Search

Epidermal necrolysis (EN) compromises a spectrum of life-threatening dermatoses (Stevens-Johnson Syndrome [SJS], overlap syndrome and toxic epidermal necrolysis [TEN]). Currently, no active therapeutic regimen with unequivocal benefit exists for SJS/TEN. SCORTEN is the widely-used prognostic scale specific for SJS/TEN. Nevertheless, a new prognostic scale, the ABCD-10, has been recently proposed. In this context, acute renal failure (ARF) seems to be an important comorbidity that could influence prognosis in SJS/TEN patients more than it is assumed by these two scales. Our objectives were to compare the accuracy of the SCORTEN and ABCD-10 scales in predicting the mortality in SJS/TEN, and to investigate the influence of renal failure on prognosis. The prognostic results of 18 patients with EN treated in two referral centers between 2013 and 2018 are presented. SCORTEN, ABCD-10 and renal function values were retrospectively collected for all patients. Out of the 18 patients who were analyzed, nine (50%) received only supportive therapy, four were treated with etanercept 50 mg in a single dose (22.2%) and five with corticosteroids (27.8%). Five patients developed ARF. Predicted mortality was 3.48 for SCORTEN and 2.33 for ABCD-10. Eventually, four patients died (22.2%), all had ARF and none of them received active treatment. Despite study limitations and in the absence of active treatment of choice, SCORTEN behaved as a reliable predictor of mortality in patients with EN, outperforming the newer ABCD-10. ARF was an early event associated with a poor prognosis, which could represent a prognostic marker to consider in the future.

Introduction: Hand eczema is a frequent disease in adults. Diagnosing the cause of hand eczema is difficult due to different classifications. There is lack of evidence on hand eczema and its causes in children. Material and method: A total of 389 children between 0 and 16 years were identified between 1996 and 2016, from whom 42 (10.8%) with exclusively hand eczema were selected. In all cases a standard battery of epicutaneous patch tests was performed, as well as additional batteries depending on the clinical suspicion. The clinical and epidemiological features of these children were recorded and compared against children with eczema in other locations. Results: The 42 children with hand eczema included 25 (59.5%) girls, and 17 (40.5%) boys, with a mean age of 10.6 ± 3.9 years, and did not differ from that of children with eczema in other locations. The definitive diagnosis after patch-testing was Atopic Dermatitis in 15 cases, Allergic Contact Dermatitis in 14 patients, Endogenous Vesiculous Eczema in 6 cases, Endogenous Hyperkeratotic Eczema in 5 cases, and Irritant Contact Dermatitis in 2 cases. The most frequent allergens detected were thiomersal (9 cases), nickel (5 cases), mercury (5 cases), and cobalt (4 cases). Conclusion: Hand eczema is a common condition in children. The most common cause is atopic dermatitis, although cases of allergic contact dermatitis manifesting as hand eczema are not uncommon. Any child with eczema of hands in whom an allergic cause is suspected should be referred for patch- testing. Resumen: Introducción: El eccema de manos es una forma frecuente de eccema en adultos. Su diagnóstico en ocasiones es complejo debido a la existencia de diferentes clasificaciones diagnósticas. Existen pocos trabajos que estudien el eccema de manos y su clasificación en niños. Material y método: Se ha identificado a 389 niños entre 0 y 16 años remitidos a la Unidad de Alergia Cutánea de nuestro servicio para estudio con pruebas epicutáneas en el periodo 1996-2016. De entre todos los casos se han seleccionado 42 casos con dermatitis localizada exclusivamente en la mano (10,8% de todos los niños remitidos). En todos los casos se realizaron pruebas epicutáneas parchando la batería estándar, así como baterías adicionales en función de la sospecha clínica. Se recogieron datos epidemiológicos (edad, sexo, antecedentes de dermatitis atópica…), así como clínicos (localización de las lesiones). Resultados: De los 42 niños remitidos con dermatitis de la mano, 25 (59,5%) eran niñas y 17 (40,5%) niños. La edad media de los pacientes con dermatitis de la mano fue de 10,6 ± 3,9 años. El diagnóstico definitivo tras la realización de pruebas epicutáneas fue dermatitis atópica en 15 casos, dermatitis alérgica de contacto en 14 pacientes, eccema endógeno vesiculoso en 6 casos, eccema endógeno hiperqueratósico en 5 casos y dermatitis irritativa de contacto en 2 casos. Los alérgenos detectados más frecuentes fueron tiomersal (9 casos), niíquel (5 casos), mercurio (5 casos) y cobalto (4 casos). Conclusión: El eccema de manos es una entidad frecuente en niños. La causa más frecuente es la dermatitis atoípica, aunque no son infrecuentes los casos de dermatitis alérgica de contacto que se manifiestan como eccema de manos. Todo niño con eccema de manos en el que se sospeche una causa alérgica debe ser remitido para realización de pruebas epicutáneas.

The differential diagnosis of Grover disease (GD) includes pemphigus foliaceus. However, the focal nature of the histologic findings and negative direct immunofluorescent point to the diagnosis of GD. It has been associated with a variety of systemic and cutaneous diseases, but its association with other primary acantholytic disorders has been rarely documented. In this article, we describe the first case of GD in association with pemphigus foliaceus.

Pemphigus foliaceus is an autoimmune bullous disease with autoantibodies against desmoglein 1. Case reports of pemphigus after surgery have also been described, which may simulate an infection of the surgical wound, a contact dermatitis, or even a tumor recurrence. Cytoimmunofluorescence can help to establish a rapid diagnosis.

Eosinophilic fasciitis (EF) is an uncommon fibrosing disease of the fascia with characteristic cutaneous and hematologic manifestations. Although EF is most commonly treated with corticosteroids at the beginning, a considerable number of patients show an inadequate response and hence various therapeutic strategies have been tried, including extracorporeal photopheresis (ECP). We describe the case of a 66-year-old woman with steroid-resistant EF that improved significantly after ECP was added to her treatment regimen. To date, only six cases of this therapeutic strategy have been reported in English literature.

Background Melanocytic acral nevi have a series of distinguishing features, including their location, patient age at onset, clinical progression, and histological findings. In particular, histopathological analysis often reveals a melanocytic acral nevus with intraepidermal ascent of cells (MANIAC nevus), which in some cases can be mistaken for atypia or malignancy. Aim This study describes the clinicopathological characteristics of acral nevi in patients under 18 years old and contrasts the clinical and histological features between MANIAC vs non-MANIAC nevi. Methods This was a retrospective observational study, performed in our department in the decade between January 2007 and January 2017. We included patients younger than 18 years of age who were subjected to the removal of melanocytic acral nevi. Results A total of 70 patients were studied. 54.2% (38/70) were females and 45.8% (32/70) were males. With regard to the type of nevus, 34 were compound, 27 were junctional, and 9 were predominantly intradermal lesions. We identified a total of 41 MANIAC nevi and 29 non-MANIAC nevi. Statistically significant differences between these two groups were identified in nevus size (larger in MANIAC) and the frequency of compound nevi (higher in the MANIAC group), but not in the remainder of the histological parameters studied.

We report the case of a 32-year-old man with measles in which skin biopsy helped to establish a definitive diagnosis. Follicular involvement is a common histopathologic feature of measles. Multinucleated epidermal and follicular cells are distinctive findings.

Sarcoidosis is an uncommon systemic granulomatous disease of unknown origin affecting lung, skin, liver, and other tissues. Noncaseating granulomas in the involved organs are the hallmark of this disease. An exaggerated immune response to an unknown antigenic stimulus could play a role in sarcoidosis development. Lung is one of the most frequently involved organs.1 Manifestations range from alveolitis to granulomatous infiltration of alveoli, bronchi, and blood vessels. The end stage of lung sarcoidosis is development of interstitial fibrosis with “honeycombing” of lung parenchyma. Interferon alfa in association with ribavirin is the treatment of choice for hepatitis C. Early treatment of acute hepatitis C with interferon alfa-2b will prevent the development of hepatic cirrhosis, but adverse effects are frequent and often can result in discontinuation of treatment.2 Most frequent adverse effects related to interferon are malaise, fever, arthralgia, and cough. Pulmonary events such as bronchial asthma, bronchiolitis obliterans, and interstitial pneumonitis have been reported. Interferon alfa is also used in the treatment of malignant melanoma, multiple myeloma, hairy-cell leukemia and HIV-associated Kaposi's sarcoma.3 Some cases of sarcoidosis after treatment with interferon alfa have been reported in the literature.4, 5, 6 Improvement of sarcoidosis has been reported with discontinuation of treatment, but in other cases an independent course of disease has been proposed, raising the belief that interferon discontinuation is unnecessary in mild-to-moderate cases of interferon-related sarcoidosis.7 We present the case of a patient with hepatitis C treated with interferon in whom pulmonary sarcoidosis developed. The sarcoidosis was initially believed to be a pulmonary neoplasm, but skin lesions developed that indicated the diagnosis of systemic sarcoidosis. The sarcoidosis finally resolved without discontinuation of interferon alfa.

Background Mycosis fungoides (MF) is the most frequent type of cutaneous T cell lymphoma. Its clinicopathological spectrum is wide, and the resulting diversity makes it difficult to establish a differential diagnosis among pityriasis lichenoides (PL), lymphomatoid papulosis (LyP), and atypical MF. Objectives This study describes four patients with longstanding PL-like lesions, in whom clinicopathological correlations contributed towards the establishment of definitive diagnoses of MF. Methods The clinical histories of the four patients were reviewed. Skin biopsies were processed by hematoxylin and eosin staining and immunohistochemical techniques. Disease spread was studied according to laboratory tests, complete blood counts, biochemical parameters, lactate dehydrogenase, lymphocyte populations, and Sezary cells. Thoracoabdominopelvic computed tomography was performed. Results The four patients included two women and two men, aged 35–70 years, all of whom had chronic skin lesions located mainly on the trunk and extremities. In three patients, initial clinicopathological correlations led to the diagnosis of PL; further biopsies were required to diagnose MF. In all patients, the atypical lymphocytic infiltrate showed a lichenoid or perivascular distribution with focal epidermotropism and parakeratosis. All cases proved positive for CD4 and CD3, and negative for CD20, CD8, and CD30. Polymerase chain reaction showed monoclonal-type T cells in one and polyclonal infiltrates in three patients. All patients were initially treated with topical corticosteroids and photochemotherapy. None of the treatments proved completely successful, but subsequent tests have yielded no evidence of disease progression in any patient. Conclusions In patients with clinical features of longstanding PL and histological findings consistent with MF, differential diagnosis must include PL, LyP, and papular MF. As some forms of PL evolve towards MF and PL-like forms of MF, these patients should undergo regular follow-up and repeated biopsies in order to discard diagnoses of atypical forms of MF.

Background According to the International Society for the study of vascular Anomalies, vascular anomalies are classified as vascular neoplasms and vascular malformations. In some vascular lesions, categorization as a neoplasm or malformation has not been established with confidence so far. In order to further clarify the nosology of verrucous hemangioma, we studied 13 cases. Objective To analyze immunohistochemical characteristics of verrucous hemangiomas in order to gain further insight in its histogenesis. Methods We carried out a retrospective review. Immunohistochemical expression for Wilms tumor 1 (WT1), Glut-1 and D2-40 was performed in 13 cases. Results Immunohistochemistry performed with Glut-1 and WT1 showed positive staining in all lesions. All verrucous hemangiomas lacked D2-40 immunostaining. Conclusions This is the first report in the literature investigating WT1 in verrucous hemangioma in order to further clarify the nosology of this vascular anomaly. Despite the clinical features of verrucous hemangioma, which are similar to those seen in vascular malformations, verrucous hemangioma exhibited an immunoprofile similar to vascular neoplasms, according to WT1 and Glut-1 positivity.

Molecular diagnostics are increasingly performed routinely in the diagnosis and management of patients with melanoma due to the development of novel therapies that target specific genetic mutations. The development of next-generation sequencing (NGS) technologies has enabled to sequence multiple cancer-driving genes in a single assay, with improved sensitivity in mutation detection. The main objective of this study was the design and implementation of a melanoma-specific sequencing panel, and the identification of the spectrum of somatic mutations in a series of primary melanoma samples. A custom panel was designed to cover the coding regions of 35 melanoma-related genes. Panel average coverage was 2,575.5 reads per amplicon, with 92,8% of targeted bases covered ≥500×. Deep coverage enabled sensitive discovery of mutations in as low as 0.5% mutant allele frequency. Eighty-five percent (85/100) of the melanomas had at least one somatic mutation. The most prevalent mutated genes were BRAF (50%;50/199), NRAS (15%;15/100), PREX2 (14%;14/100), GRIN2A (13%;13/100), and ERBB4 (12%;12/100). Turn-around-time and costs for NGS-based analysis was reduced in comparison to conventional molecular approaches. The results of this study demonstrate the cost-effectiveness and feasibility of a custom-designed targeted NGS panel, and suggest the implementation of targeted NGS into daily routine practice.

Superficial angiomyxoma is a distinctive cutaneous soft tissue neoplasm that is clinically variable, infrequent, and benign. However, this tumor has a high propensity for local recurrence. There is a known association of angiomyxomas with Carney complex. We report a case of superficial angiomyxoma in a 28-year-old woman who presented with an erythematous, mul-tilobulated nodule on the nipple. This tumor has a predilection for the trunk, head and neck, extremities, and genitalia. The present case, hence, is unusual, because the lesion developed on the nipple. The appearance of a solitary cutaneous angiomyx-oma warrants an examination to rule out the presence of Carney complex. After clinical examination, echocardiogram and en-docrine analyses, Carney complex was ruled out in our patient.

Primary cutaneous neuroendocrine carcinoma (PCNC), previously known as Merkel cell carcinoma (MCC), is a rare tumor of the skin with aggressive behavior and poor prognosis. Typically, PCNC is positive for Cytokeratin-20 (CK20) and negative for Thyroid Transcription Factor-1 (TTF-1). Rarely, CK-20 negative and TTF-1 positive PCNC have been described. We present the case of two patients with skin lesions histologically compatible with MCCs and a behavior characteristic of this disease, but with expression of TTF-1 instead of CK-20. In conclusion, there are increasing reports of TTF1+ CK20− skin lesions without signs of systemic disease which behave clinically and prognostically like a PCNC. The origin of these TTF1 tumors are, to date, unknown.

Psoriasis is a disabling chronic inflammatory condition of the skin and joints that typically requires long-term treatment. Recommended treatments for psoriasis include a wide range of topical and systemic options, from topical agents and targeted phototherapy for mild psoriasis to traditional systemic agents such as methotrexate, cyclosporine and acitretin for more serious disease. The introduction of targeted biological agents such as T-cell-modulating agents, tumor necrosis factor α (TNFα) antagonists and interleukin (IL)-12 and IL-23 inhibitors has provided new choices for the management of psoriasis and psoriatic arthritis that may offer better long-term efficacy and tolerability than traditional approaches. Most biological agents are administered by subcutaneous injection. Infliximab, a TNFα antagonist, is the only biological agent approved for psoriasis that is administered by intravenous infusion, in the setting of hospital-based or specialized infusion center-based clinics. Infliximab allows weight-based dosing and may offer more rapid disease control than other biological agents, with significant improvements seen as early as 1 week after treatment initiation. This article gives an overview of psoriasis management, focusing on clinic-based infusion therapy with infliximab.

Palisaded neutrophilic granulomatous dermatitis (PNGD) is a rare neutrophilic dermatosis that shows a broad clinical and histopathological spectrum. The clinical presentation of PNGD varies from asymptomatic papules, to nodules, to annular plaques. The most common clinical presentation is erythematous papules on the extensor surface of extremities, especially fingers and elbows. Histopathological findings demonstrate a spectrum of changes that reflect the evolution of the lesions, from only sparse mixed perivascular infiltrates to interstitial or palisaded granuloma formation with dermal fibrosis. The cause of PNGD is unknown, but there is a prominent association with systemic conditions, particularly with autoimmune diseases, representing rheumatoid arthritis and systemic lupus erythematosus, the most common associations. It has also been described associated to systemic sclerosis, sarcoidosis, systemic vasculitis, inflammatory bowel disease, and lymphoproliferative disorders, and also drugs have been implicated. Recently, it has been described the potential association with tumor necrosis factor alpha inhibitors. This dermatosis should be considered as a marker of systemic disease and particularly it should be considered in patients with history of autoimmune disorders who present with papular eruptions on the extremities.

Dear Editor: Retiform purpura is a dermatological condition characterized by reticulated, stellate, or serpentine shaped purple lesions on the skin and mucous membranes. New, multiple cases of retiform purpura after the use of levamisole adulterated cocaine have been reported. Levamisole is an anthelmintic drug with immunomodulatory and immunostimulating properties. It has been used in humans to treat rheumatoid arthritis, cancer of the colon and nephritic syndrome in children. It was withdrawn from use in the United States in 2000 because of the risk of agranulocytosis1. We report the case of a 52-year-old woman receiving treatment with levothyroxine for hypothyroidism. Two days after consuming cocaine, she developed painful skin lesions with arthralgia on both wrists. Physical examination revealed plaques and papules infiltrated to touch, purpuric on the edges and necrotic in the center, with reticular and stellate lesions on both cheeks, the tip of the nose, outer left ear, and lower limbs (Fig. 1). Biopsy revealed thrombotic vasculopathy of the small and medium blood vessels in the dermis and subcutaneous cell tissue (Fig. 2). Blood tests revealed leukopenia, neutropenia, and lymphopenia. Antinuclear antibodies (ANA, titer 1 : 1.280) in anti-neutrophil cytoplasm antibodies (ANCAs) against myeloperoxidase with a p-ANCA pattern (titer, >100 [0~5]), and ANCAs against proteinase 3 with a c-ANCA pattern (titer, 6.8 [0~5]) were also found. Hypocomplementemia of C3 was detected. The tests for thrombosis and coagulation, serology, cryoglobulins and antiphospholipid antibodies were normal or negative. Cocaine was detected in the urine sample. The results of chest radiography and urine sediment test were normal. A diagnosis of retiform purpura resulting from the use of cocaine, that was probably adulterated with levamisole was made. She was prescribed with low dose oral prednisone. The hematological symptoms cleared 5 days later, after one month, the skin lesions had healed without sequelae. Fig. 1 (A) A reticular plaque with purpuric edges and necrotic center on the left cheek. (B) Reticular and stellate plaques with purpuric edges and necrotic center on low limbs. Fig. 2 Hematoxylin-eosin staining image (×10) showing thrombotic vasculopathy of the small and medium blood vessels in the dermis and subcutaneous cell tissue. Levamisole is currently only used in veterinary medicine as an anthelmintic; however, it has recently begun to be used as an adulterant in cocaine in many countries, including Spain2. Levamisole poisoning should be suspected in the case of a young patient presenting bilateral cutaneous necrosis on the earlobes, cheeks, or nose, that may be accompanied with retiform purpura lesions, primarily on the legs3. There may also be signs of arthralgia, fatigue, and general malaise. In the literature, some cases showing convulsions, kidney failure, or pulmonary haemorrhage have also been reported. The most common alterations in laboratory tests are neutropenia, a p-ANCA pattern, and the presence of antiphospholipid antibodies. More specific immune markers include antielastase antibodies. However, to confirm the diagnosis, levamisole detection in urine by using gas chromatography-mass spectrometry is required. However, most of the reported cases, including ours, are unconfirmed. This is because of the unavailability of testing techniques4 and the short half-life of levamisole (5.5~6 h)5. Two histological patterns have been documented: leukocytoclastic vasculitis and thrombotic vasculopathy. In most patients, skin lesions resolve spontaneously 2~3 weeks after stopping levamisole. However, the serological alterations may persist for up to 14 months3.

Intracranial invasion of cellular blue nevus is extremely rare, and its malignant transformation is even less common. The differential diagnosis includes neurocutaneous melanosis and neurocristic cutaneous hamartoma. A 50-year-old female presented with intracranial melanoma in contiguity with a congenital blue nevus on the scalp. The patient showed a wide pigmented lesion on the scalp that had grown in the last few years over the congenital blue nevus. Magnetic resonance imaging revealed an intracranial tumor lying contiguous to the nevus. Despite aggressive surgery, the tumor relapsed and the patient developed systemic metastases. We report a rare case of cellular blue nevus showing an unexpected aggressive behavior with extensive extra- and intracranial expansion and distant metastases.