melphalan and Facial-Dermatoses

A 63-year-old patient had experienced bilateral spontaneous periorbital hemorrhage for one year. After hospitalization because of recurring hemoptysis, biopsies of skin and colon revealed systemic ALlambda amyloidosis. In addition, the heart and lungs appeared to be involved. Monoclonal gammopathy was excluded by a normal plasma cell count and a polyclonal expression pattern in a bone marrow sample and by radiographic examination. The patient was treated with a relatively non-aggressive regimen of melphalan and prednisolone monthly with careful hematologic monitoring. This approach led to a significant improvement in relevant parameters. Recent advances in diagnosis, monitoring and therapy have made it easier to manage patients with amyloidosis with their poor prognosis.

Topics: Amyloidosis; Drug Combinations; Eye Hemorrhage; Facial Dermatoses; Humans; Male; Melphalan; Middle Aged; Prednisolone; Treatment Outcome; Vascular Diseases

Scleromyxedema is a rare mucinous disease without thyroid dysfunction, associated with a monoclonal gammopathy. The deposits of mucin in the skin and other organs such as the cardiovasculary system, determine the prognosis of the disease. A 74-year old patient with initial scleromyxedema is described, in whom a pulse therapy with melphalan was initiated already in the early stages of the disease. Four cycles of melphalan in combination with prednisolon led to a complete and sofar stable remission of the disease.

Topics: Aged; Biopsy; Diagnosis, Differential; Drug Therapy, Combination; Facial Dermatoses; Female; Humans; Lichenoid Eruptions; Melphalan; Myxedema; Prednisolone; Skin

Topics: Adult; Allergens; Chlorambucil; Cross Reactions; Dermatitis, Allergic Contact; Dermatitis, Occupational; Diagnosis, Differential; Facial Dermatoses; Humans; Male; Medical Laboratory Personnel; Melphalan; Patch Tests

Scleredema is an uncommon disease of unknown origin. Characteristic thick skin with symmetrical diffuse induration develops. The infiltration begins on the face and neck then extends to the root of the upper limbs and trunk. There are three clinical types of scleredema. The first is preceded by an upper airway infection and progresses rapidly before regressing spontaneously within a few months. The second type is associated with chronic diabetes. The third type is associated with monoclonal gammapathy, rarely of myelomatous type, and develops insidiously. Acanthosis nigricans can be a paraneoplastic syndrome, often associated with a gastrointestinal cancer. Few cases associating scleredema and acanathosis nigricans have been reported.. A 56-year old woman had developed scleredema over the last 6 years when acanthosis nigricans appeared together with IgA kappa multiple myeloma. Treatment with melphalan and prednisolone was effective against the myeloma as well as the scleredema and acanthosis nigricans.. Only five cases of associated scleredema and multiple myeloma have been reported, four with kappa IgG myeloma and one with IgA myeloma. An association between acanthosis nigricans and sclerederma could be coincidental although the fact that the different manifestations regressed together after the myeloma treatment would suggest some relationship between these three diseases.

Topics: Acanthosis Nigricans; Antineoplastic Agents, Alkylating; Facial Dermatoses; Female; Humans; Immunoglobulin A; Immunoglobulin kappa-Chains; Melphalan; Middle Aged; Multiple Myeloma; Prednisolone; Scleredema Adultorum; Treatment Outcome