ABSTRACT
Background: Majocchi's granuloma (MG) is an uncommon deep fungal folliculitis predominantly caused by dermatophytes. Given the rarity of this condition, available data regarding predisposing comorbidities/risk factors, clinical characteristics, offending microbiologic pathogens, diagnostics, pathologic findings, and treatment approaches has been inferred from historical cases. Objectives: To review our institutional experience with MG. Methods: We retrospectively analyzed a multicenter cohort of adult patients diagnosed with MG between 1992 and 2022. Results: We analyzed 147 patients with MG, 105 of which were male with a median age of 55.6 years. Immunosuppressant and topical corticosteroid use were common prior to development of MG. Dermatologic lesions and their sites of involvement did not differ based on the immune status of patients. Trichophyton rubrum was the most common causative pathogen of MG, in addition to other dermatophytes. Treatment duration for all prescribed agents was median 31.5 days with oral terbinafine being the most frequently utilized agent. Clinical resolution was achieved in 96.6% of cases. Limitations: Retrospective, nonrandomized study. Conclusions: Although rare and clinically variable in presentation, diagnosis of MG often requires histopathologic confirmation to subsequently direct prolonged treatment with systemic antifungal therapy for mycological cure.
Subject(s)
Dermatitis , Hematologic Neoplasms , Lung Diseases , Pyoderma Gangrenosum , Sweet Syndrome , Humans , Retrospective Studies , Pyoderma Gangrenosum/complications , Sweet Syndrome/complications , Sweet Syndrome/diagnosis , Sweet Syndrome/pathology , Lung Diseases/complications , Dermatitis/complications , Hematologic Neoplasms/complicationsABSTRACT
Subepidermal (subepithelial) autoimmune blistering dermatoses are a group of rare skin disorders characterized by the disruption of the dermal-epidermal junction through the action of autoantibodies. The fourth article in this continuing medical education series presents the current validated disease activity scoring systems, serologic parameters, treatments, and clinical trials for bullous pemphigoid, mucous membrane pemphigoid, epidermolysis bullosa acquisita, bullous systemic lupus erythematosus, anti-p200 pemphigoid, linear IgA bullous dermatosis, and dermatitis herpetiformis.
Subject(s)
Autoantibodies/blood , Autoimmune Diseases/drug therapy , Immunologic Factors/administration & dosage , Photochemotherapy/methods , Skin Diseases, Vesiculobullous/drug therapy , Administration, Cutaneous , Administration, Oral , Autoantibodies/immunology , Autoimmune Diseases/blood , Autoimmune Diseases/diagnosis , Autoimmune Diseases/immunology , Biomarkers/blood , Clinical Trials as Topic , Cytokines/blood , Cytokines/immunology , Dermis/immunology , Dermis/pathology , Drug Therapy, Combination/methods , Glucocorticoids/administration & dosage , Humans , Severity of Illness Index , Skin Diseases, Vesiculobullous/blood , Skin Diseases, Vesiculobullous/diagnosis , Skin Diseases, Vesiculobullous/immunology , Treatment OutcomeABSTRACT
Subepithelial autoimmune blistering dermatoses are a group of rare skin disorders that are characterized by the disruption of the dermal-epidermal junction through the action of autoantibodies. The third article in this continuing medical education series explores the background, epidemiology, clinical features, and diagnostic criteria of each of the major subepithelial autoimmune blistering dermatoses, including bullous pemphigoid, pemphigoid gestationis, lichen planus pemphigoides, mucous membrane pemphigoid, linear IgA bullous dermatosis, and dermatitis herpetiformis.
Subject(s)
Autoimmune Diseases/diagnosis , Lichen Planus/diagnosis , Pemphigoid Gestationis/diagnosis , Skin Diseases, Vesiculobullous/diagnosis , Autoimmune Diseases/epidemiology , Autoimmune Diseases/immunology , Autoimmune Diseases/pathology , Dermis/immunology , Dermis/pathology , Female , Humans , Lichen Planus/epidemiology , Lichen Planus/immunology , Lichen Planus/pathology , Pemphigoid Gestationis/epidemiology , Pemphigoid Gestationis/immunology , Pemphigoid Gestationis/pathology , Pregnancy , Skin Diseases, Vesiculobullous/epidemiology , Skin Diseases, Vesiculobullous/immunology , Skin Diseases, Vesiculobullous/pathologyABSTRACT
Intraepithelial autoimmune blistering dermatoses are a rare group of skin disorders characterized by the intraepithelial disruption of intercellular connections through the action of autoantibodies. The first article in this continuing medical education series explores the background, epidemiology, clinical features, and diagnostic criteria of each of the major intraepithelial autoimmune blistering dermatoses, including pemphigus foliaceus, pemphigus erythematosus, pemphigus herpetiformis, fogo selvagem, pemphigus vulgaris, pemphigus vegetans, drug-induced pemphigus, IgA pemphigus, IgG/IgA pemphigus, and paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome.
Subject(s)
Autoimmune Diseases/diagnosis , Skin Diseases, Vesiculobullous/diagnosis , Skin/pathology , Autoimmune Diseases/epidemiology , Autoimmune Diseases/immunology , Autoimmune Diseases/pathology , Diagnosis, Differential , Humans , Skin/immunology , Skin Diseases, Vesiculobullous/epidemiology , Skin Diseases, Vesiculobullous/immunology , Skin Diseases, Vesiculobullous/pathologyABSTRACT
Intraepithelial autoimmune blistering dermatoses are a rare group of skin disorders characterized by disruptions of inter-keratinocyte connections within the epidermis through the action of autoantibodies. The second article in this continuing medical education series presents validated disease activity scoring systems, serologic parameters of disease, treatments, and clinical trials for pemphigus and its subtypes.
Subject(s)
Autoantibodies/blood , Autoimmune Diseases/therapy , Immunologic Factors/therapeutic use , Plasma Exchange , Skin Diseases, Vesiculobullous/therapy , Administration, Cutaneous , Administration, Oral , Autoantibodies/immunology , Autoimmune Diseases/blood , Autoimmune Diseases/diagnosis , Autoimmune Diseases/immunology , Drug Therapy, Combination/methods , Humans , Injections, Intralesional , Severity of Illness Index , Skin/immunology , Skin/pathology , Skin Diseases, Vesiculobullous/blood , Skin Diseases, Vesiculobullous/diagnosis , Skin Diseases, Vesiculobullous/immunology , Treatment OutcomeABSTRACT
BACKGROUND: Pyoderma gangrenosum (PG) is a devastating neutrophilic dermatosis that may be associated with trauma or systemic diseases. The associations, characteristics, and temporal relationship of PG with hematologic malignancies are not well understood. OBJECTIVE: We performed a systematic review of PG associated with hematologic malignancies using data from case reports, case series, and retrospective studies. METHODS: We searched MEDLINE, EMBASE, Scopus, and Web of Science from each database's inception to December 12, 2018. Two reviewers independently selected studies and extracted data. RESULTS: Two hundred seventy-nine publications met the inclusion criteria (340 cases). Myelodysplastic syndrome (MDS) was the most commonly reported hematologic malignancy associated with PG, followed by monoclonal gammopathy of undetermined significance and acute myeloid leukemia. The mean age of patients was 56.5 years, with males being more common. There was a predominance of the ulcerative PG subtype and multifocal distributions across all hematologic malignancies. The majority of MDS cases preceded PG, which was reversed for MGUS. LIMITATIONS: The data were limited by reporting bias because PG subtypes rely on the rendered diagnosis reported. In addition, the classification for hematologic malignancies has evolved since 1978. CONCLUSION: Patients with PG should be evaluated for hematologic malignancies, with MDS being the most common.