ABSTRACT
Cheilitis granulomatosa is a rare inflammatory disorder with unclear aetiology. It is a disorder characterized by recurrent or persistent swelling of one or both lips that may be part of the Melkersson-Rosenthal syndrome or may be a manifestation of Crohn's disease. An overview of the clinical features, histopathology, differential diagnosis, management strategies and prognosis of cheilitis granulomatosa is presented and discussed with regard to the literature.
Subject(s)
Cheilitis/drug therapy , Cheilitis/etiology , Crohn Disease/complications , Melkersson-Rosenthal Syndrome/complications , Anti-Inflammatory Agents/administration & dosage , Anti-Inflammatory Agents, Non-Steroidal/administration & dosage , Biopsy, Needle , Crohn Disease/diagnosis , Diagnosis, Differential , Female , Humans , Immunohistochemistry , Male , Melkersson-Rosenthal Syndrome/diagnosis , Prognosis , SteroidsABSTRACT
In a 45-year-old man who from early childhood had been suffering of periodic fever, which did not respond to any therapy attempted, the ultimate diagnosis was hyperimmunoglobulinaemia D syndrome (HIDS). HIDS attacks typically occur every 4-6 weeks and last 3-7 days. The most frequent symptoms are fever, diarrhoea, arthralgias, cold shivers, abdominal pain, vomiting and headache. Physical examination often reveals lymphadenopathy, skin lesions, arthritides, splenomegaly and serositis. Laboratory investigation includes an acute-phase response with granulocytosis and enhanced erythrocyte sedimentation rate. The serum concentration of IgD is increased as is the concentration of IgA. There is no causal therapy. A causative gene mutation was recently identified.
Subject(s)
Familial Mediterranean Fever/diagnosis , Familial Mediterranean Fever/genetics , Hypergammaglobulinemia/diagnosis , Immunoglobulin D/blood , Acute-Phase Reaction , Anti-Inflammatory Agents, Non-Steroidal/therapeutic use , Diagnosis, Differential , Familial Mediterranean Fever/drug therapy , Humans , Hypergammaglobulinemia/drug therapy , Hypergammaglobulinemia/genetics , Immunoglobulin A/blood , Immunoglobulin D/genetics , Male , Middle Aged , Treatment OutcomeABSTRACT
In three patients, a 12-year-old Muslim Moroccan girl with polyarthralgia and a wobbling gait, a 47-year-old emaciated Hindustani man with muscle weakness and on antiepileptic drugs, and a heavily-veiled native Dutch woman aged 34 years with polyarthralgia and psoriasis, clinical, laboratory and radiologic examination led to the diagnoses of rickets (the first patient) and of osteomalacia (the other two). They recovered after calcium and vitamin D suppletion. After the discovery of vitamin D and the beneficial effects of sunlight and cod liver oil rickets and osteomalacia became rare. Recently these diseases have to be considered more often again because of changes in the composition of the Dutch population: more immigrants and (institutionalized) elderly who are prone to develop a vitamin D deficiency. Dietary and clothing habits should be investigated when a patient complains about diffuse (bone) pains. In case of minimal exposure to sunlight and a diet low in vitamin D the symptoms may be explained easily by a vitamin D deficiency. Once hypovitaminosis D is diagnosed, the therapy is simple and effective.
