleredemului similar, with diffuse thickening of the skin. Mobilitatea fetei si a extremitatilor este diminuata. Face and extremities, mobility is diminished.
Manifestarile sistemice includ disfunctie obstructiva si restrictie pulmonara, anomalii cardiovasculare si poliartrita. Boala de plamini se manifesta prin dispnee de efort . Systemic manifestations include dysfunction and obstructive pulmonary restriction, cardiovascular abnormalities and arthritis. Lung disease is manifested by dyspnoea of effort . Simptomele gastrointestinale sunt relationate cu lipsa peristalticii esofagiene . Gastrointestinal symptoms are related to the lack peristalticii esophagus. Se mai raporteaza slabiciune musculara, poliartrita, afectare cerebrala, miopatie inflamatorie . It is also reported muscle weakness, arthritis, brain damage, inflammatory myopathy. Simptomele oftalmologice cuprind ectroprion si opacitati corneene. Anomaliile cardiac apar la 10% din cazuri. Ectroprion Symptoms include eye and corneal opacity. Cardiac anomalies occur in 10% of cases.

Diagnostic Diagnosis
Studii de laborator: Laboratory studies:
-imunoelectroforeza proteinelor serice arata prezenta paraproteinei Show the presence of serum proteins, immunoelectrophoresis paraproteinemic
-putini pacienti prezinta mielom sau macroglobulinemie Waldenstrom -Few patients have myeloma or Waldenstrom's macroglobulinemia
-functiile tiroidei sunt normale Thyroid functions are normal-
-alte teste de laborator sunt normale. -Other laboratory tests are normal.
Diagnosticul diferential se face cu urmatoarele afectiuni: amiloidoza nodular localizata cutanata, milium colloidal, granulomul inelar, lepra , morfea , porfiria tarda cutanata, sarcoidoza , scleredemul, xantoamele. The differential diagnosis is made with the following conditions: amyloidosis Nodular localized cutaneous milium colloidal, annular granuloma, leprosy , Morf , porphyria cutanea tarda, sarcoidosis , scleredemul, xantoamele.

Tratament Treatment

Tratamentul este dificil si ineficient. Treatment is difficult and inefficient. Au fost incercate numeroase regimuri terapeutice incluzind PUVA, retinoizii, corticosteroizii, plasmafereza, laserul, dermoabraziune. Tacrolimusul topic a dovedit beneficii in boala localizata. Regimens have been tried many including PUVA, retinoids, corticosteroids, plasmapheresis, laser, dermoabraziune. Tacrolimus topic has proven benefit in localized disease.
Prognostic. Prognosis.
Pentru licehnul mixedematos evolutia este cronica fara tendinta la remisiune. For licehnul mixedematos evolution is chronic without remission tendency. Scleromixedemul are un prognostic negativ fata de alte forme. Scleromixedemul has a negative prognosis than other forms. Cauza frecventa de deces include complicatiile agentilor terapeutici sistemici precum melfalan sau afectarea cardiaca. Common cause of death include complications of systemic therapeutic agents such as melphalan or heart disease. Desi multi pacienti au paraproteinemie clonala putini dezvolta mielom multiplu , daca acesta este prezent prognosticul este nefavorabil. Although many patients have few clonal paraproteinemias develop multiple myeloma , if it is present the prognosis is unfavorable. Pacientii cu afectare pulmonara au de asemeni un prognostic negativ. Patients with pulmonary disease also have a negative prognosis.