Nodular (keloidal) scleroderma.

Keloidal or nodular scleroderma (NS) is a variant of localized scleroderma (LS) frequently seen in patients with limited or diffuse systemic sclerosis (SSc). It presents as raised, firm plaques or nodules with extensive dermal fibrosis and hyalinized collagen bundles. We present a patient with SSc who presented with this rare entity.

Esclerodermia nodular (queloidea) / Nodular (keloidal) scleroderma

Keloidal or nodular scleroderma (NS) is a variant of localized scleroderma (LS) frequently seen in patients with limited or diffuse systemic sclerosis (SSc). It presents as raised, firm plaques or nodules with extensive dermal fibrosis and hyalinized collagen bundles. We present a patient with SSc who presented with this rare entity. (AU)

La esclerodermia nodular o queloidea es una variante de esclerodermia localizada que se encuentra predominantemente en pacientes con esclerosis sistémica limitada o difusa (SSc). La presentación clínica es de placas o nódulos firmes y sobreelevados con fibrosis dérmica y haces de colágeno hialinizados. En este reporte de caso presentamos a una paciente con SSc con esta entidad rara. (AU)

Cutaneous Angiomyolipoma-A Distinct Entity That Should Be Separated From Classic Angiomyolipoma: Complete Review of Existing Cases and Defining Fundamental Features.

Cutaneous angiomyolipoma is a rare mesenchymal tumor that is demographically, clinically, and immunohistochemically distinct from its renal and extrarenal counterparts. We present a case of cutaneous angiomyolipoma in the right retroauricular area of a 35-year-old male patient and provide a broad systematic review of the literature and the largest compilation of cutaneous angiomyolipomas reported to date. According to the findings presented in this review, we conclude that cutaneous angiomyolipoma should be completely separated from renal and extrarenal angiomyolipomas and therefore be considered a distinct entity in the classification of skin tumors.

Quantification of Hair Follicles in the Scalp in Mexican Mestizo Population.

INTRODUCTION: There are no reports of the density of hair follicles in the scalp of Mexican mestizo population, necessitating the determination of normal references values for this population compared with other ethnic groups. OBJECTIVE: To determine the average hair follicle count on the scalp in Mexican mestizo population. MATERIALS AND METHODS: A total of 50 scalp samples (25 men and 25 women) from Mexican mestizo individuals aged between 19 and 60 years, with no clinical evidence of hair disease, obtained by biopsy punch at General Hospital "Dr. Manuel Gea González" were collected over 2 years. The total follicular density, vellus and terminal hair follicles, and the percentage in anagen and catagen-telogen phase were measured. χ2 was used as the basic statistical test. RESULTS: The mean number of total hair follicles in our Mexican mestizo population was 23.2 ± 4.2, which is lower compared with Thais, Iranians, and Caucasians. However, the ratio of terminal and vellus hair follicles was higher than in Thais, Caucasians, and African-Americans. The percentage of terminal hairs in anagen phase was lower than in the other populations, and higher in telogen, without exceeding 15%, established as the normal reference value. There was an association between sex and terminal hairs in telogen phase (p < 0.05). The average follicular density per mm2 was slightly higher compared with African-Americans and Asians. Women had more terminal hairs than men. CONCLUSIONS: The density of total follicles is lower in the Mexican mestizo population compared with Iranians, Thais, and Caucasians. However, the greater number of terminal hairs compared to vellus hairs gives the appearance of greater overall volume. The results of this study can be used as a reference for diseases of the scalp in the Mexican population.

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).

Atypical dermoscopic presentation of an acral congenital melanocytic nevus in an adult: parallel ridge pattern and its histologic correlation.

Acral melanoma is the most frequent subtype in the Asian and Mexican mestizo populations. Dermoscopy is a noninvasive diagnostic technique that helps the differential diagnosis of pigmented skin lesions on acral volar skin. We, herein, present a case of acral congenital melanocytic nevus with a parallel ridge dermoscopic pattern. Since the parallel ridge pattern in a melanocytic lesion of the acral skin is classically ascribed to melanoma, the present case can be definitely labeled as "atypical" and worth of being elucidated in its histopathological correlates.

Inmunohistochemical detection of mastocytes in tissue from patients with actinic prurigo.

BACKGROUND: Actinic prurigo (AP) is a type of photodermatosis, the pathophysiology of which has not been determined. AP has been suggested to be a hypersensitivity reaction to the presence of eosinophils and the local production of IgE. MATERIAL AND METHODS: Descriptive study, using paraffin blocks of tissue that have been diagnosed with AP from the Dermopathology department, Hospital General Dr. Manuel Gea González. In 66 blocks from 63 patients, eosinophils were identified by hematoxylin and eosin staining, and mastocytes were labeled by immunohistochemistry. Three random microphotographs (40x) were used, and cell counts were calculated as the mean count in the 3 microphotographs. RESULTS: Forty cases (63.5%) were female, and 23 (36.5%) were male. The mean age was 26.49 ±14.09 years; regarding the evolution time of the disease, the average was 11.93 years ±11.39. In 38 of 63 cases (60%), the lip, skin, and conjunctiva were affected clinically. In 22 of 63 cases (34%), AP cheilitis was the sole manifestation, and in 4 of 63 cases (6%), there were lesions in the skin and conjunctiva. The mean eosinophil count was 9 per case, the average number of mastocytes/field was 28.48 (range 0 to 66) Kruskal-Wallis p=0.001. CONCLUSIONS: There are elements in AP that mediate the reaction of hypersensitivity type IV b, necessitating the identification of triggering factors. KEY WORDS: Actinic prurigo, eosinophil, hypersensitivity IV b, IgE, mastocytes.

Apoptosis in chronic cutaneous lupus erythematosus, discoid lupus, and lupus profundus.

INTRODUCTION: Lupus erythematosus is a multisystemic disease that is characterized by autoantibody production and immune complex deposition in such tissues as the mucosa, joints, the central nervous system, and skin. Cutaneous lupus erythematosus is categorized as acute, subacute, and chronic. Chronic cutaneous lupus erythematosus comprises discoid lupus erythematosus (DLE) and lupus profundus (LP). AIM: To analyze the expression of proapoptotic molecules in patients with lupus erythematosus discoid and lupus profundus. MATERIAL AND METHODS: Descriptive study, the study groups comprised 10 cases of LP and 10 cases of DLE, and a control. Skin samples of cases and controls were processed for immunohistochemistry and by TUNEL technique. The database and statistical analysis was performed (statistical test X(2)) SPSS (Chicago, IL, USA). RESULTS: Apoptotic features were broadly distributed along the skin biopsies in epidermal keratinocytes as well as at dermis. By immunohistochemistry the expression of Fas receptor and Fas-L was higher in the skin of lupus patients compared with controls. We also noted differences in Fas-L, -Fas, and -Bax proteins expression intensity in discoid lupus erythematosus patients in the epidermis, and hair follicles. CONCLUSIONS: Fas and Fas-L are expressed similarly in LP and DLE.

Vestibular papillomatosis as a normal vulvar anatomical condition.

At the beginning of the nineteen-eighties, vulvar vestibular papillomatosis (VVP) was thought to be a human papilloma virus (HPV) disease. Based upon these findings many clinicians have been treating this condition with laser ablation or by topical application of podophyllin or trichloroacetic acid. Currently, most authors believe that VVP should be considered an anatomical variant of the vestibular mucosa and not HPV related. We present a case of VVP in which there was no histological or molecular evidence of HPV; unnecessary treatment should be avoided.

[Oral erythema multiforme, an entity to be considered in the differential diagnosis of chronic and recurrent vesiculobullous diseases. Report of a case]. / Eritema multiforme bucal, un padecimiento a considerar en el diagnóstico diferencial de enfermedades vesiculoampollares crónicas y recurrentes. A propósito de un caso.

We present the case of a 23-year-old woman who was referred with an 18-month history, of oral ulcers finally diagnosed as erythema multiforme (EM). During this time she received multiple diagnoses and treatments, without resolution of the symptoms. The patient had a history of frequent infections by herpes simplex virus (HSV) in mouth, and apparently the drugs used to control it extended the signs and symptoms of EM. The patient showed rapid improvement after discontinuation of the drugs, and treatment for episodes of HSV infection was prescribed.

Dermoscopy Clues in Pigmented Bowen's Disease.

Pigmented tumors have similar clinical features that overlap and hamper diagnosis. Dermoscopy increases the diagnostic accuracy of doubtful melanocytic lesions and has been used as a noninvasive tool in the detection of pigmented lesions (PLs) like melanoma, basal cell carcinoma, and pigmented Bowen's disease (pBD). Our objective was to show the dermoscopic features of 2 cases of pBD and compare with the findings reported in the literature. Two dermoscopic images of biopsy proven pBD were retrospectively analyzed for dermoscopic patterns. Both cases showed brown regular globules, structureless brown and blue pigmentation, glomerular vessels, hypopigmented regression-like areas, and keratosis. These findings were similar to the cases reported previously. The dermoscopic diagnosis of pBD is based on the absence of criteria for a melanocytic lesion in the presence of glomerular vessels, regular brown globules and keratosis. Although pBD is rare, it should be included in the differential diagnosis of PLs, especially melanoma.

Síndrome de hiperinmunoglobulinemia E: Reporte de dos casos / Hyperimmunoglobulin E syndrome: report of two cases

Introducción. El síndrome de hiperinmunoglobulinemia E es una inmunodeficiencia sistémica poco frecuente, caracterizada por dermatitis eccematosa, abscesos fríos recurrentes, infecciones pulmonares con formación de neumatoceles, facies tosca, niveles elevados de inmunoglobulina E (IgE) en suero y eosinofilia. Casos clínicos. Caso 1. Femenino de 11 años de edad con antecedentes de neumonía recurrente, gastroenteritis de repetición, dermatitis eccematosa de predominio en pliegues, y abscesos fríos; en estudios de laboratorio destacó el hallazgo de 16 070 eosinófilos e IgE de 4 864 Ul. Manejada con gammaglobulina se observó buena respuesta clínica. Caso 2. Femenino de 12 años de edad con historia de otitis recurrente y conjuntivitis supurativa, presentaba eccema crónico generalizado e impetiginizado. En estudios de laboratorio se reportó IgE de 3 000 UI; fue manejada con dapsona, trimetropim/sulfametoxazol y metotrexate. Conclusión. Los 2 casos aquí informados presentaron piel eccematosa, infecciones recurrentes e incremento de los niveles de IgE, compatibles con síndrome de hiperinmunoglobulinemia E en la forma autosómica recesiva.

Background. Hyperimmunoglobulin E syndrome is a rare systemic immunodeficiency characterized by eczematous dermatitis, recurrent cold abscesses, lung infections with pneumatoceles, coarse facial appearance, high IgE levels and eosinophilia. Case reports. Case 1: We report the case of an 11-year-old female with a history of recurrent lung infections, recurrent gastroenteritis, eczematous dermatitis affecting the skin folds and cold abscesses. Laboratory studies showed elevated eosinophils (16 070) and IgE 4864 IU. The patient received treatment with g-globulin, showing adequate clinic response to treatment. Case 2: We present the case of a 12-year-old female with a history of recurrent otitis and suppurative conjunctivitis, showing widespread and chronic infected eczema. Laboratory studies showed elevated IgE (3 000 IU). She was treated with dapsone, trimethoprim/sulfamethoxazole and methotrexate. Conclusions. We presented two patients with eczematous skin, recurrent infections and increased IgE levels, which are compatible with hyperimmunoglobulin E autosomal recessive syndrome.

The emergence of mucormycosis as an important opportunistic fungal infection: five cases presenting to a tertiary referral center for mycology.

BACKGROUND: Mucormycosis, a rare opportunistic fungal infection, is re-emerging in importance with the increase in prevalence of immunosuppressive states, both as a result of therapy and disease. METHODS: We report five cases of mucormycosis diagnosed by the Dermatology Department and managed jointly with the Medical and Surgical Services of "Dr Manuel Gea Gonzalez" General Hospital in Mexico City, a tertiary referral center for mycology. We also review the current literature including recent advances in medical therapy. RESULTS: Four of the five cases were of the rhino-orbital-cerebral variant, commonly associated with significant mortality, and one of these patients died despite early diagnosis and aggressive management. The fifth case was primary cutaneous mucormycosis and this patient survived infection without relapse. Diabetic ketoacidosis predisposed to infection in four cases and the other was associated with advanced human immunodeficiency virus infection. Radiologic imaging was important in cases of facial involvement in order to evaluate the extent of disease and possible intracranial involvement. All cases were managed with systemic antifungals and surgical debridement, together with the treatment of predisposing factors. CONCLUSIONS: These cases illustrate the need for early clinical recognition and prompt therapy, as well as the requirement for tissue biopsy in order to demonstrate the characteristic morphologic features of this fungal agent in the absence of positive mycology culture results. This report also highlights that, although rhino-orbital-cerebral mucormycosis requires effective multidisciplinary management, the disease not uncommonly presents to dermatologists for diagnosis.

[Vulvar syringoma: a report of two cases]. / Siringomas vulvares: informe de dos casos.

Syringoma is a benign neoplasm of eccrine origin. Lesions often arise around the eyes and on the upper cheeks of middle-aged women. Syringomas involving the genitalia are rare, they present as multiple, bilateral, skin-colored papules over the labia majora. The lesions should therefore be considered in the differential diagnosis of multiple papular lesions of the vulva. We report two females of 37 and 62 years of age, both observed in a dermatological service.