Aquagenic syringeal acrokeratoderma: report of two teenage cases.

Transient reactive papulotranslucent acrokeratoderma is an unusual condition of the palms that has been characterized by symmetric, hypopigmented, flat-topped papules and plaques with eccrine duct prominence that becomes more pronounced on exposure to water. Histopathologically, hyperkeratosis and dilated eccrine ostia are seen. Two teenagers with papulotranslucent acrokeratoderma are described. We propose the term aquagenic syringeal acrokeratoderma, which accurately describes the appearance after exposure to water and the prominent eccrine ducts seen clinically and histologically in the setting of acrokeratoderma.

An unusual pustular eruption in an infant with Down syndrome and a congenital leukemoid reaction.

Congenital leukemia and leukemoid reactions may be indistinguishable on clinical and histologic grounds and are highly associated with trisomy 21. This report characterizes a specific vesiculopustular skin eruption in an infant with Down syndrome and a congenital leukemoid reaction. On the first day of life an unusual vesiculopustular eruption developed, starting in areas of cutaneous trauma. A biopsy revealed immature myeloid cells in an epidermal spongiotic vesiculopustule and in a perivascular distribution, suggestive of leukemia cutis. As the peripheral blood smear normalized, the eruption cleared. Myelodysplasia subsequently developed and evolved into acute myelogenous leukemia. This is the first detailed report of a specific skin infiltrate caused by the immature cells of a leukemoid reaction. Skin infiltration by immature myeloid cells during a congenital leukemoid reaction may portend an aggressive course of the myeloproliferative disorder.

Clotting and thrombotic disorders of the skin in children.

The skin is often the presenting organ for thrombotic disorders. Purpura fulminans, with its widespread cutaneous necrosis, may be the result of severe infection, of an inherited or acquired disorder of the protein C procoagulant pathway, or of unknown causes (the idiopathic form). Recurrent venous or arterial thrombosis and the associated skin findings at an early age may be clues to an inherited disorder of protein C, protein S, or antithrombin III. Livedo reticularis, leg ulcerations, and hemorrhagic purpura may suggest the antiphospholipid antibody syndrome.

Neonatal lupus erythematosus and maternal lupus erythematosus mimicking HELLP syndrome.

A 4-month-old boy had a faint, confluent, macular erythema involving the forehead, nose, and philtrum, and matlike telangiectases over the forehead and eyelids. The clinical features, course, and laboratory studies were characteristic of neonatal lupus erythematosus. He was otherwise healthy and the product of a preterm twin pregnancy complicated by the development of maternal HELLP (hemolysis, elevated liver enzymes, low platelets) syndrome. The patient had elevated Ro(SSA) and La(SSB) antibody titers, and studies performed on maternal blood disclosed positive antinuclear antibodies and elevated Ro and La antibody titers.

Pyogenic granuloma (lobular capillary hemangioma): a clinicopathologic study of 178 cases.

Pyogenic granuloma (lobular capillary hemangioma) is a common acquired vascular lesion of the skin and mucous membranes in the pediatric age group. This is a retrospective analysis of 178 patients, 17 years of age and younger (mean age 6.7 yrs). Forty-two percent of the lesions occurred in the first five years of life; only 12% appeared in infants less than 1 year old. The male:female ratio was 3:2. Most patients (74.2%) had no history of trauma or predisposing dermatologic condition. The mean lesional size was 6.5 mm and the mean duration at diagnosis was 3.8 months. The granulomas were most commonly located in the head and neck area (62.4%), followed in order of decreasing frequency by trunk (19.7%), upper extremity (12.9%), and lower extremity (5.0%). The preponderance (88.2%) occurred on the skin, the remaining ones involved the mucous membranes of the oral cavity and conjunctivae. Histologic examination demonstrated normal numbers of mast cells, in contrast to increased mast cells characteristic of proliferative phase hemangiomas. Most lesions (n = 149) were treated by full-thickness skin excision and linear closure; there were no recurrences in this group. The recurrence rate in 23 lesions treated by shave (intradermal) excision and cautery or cautery alone was 43.5%.

Lasers, tissue expansion, and sun protection in pediatric dermatology.

As our knowledge of cutaneous disease increases, areas emerge in which our intervention with patients at an early age can significantly alter the progression of disease. This article discusses three major topics of new advances in the treatment of children with cutaneous disorders. These include laser therapy of various skin anomalies, the use of tissue expansion in congenital nevi, and the role of sun protection in children.

Disorders affecting the skin and the heart.

Numerous disorders affect both the skin and the heart. These include inherited and metabolic disorders, rheumatologic diseases, infectious diseases, and a variety of others. In many instances, awareness of this association allows the dermatologist to assist in identifying potentially life-threatening cardiac conditions.

Rhabdomyosarcoma presenting as a cutaneous nodule.

Two patients with rhabdomyosarcoma presented clinically with dermal nodules on the face. The first patient was a 12-month-old girl with a 2 X 3-cm-diameter red nodule on her left cheek that had begun as a small red papule when she was 3 months old and had progressively increased in size. The second patient was a 19-year-old girl who presented with an enlarging mass on her right cheek that had begun as a pea-sized nodule three months previously. In both cases, biopsies revealed a rhabdomyosarcoma in the underlying soft tissue with extension into the overlying dermis. Rhabdomyosarcoma presenting as a dermal nodule is rare. It usually presents as an asymptomatic papule without distinctive clinical features and therefore may result in delayed diagnosis unless a biopsy is performed.

Recurrent subepidermal calcified nodule of the nose.

A form of idiopathic calcinosis cutis, subepidermal calcified nodule (SCN), recurred twice on the nasal ala of a healthy 6-year-old girl whose serum calcium and phosphorus levels were within normal limits. Basophilic granular material and numerous mast cells were present in the superficial dermis. The pathogenesis of SCN is uncertain, but the clinical and histologic features of this lesion are distinctive.

Nontypable Haemophilus influenzae (biotype 4) as a neonatal, maternal, and genital pathogen.

Between 1976 and 1981 Haemophilus influenzae was identified in 16 women with postpartum bacteremia and 36 neonates with bacteremia or meningitis. H. influenzae was also recovered from neonatal or genital cultures of 50 additional patients. By counter-immunoelectrophoresis 17% of neonatal isolates from blood or cerebrospinal fluid (CSF) were type b. All remaining strains (94% overall) were nontypable (NT). Of the NT blood or CSF isolates, 38% belonged to biotype 4. Of all the NT biotype 4 isolates referred to the Centers for Disease Control during the study, 82% were of genital, neonatal, or maternal origin, a finding that suggests that this isolate is a genital biotype. Clinical disease was similar to that observed in patients infected with group B Streptococcus except for the infrequent (11%) occurrence of meningitis. Maternal bacteremia resulted in mild febrile illness, while neonatal bacteremia was associated with a high incidence of shock, respiratory distress (50%), and death (30%). H. influenzae bacteremia in these two patient groups was rare in Houston before 1976, but since then it has been responsible for 2.5% of cases of significant bacteremia. NT H. influenzae should be recognized as a definite neonatal, maternal, and genital pathogen.

Differences among Nocardia spp. in susceptibility to aminoglycosides and beta-lactam antibiotics and their potential use in taxonomy.

Using disk diffusion and agar dilution methods, 109 clinical isolates of Nocardia were tested against 10 antimicrobial agents. A species difference in susceptibility was noted for cefamandole, carbenicillin, gentamicin, and kanamycin. These differences in susceptibility may be of use taxonomically as well as having some potential use clinically.

In vitro activity of trimethoprim and sulfamethoxazole against the nontuberculous mycobacteria.

Trimethoprim (TMP) and sulfamethoxazole (SMZ) were studied alone and in combination (TMP-SMZ) against 141 nontuberculous mycobacteria; an agar dilution method was used. All strains of Mycobacterium kansasii (8), Mycobacterium marinum (16), and Mycobacterium scrofulaceum (3); 97% (63 of 65) of strains of Mycobacterium fortuitum; and 27% (3 of 11) of strains of Mycobacterium avium-intracellulare were inhibited by less than or equal to 32 micrograms of SMZ/ml. In contrast, all 38 isolates of Mycobacterium chelonei were resistant to 32 micrograms of SMZ/ml. All species were highly resistant to TMP, with minimal inhibitory concentrations (MICs) of greater than or equal to 64 micrograms/ml. The MICs of TMP-SMZ for these organisms were similar to the MICs of SMZ alone. These results support the use of sulfonamides for treatment of infections due to M. fortuitum and M. marinum and suggest the need for further clinical and laboratory studies of the activity of these drugs against several additional mycobacterial species. TMP-SMZ appears to offer no advantage (in vitro) over SMZ alone against any of these organisms.

Comparative trial of cefonicid and cefamandole in the therapy of community-acquired pneumonia.

Cefonicid (Smith Kline & French Laboratories; D-75073) is a new parenteral cephalosporin with a markedly long half-life, high serum levels, and good in vitro activity against Haemophilus influenzae. Patients with community-acquired pneumonia were randomized 2:1 to receive cefonicid, 1 g daily (21 cases) or cefamandole, 1 g every 6 h (12 cases). The two groups were similar, except that the cefonicid patients were older (mean 42 versus 31 years). Peak serum levels of cefonicid averaged 133 microgram/ml after intravenous and 83 microgram/ml after intramuscular administration compared with 55 microgram/ml with intravenous cefamandole. All 9 patients on intramuscular cefonicid and 8 or 12 patients on intravenous cefonicid had trough serum levels of greater than 2.0 microgram/ml at 24 h. Sputum levels of cefonicid were usually between 2.0 and 4.0 microgram/ml and did not correlate with serum levels. Cefonicid was well tolerated, and all cefonicid patients responded clinically. Sputum cultures for H. influenzae or Streptococcus pneumoniae became negative in 6 of 7 cefamandole patients and 13 or 15 cefonicid patients. In in vitro studies, cefonicid inhibited 90% of beta-lactamase-negative h. influenzae at 0.5 microgram/ml and beta-lactamase-positive strains at 2.0 microgram/ml. Cefonicid inhibited 50% of S. pneumoniae at 1.6 microgram/ml, but required 6.4 microgram/ml to inhibit 90%. Cefonicid once a day appears to be as safe and as effective as cefamandole four times a day for therapy of community-acquired pneumonia.

Susceptibility of Mycobacterium marinum to tetracyclines and aminoglycosides.

Current agar dilution methods for susceptibility testing of Mycobacterium marinum versus the tetracyclines have failed to show in vitro susceptibility despite good in vivo results. We found that the tetracyclines were unstable in agar and resulted in a fine haze of growth for several concentrations before complete inhibition of growth was seen. In contrast, the aminoglycosides resulted in sharp minimal inhibitory concentration endpoints, which were generally independent of the length of incubation. The problems with the tetracyclines can be lessened by shortening the incubation time to 5 days and redefining the minimal inhibitory concentration as the lowest concentration resulting in no growth or a slight haze of growth. By this methodology, 19 clinical isolates of M. marinum were tested for susceptibility to the tetracyclines and aminoglycosides. Minocycline inhibited greater than 50% of isolates at 2.0 microgram/ml, whereas both minocycline and doxycycline inhibited greater than 90% of isolates at 4.0 microgram/ml. These studies support the usage of the tetracyclines in the treatment of clinical diseases due to M. marinum and suggest a susceptibility method which is more predictive of clinical results.

Sulfonamide activity against Mycobacterium fortuitum and Mycobacterium chelonei.

Forty-eight clinical strains of Mycobacterium fortuitum and 15 clinical strains of Mycobacterium chelonei were evaluated for susceptibility to sulfonamides, including trimethoprim-sulfamethoxazole (TMP-SMZ). Sensitivity tests were carried out with use of agar dilutions in Mueller-Hinton agar and a plate inoculum of 10(2) cfu. Thirty-six percent of the isolates of M. fortuitum were inhibited by 8 micrograms of sulfonamide/ml, and 98% were inhibited by 32 micrograms/ml. None of the isolates of M. chelonei were inhibited at these concentrations, but 73% were inhibited by 128 micrograms/ml, and 87% were inhibited by 256 micrograms/ml. Both species were highly resistant to TMP, and the combination TMP-SMZ (1:20) was no more active than was SMZ alone. The growth of M. chelonei on Mueller-Hinton agar required the addition of 10% OADC (oleic acid, albumin, dextrose, and catalase), and exact MICs were difficult to determine by agar dilutions because growth of the organism tended to diminish gradually over several dilutions. Six patients with disease due to rapidly growing mycobacteria were treated with sulfonamides, and all showed a good response to therapy. Sulfonamides may be the treatment of choice for infections due to M. fortuitum and offer potential for the therapy of disease due to M. chelonei.

Haemophilus influenzae infections in adults: characterization of strains by serotypes, biotypes, and beta-lactamase production.

One hundred three cases of bacteremia or meningitis due to Haemophilus influenzae in adults were evaluated. Among 96 episodes of bacteremia, 60% were due to pneumonia and 15% to genital-related infections; 10% had no apparent source of infection. Of 42 isolates serotyped in routine fashion by slide agglutination, 79% were reported as type b. In contrast, of 45 isolates from the same interval with confirmed serotyping (usually by counterimmunoelectrophoresis), only 29% were type b and 64% were nontypable; 26% had been misidentified by routine slide agglutination. The majority (85%) of confirmed typable strains were biotype I. Four (40%) of 10 nontypable obstetrical isolates belonged to the relatively rare biotype IV. Only 2% of isolates were ampicillin-resistant, despite a high resistance rate among pediatric isolates in the same communities. When serotyping is carefully performed, nontypable organisms appear to be the major cause of invasive H. influenzae disease in adults.