Menstrual and hormonal alterations in juvenile dermatomyositis.

OBJECTIVES: To evaluate age at menarche, menstrual cycles and hormone profile in juvenile dermatomyositis (JDM) patients and controls. METHODS: Twelve consecutive JDM patients were compared to 24 age-matched healthy subjects. Age at menarche and age of maternal menarche were recorded. Menstrual cycle was evaluated prospectively for 6 consecutive months and the mean cycle length and flow were calculated. The hormone profile was collected on the last menstrual cycle. Demographic data, clinical features, muscle enzymes, JDM scores and treatment were analysed. RESULTS: The median of current age of JDM patients and controls was similar (18 vs. 17 years, p=0.99). The median age at menarche of the JDM patients was higher than in the control group (13 vs. 11 years, p=0.02) whereas the median age of maternal menarche was alike in both groups (12 vs. 13 years, p=0.67). Menstrual disturbances were not observed, except for one patient who had longer length of menstrual cycle. The median of follicle stimulating hormone (FSH) was significantly higher in JDM patients compared to controls (4.5 vs. 3.0 IU/L, p=0.02) and none of them had premature ovarian failure (POF). The median of progesterone was significantly lower in JDM patients (0.3 vs. 0.7 ng/mL, p=0.01) with a higher frequency of decreased progesterone compared to controls (75% vs. 29%, p=0.01). CONCLUSIONS: Our study identifies in JDM patients delayed menarche with normal cycles and low follicular reserve. The decreased progesterone levels may suggest an underlying subclinical corpus luteum dysfunction in this disease.

Gonadal function in adolescents and young women with juvenile systemic lupus erythematosus.

The authors analysed the gonadal function and age of menarche of 23 female adolescents and young women with SLE, and correlated these with clinical, SLEDAI and therapeutic parameters. The presence of one or more clinical and laboratory parameters defined normal gonadal function: normal menstrual cycles with or without dysmenorrhea; elevated cervical mucus length; normal levels of plasma FSH, LH, estradiol, progesterone, prolactin and testosterone; normal urinary hormonal cytology; serial pelvic ultrasound compatible with ovulatory pattern; and present or previous pregnancy. The mean age of menarche (13.5 +/- 1.4 years) was greater than that found among 2578 healthy Brazilian adolescents (12.5 +/- 1.3 years; P = 0.0002). The delay in menarche correlated with an increase in the duration of the disease (P = 0.0085) and the cumulative dose of prednisone (P = 0.0013) used until the appearance of the menarche. The mean phase length in SLE was 31.5 +/- 10.3. Sixteen female (70%) patients showed normal and seven (30%) abnormal gonadal function. Gonadal function was not correlated with parameters of SLE. These results suggest that the patients of this study reach adulthood with a high chance of fertility.

[Albopapuloid epidermolysis bullosa (Pasini's disease)]. / Epidermólise bolhosa albopapulóide (doença de Pasini).

The aim for this communication is one case of the dystrophic Epidermolysis bullosa in its variant just called as "albopapuloid". The patient a male of 25 years, suffers from the disease since the age of 2. His mother and one brother show the same disease. The clinical manifestations began as bulloe on the superior and inferior limbs. The bullae heal with atrophic scars and the eruption is constantly relapsing after subsiding. Besides the bullous eruption there are other lesions persistently coming out as whitish elevations which are elongate as to form numerous streaks on the surface of the skin. Such lesions started at the age of 5 and do not come from the bullae but they are quite independent, and constitute the essential characteristic for the albopapuloid clinical variant. Small miliary epidermal cysts are seen over the scars. Dystrophic changes of the nails with absence of many of them and the toes show the lst phalanges partially absorbed. On histological examination the bullae are subepidermal and contain a fibrin-leukocytic exudate. The albopapuloid lesions reveal keratosis, epithelial atrophy, diffuse and condensed fibrosis in the corium and around the hair follicles.