Hematidrosis: a pathologic process or stigmata. A case report with comprehensive histopathologic and immunoperoxidase studies

Cases of hematidrosis (bloody sweat) are extremely rare. This disease has been described in various terms and has been often tied to religious belief as stigmatization. We report a typical patient with hematidrosis in a 14-year-old girl who frequently bled from her scalp and palms, and, occasionally, from trunk, soles, and legs. The bloody sweat from her scalp contained all blood elements. Immediate biopsy after there was bleeding on her scalp showed multiple blood-filled spaces that opened directly into the follicular canals or on to the skin surface. Immunoperoxidase studies failed to demonstrate vascular nature of these spaces. Our study explained how and why there was bleeding in our patient and in patients with related conditions as described in earlier literatures. We also explained why this phenomenon was intermittent because the spaces indicated above will disappear after exuding their content but then reoccurred after the blood flow was reestablished.     

Pruritic papular eruption in HIV seropositive patients: a cutaneous marker for immunosuppression

BACKGROUND: Previous reports have shown the correlation between certain skin disorders and immune status in human immunodeficiency virus (HIV) infected patients. Pruritic papular eruption (PPE) is the most common cutaneous manifestation in HIV infected patients. The purpose of this study is to define the relationship between the presentation of PPE and the immune status in HIV infection, as measured by the T-cell subset, and to establish the usefulness of this common eruption as a predictor of CD4 count. METHOD: In this cross-sectional study, 20 HIV-positive patients with characteristics of PPE were studied. Clinical data, skin biopsy, and immune status, evaluated by measuring CD4, CD8, and CD4/CD8, were investigated. RESULTS: Seventy-five per cent of patients already had antecedent skin disorders, so PPE is not a leading symptom in HIV infected patients; 81.25% of PPE patients had an advanced degree of immunosuppression with a CD4 count below 100/mm3 and 75% below 50/mm3. CONCLUSIONS: PPE can be regarded as a cutaneous marker of advanced HIV infection.     

Satoyoshi Syndrome

Satoyoshi syndrome is a very rare disorder of unknown etiology, characterized by progressive, intermittent, painful muscle spasms, alopecia universalis, diarrhea or unusual malabsorption, various endocrine disorders, and secondary skeletal abnormalities. This report describes a 9-year-old Thai girl who developed alopecia universalis when she was 6 years old. At age 7 years, she began to have recurrent, painful muscle spasms. The spasms progressed in time, producing recurrent patella dislocation. The laboratory investigations and radiologic study were compatible with Satoyoshi syndrome. She was treated with oral corticosteroid therapy, with marked improvement of her muscle spasms and alopecia. She underwent corrective surgery for deformities of both knees with a normal healing process.     

Mast cells in leprosy and leprosy reaction

BACKGROUND: Mast cells can be visualized in routine, acid-fast-staining, paraffin tissue section as metachromatic staining cells, and can be activated to release inflammatory mediators which play a role in the cell-mediated immune response. METHODS: Skin biopsy tissues were taken from the most active skin lesion of each leprosy patient at the time of diagnosis (nonreactional group) and at the time of reaction (reactional group) during the years 1994-1997 in the leprosy clinic at the Department of Dermatology, Faculty of Medicine, Siriraj Hospital, Mahidol University, Thailand. Mast cells were identified by metachromatic staining (purple) in Fite's stain sections and reported as the average number of cells per high power field in three compartments: at the center and periphery of the granuloma and in the interstitium. The data were analyzed in three groups: nonreactional group, type I, and type II leprosy reactions. The mast cell count of each group and each compartment of the section, expressed as the mean +/- standard error, was compared. RESULTS: A total of 95 persons were included in the study, but 108 tissue sections were obtained due to nine cases having more than one section. Of these patients, 63 cases (66.32%) had no reaction, 19 cases (20%) had type I reaction, and 13 cases had type II reaction. There was no difference in age and sex among these groups. The mast cell count in the interstitium was higher than that within the granuloma, both at the center and at the periphery, in every type, and the count in this area reduced significantly in leprosy reactions, both type I and type II, compared with the nonreactional group. CONCLUSIONS: The change in the average mast cell number in nonreactional leprosy and leprosy reactions may indicate the important role of mast cells in dynamic changes in the cell-mediated immune response in leprosy and leprosy reactions.     

Scrofuloderma and Sweet's syndrome

BACKGROUND: In recent years, the rare association of Sweet's syndrome with nontuberculous mycobacterial lymphadenitis has been reported. OBJECTIVE: To report the clinical, demographic, and bacteriologic data and association with Sweet's syndrome of 18 patients with scrofuloderma and scrofuloderma-like condition caused by nontuberculous mycobacterial infections seen during the past 7 years (1994-2000). METHODS: In all patients, a biopsy specimen was obtained for histopathologic and microbiologic studies. Patients from whom Mycobacterium tuberculosis and nontuberculous mycobacteria were isolated from the culture of skin biopsy specimens were included. Deep fungal infection was excluded by the lack of a fungal element in histologic section and cultural methods. The patients were treated with antimicrobials or antituberculous drugs according to the causative species. RESULTS: Eighteen cases of scrofuloderma (nine male, nine female; mean age, 36.9 years) were found among 104 patients with cutaneous tuberculosis and nontuberculous mycobacterial cutaneous infections. Sixteen of the 18 cases had lymphadenitis as the underlying focus of scrofuloderma: 15 cases occurred in the cervical group and one case in the inguinal area. One case drained from the soft tissue and one from the paranasal air sinus. Five cases had multiple episodes of Sweet' s syndrome during the course of treatment. Most cases in this group (four of the five) were middle-aged women with cervical lymphadenitis, and the most common species were rapid growers. CONCLUSIONS: Age, sex, and the site of infection may have some influence on the association with Sweet's syndrome in nontuberculous mycobacterial infections.     

Melanocytic matricoma: two cases of a rare entity in women

Melanocytic matricoma is a rare cutaneous adnexal tumor occurring in humans with only 13 cases reported in the literature. The typical lesion is a circumscribed pigmented nodule on sun‐damaged skin. Most cases have occurred in elderly men. The tumor contains a mixed population of matrical cells, supramatrical cells, shadow or ghost cells, and dendritic melanocytes. We report two cases of melanocytic matricoma in two elderly women with unusual histopathological features such as cystic degeneration and focal granulomatous inflammation, which are considered to be atypical for this entity.     

Severe congenital systemic juvenile xanthogranuloma in monozygotic twins

Abstract:  Juvenile xanthogranuloma, a histiocyte disorder, usually presents with a solitary cutaneous lesion. Juvenile xanthogranuloma with extracutaneous involvement is a rare disease in which significant morbidity and occasional deaths may occur. Monozygotic twins with congenital systemic juvenile xanthogranuloma who presented with multiple skin lesions, hepatosplenomegaly, liver failure, and bone marrow involvement were reported. The diagnosis of systemic juvenile xanthogranuloma was confirmed by histology and immunohistochemical stains of the skin with liver biopsies revealing dense infiltration of lymphohistiocytes with typical Touton giant cells staining positive for CD68 and negative for CD1a and S-100 protein. Both of them received systemic prednisolone 1 mg/kg/day which was gradually tapered off with time according to clinical and investigative responses. At the 17-month follow-up period, both patients showed remarkable regression in all symptoms and laboratory studies.     

The Effect of Vitamin D Status on Pediatric Asthma at a University Hospital,Thailand

Purpose

In the USA and Europe, hypovitaminosis D is associated with increased asthma severity, emergency department (ED) visit, and impaired pulmonary function in asthmatic patients. However, in tropical countries, data on the effect of vitamin D status on asthma is limited. This study evaluates the relationship between vitamin D status and the level of asthma control as well as other asthmatic parameters.

Methods

Asthmatic children were evaluated for serum 25-hydroxyvitamin D, pulmonary function tests, a skin prick test, and the level of asthma control.

Results

A total of 125 asthmatic children were recruited (boys, 66.4%). Their mean age±SD was 10.8±3.0 years. Vitamin D statuses were: deficiency (<20 ng/mL) in 19.2% of the patients, insufficiency (20-30 ng/mL) in 44.8%, and sufficiency (>30 ng/mL) in 36%. The vitamin D levels were 25.9±9.4 ng/mL in uncontrolled patients, 29.2±8.6 ng/mL in partly controlled patients, and 27.9±8.0 ng/mL in controlled patients (P>0.05). There were no significant differences in pulmonary function, asthma exacerbation, inhaled-corticosteroid (ICS) dose, anti-inflammatory drugs, or ED visit or hospitalization between different vitamin D statuses. Vitamin D deficiency patients were older and had a delayed onset of asthma than insufficiency or sufficiency patients. There was no significant correlation between serum vitamin D and pulmonary function/doses of ICS.

Conclusions

High prevalences of vitamin D deficiency and insufficiency were found in asthmatic children in Thailand; however, there was no significant relationship between vitamin D status and the level of asthma control or other asthma parameters.