Subject(s)
Acanthosis Nigricans/drug therapy , Glycolates/administration & dosage , Keratolytic Agents/administration & dosage , Receptor, Fibroblast Growth Factor, Type 3/genetics , Acanthosis Nigricans/genetics , Acanthosis Nigricans/pathology , Administration, Cutaneous , Adolescent , Adult , Drug Administration Schedule , Female , Humans , Middle Aged , Mutation , Pedigree , Skin/drug effects , Skin/pathology , Treatment Outcome , Young AdultSubject(s)
Glucocorticoids/therapeutic use , IgA Vasculitis/diagnosis , IgA Vasculitis/drug therapy , Immunosuppressive Agents/therapeutic use , Nephritis/diagnosis , Nephritis/drug therapy , Prednisolone/therapeutic use , Ribonucleosides/therapeutic use , Tacrolimus/therapeutic use , Adolescent , Diagnosis, Differential , Drug Therapy, Combination , Humans , MaleABSTRACT
A 72-year-old woman with a history of diffuse large B cell lymphoma and recent recurrence visited our department complaining of several painful edematous nodules with blisters on her face. She had iteratively developed cutaneous eruptions after every treatment with granulocyte colony-stimulating factor (G-CSF) for neutropenia, and each time the eruption improved after the cessation of the G-CSF treatment. The blisters became crusty and the skin lesions slightly improved, but on the 24th hospital day, the eruption formed painful erythematous nodules with erosion, and the patient also developed a high fever of up to 38°C. A biopsy specimen showed a dermal infiltrate of increased and enlarged plump histiocytes, some of which indicated karyomitosis with a small number of lymphocytes. No increase in the number of eosinophils or neutrophils was noted. These eruptions lasted for 15 days and disappeared with the recovery of the peripheral blood count and attendant cessation of G-CSF. We diagnosed this case as G-CSF-induced granulomatous dermatitis with enlarged histiocytes. Several cases with maculopapular rash and dermal inflammatory infiltrate composed of interstitially arranged large histiocytes have been reported. However, to the best of our knowledge, this is the first case report of G-CSF-induced granulomatous dermatitis with enlarged histiocytes clinically manifesting as painful edematous nodules with a high fever, similar to Sweet's syndrome. We speculated that the infiltrating cells were not neutrophils but histiocytes, presumably because of agranulocytosis.
Subject(s)
Drug Eruptions/etiology , Filgrastim/adverse effects , Aged , Drug Eruptions/pathology , Edema/chemically induced , Edema/pathology , Female , Fever/chemically induced , Hematologic Agents/adverse effects , Histiocytes/pathology , Humans , Neutropenia/drug therapy , Sweet Syndrome/pathologyABSTRACT
We report on a 59-year-old man with a 1-year history of forearm erythema, bilateral limb arthralgia, and muscle weakness. During the initial examination we observed infiltrative erythema of the forearm and muscle weakness and atrophy of the limbs. Blood tests revealed marked increases in myogenic enzymes. Because histopathological studies showed lymphocytic infiltration around the small blood vessels in the dermis and mucin deposition, we made a tentative diagnosis of dermatomyositis. However, the specific cutaneous manifestations of dermatomyositis, including heliotrope erythema and Gottron's sign, were absent, and the findings of electromyography were normal. A subsequent detailed examination revealed hypothyroidism and high titers of antithyroglobulin and antimicrosome antibodies, and we made a definitive diagnosis of Hashimoto's thyroiditis. The thyroid function and skin manifestations both improved after treatment with levothyroxine sodium. Dermatomyositis and Hashimoto's thyroiditis can exhibit similar characteristics, and caution is required because of the possibility of misdiagnosis.
Subject(s)
Dermatomyositis/diagnosis , Hashimoto Disease/diagnosis , Diagnosis, Differential , Hashimoto Disease/drug therapy , Humans , Male , Middle Aged , Thyroxine/therapeutic useABSTRACT
BACKGROUND: A correlation between decreased blood coagulation factor XIII activity and the severity of organ disorders in pediatric Henoch-Schönlein purpura (HSP) has been demonstrated, but possible correlations in adult HSP have not been thoroughly investigated. OBJECTIVES: To investigate the association between factor XIII activity with varying clinical severities of HSP and the severity of organ disorders and to examine the efficacy of factor XIII substitution therapy. METHODS: The distribution of purpura and the severities of joint, abdominal, and renal symptoms were scored in 44 adults with HSP. Plasma factor XIII activity was measured with the latex agglutination immunoturbidity method. RESULTS: Reduced factor XIII activities were correlated with clinical severity scores (the total of all scores), organ disorder severity scores (the total score excluding the purpura score), joint symptom scores, and abdominal symptom scores but not with renal disorder scores. Factor XIII activities were increased in patients during posttreatment remission. Factor XIII substitution therapy was performed in 7 patients with severe organ disorders. Consequently, joint and abdominal symptoms markedly improved, but renal symptoms did not. CONCLUSION: Measurement of plasma factor XIII activity in adult HSP is clinically useful because it indicates disease severity and the severity of digestive tract and joint disorders. Factor XIII substitution therapy is effective for joint and abdominal symptoms but not for renal symptoms. Further investigation of the effect of this treatment on renal symptoms is necessary.