We report on the case of a male who from the start of life displayed vesicular lesions; on the trunk these were clustered and on the limbs they adopted a linear configuration. After biopsy of one such lesion, the histopathological study was compatible with a diagnosis of incontinentia pigmenti (IP). In the following months, hyperkeratotic lesions appeared which later became pigmented. The mother and other female members of the family also showed different degrees of alteration related to the same disease. The karyotype study showed the existence of 47,XXY (Klinefelter syndrome). The exceptional nature of this case is that although it is the third case reported in the literature of a male affected by incontinentia pigmenti with a previous family history, it is the only one combining this characteristic with the presence of a 47,XXY karyotype.
Apocrine mixed tumors of the skin illustrate the ability of pluripotential primordial cells to differentiate in different directions within an individual tumor. Moreover, mixed tumors appear as a compendium of different folliculo-sebaceous neoplasms. We suggest that these proliferations should be interpreted as "complex neoplasms of the primary epithelial germ."
A 12-year-old girl had a lesion located on the upper part of the scalp that was clinically interpreted as a melanocytic nevus and corresponded histologically to a basal cell carcinoma. At age 2 years she had been diagnosed as having acute lymphoblastic leukemia L1, for which she was treated with systemic chemotherapy. She also received telecobalt therapy to the whole cranium for prophylaxis of meningeal leukosis. Having rejected other possible causes that can favor the appearance of basal cell carcinoma during childhood (basal cell nevus syndrome, nevus sebaceus, albinism, etc.), we believe that this case should be added to the five previously reported in the literature as basal cell carcinoma developing at an early age in cranial radiation portals with megavoltage radiotherapy. We draw attention to the need to explore radiated areas of skin as part of the long-term follow-up of these patients, and to the advisability of obtaining a biopsy from any persistent lesion in these areas.
A 3-year-old boy had maculopapules on his face and neck since age 6 months. These were yellow-brown, asymptomatic, and clinically similar to flat warts. Histopathologic study revealed a fibrohistiocytic infiltrate in the superficial dermis. Ultrastructurally, comma-shaped bodies, desmosome-like junctions, and coated vesicles were seen; there were no lipid droplets or Birbeck granules. With these data, a diagnosis of benign cephalic histiocytosis was made. Twenty-five cases are reported in the literature: 17 males and 8 females (male:female ratio 2:1). Sixteen patients had lesions on parts of the body other than the head, neck, and shoulders.
The title "digital mucoid cyst" is generic and encompasses cutaneous myxomas, which are focal intradermic mucinoses that are at times cystic, and ganglia, which are subcutaneous teno-arthro-synovial "hernias." A large myxoma over the second and third phalanges of the middle finger of the left hand is reported that caused progressive functional incapacity. It was managed by surgery, the technique of which is described. The need to differentiate cutaneous myxomas from ganglia is emphasized because the treatment of them is different.
Patients and Methods
This parallel‐group, double‐blind trial compared the efficacy and tolerability of topical prednicarbate, 0.25% ointment, with that of fluocortin butyl ester, 0.75% ointment.
The trial included 79 outpatients at three participating centers. All patients had a diagnosis of atopic dermatitis on the basis of Hanifin and Rajka's criteria.1 Other inclusion criteria were: age ≥ 18 years and stability or worsening of the condition for at least 1 week in the current episode. Patients were required to have been treatment‐free for minimum periods of: 7 days if previously treated with other topical corticosteroids; 1 month if previously treated with systemic corticosteroids; 3 days if previously treated with shortacting antihistamines; 2 months if previously treated with astemizole.
Exclusion criteria were: severe incapacitating dermatitis; infectious processes or association with other skin changes; need for the concomitant administration of antihistamines or antimicrobials; serious concurrent illness. Women not using adequate contraception were also excluded.
A thin layer of topical prednicarbate (N = 42) or fluocortin butyl ester (N = 37) was applied to the affected area of skin twice daily, without occlusion. The duration of treatment was set at 21 days, but it could be interrupted earlier, at the discretion of the investigator, on the grounds of total disappearance of the condition, inefficacy, or the appearance of any adverse reaction. A daily application of an emollient oil and/or a cleaning or emollient aqueous cream could be used at the investigator's discretion.
At the start of treatment and at 4, 7, 14, and 21 days, each of the following features was rated on a scale of 0 to 3 (0; absent, 1: mild, 2: moderate, 3: severe): pruritus; excoriations; erythema; scaling; lichenification; vesiculation; hyper‐keratosis. On the basis of these scores, the overall efficacy of treatment was rated as: improvement > improvement of > 50 to 75%; improvement of > 25 to 50%; improvement < 25%; no improvement; worsening.
If the treatment ended before 21 days, owing to either cure or inefficacy, the score for the last evaluation was used for all subsequent followup points. At the end of treatment the investigator and the patient rated treatment as excellent, good, fair, or poor. Cosmetic acceptance by the patient was evaluated in the same terms.
We report on a female aged 13 years, whose scalp hair began to disappear at the age of 9 years, leaving only sparse wispy hairs in the parietal-occipital region. Eyelashes, eyebrows and body hair were unaffected. There were no signs of ectodermal dysplasia on the skin, nails, teeth and eyes nor other congenital abnormalities. The family pedigree showed 15 relatives similarly affected according to an autosomal dominant mode of transmission. Clinical, genetic, histological and ultrastructural aspects led to a diagnosis of hereditary hypotrichosis simplex of the scalp (Toribio-Quiñones type).
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