Primary cutaneous mucormycosis due to Rhizopus arrhizus in an immunosuppressed patient with paroxysmal nocturnal haemoglobinuria.

OBJECTIVE: We present a case of primary cutaneous mucormycosis in a patient with bone marrow failure secondary to paroxysmal nocturnal haemoglobinuria (PNH). CLINICAL CASE: A 60-year-old male patient with a history of PNH, complicated to a severe aplastic anaemia, presented to the emergency department complaining of papules on the lower limbs that rapidly turned into necrotic plaques within 2 months. Histopathological examination showed granulomatous and suppurative dermatitis with tissue necrosis and the presence of non-septate hyphae. Molecular identification was achieved by amplification and sequencing of the 18S-ITS1-5.8S-ITS2-28S rRNA region using the polymerase chain reaction. The sequence showed 100% identity with Rhizopus arrhizus. The patient received treatment with liposomal amphotericin B and surgical debridement. Nonetheless, the patient suffered from severe low red blood cells and platelets and also underwent septic shock; he died 6 days after admission to the hospital. CONCLUSION: Mucormycosis in the setting of immunosuppression is challenging. Upon suspicion of a diagnosis, immediate treatment is required. Adjunctive therapies may be considered; however, the case fatality rate remains high.

Clinical, trichoscopy, and light microscopic findings in hypohidrotic ectodermal dysplasia: Report of 21 patients and a review of the literature.

INTRODUCTION: Hypohidrotic ectodermal dysplasia (HED) is a genetic condition typified by alterations in skin structures including sweat glands, hair, nails, and teeth. Hair findings in HED have been poorly characterized in larger series. OBJECTIVE: To characterize scalp and hair findings of patients with HED clinically and with trichoscopy and light microscopy. METHODS: A cross-sectional study in 21 pediatric HED patients was performed using available clinical and scalp dermatoscopic images, as well as pulled-hair samples for clinical evaluation, trichoscopic, and light microscopic analyses. RESULTS: Seventeen out of 21 patients (81%) were men. Twenty patients had straight hair. Sixteen patients had decreased hair density, 6 of whom had hair loss mainly in the temporal and occipital regions. Fourteen patients had hair whorls. On trichoscopy, we observed: single-hair follicular units (n = 19, 90%), scalp hyperpigmentation (n = 13, 62%), variable diameter of the hair shafts (n = 12, 57%), perifollicular scales (n = 8, 38%), scalp erythema (n = 8, 38%), and short curly pigtail hairs (n = 6, 29%). On light microscopy, findings included: hair shafts with irregular diameter (n = 7, 33%), heterogeneous hair color (n = 6, 29%), trichoptilosis (n = 2, 10%), and pili torti (n = 1, 5%). CONCLUSIONS: In this series, hair findings in HED were similar to those described in previous studies. However, we describe two new clinical and two trichoscopic findings: decreased hair density mainly in the temporal and occipital regions, oblique upwards occipital hair follicles orientation, angled hairs, and short curly pigtail hairs. These heterogeneous findings may reflect the multiple factors and signaling pathways that can be affected in these syndromes.

Primary cutaneous blastic plasmacytoid dendritic cell neoplasm in a child: A challenging diagnosis and management.

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive malignancy of the skin and hematopoietic system. There are few pediatric cases reported in the literature. Management of primary cutaneous BPDCN is challenging because, despite an apparently indolent clinical presentation, rapid dissemination with high mortality can occur. We describe a child with isolated cutaneous involvement who had a good response to chemotherapy as first-line treatment of BPDCN.

The great imitator with no diagnostic test: pyoderma gangrenosum.

Pyoderma gangrenosum is a rare, neutrophil-mediated, auto-inflammatory dermatosis that wound care specialists must be prepared to recognise. This condition has clinical features analogous to infectious processes. There is no specific diagnostic test and the diagnosis is usually obtained from exclusion. Its early recognition and proper management with prompt initiation of immunosuppressive therapy are essential to improve the quality of life and the prognosis of patients.

First Report of Bacillary Angiomatosis by Bartonella elizabethae in an HIV-Positive Patient.

We present the case of an HIV-positive patient who developed polymorphous lesions in which the evidence in the skin biopsy corresponds to the diagnosis of bacillary angiomatosis, and further tests proved the pathological agent involved in this case is not the usual Bartonella species, B. henselae and B. quintana, but B. elizabethae. As far as we know, this is the first case of bacillary angiomatosis secondary to this etiological agent.

Linfoma anaplásico de células T grandes primario cutáneo CD30+. Serie de nueve casos.

INTRODUCTION: CD30+ anaplastic large T cell lymphoma is a cutaneous primary lymphoma in which there is no evidence of systemic disease; histopathological study is required for its diagnosis. OBJECTIVE: To present the cases diagnosed with primary cutaneous CD30+ anaplastic large T-cell lymphoma over a 24-year period in Hospital General "Dr. Manuel Gea González" Department of Dermatology. METHOD: Retrospective study. Descriptive statistics was carried out. Information was collected on gender, age, clinical characteristics, complementary test results, previous and current treatments, histopathological studies reports and immunohistochemistry test results. RESULTS: Of 29 309 records, nine patients (0.000034%) with a diagnosis of CD30+ anaplastic T cell lymphoma were found. Histopathological and immunohistochemical diagnosis was confirmed by two certified dermatopathologists. Average age was 61.2 years, and there was a predominance of the female gender, with initial clinical presentation as a papular or nodular lesion and varied topography. CONCLUSIONS: The prognosis of CD30+ anaplastic large T cell lymphoma in the studied population was dependent on clinical stage. The treatment at early stages has favorable results.

INTRODUCCIÓN: El linfoma anaplásico de células T grandes CD30+ es un linfoma primario cutáneo en el cual no hay evidencia de enfermedad sistémica; para su diagnóstico es necesario el estudio histopatológico. OBJETIVO: Presentar los casos diagnosticados en el Departamento de Dermatología del Hospital General "Dr. Manuel Gea González" con linfomas anaplásicos de células T grandes primarios cutáneos CD30+ durante un periodo de 24 años. MÉTODO: Estudio retrospectivo en el que realizó estadística descriptiva. Se recopiló información de sexo, edad, características clínicas, resultados de pruebas complementarias, tratamientos previos y actuales, reportes de los estudios histopatológicos y de inmunohistoquímica. RESULTADOS: Entre 29 309 expedientes, se encontraron nueve casos (0.000034 %) con diagnóstico de linfoma anaplásico de células T CD30+. Se hizo la confirmación del diagnóstico histopatológico e inmunohistoquímico por dos dermatopatólogos certificados. La edad promedio fue de 61.2 años, hubo predominio del sexo femenino y de lesión papular o nodular y topografía variada como presentación clínica inicial. CONCLUSIONES: El pronóstico del linfoma anaplásico de células T grandes CD30+ en la población estudiada fue dependiente del estadio clínico. El tratamiento en etapas tempranas tiene resultados favorables.

Myopericytoma in an unusual location.

Myopericytoma is a soft-tissue tumor of perivascular cells (pericytes). It is slow-growing, usually asymptomatic, and generally benign, although a malignant variant has been described. The etiology is unknown, but it has been associated with local trauma. The most common location is on the distal extremities. Histologically, it is characterized by a well-circumscribed, non-encapsulated proliferation of spindle shaped cells similar to myofibroblasts with oval nuclei and eosinophilic cytoplasm, arranged in perivascular concentric rings. There are few mitoses and no necrosis is reported. The immunohistochemical analysis is positive for smooth muscle actin and negative or weakly positive for desmin. A low Ki-67 proliferation index is typical. Treatment is surgical excision with free margins. Recurrences after adequate excision are uncommon. We describe a 48-year-old woman with a myopericytoma in an unusual location (next to the inner corner of her left eye) who was treated with surgical excision; there has been no recurrence after 5 years of follow up.

[Discrasia linfoide epiteliotrópica de células T. Tratamiento con radiación ultravioleta].

INTRODUCTION: T-cell lymphoid dyscrasia is a group of different, infrequent, epitheliotropic inflammatory dermatosis that precede cutaneous T-cell lymphomas. Treatment with ultraviolet radiation is safe and efficacious. OBJECTIVE: To describe responses to treatment, secondary effects and complications. METHOD: Eleven patients with different T-cell lymphoid dyscrasias were treated. Two received psoralens plus ultraviolet A radiation (PUVA) and nine narrow band ultraviolet B radiation (NB-UVB). RESULTS: Seven women and four men from 5 to 30 years-old and phototypes III and IV were included. They had hypopigmented epitheliotropic lymphoid dyscrasia, chronic pityriasis lichenoides, hypopigmented parapsoriasis and pigmented purpuric dermatoses. Two received PUVA and 9 NB-UVB. Eight achieved complete response and three partial response with 10 to 119 sessions. Any patient presented neither relapse nor progression to cutaneous T-cell lymphoma during treatment or in the follow up period after 34 to 247 sessions. Secondary effects were xerosis and mild pruritus. CONCLUSIONS: The majority of patients with T-cell lymphoid dyscrasias can be successfully and safely treated with minimal secondary effects after a variable number of sessions and periods of time with ultraviolet radiation.

INTRODUCCIÓN: Las discrasias linfoides epiteliotrópicas de células T son un grupo de dermatosis inflamatorias poco frecuentes que pueden progresar a linfomas cutáneos. El tratamiento con radiación ultravioleta ha sido descrito como seguro y eficaz. OBJETIVO: Describir la respuesta al tratamiento, sus efectos secundarios y complicaciones. MÉTODO: Se administró radiación ultravioleta A con psoralenos (PUVA) a dos pacientes y radiación ultravioleta B de banda estrecha (NB-UVB) a nueve pacientes, con diferentes discrasias. RESULTADOS: Conformaron la serie siete mujeres y cuatro hombres, con edades de 5 a 30 años y con fototipos III y IV, con diagnóstico de discrasia linfoide epiteliotrópica hipopigmentada, pitiriasis liquenoide crónica, parapsoriasis hipopigmentada y púrpura pigmentada. Dos recibieron PUVA y nueve recibieron NB-UVB. Ocho alcanzaron respuesta completa y tres respuesta parcial con 10 a 119 sesiones de tratamiento. Ningún paciente presentó reactivación de la enfermedad después de 34 a 247 sesiones, y ninguno en tratamiento o seguimiento evolucionó a linfoma cutáneo. Los efectos secundarios fueron xerosis y prurito. CONCLUSIÓN: El tratamiento de las discrasias linfoides con radiación ultravioleta es eficaz y seguro, con efectos secundarios leves y con respuestas exitosas en la mayoría de los pacientes, en tiempo y número de sesiones variables.

From Classical to Unusual: Report on 5 Cases of Transient Reactive Aquagenic Pseudokeratoderma and Review of the Literature.

Transient reactive aquagenic pseudokeratoderma is characterized by transient whitish and translucent papules after water exposure in palms and soles. We report 5 additional cases and their histopathologic features. Patient 5 showed a topography that had not been reported before; this demonstrates that it is not an exclusive condition of palms and soles. Patient 2 presents very subtle clinical findings, making difficult the diagnosis. And the other 3 patients have a typical presentation. Transient reactive aquagenic pseudokeratoderma has heterogeneous clinical features; in some cases, histopathologic findings, although subtle, help to confirm the diagnosis.

Neutrophilic Eccrine Hidradenitis in an HIV-Infected Patient.

A 35-year-old man presented with a 2-day history of a maculopapular pruritic eruption that had affected his general state of health and was accompanied by chills. The patient had been diagnosed with HIV in 2008, and was undergoing treatment with a combination of emcitrabine/tenofovir plus nevirapine. He had a current viral load of 1,558,160 copies/mL and a lymphocyte count CD4+ count of 230/mm3. Physical examination revealed an eruption involving the face, nape of the neck, chest, abdomen, extremities, palms, and soles. This was characterized by erythematous papules approximately 2 mm in diameter coalescing on the palms and soles, where they formed plaques (Figures 1 and 2).

A case series and a review of the literature on foreign modelling agent reaction: an emerging problem.

Foreign modelling agent reactions (FMAR) are the result of the injection of unapproved high-viscosity fluids with the purpose of cosmetic body modelling. Its consequences lead to ulceration, disfigurement and even death, and it has reached epidemic proportions in several regions of the world. We describe a series of patients treated for FMARs in a specialised wound care centre and a thorough review of the literature. A retrospective chart review was performed from January 1999 to September 2015 of patients who had been injected with non-medical foreign agents and who developed cutaneous ulceration needing treatment at the dermatology wound care centre. This study involved 23 patients whose ages ranged from 22 to 67 years with higher proportion of women and homosexual men. The most commonly injected sites were the buttocks (38·5%), legs (18%), thighs (15·4%) and breasts (11·8%). Mineral oil (39%) and other unknown substances (30·4%) were the most commonly injected. The latency period ranged from 1 week to 17 years. Complications included several skin changes such as sclerosis and ulceration as well as systemic complications. FMAR is a severe syndrome that may lead to deadly complications, and is still very common in Latin America.

Histoplasmosis and skin lesions in HIV: a safe and accurate diagnosis.

Human histoplasmosis is caused by the dimorphic fungus Histoplasma capsulatum. This infection can run asymptomatic or be life-threatening, depending fundamentally on the host's immune status. Immunocompromised patients can present disseminated disease to the skin, making the biopsy an accessible approach. The current diagnosis gold standard is fungal culture which takes several days or weeks to grow and must be handled in a biosafe laboratory which is avoided if we use the technique here described. We propose the use of molecular biology for diagnosis confirmation, considering it can shorten diagnosis lapse, has good specificity and sensitivity and reduces the risk of infection for the medical and laboratory personnel. Seven paraffin-embedded skin biopsy samples were included from patients with confirmed HIV and histoplasmosis diagnosis. Total DNA was isolated and molecular typing of H. capsulatum var. capsulatum. All samples were positive. This is a safe and accurate method for skin histoplasmosis diagnosis.

[Cutaneous metastasis of renal cell carcinoma: A case report and review of the literature]. / Metástasis cutánea de carcinoma de células renales (MCCCR): reporte de caso y revisión de la literatura.

INTRODUCTION: Renal cell carcinoma (RCC) accounts for 2-3% of adult solid malignancies. About 25-30% develop metastasis at the time of diagnosis and 60% corresponds to clear cell type. CASE PRESENTATION: A 66-year-old man, with a personal history of left radical nephrectomy, presents with an asymptomatic skin tumor he noticed one month earlier. Histopathologic study reported metastatic cutaneous infiltration of clear cell carcinoma, suggestive of a primary renal carcinoma. DISCUSSION: Cutaneous metastasis of RCC represents 6.0-6.8% of all cutaneous metastases. These patients have poor prognosis and, therefore, their treatment is palliative. It is essential to perform a complete periodic dermatologic examination for proper restaging and treatment.

[Sclerosing acral skin perineurioma: clinicopathologic study of ten cases (eight classical and two with xanthomatous changes)]. / Perineuroma esclerosante de piel acral: estudio histopatológico de diez casos (ocho clásicos y dos con cambios xantomatosos).

INTRODUCTION: Perineurioma is an infrequent and benign cutaneous neoplasm characterized by proliferation of perineurial cells. It is classified into two main types: intraneural and the extraneural or soft tissue perineurioma, in which the sclerosing variant is included. Sclerosing perineurioma is more frequently found on acral skin. Clinically, they are well-circumscribed,skin colored, nodular tumors. OBJECTIVE: Describe and communicate clinicopathologic findings from a case series of sclerosing acral perineurioma. MATERIAL AND METHODS: This is a clinical, morphological and immunohistologic case study of eight patients with the diagnosis of sclerosing perineurioma. RESULTS: It included five men and five women, with ages ranging between nine and 66 years. All of them had lesion on acral skin. At microscopy study, the lesions showed a proliferation of epithelioid and spindle-shaped perineurial cells, arranged in small aggregates and short fascicles between thickened collagen bundles. Immunohistochemistry studies revealed that the proliferating cells expressed EMA, Claudin-1 and Glut-1, and were negative for S-100 protein. CONCLUSIONS: It is important to report these infrequent skin tumors, so they can be taken into account in the differential diagnoses of acral lesions.

[Multiple primary melanoma: a clinical case and review of the literature]. / Melanoma primario múltiple (MMP) sincrónico: caso clínico y revisión de la literatura.

Patients with a first primary melanoma are known to have an increased risk of being diagnosed with another primary melanoma. We present the case of a 42-year-old male patient with multiple primary melanoma who was admitted to the dermatology department at the general hospital "Dr Manuel Gea González" in Mexico City. Incidence, risk factors and prognosis are discussed in this review.

[Trichoepithelioma: a retrospective study 1993-2012 - Dr Manuel Gea González General Hospital]. / Tricoepitelioma: estudio retrospectivo (1993-2012, Hospital General «Dr. Manuel Gea González¼).

BACKGROUND: Trichoepithelioma is a benign follicular tumor, affects young female adults, and has three clinical forms: solitary, multiple, or desmoplastic. Diagnosis represents a clinical challenge for the dermatologist. There are many differential diagnoses, and the most important is basal cell carcinoma because they share histological features. It has good prognosis and therefore treatment is usually for aesthetic purposes, even though it is associated with recurrence. METHODS: This is a retrospective study carried out from January 1993 to December 2012 of all the patients who were diagnosed by histopathology to have trichoepithelioma in the dermatology department of the Dr. Manuel Gea González General Hospital. RESULTS: There were 47 cases, of which 70.2% were female, with an average age of 43.6 years. Solitary trichoepithelioma was the most frequent clinical form (66%) and only three of 14 cases of multiple trichoepithelioma had a positive family history.

Assessment of syndecan-1 (CD138) and Ki-67 expression for differentiating keratoacanthoma and squamous cell carcinoma.

BACKGROUND: There is a major controversy over the natural behavior of keratoacanthoma (KA). KAs have been described as benign lesions, but also as variants of squamous cell carcinoma (SCC). Microscopic differentiation between these 2 entities is problematic, and sometimes impossible. Syndecan-1 (CD138) is an adhesion molecule whose expression appears to be inversely correlated with tumor invasiveness. Elevated Ki-67 expression is indicative of a high proliferation index, a feature of malignant tumors. METHODS: Syndecan-1 and Ki-67 expression were assessed in 22 KA skin samples and in 17 SCC skin biopsies. RESULTS: Syndecan-1 expression was diminished in the SCC specimens compared with the KA specimens ( P =.000). Ki-67 expression was increased in the SCC specimens compared with the KA specimens, with mean values of 9 and 0.08, respectively ( P =.000). LIMITATIONS: Further studies that compare intermediate risk KAs to typical KAs and SCCs are required to corroborate these findings. CONCLUSION: The assessment of syndecan-1 and Ki-67 expression in skin biopsies is a helpful tool for differentiating KA and SCC

Sporadic progressive mucinous histiocytosis in a Mexican patient.

A 33 year-old woman presented with numerous 3- to 5-mm red-brown and yellow-brown dome-shaped nodules, primarily located on the scalp, dorsal aspects of the forearms, and lower extremities (Figure 1 and Figure 2). Her lesions started to appear 5 years prior to her consultation with increasing number and without spontaneous regression. Findings from a previous biopsy revealed epithelioid dermatofibroma. The remainder of the physical examination was unremarkable. There were no familial cases of this condition (both the mother and two older sisters were examined).

Atypical Presentation of Spitz Nevus: A Case Report.

Introduction: The Spitz nevus (SN) is an acquired melanocytic neoplasm composed of epithelioid and/or spindle cells, which tends to develop in childhood. In pediatric patients, it is usually located on the face and neck. Unusual locations have been found in the literature, such as the penis, mouth, and tongue, as well as 2 cases of ungual SN. Case Report: A 15-year-old male evaluated for dark brown-black longitudinal melanonychia that covered 40% of the nail, with pseudo-Hutchinson's sign, of 1 year of evolution. Discussion: The SN accounts for 1% of the melanocytic neoplasms. In the present paper, we show the third case of ungual SN never previously disclosed, which presents a zigzag pattern reported in the literature for its association with the pediatric population.

Lichenoid drug reaction after ipilimumab/nivolumab combination therapy: A case report.

Nivolumab (PD-1 inhibitor) and ipilumumab (CTLA-4 inhibitor) are recently approved checkpoint inhibitors for treatment of non-small cell lung cancer. Immune-related adverse events related to the usage of checkpoint inhibitors are growing with their popularity. We present the case of a patient in combination treatment of nivolumab and ipilimumab who developed a lichenoid drug reaction, notable because it worsened to a bullous lichenoid drug reaction. Treatment with prednisone and withdrawal of checkpoint inhibitors aided in clinical resolution. Initial presentation of a lichenoid reaction that progressed to a bullous, desquamated presentation indicates the possibility of the prodromal rash progressing to a Stevens-Johnson Syndrome-like dermatosis. When dermatologists are consulted for rashes developed during checkpoint-inhibitor therapy, they should be aware that early treatment may prevent progression to bullae formation and desquamation and develop their treatment plans with this in mind.