ABSTRACT
Eight cases of sporadic acquired primary osteoma cutis have been reported in the literature. Age at onset varies from 16 to 55 years with a mean of about 35 years. The sex ratio is 1 and a wide range of localizations have been reported. There is no known treatment. We report a new case of primary osteoma cutis observed in an adult. The monomelic feature of this case has not been reported previously. The patient was 76 years old and had multiple painless, stone-like formations at several sites on the left thigh and leg since the age of 40. Histological examination of skin biopsies showed a perfectly differentiated bone tissue in the dermal layer. There was no similar family history nor abnormal morphotype. Likewise, the absence of laboratory signs of pseudohypoparathyroidism, together with the late and spontaneous onset allowed us to eliminate hereditary Albright's osteodystropy or secondary osteomatosis due to a local pathological process. Despite the late onset, the monomelic character of the osteomas observed and the association of hemicorporeal hypertrophy and linear basocellular naevi reported in the literature would suggest a hamartomatous origin rather than a metaplasic process in this patient.
Subject(s)
Osteoma/diagnosis , Skin Neoplasms/diagnosis , Adolescent , Adult , Aged , Calcinosis/diagnosis , Diagnosis, Differential , Female , Fibrous Dysplasia, Polyostotic/diagnosis , Humans , Male , Middle Aged , Osteoma/etiology , Osteoma/pathology , Skin Neoplasms/etiology , Skin Neoplasms/pathologySubject(s)
Cellulitis/etiology , Facial Dermatoses/etiology , Leukemia, Myelogenous, Chronic, BCR-ABL Positive/complications , Sweet Syndrome/etiology , Aged , Cellulitis/pathology , Diagnosis, Differential , Facial Dermatoses/pathology , Female , Humans , Pyoderma Gangrenosum/diagnosis , Sweet Syndrome/pathologyABSTRACT
INTRODUCTION: the association of acute febrile neutrophilic dermatosis (Sweet's syndrome) with malignant haemopathies is well known and characterized by an usual lack of hyperleukocytosis: indeed, moderate neutropenia is often reported. However, cases of Sweet's syndrome in the agranulocytosis stage are exceptional (7 in the literature). CASE-REPORT: We report the case of a woman with acute myeloblastic leukaemia who had presented with Sweet's syndrome in the phase of therapeutic aplasia during induction of treatment, in the absence of white blood cells transfusion or treatment with haematopoietic growth factor (GM CSF, GCSF). COMMENTS: the physiopathology of Sweet's syndrome is unknown. Various mechanisms have been suggested, including immune reaction type III, increased interleukin-1 synthesis, increased chemotaxis of neutrophils, action of haematopoietic growth factors, iatrogenic effect of some drugs (e.g. cotrimoxazole, furosemide or minocycline). Yet none of these mechanisms involving circulating polymorphonuclears or their bone marrow precursors can explain the occurrence of Sweet's syndrome in the phase of agranulocytosis. CONCLUSION: the diagnosis of Sweet's syndrome must be considered in patients with agranulocytosis in order to avoid ineffective antibiotics and to initiate a corticosteroid therapy that will accelerate the cure of this benign dermatosis.
Subject(s)
Agranulocytosis/complications , Leukemia, Myeloid, Acute/complications , Sweet Syndrome/etiology , Agranulocytosis/chemically induced , Cytarabine/adverse effects , Cytarabine/therapeutic use , Female , Humans , Hydrocortisone/therapeutic use , Leukemia, Myeloid, Acute/drug therapy , Middle Aged , Sweet Syndrome/drug therapy , Sweet Syndrome/pathologyABSTRACT
A case of basal cell carcinoma in a 17-year old male patient complicated, 5 years later, by inguinal and pulmonary metastases is reported. This clinical case raises two problems: the reality of the entity and the long-term follow-up of this type of tumours.
Subject(s)
Carcinoma, Basal Cell/pathology , Lung Neoplasms/secondary , Lymphatic Metastasis , Skin Neoplasms/pathology , Adolescent , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Carcinoma, Basal Cell/therapy , Combined Modality Therapy , Humans , Inguinal Canal , Lung Neoplasms/drug therapy , Male , Skin Neoplasms/therapyABSTRACT
An 84-year-old man who had the typical clinical features of Brunsting-Perry cicatricial pemphigoid is described. Direct immunofluorescence microscopic examination of salt-split skin revealed linear deposits of IgG and C3 on the floor of the artificial bullae. Direct immunoelectron microscopic examination of peribullous skin showed dermal cleavage level below the lamina densa and granular deposits of IgG and C3 attached to and below the lamina densa in a pattern identical to epidermolysis bullosa acquisita. These findings suggest that Brunsting-Perry cicatricial pemphigoid may represent a clinical variant of epidermolysis bullosa acquisita.
Subject(s)
Epidermolysis Bullosa Acquisita/complications , Pemphigoid, Benign Mucous Membrane/diagnosis , Aged , Aged, 80 and over , Biopsy , Blister/pathology , Chronic Disease , Complement C3/analysis , Epidermolysis Bullosa Acquisita/diagnosis , Epidermolysis Bullosa Acquisita/immunology , Fluorescent Antibody Technique , Humans , Immunoglobulin G/analysis , Male , Pemphigoid, Benign Mucous Membrane/immunology , Pemphigoid, Benign Mucous Membrane/pathology , Skin/immunologyABSTRACT
Werner's syndrome (adult progeria) is a rare autosomal recessive condition characterized mainly by a characteristic habitus (short stature, light body weight) scleroderma like changes of the limbs and premature aging. Chronic leg ulcers appears in about fifty per cent of the patients. These ulcers can be related to the combination of mechanical factors on atrophic subcutaneous tissue and skin of the feet and leg associated with early arteriosclerosis (20%) and diabetes mellitus (60%).
