Search

Eosinophilic fasciitis (EF) is an uncommon autoimmune connective tissue disorder characterized by edema, erythema, and subsequent induration of the extremities. It is commonly treated with corticosteroids but there is no treatment ladder for immunosuppressants or steroid-sparing agents. We report two EF cases treated effectively with mycophenolate mofetil (MMF) or mycophenolic acid (MPA) and present a literature review. We performed a MEDLINE search using the keywords 'eosinophilic fasciitis', 'Shulman syndrome', 'mycophenolic acid', or 'mofetil mycophenolate', and found 8 articles with 27 cases in which MMF or MPA was used. Twenty-nine cases were reviewed (2 reported herein and 27 from the literature search); all patients received a combination of systemic corticosteroids and MMF. MMF/MPA were given as a steroid-sparing agent in 27 (93.1%), in 1 (3.4%) as adjunctive therapy with other immunosuppressants, and in one, as monotherapy 1 (3.4%). Nineteen had a complete response, 6, a partial response, and 2 were unresponsive to diverse immunomodulators; in 2 cases, the outcome was not reported. MMF and MPA show promising therapeutic results and could be a treatment option to reduce corticosteroid related side effects.

We describe two cases of nodular basal cell carcinoma presenting with novel morphology of linear looped hairpin vessels under dermoscopy.

Dermoscopy of mycetoma has white structures as the predominant feature, while white scale and yellowish structures were also consistent in our findings with available literature.

Dear Editor, Primary cutaneous diffuse large B-cell lymphoma, leg-type (PCDLBCL-LT) is a rare and aggressive neoplasm. A timely diagnosis may prevent fatal outcomes; physicians should take this entity into consideration when assessing non-specific lesions on the lower limbs. We present a 69-year-old woman with a 1-month history of a firm plaque on her left leg. Physical examination revealed an asymptomatic, indurated, smooth, and erythematous plaque on the pretibial region of her left extremity (Figure 1, a). The rest of the physical examination was normal. Histological examination revealed cohesive sheets of a dense cell infiltrate in the dermis, composed of large round immunoblast-type cells with prominent nucleoli, and the presence of mitoses. Immunohistochemical stains were positive for CD20, Bcl2, and MUM1 (Figure 1, b-d). Additionally, c-MYC and Ki67 exhibited a 20% positivity; CD3 and CD10 were negative. The diagnosis of PCDLBCL-LT was established. Imaging and blood workup ruled out systemic involvement. Treatment with R-CHOP chemotherapy was initiated, with complete tumor regression by the third cycle. The patient completed 6 cycles and has remained disease-free after 18 months. Primary cutaneous B-cell lymphomas (CBCL) are lymphoproliferative disorders that appear on the skin without evidence of extracutaneous manifestations at the time of diagnosis (1). They represent 25 to 35% of all primary cutaneous lymphomas (2). In 2018, an updated version of the 2008 WHO-EORTC classification divided CBCLs into 5 subtypes: PCDLBCL-LT, primary cutaneous marginal zone B-cell lymphoma (PCMZL), primary cutaneous follicle center lymphoma (PCFCL), Epstein-Barr virus-positive mucocutaneous ulcer (EBVMCU), and intravascular large B-cell lymphoma (3). PCDLBCL-LT is the least common subtype, representing approximately 10% of all CBCLs and only 4% of all cutaneous lymphomas (2,3). Although the pathogenesis for most CBCLs is still unknown, positive serology for Lyme disease in a significant number of patients has been recognized as a probable etiologic association (4). PCDLBCL-LT is more frequent in women, and the mean age of presentation is 76 years. It usually presents as erythematous or bluish nodules, and up to 75% of the cases appears on one or both legs (1). Although infrequent, other locations have been reported, including the head, neck, trunk, and upper extremities (5). Workup should include a complete physical exam, skin biopsy, blood tests, and imaging (2,3). Histopathology shows a diffuse infiltrate in the dermis composed of large B-cells (centroblasts and/or immunoblasts) with extension to subcutaneous cellular tissue. These cells have round nuclei that are more than twice the size of normal lymphocytes, with prominent nucleoli. The immunophenotype of PCDLBCL-LT is CD20+, CD79a+, CD10-, and Bcl-6+/-, and strongly expresses Bcl-2, MUM1/IRF4, and FOX-P1 (1-3). Unlike the other indolent subtypes, PCDLBCL-LT is generally more aggressive with a poor prognosis. The 5-year disease survival rate is of approximately 50% (5). Management depends on the body surface area, location, and the patient's age and general health. To date, chemotherapy with R-CHOP remains the first line of therapy for PCDLBCL-LT, resulting in complete remission in up to 92% of cases (2). The prognostic characteristics of most PCDLBCL-LTs require timely and appropriate diagnosis and treatment.

Chikungunya and Zika virus infections are emerging diseases in the Americas, and dengue continues to be the most prevalent arthropod-borne virus in the world. These arbovirus diseases may spread by endemic transmission or as travel-related infections and have rapidly expanded their geographic distribution secondary to vector spread. All 3 share a similar clinical picture that includes a maculopapular rash. Zika is characterized by pruritic rash, low-grade fever, and arthralgia. Congenital nervous system malformations are a growing public-health concern. Chikungunya distinctive dermatologic manifestations include facial melanosis and bullous eruption. Dengue bleeding complications may be life-threatening and require inpatient management.

Mycetoma is an infrequent subcutaneous infection caused by true fungi (eumycetoma) or aerobic actinomycetes (actinomycetoma). We report the case of a 62-year-old man with eumycetoma involving the left foot and ankle. Skin biopsy revealed black-brown grains, and in culture, a white colony fungus grew at day 8. Molecular sequencing using ITS1-ITS4 primers identified the species as Aspergillus sydowii. The patient was treated with itraconazole 200 mg twice daily and terbinafine 250 mg daily for 8 months, with complete response and no recurrence after 2.5 years of follow-up. Aspergillus sydowii is a saprotrophic fungus that rarely causes skin or nail disease. No cases of eumycetoma caused by this agent have been previously reported. As its geographic distribution continues to expand, it may increasingly be recognized as a cause of human disease.

Author summary Patients who are immunocompromised or have cognitive or physical disabilities are at a higher risk of being affected with infections such as crusted scabies. This is a rare skin hyperinfestation by Sarcoptes scabiei var. hominis. The main characteristic of this dermatosis is a thick crust due to the high concentration of mites; in addition, other manifestations such as papules, excoriations, and burrows may be absent. In severe cases, thick yellow-brown crusts and plaques with deep fissures are present. Diagnosis can be made by observing mites, ova, or feces from skin scrapings. Multiple therapies can be used in patients with this condition. Management with patient isolation is important to prevent institutional outbreaks. This disease can have high mortality, primarily due to sepsis. Awareness of this condition and its serious consequences is important to reduce its mortality and morbidity.

Background Mycetoma is a neglected tropical disease characterized by nodules, scars, abscesses, and fistulae that drain serous or purulent material containing the etiological agent. Mycetoma may be caused by true fungi (eumycetoma) or filamentous aerobic bacteria (actinomycetoma). Mycetoma is more frequent in the so-called mycetoma belt (latitude 15° south and 30° north around the Tropic of Cancer), especially in Sudan, Nigeria, Somalia, India, Mexico, and Venezuela. The introduction of new antibiotics with fewer side effects, broader susceptibility profiles, and different administration routes has made information on actinomycetoma treatment and outcomes necessary. The objective of this report was to provide an update on clinical, therapeutic, and outcome data for patients with actinomycetoma attending a reference center in northeast Mexico. Methodology/principal findings This was a retrospective, cross-sectional, descriptive study of 31 patients (male to female ratio 3.4:1) diagnosed with actinomycetoma by direct grain examination, histopathology, culture, or serology from January 2009 to September 2018. Most lesions were caused by Nocardia brasiliensis (83.9%) followed by Actinomadura madurae (12.9%) and Actinomadura pelletieri (3.2%). About 50% of patients had bone involvement, and the right leg was the most commonly affected region in 38.7% of cases. Farmers/agriculture workers were most commonly affected, representing 41.9% of patients. The most commonly used treatment regimen was the Welsh regimen (35.5% of cases), a combination of trimethoprim/sulfamethoxazole (TMP/SMX) plus amikacin, which had a 90% cure rate, followed by TMP/SMX plus amoxicillin/clavulanic acid in 19.4% of cases with a cure rate of 100%. In our setting, 28 (90.3%) patients were completely cured and three (9.7%) were lost to follow-up. Four patients required multiple antibiotic regimens due to recurrences and adverse effects. Conclusions/significance In our sample, actinomycetoma was predominantly caused by N. brasiliensis. Most cases responded well to therapy with a combination of TMP/SMX with amikacin or TMP/SMX and amoxicillin/clavulanic acid. Four patients required multiple antibiotics and intrahospital care., Author summary Mycetoma is a chronic skin disease that can invade bone or underlying organs. Inadequate treatment may lead to long-lasting disability. Information about treatment is scarce. We report the outcomes of 31 patients with a diagnosis of bacterial actinomycetoma attending a tertiary care hospital in northeast Mexico. Most cases were treated with a combination of antibiotics including TMP/SMX plus amikacin or TMP/SMX plus amoxicillin/clavulanic acid. Cure was achieved in 90% of patients and only one required surgery.

Erythroderma is an inflammatory skin syndrome that involves desquamation and erythema of more than 90% of the body surface area. It represents a final clinical endpoint for many adult dermatological conditions. The most frequent cause of erythroderma is psoriasis followed by eczematous conditions, drug-induced reactions, pityriasis rubra pilaris and cutaneous T-cell lymphomas. Diagnostic approach must include a thorough history and clinical examination. If the etiology of erythroderma is uncertain multiple skin biopsies may enhance diagnostic accuracy. The initial management of erythroderma must include a nutrition expert evaluation, fluid imbalance assessment, maintaining skin barrier function, sedative antihistamines and exclusion of secondary bacterial infection. We present a practical review of the etiology, diagnosis, and treatment of this entity.

Resumen La eritrodermia es un sindrome inflamatorio de la piel caracterizado por descamacion y eritema en mas del 90% de la superficie corporal. Representa la etapa final de muchas enfermedades dermatologicas en el adulto. La causa mas frecuente es la psoriasis, le siguen las enfermedades eccematosas, las reacciones medicamentosas, la pitiriasis rubra pilaris y los linfomas cutaneos de celulas T. El abordaje diagnostico debe incluir una historia y examen fisicos exhaustivos. Si se desconoce la etiologia de la eritrodermia es posible que multiples biopsias a lo largo del curso de la enfermedad aumenten las posibilidades de un diagnostico correcto. El abordaje inicial de la eritrodermia debe incluir la evaluacion de un experto en nutricion, la valoracion del balance hidroelectrolitico, medidas para mantener la funcion de barrera de la piel, antihistaminicos con efecto sedante y la exclusion de infecciones bacterianas secundarias. Presentamos una revision practica de la etiologia, diagnostico y tratamiento de esta entidad.

Lichen sclerosus (LS) is an uncommon, chronic, lymphocyte-mediated, inflammatory dermatosis characterized by ivory-white patches with scar-like atrophy. Extragenital bullous lichen sclerosus may rarely affect palms and soles, causing severe pain and substantially impairing quality of life. We present the first case of acral bullous lichen sclerosus intolerant to UVA-1 phototherapy successfully treated with low doses of narrowband ultraviolet B phototherapy.

Alopecia areata (AA) is an autoimmune disease of the hair follicle. Keratinocytes of the hair follicle generate an immunosuppressive environment by the local secretion of hormones of the hypothalamic-pituitary-adrenal axis of the skin (skin HPA analog). Our objective was to measure the local production of corticotropin-releasing hormone (CRH), adrenocorticotropic hormone (ACTH), and α-melanocyte-stimulating hormone (α-MSH) in the scalp tissue of patients with AA before and after ultraviolet A1 (UVA-1) phototherapy to determine their role in the pathogenesis of AA and the effect of UVA-1 on the AA hormonal environment. This was a retrospective and descriptive study of skin samples from 22 patients with AA before and after UVA-1 treatment. We compared the changes in the local hormonal environment by measuring CRH, ACTH, type 2 melanocortin receptor (ACTH receptor) and α-MSH with immunohistochemical stains. The positivity of MSH was significantly higher (P = .037) in the post-treatment samples compared with the baseline value. ACTH was significantly higher in intensity (P = .032) in the post-treatment samples compared with the initial value. CRH was significantly higher in intensity (P = .013) in baseline samples compared with the final biopsies. The positivity of the ACTH receptor MC2R was not different between the two groups (P = .626). In AA, an interruption in the signalling of CRH could decrease the local concentration of ACTH and MSH, and consequently, the immunosuppressive effect of these hormones. This phenomenon is normalized in the skin treated with UVA-1. A defective signalling system in the cutaneous HPA axis may be involved in the pathogenesis of AA.

Background Mycetoma is a chronic, granulomatous infection of subcutaneous tissue, that may involve deep structures and bone. It can be caused by bacteria (actinomycetoma) or fungi (eumycetoma). There is an epidemiological association between mycetoma and the environment, including rainfall, temperature and humidity but there are still many knowledge gaps in the identification of the natural habitat of actinomycetes, their primary reservoir, and their precise geographical distribution. Knowing the potential distribution of this infection and its ecological niche in endemic areas is relevant to determine disease management strategies and etiological agent habitat or reservoirs. Methodology/principal findings This was an ambispective descriptive study of 31 patients with actinomycetoma. We determined the biophysical characteristics including temperature, precipitation, soil type, vegetation, etiological agents, and mapped actinomycetoma cases in Northeast Mexico. We identified two disease cluster areas. One in Nuevo Leon, with a predominantly kastanozems soil type, with a mean annual temperature of 22°, and a mean annual precipitation of 585.2 mm. Herein, mycetoma cases were produced by Actinomadura pelletieri, Actinomadura madurae, Nocardia brasiliensis, and Nocardia spp. The second cluster was in San Luis Potosi, where lithosols soil type predominates, with a mean annual temperature of 23.5° and a mean annual precipitation of 635.4 mm. In this area, all the cases were caused by N. brasiliensis. A. madurae cases were identified in rendzinas, kastanozems, vertisols, and lithosols soils, and A. pelletieri cases in xerosols, kastanozems, and rendzinas soils. Previous thorn trauma with Acacia or Prosopis plants was referred by 35.4% of subjects. In these states, the presence of thorny plants, such as Acacia spp., Prosopis spp., Senegalia greggi, Vachellia farnesiana and Vachellia rigidula, are common. Conclusions/significance Mapping this neglected tropical infection aids in the detection of disease cluster areas, the development of public health strategies for early diagnosis and disease prediction models; this paves the way for more ecological niche etiological agent research.

This retrospective medical record review evaluates the efficacy of methotrexate for treatment of recalcitrant erosive lichen planus of the vulva in women treated at a vulvar clinic.

Chikungunya and Zika virus infections are emerging diseases in the Americas, and dengue continues to be the most prevalent arthropod-borne virus in the world. These arbovirus diseases may spread by endemic transmission or as travel-related infections and have rapidly expanded their geographic distribution secondary to vector spread. All 3 share a similar clinical picture that includes a maculopapular rash. Zika is characterized by pruritic rash, low-grade fever, and arthralgia. Congenital nervous system malformations are a growing public-health concern. Chikungunya distinctive dermatologic manifestations include facial melanosis and bullous eruption. Dengue bleeding complications may be life-threatening and require inpatient management.

Bullous morphea is rare clinical variant of localized scleroderma characterized by the formation of bullae on sclerotic morphea plaques. Severe disease may be highly disabling and greatly impair quality of life. Current treatment strategies are based on anecdotal reports of clinical experience and include topical corticosteroids, methotrexate and phototherapy. Herein, we describe the case of a 56-year-old woman with progressive bullous sclerotic lesions who was successfully treated with mycophenolate mofetil after treatment failure with psoralen plus ultraviolet A therapy, ultraviolet A1 phototherapy, and methotrexate. Treatment with mycophenolate mofetil halted disease progression after 8 weeks. No major adverse effects were recorded in a 3-year follow-up with continuous treatment. This case suggests mycophenolate mofetil may be considered as an alternative for the treatment of resistant bullous morphea lesions. J Drugs Dermatol. 2018;17(10):1123-1125.