Vânia Oliveira Carvalho

Cutis verticis gyrata in a child with Turner syndrome.

To the Editors: Cutis verticis gyrata (CVG) is a rare disorder characterized by elevations and depressions of the skin that form cerebriform folds. Data on the association of CVG with Turner syndrome (TS) are scarce (1,2). This report emphasizes the association of CVG with chromosomal abnormalities and proposes inclusion of these syndromes as secondary causes of CVG. We saw a Caucasian female infant 18 days old, the product of a full-term, uncomplicated pregnancy who presented with abnormal scalp folds at birth (Fig. 1). Her parents were nonconsanguenous. The infant’s weight and height were in the 5th percentile. She had surplus skin over the nape of the neck, edema of the feet, epicanthi, ogival palate, and furrows resembling gyri on the anterior biparietal portion of the head measuring 3 cm × 2 cm, with absence of hair. The neurologic and cardiac evaluations were normal. Laboratory tests found negative chromatin in buccal smears, with a 45-XO karyotype. A skin biopsy specimen from a lesion revealed a normal epidermis and thick fibrous bands of collagen in the superficial and reticular dermis. At 5 months of age, the edema of the feet and folds on the scalp persisted and a winged neck was also noted. At 10 months her height was in the 3rd percentile and weight in the 10th percentile, with normal growth velocity and normal mental status. In 1953, Polan and Butterworth (3) classified CVG as primary (or idiopathic) and secondary. As a primary illness, it is usually associated with neurologic abnormalities. When secondary, it can be due to inflammatory changes; tumors, nevi, neurofibroma and fibroma; trauma; and miscellaneous disorders such as acromegaly, myxedema, cretinism, leukemia, syphilis, acanthosis nigricans, and tuberous sclerosis. The presence of CVG without other manifestations is termed primary essential CVG (4). TS manifests clinically at birth with edema over the dorsum of the hands and feet and accentuation of the nuchal folds. Marini et al. (1) reported a case of CVG associated with TS and suggested that the congenital lymphedema presented in this syndrome might be the cause of the CVG. Lymphedema in TS is thought to originate from a congenital abnormality of the lymphatic vessels, which can be caused by hypoplasia or by obstruction of their drainage (5). Embryologically the lymphatic vessels of the scalp drain to the jugular lateral sacs and these normally communicate with the final portion of the right lymphatic duct and the thoracic ducts. In the absence of a connection between the venous and lymphatic systems, as occurs in some chromosomal abnormalities, there is lymph accumulation in the jugular sacs and in the tissues, causing cystic hygroma and hydrops. If there is intrauterine correction or if an alternative route of lymphatic drainage develops, there will be reabsorption of the edema and regression of the cystic hygroma. In TS and other aneuploid syndromes this results in congenital winged neck and puffiness of the dorsum of the hands and feet (6–8). Approximately 80% of children with TS are born with lymphedema, mainly of the dorsum of the hands and feet. Cystic hygroma occurs in 50% of patients. Usually the lymphedema resolves by recanalization of the lymphatic vessels. The resolution of both edema and cystic hygroma could explain the dermatoglyphic abnormalities, the pterygium coli, and the low hairline that occurs in TS (5,9–11). It is postulated that CVG might also result from resolved intrauterine scalp lymphedema (1,2). The echographic detection of lymphedema in a fetus with CVG and TS has been reported (12). Lymphedema and cystic hygroma may also occur in Down and Edwards syndromes (13). Newborns with these chromosomal abnormalities, characterized by “pterygium coli” at birth, were noted by ultrasound between the 14th and 16th weeks of gestation (13) to have cystic hygroma and generalized edema (including the head). At present, in Polan’s classification, chromosomal alterations are not listed as a cause of CVG. Some authors have shown the association of CVG with TS (1,2) and we Figure 1. Furrows on the anterior biparietal portion of the head.

Alterações dermatológicas em crianças com Aids e sua relação com categorias clínico-imunológicas e carga viral

BACKGROUND: Ever since the beginning of the Acquired Immunodeficiency Syndrome (Aids) epidemic, a number of dermatoses have been frequently described in patients with this disease. In the literature, case reports and studies in series of adult patients with atypical lesions are frequent found. By contrast, there are few reports on cutaneous alterations in pediatric patients with Aids. OBJECTIVE: The purpose of the following prospective survey is to evaluate the presence of dermatoses in 40 pediatric patients with Aids. METHOD: Forty patients diagnosed with Aids and less than 13 years of age were seen for six months. The following features were evaluated: number of dermatoses, clinical characteristics, distribution in accordance with the clinical-immunological categories in relation to CD4 T-lymphocyte and viral load values. RESULTS: The prevalence of dermatoses in the first evaluation was 82.4%. In a longitudinal follow-up, 92.5% of the patients developed some kind of skin problem. Children belonging to the worst clinical-immunological category, with a viral load higher than 100,000 copies/ml showed a higher number of dermatoses when compared to the mildest categories. The number of dermatologic alterations per patient was 6.8 in clinical category C and 3.6 in A. In the immunological severe category, it was 7.0, while in the mild one, it was 3.7. For viral load > 100,000, the number was 7.3, and for < 100,000, it was 4,2 (all with statistical significance). CONCLUSION: Dermatological alterations were frequent and directly related to advanced stages of Aids in pediatric patients. Higher frequencies of skin alterations in pediatric patients with Aids indicate that it is necessary to perform careful and frequent dermatological examinations of these patients.

An inflammatory reaction surrounding molluscum contagiosum as possible manifestation of immune reconstitution inflammatory syndrome in HIV infection.

Abstract:  Highly active antiretroviral therapy can restore specific immune responses and control of microorganism infections in human immunodeficiency virus‐positive patients. This immune recovery may cause an inflammatory reaction to microbial and autoimmune antigens known as immune reconstitution inflammatory syndrome. We describe a clinical case with an intense inflammatory response surrounding molluscum contagiosum after highly active antiretroviral therapy. The clinical and laboratory findings suggested that the reaction was due to immune reconstitution inflammatory syndrome occurring during a period of immune recovery in a child with acquired immune deficiency syndrome.

The relevance of recognizing clinical and morphologic features of pityriasis lichenoides: clinicopathological study of 29 cases.

Background: Pityriasis lichenoides (PL) is a lymphoproliferative disease of unknown origin; its diagnosis is based on clinical characteristics and confirmed by histology. Objectives: To describe clinical and histological features of PL in 29 pediatric patients. Materials and Methods: Retrospective descriptive study of children (patients less than 15 years old) diagnosed with PL between 1986 and 2010 at a Reference Service in Pediatric Dermatology from South Brazil. Results: Twenty-nine PL cases were found by chart review in 24 years. Mean age of diagnosis was 8 years (22 to 178 months) and a mean time of diagnosis was 13.8 months (1 to 120 months). Twenty cases (69%) were male. Seasonal correlation was found with colder months in 62% of cases (p<0.01). Clinical diagnosis was pityriasis lichenoides chronica (PLC) in 25 cases, and pityriasis lichenoides et varioliformis acuta (PLEVA) in four. Itching was the main reported symptom occurring in 13 (45%). Fourteen cases had been histologically evaluated. In six, microscopic findings were consistent with PLC, in four consistent with PLEVA, and four biopsies exhibited mixed characteristics of both forms. Concordance between clinical and histological diagnosis was seen in most cases. Conclusion: PL occurs in children and young adults, more commonly in males, and during cold months. PLC was the more frequent clinicohistologic form, and necrotic lesions characterized PLEVA. Associating clinical and histological findings is important for differentiating between PLC and PLEVA diagnosis.

Polyomavirus BK: possibly associated skin eruption in a patient with hemorrhagic cystitis.

Abstract:  A 14‐year‐old girl with Fanconi anemia was submitted to allogeneic hematopoietic stem cell transplantation. After 17 days she developed hemorrhagic cystitis due to polyoma BK virus (BKV), confirmed by PCR (polymerase chain reaction). Two weeks after the appearance of the urinary symptoms the patient presented numerous papules and vesicles on both hands and feet. PCR of the skin lesions and plasma was positive for BKV. The relationship of BKV with frequent infections in immunocompromised patients is well established. The positive PCR of vesicular fluid suggests that this was the causative agent of the skin lesion in this case. There are no reports of skin lesions with positive PCR for BKV.

Infectious and inflammatory skin diseases in children with HIV infection and their relation with the immune status--evaluation of 127 patients.

Abstract:  Human immunodeficiency virus infection causes changes in the immune system and disease evolution can be partially measured by levels of T CD4+ lymphocytes. Knowledge of the chronology of skin disease in relationship to the immune status of the patient may help understand the pathogenesis of AIDS. One hundred twenty‐seven children were prospectively evaluated for skin diseases and their relationship to immune status. Immunodeficiency in human immunodeficiency virus‐infected children was correlated with infectious dermatoses whereas normal CD4+ T lymphocyte levels was correlated with diseases mediated by hypersensitivity mechanisms.

Paraqueratose granular: relato de seis casos em crianças

Granular parakeratosis is an alteration of keratinization that was first described in adults. It is characterized by hiperkeratotic plaques and papules in intertriginous areas. The authors describe six cases of granular parakeratosis in children. One patient had lesions on the buttocks; two children presented papules in both axillae and cervical region (presentations never described before in the literature). The remaining three patients presented with lesions in the inguinal folds. Review of the literature and discussion on the pathogenesis of this rare dermatosis are presented.

Hematohidrosis: insights in the pathophysiology

Hematohidrosis: insights in the pathophysiology The spontaneous discharge of bloody secretion through normal skin is known as hematohidrosis. We present a case of this rare condition and discuss possible mechanisms to explain the clinical manifestations. Written consent by the patient for this submission was obtained. An 18-year-old Caucasian female presented with episodes of spontaneous bleeding of the palms, back of the hands, forehead (Fig. 1), and tear ducts since the age of 12. No skin lesions were present, and they stopped spontaneously after a few minutes. There was no relation with the menstrual cycle, and sometimes extensive bleeding occurred during her sleep. Physical examination, gynecological evaluation, complete blood cell count, metabolic panel, and coagulation tests were normal. On dermatologic evaluation, no skin lesions were found, and a punch biopsy was performed on the back of the left hand, where spontaneous bleeding had just started. Smear preparation of the bloody secretion had peripheral blood characteristics – many erythrocytes, few leukocytes. Skin biopsy – stained with hematoxylin–eosin and Pearl – revealed normal skin, preserved adnexa, and normal blood vessels. Over 120 histological sections were evaluated, and no erythrocytes were found anywhere outside the vessels (Fig. 2). The patient was admitted to the hospital and carefully watched to rule out malingering; in fact, she presented several bleeding episodes under our close visual monitoring. During follow-up, she was diagnosed with conversion, dissociative, and generalized anxiety disorders, which were controlled with clonazepam, sertraline, valproic acid, and risperidone, but this treatment did not interfere with the bleeding. Although there was no psychiatric evaluation of the patient prior to the bleeding episodes, we can only suppose that the impact of the skin condition helped develop or exacerbate the psychiatric diseases. Curiously, it was noticed that the bleeding episodes were coincident with high blood pressure peaks (180 9 90 mmHg), but the 24-hour ambulatory blood pressure monitoring, Holter, and echocardiogram were normal. To control blood pressure levels, she was started on atenolol. During six months, she showed marked improvement with no more bleeding episodes. Owing to irregular intake of the medication, the bleedings restarted sporadically. Hematohidrosis is a rare clinical condition that manifests as self-limiting episodes of spontaneous discharge of bloody secretion through intact skin or sweat gland orifices, with an unknown cause. Some theories have been proposed, including: increased vascular pressure leading to the passage of blood cells through the ducts of the sweat glands; vasculitis of dermal vessels; and exacerbated sympathetic activation leading to periglandular vessel constriction and subsequent expansion, allowing the passage of blood content into the ducts. Only five reported cases in the literature present a histological description of a skin biopsy performed immediately after the bleeding. They show sporadic and inconsistent findings, such as: periglandular congested vessels; red blood cells in the follicular lumen and among the collagen fibers; leakage of blood around dermal capillaries; or even normal skin. In the patient described herein, the skin biopsy was completely normal. This excludes other causes of bleeding and may also indicate that no permanent anatomical changes occur in the pathophysiology of the disease. Differential diagnosis should include chromhidrosis, factitious dermatitis, vicarious menstruation, vasculitis, and platelet and coagulation disorders.

Clinical features and nail clippings in 52 children with psoriasis

Nail clipping, the act of cutting the distal portion of a nail for microscopic analysis, can complement the diagnosis of skin diseases with nail involvement, such as psoriasis. This study aimed to describe histopathologic findings on 81 nails from 52 children and adolescents with skin psoriasis and to determine whether these changes correlated with the severity of skin and nail involvement.

Nail changes in psoriatic children

Indian Journal of Dermatology, Venereology, and Leprology | May-June 2016 | Vol 82 | Issue 3 314 and mean facial sebum excretion have been detected.[5] Therefore, sleep may affect sebum levels via its interactions with these hormones. We propose that our findings regarding the positive relationship between good sleep quality and higher sebum levels may be related to these physiologic characteristics of sleep and sebum.