Tofacitinib for the treatment of refractory pyoderma gangrenosum.

Pyoderma gangrenosum (PG) is a rare, debilitating, inflammatory skin disease associated with a variety of systemic diseases. Because of its rarity, PG is treated with miscellaneous immunosuppressive agents as there is no US Food and Drug Administration-approved standardized treatment approach. We present four patients with PG treated with tofacitinib in the context of the six existing cases in the literature. Tofacitinib appeared to be beneficial in the small sample of patients (n = 10) who failed an average of four other systemic therapies. The majority of patients had classic PG located on the legs (80%, 8/10), while 20% of cases (2/10) were peristomal. The most common comorbidity was inflammatory bowel disease (78%, 7/9). There were no negative treatment results and 40% (4/10) of patients had complete healing of their ulcers, while the other 60% (6/10) had marked clinical improvement. From our observation, tofacitinib appears to be a promising steroid-sparing adjuvant treatment in patients with refractory PG who have failed on other systemic therapies.

Unusual presentations of cutaneous tuberculosis.

Cutaneous tuberculosis occurs in 1-2% of world cases of tuberculosis and more common in tropical countries. It presents with different clinical forms. Unusual clinical presentations are not uncommon and awareness of these will help in suspecting and managing these patients successfully. Lupus pernio like lupus vulgaris, tuberculosis of glans penis and lichen scrofulosorum on the distal parts of limbs are presented here because of their unusual clinical presentation.

Recurrent Henoch-Schönlein Purpura with bullous rash and pulmonary nodules.

BACKGROUND: Henoch-Schönlein purpura (HSP) is the most common vasculitis of childhood. It has a characteristic rash described as palpable purpura that most frequently affects the distal lower extremities and buttocks. HSP rarely presents with bullous rash nor pulmonary nodules. CASE PRESENTATION: We present a novel case of a 12-years-old female with recurrent pediatric HSP with a combination of the rare manifestations of bullous rash and pulmonary nodules. She initially presented with the bullous rash, chest pain, cough, and abdominal pain. Patient was successfully treated with intravenous pulse corticosteroids followed by a high dose oral corticosteroid taper, with resolution of the bullous rash and pulmonary nodules. CONCLUSION: The rare manifestations of scarring bullous rash and pulmonary nodules can be presenting features of pediatric HSP, the combination of which has not been previously reported. The treatment of intravenous corticosteroid resolved patient's abdominal symptoms, rash and pulmonary nodules.

Treatment of erosive pustular dermatosis: a systematic review of the literature.

IMPORTANCE: Erosive pustular dermatosis (EPD) is a rare chronic inflammatory condition of the scalp and legs that is often difficult to manage. Currently, there are no treatment guidelines. OBJECTIVE: To systematically assess the existing literature on various treatment modalities and their efficacies when used in the management of EPD. EVIDENCE REVIEW: We searched PubMed, Cochrane Libraries, Scopus, and clicnialtrial.gov databases for articles in the English language with no limited time frame. Emphasis was placed on articles that reported on treatment for EPD. FINDINGS: Of the 168 articles identified by the literature search, 92 met eligibility criteria and were included for qualitative analysis. Efficacious topical treatments included clobetasol, betamethasone, and tacrolimus. Ninety-three and 88% of cases utilizing clobetasol and betamethasone respectively demonstrated improvement or resolution. All 32 cases utilizing tacrolimus reported improvement. Efficacious systemic treatments included oral steroids such as prednisone, methylprednisolone, and dexamethasone. Topical dapsone, photodynamic therapy, systemic steroids, cyclosporine, and oral zinc derivatives were also described with some success. CONCLUSIONS AND RELEVANCE: According to available data, limited solely to case reports and case series, potent topical steroids are an effective treatment option for EPD. Topical tacrolimus may also be considered in cases that require long-term use or maintenance. Other treatment modalities shown to be successful based on high reported efficacy and low rates of recurrence after treatment include topical dapsone, systemic steroids, zinc derivatives, and cyclosporine. Further studies are needed to compare treatment modalities and to establish treatment protocols.

Dermatosis ampollar IgA lineal: serie de 17 casos / Linear IgA Bullous Dermatosis: A Series of 17 Cases

La dermatosis ampollar IgA lineal es una enfermedad vesicoampollar subepidérmica, adquirida, mediada por inmunoglobulinas. Presentamos nuestra serie con el objetivo de describir las características clínicas, evolución y tratamientos instaurados. Se realizó un estudio descriptivo, observacional retrospectivo. Se incluyeron 17 pacientes. Como antecedentes 2 niños recibieron vacunas 2 semanas antes del inicio de los síntomas; en 2 casos la enfermedad estuvo precedida por cuadros respiratorios broncoobstructivos. Un paciente recibió antibioticoterapia endovenosa antes del inicio del cuadro. Hallamos asociación con hepatitis autoinmune en un caso y con alopecia areata en otro. Un niño padecía asociación VACTERL. El diagnóstico se confirmó con histopatología e inmunofluorescencia directa. Como tratamiento 16 pacientes recibieron dapsona, 8 de ellos asociaron corticoides orales y 2 esteroides tópicos. Destacamos la presencia de rebrotes con compromiso perioral ante cuadros infecciosos e inmunizaciones, la asociación con síndrome de VACTERL y con hepatitis autoinmune

Linear IgA bullous dermatosis is an acquired subepidermal immunoglobulin-mediated vesiculobullous disease. In this retrospective, observational, descriptive study, we describe the clinical characteristics, treatments, and outcomes of 17 patients with linear IgA bullous dermatosis. Two children had been vaccinated 2 weeks before the onset of symptoms, 2 had had bronco-obstructive respiratory symptoms, and 1 had received intravenous antibiotic therapy. We also observed an association with autoimmune hepatitis in one patient and alopecia areata in another. One boy had VACTERL association. Diagnosis was confirmed by histopathology and direct immunofluorescence. Sixteen patients were treated with dapsone, which was combined with oral corticosteroids in 8 cases and topical corticosteroids in two. Of note in this series was the occurrence of relapses in the perioral area coinciding with infections and vaccination, and the association between linear IgA bullous dermatosis and autoimmune hepatitis and VACTERL association

[Successful treatment of gangrenous pyoderma with methotrexate in a patient withCrohn's disease]. / Tratamiento exitoso del pioderma grangrenoso con metotrexate en pacientecon enfermedad de Crohn.

Pyoderma gangrenosum (PG) is a neutrophilic, cutaneous-ulcerative, non-infectious dermatosis that occurs in up to 2% of patients with Crohn's disease (CD). Its appearance is usually independent of the clinical course of CD. The most used therapies have been systemic corticosteroids, cyclosporine and biological therapy, which have shown good results. Methotrexate (MTX) is an antimetabolite drug with anti-inflammatory activity characterized by a weekly dosage, a faster onset of action, with a good safety profile related to malignant neoplasms and, above all, a lower cost. It is effective in the induction of remission in patients with CD, however its efficacy in the management of extraintestinal manifestations such as PG is uncertain. We present the case of a young man who comes to the emergency room with an history of chronic diarrhea, weight loss, and fever accompanied by a painful, erythematous nodule, located at the lower extremity compatible with PG in the context of CD. It was initially managed with systemic corticosteroids without improvement. Concomitant therapy with MTX was started with a favorable clinical outcome. In conclusion, the concomitant therapy of systemic corticosteroids and MTX in patients with CD with PG could be an alternative treatment in cases where cyclosporine or biological therapy is not available.

Tratamiento exitoso del pioderma grangrenoso con metotrexate en paciente con enfermedad de Crohn / Successful treatment of gangrenous pyoderma with methotrexate in a patient with Crohn's disease

El pioderma gangrenoso (PG), es una dermatosis neutrofílica, cutáneo-ulcerativa, no infecciosa, que ocurre hasta en un 2% de los pacientes con enfermedad de Crohn (EC). Su aparición suele ser independiente del curso clínico de la EC. Las terapias más utilizadas han sido los corticoides sistémicos, la ciclosporina y la terapia biológica, los cuales han mostrado buenos resultados. El Metotrexate (MTX) es un fármaco antimetabolito con actividad antiinflamatoriaque se caracteriza por una dosificación semanal, un inicio de acción más rápida, con buen perfil de seguridad relacionado con neoplasias malignas y sobretodo un menor costo. Posee eficacia en la inducción de la remisión en pacientes con EC, sin embargo su eficacia en el manejo de manifestaciones extraintestinales como el PG es incierta. Presentamos el caso de un varón joven que acude a urgencias por diarrea crónica, pérdida de peso, y fiebre acompañado de un nódulo doloroso, eritematoso, situado a nivel pretibial compatible con PG en el contexto de EC. Fue manejado inicialmente con corticoides sistémicos sin mejoría. De tal forma, se inició terapia concomitante con MTX con respuesta clínica y analítica favorable. En conclusión, la terapia concomitante de cortiocoides sistémicos y MTX en pacientes con EC con PG podría suponer un tratamiento alternativo al no contar con disponibilidad de ciclosporina o terapia biológica en nuestro medio.

Pyoderma gangrenosum (PG) is a neutrophilic, cutaneous-ulcerative, non-infectious dermatosis that occurs in up to 2% of patients with Crohn's disease (CD). Its appearance is usually independent of the clinical course of CD. The most used therapies have been systemic corticosteroids, cyclosporine and biological therapy, which have shown good results. Methotrexate (MTX) is an antimetabolite drug with anti-inflammatory activity characterized by a weekly dosage, a faster onset of action, with a good safety profile related to malignant neoplasms and, above all, a lower cost. It is effective in the induction of remission in patients with CD, however its efficacy in the management of extraintestinal manifestations such as PG is uncertain. We present the case of a young man who comes to the emergency room with an history of chronic diarrhea, weight loss, and fever accompanied by a painful, erythematous nodule, located at the lower extremity compatible with PG in the context of CD. It was initially managed with systemic corticosteroids without improvement. Concomitant therapy with MTX was started with a favorable clinical outcome. In conclusion, the concomitant therapy of systemic corticosteroids and MTX in patients with CD with PG could be an alternative treatment in cases where cyclosporine or biological therapy is not available.

Clinical Experience with Rituximab and Intravenous Immunoglobulin for Pretibial Myxedema: A Case Series.

Background: Severe pretibial myxedema (PTM) can be difficult to manage, highlighting the need to investigate newer therapies. Rituximab (RTX) and intravenous immunoglobulin (IVIg) have been tried in Graves' orbitopathy. Since PTM and orbitopathy share a similar underlying pathophysiology, this study aimed to explore these therapies for progressive PTM. Methods: The electronic database was screened for PTM patients evaluated at the Mayo Clinic, Rochester, from 2002 to 2016, and three patients who received IVIg and five who received RTX are reported. PTM pattern was classified as non-pitting edema, plaque and induration, nodular/nummular, and elephantiasis. PTM was confirmed by biopsy in six patients. Results: The patients' median age was 53.8 years, 75% were female, and all but one patient were either active or former smokers. All patients were euthyroid and had progressed despite various therapies prior to starting these agents. Six patients had a plaque and induration pattern, and two had a nodular pattern with elephantiasis. After therapy, six (75%) patients had PTM stability or improvement both subjectively and objectively (80% with RTX and 66% with IVIg). The three patients (one in the IVIg group and two in the RTX group) who had subjective improvement had a plaque pattern. One patient with elephantiasis had a transient response to IVIg and another had stability after RTX. Thyrotropin receptor antibody values and orbitopathy also improved in patients who demonstrated PTM improvement. No serious adverse events were reported, but one patient each had transient hypertension and injection-site thrombophlebitis after IVIg. Conclusions: Immunomodulation therapy was followed by PTM improvement or stability in most patients, with a slightly better response after RTX compared to IVIg. A validated response assessment instrument and larger series of patients are required to determine if the underlying disease process could be curtailed with these agents.