ABSTRACT
BACKGROUND: Many medications, including tumor necrosis factor antagonists, have been anecdotally reported to be effective in treating cutaneous sarcoidosis, but controlled study is lacking. OBJECTIVE: We sought to determine if adalimumab is a safe and effective treatment for cutaneous sarcoidosis. METHODS: Adalimumab or placebo was administered to 10 and 6 patients, respectively, in double-blind, randomized fashion for 12 weeks, followed by open-label treatment for an additional 12 weeks, followed by 8 weeks of no treatment. Assessments were made of cutaneous lesions, quality-of-life issues, laboratory findings, pulmonary function, and radiographic findings. RESULTS: At the end of the 12-week, double-blind phase, there was improvement in a number of cutaneous findings in the adalimumab-treated patients (group 1) relative to placebo recipients (group 2), most notably in target lesion area (P = .0203). At the end of the additional 12-week open-label phase, significant improvement relative to baseline was found for target lesion area (P = .0063), target lesion volume (P = .0225), and Dermatology Life Quality Index score (P = .0034). No significant changes were seen in pulmonary function tests, radiographic findings, or laboratory studies. After 8 weeks off treatment, there was some loss of this improvement. LIMITATIONS: Standardized, validated measures for cutaneous sarcoidosis are lacking. There may be observer bias in the open-label portion of this study. The small size of this study makes it difficult to generalize results. CONCLUSIONS: Adalimumab, at the dose and duration of treatment used in this study, is likely to be an effective and relatively safe suppressive treatment for cutaneous sarcoidosis.
Subject(s)
Anti-Inflammatory Agents/administration & dosage , Antibodies, Monoclonal, Humanized/administration & dosage , Sarcoidosis/drug therapy , Skin Diseases/drug therapy , Adalimumab , Anti-Inflammatory Agents/adverse effects , Antibodies, Monoclonal, Humanized/adverse effects , Biopsy , Double-Blind Method , Female , Humans , Male , Middle Aged , Placebos , Quality of Life , Sarcoidosis/pathology , Skin Diseases/pathology , Treatment Outcome , Tumor Necrosis Factor-alpha/antagonists & inhibitorsABSTRACT
Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue tumor arising in the dermis. It is notorious for high rates of local recurrence despite its low metastatic potential. Although the etiology is unknown, DFSP often is considered to arise within scars and at sites of prior vaccination or trauma. Clinically, DFSP can be highly variable and mimic other soft tissue proliferations. We present a case of recurrent DFSP arising at the site of a Rho(D) immune globulin (Rhlg) injection that was administered 7 years prior. We also discuss the diagnostic challenges of DFSP as well as the indolent and locally recurrent nature of the tumor. This case serves to remind dermatologists of the highly variable clinical appearance of DFSP as well as to warn against presumptive diagnoses of lesions that mimic keloids and hypertrophic scars.
Subject(s)
Cicatrix/complications , Dermatofibrosarcoma/etiology , Neoplasm Recurrence, Local/pathology , Rho(D) Immune Globulin/adverse effects , Skin Neoplasms/etiology , Adult , Buttocks , Dermatofibrosarcoma/pathology , Dermatofibrosarcoma/surgery , Female , Humans , Injections, Subcutaneous , Rho(D) Immune Globulin/administration & dosage , Skin Neoplasms/pathology , Skin Neoplasms/surgerySubject(s)
Fungemia/diagnosis , Liver Cirrhosis, Alcoholic/diagnosis , Meningitis, Fungal/diagnosis , Mucormycosis/diagnosis , Opportunistic Infections/diagnosis , Paranasal Sinus Diseases/diagnosis , Biopsy, Needle , Combined Modality Therapy , Critical Illness , Disease Progression , Emergency Service, Hospital , Fatal Outcome , Female , Fungemia/complications , Fungemia/immunology , Fungemia/therapy , Humans , Immunohistochemistry , Liver Cirrhosis, Alcoholic/complications , Liver Cirrhosis, Alcoholic/immunology , Meningitis, Fungal/complications , Meningitis, Fungal/immunology , Middle Aged , Mucormycosis/complications , Mucormycosis/immunology , Opportunistic Infections/complications , Opportunistic Infections/immunology , Opportunistic Infections/therapy , Paranasal Sinus Diseases/complications , Paranasal Sinus Diseases/immunology , Paranasal Sinus Diseases/therapyABSTRACT
Sweet syndrome, or acute febrile neutrophilic dermatosis, is characterized by the presence of fever, peripheral leukocytosis, painful erythematous plaques and nodules, and a predominately neutrophilic dermal infiltrate. We report a case occurring in a 10-week-old male child, with preceding upper respiratory tract, and gastrointestinal infection symptoms. Sweet syndrome occurring in an infant should prompt a work-up for immunodeficiency, as well as a review of the peripheral blood smear to rule out the rare case of malignancy.
