tacrolimus and Pemphigoid--Bullous

Bullous scabies is an uncommon subtype of scabies that frequently mimics other blistering skin diseases. Nocturnal pruritus is a hallmark symptom of bullous scabies. We report an unusual case of bullous scabies presenting in the absence of pruritus in an immunosuppressed pediatric patient. It is critical that clinicians consider the diagnosis of bullous scabies in any patient with bullae, irrespective of pruritus symptoms.

Topics: Adolescent; Alagille Syndrome; Diagnosis, Differential; Exanthema; Follow-Up Studies; Humans; Immunocompromised Host; Ivermectin; Liver Transplantation; Male; Pemphigoid, Bullous; Pruritus; Risk Assessment; Scabies; Tacrolimus

Topics: Blood Pressure; Graft Rejection; Humans; Immunosuppressive Agents; Kidney Transplantation; Male; Middle Aged; Mycophenolic Acid; Pemphigoid, Bullous; Tacrolimus

Topics: Adult; Enzyme-Linked Immunosorbent Assay; Fluorescent Antibody Technique, Indirect; Humans; Immunoglobulin G; Immunosuppressive Agents; Lupus Erythematosus, Cutaneous; Male; Pemphigoid, Bullous; Tacrolimus

Topics: Administration, Topical; Aged; Female; Fluorescent Antibody Technique, Direct; Humans; Immunosuppressive Agents; Pemphigoid, Bullous; Tacrolimus; Tibia

Pemphigoid vegetans is an exceptionally rare intertriginous variant of bullous pemphigoid characterized by vegetative and purulent lesions present in the groin, axillae, thighs, hands, eyelids and perioral regions. The clinical, histopathological and immunofluorescent profile of a new case of pemphigoid vegetans in a 79-year-old man is reported. Our patient had papillomatous plaques with pustules in the bilateral inguinal folds, which clinically resembled pemphigus vegetans. Also suggesting pemphigus vegetans, an initial skin biopsy showed eosinophilic spongiosis, while a second biopsy showed histological and immunological features diagnostic of pemphigoid. Because only a few cases of pemphigoid vegetans have been reported in the literature, clinical and morphological data are scant. Most reported cases were successfully treated with topical antibiotics or steroids; therefore, appropriate diagnosis of this rare lesion will assist management.

Topics: Aged; Anti-Infective Agents; Anti-Inflammatory Agents; Chlorhexidine; Clobetasol; Fluorescent Antibody Technique; Humans; Immunosuppressive Agents; Male; Pemphigoid, Bullous; Tacrolimus; Trimethoprim, Sulfamethoxazole Drug Combination

Ocular involvement in cicatricial pemphigoid often occurs from the onset. Certain forms are seen only in this condition.. A 31-year-old woman presented highly inflammatory conjunctivitis for several months associated with bilateral symblepharons and superficial punctuate keratitis around the left eye refractory to treatment. The patient had a history of mouth ulcers and sores on the scalp. Examination showed scalp lesions with crusts. Histological examination of these lesions revealed dermal-epidermal cleavage. Direct immunofluorescence showed sub-membrane deposits. Anti-basal membrane antibodies were positive. Immunotransfer confirmed the presence of circulating antibodies. This condition was controlled following administration of three courses of cyclophosphamide as a bolus. However, the symblepharons persisted in both eyes. Improvement lasted 4 years. The patient again consulted for inflammatory conjunctivitis and superficial punctate keratitis resulting in invalidating loss of visual acuity, associated with hypereosinophilia. Cortisone eye drops alone resulted in no improvement. Treatment was initiated with topical tacrolimus (Protopic) 0.03% comprising once-daily application to the conjunctiva in the evening. This therapy was well tolerated, and 2 daily applications could be given, followed by a dosage of 0.1%. Improvement was rapid and spectacular, with frank amelioration of visual acuity and resolution of the patient's keratitis. Treatment was continued for 4 months and gradually reduced to the 0.03% dosage level, with increasingly wide intervals between applications. There has been no relapse within the 12 months following the end of treatment.. Standard treatment of pharmacological cicatricial pemphigoid involves systemic immunosuppression since topical anti-inflammatories are ineffective. The mortality associated with this disease is due to iatrogenic complications. Tacrolimus exhibits extremely good penetration of the conjunctiva. Following administration at a concentration of 0.06% 3 times daily in 15 patients with inflammatory disease of the conjunctiva or the cornea, improvement was seen in 10 of these patients at 26 weeks. Tacrolimus appears to act through immunomodulatory and anti-inflammatory mechanisms. It induces local inhibition of T lymphocyte activation and reduces production of pro-inflammatory lymphokines. Oral tacrolimus cannot be used to control cicatricial pemphigoid refractory to standard immunomodulators. However, 3 three other cases involving topical treatment of cicatricial pemphigoid showed marked efficacy of treatment given for 2 to 6 months, with complete tolerability. Thus, topical tacrolimus appears to constitute an interesting alternative treatment in cicatricial pemphigoid.

Topics: Administration, Topical; Adult; Conjunctivitis; Cyclophosphamide; Eye Diseases; Female; Humans; Immunosuppressive Agents; Injections, Intravenous; Keratitis; Lymphocyte Activation; Pemphigoid, Bullous; T-Lymphocytes; Tacrolimus; Time Factors; Tissue Adhesions; Treatment Outcome; Visual Acuity

Bullous pemphigoid (BP) is a cutaneous autoimmune disease predominantly affecting older patients which can cause death either due to severe clinical manifestations or due to the side effects of systemic immunosuppressive treatment. Topical treatment with corticosteroids is an established alternative to systemic treatment. However, prolonged application is accompanied by side effects such as skin atrophy. Recently, the immunomodulatory calcineurin antagonists tacrolimus and pimecrolimus have been introduced for topical treatment of skin diseases. Tacrolimus has been reported to be effective in several inflammatory skin disorders such as atopic dermatitis, psoriasis, lichen planus, lupus erythematosus and pyoderma gangraenosum. Efficacy has also been described in the topical treatment of BP in some cases. Here we present the case of a 89 year old patient with BP. He was treated with 0.1% tacrolimus ointment, which was able to control the disease. We briefly review the literature and discuss the potential role of tacrolimus as a novel option for the topical treatment of BP.

Topics: Aged; Aged, 80 and over; Biopsy; Complement C3; Fluorescent Antibody Technique; Follow-Up Studies; Humans; Immunoglobulin G; Immunosuppressive Agents; Male; Ointments; Pemphigoid, Bullous; Skin; Tacrolimus; Time Factors; Treatment Outcome

Oral cicatricial pemphigoid is a chronic autoimmune blistering disease which affects predominantly the gingiva and the buccal mucosa. The pathogenesis of this disease is still incompletely understood; however, there is compelling evidence that cicatricial pemphigoid might be mediated by T lymphocytes. Therefore, we performed immunomodulatory therapy with topical tacrolimus in patients with long-standing, therapy-resistant oral cicatricial pemphigoid. Following 3 months of treatment, complete healing and ongoing remission could be achieved.

Topics: Aged; Autoimmune Diseases; Facial Dermatoses; Female; Humans; Immunosuppressive Agents; Male; Mouth Diseases; Ointments; Pemphigoid, Bullous; Tacrolimus; Time Factors; Treatment Outcome

Topics: Administration, Topical; Aged; Humans; Immunosuppressive Agents; Male; Ointments; Pemphigoid, Bullous; Tacrolimus; Treatment Outcome

Localized vulval childhood pemphigoid is a rare variant within the pemphigoid group. Although its prognosis seems favorable, the best therapeutic strategy remains unclear.. We here describe the case of an 8-year-old girl presenting with a 5-year history of relapsing vulval pain and lesions suggestive of lichen sclerosus. Clinical features, light microscopy and direct immunofluorescence microscopy were consistent with vulval cicatricial pemphigoid, although the autoantigen(s) involved could not be characterized. Her disease responded to treatment with topical tacrolimus ointment 0.1% within 3 months without any evidence for disease activity, except for slight residual scarring. After 12 months, her treatment was stopped without relapse.. This observation suggests that in this rare immune-mediated blistering disease topical tacrolimus is an interesting therapeutic option without the adverse effects associated with topical steroids.

Topics: Administration, Topical; Child; Female; Humans; Immunosuppressive Agents; Ointments; Pemphigoid, Bullous; Secondary Prevention; Tacrolimus; Vulvar Diseases

Topics: Administration, Topical; Aged; Female; Humans; Immunosuppressive Agents; Male; Middle Aged; Pemphigoid, Bullous; Tacrolimus

Topics: Aged; Aged, 80 and over; Hand Dermatoses; Humans; Immunosuppressive Agents; Male; Pemphigoid, Bullous; Tacrolimus