Bowel-bypass syndrome without bowel bypass. Bowel-associated dermatosis-arthritis syndrome.

A recurrent, episodic illness that occurs in as many as 20% of patients who undergo ileojejunal bypass surgery for morbid obesity has been well characterized and includes inflammatory cutaneous lesions with a histologic appearance like that of neutrophilic vasculitis, a nondeforming polyarthritis, and other systemic manifestations. Current concepts of pathogenesis center on overgrowth of bacterial flora in the bypassed bowel segment with subsequent development of a circulating immune complex disease. We report, for the first time to our knowledge, an identical clinicopathologic syndrome in four patients who have not had jejunoileal bypass surgery. Each patient, however, had other gastrointestinal disease that we believe predisposed to this syndrome, possibly via circulating immune complexes with bowel-associated antigens. We propose the expanded term, bowel-associated dermatosis-arthritis syndrome, to incorporate these new cases. We believe that this is not a rare syndrome and that it is easily distinguishable from other types of cutaneous necrotizing vasculitis.

Febrile neutrophilic dermatosis in acute myelogenous leukemia.

A 50-year-old woman with acute myelogenous leukemia on two occasions had fever and exquisitely tender, warm, erythematous, indurated to frankly bullous plaques on the face and upper extremities. Histologically, the lesions showed rather dense dermal infiltrates of mature polymorphonuclear leukocytes; special stains for bacteria and fungi were negative, as were all cultures for infectious agents. The lesions did not respond to antibiotics but cleared rapidly with systemic steroid treatment. Febrile neutrophilic dermatosis of acute myelogenous leukemia is believed to be a nonspecific reaction to the underlying malignant disease process. By prompt recognition of this entity, prolonged expensive courses of antibiotics may be avoided.

Needle puncture scars from midtrimester amniocentesis.

A neonate was noted to have two depressed scars on the upper part of the back at birth that we believe resulted from midtrimester amniocentesis; no underlying injury was apparent. Scar formation from needle puncture occurs in an estimated 1% to 3% of the infants whose mothers have undergone midtrimester diagnostic amniocentesis. The scars most commonly are depressed, dimplelike, and measure 1 to 2 mm in diameter, although linear scarring may also occur. Single or multiple scars may be present, and, in seven of the 36 infants previously described in the literature, internal injuries also occurred as a result of the needle puncture.

Papular xanthoma. A clinical, histologic, and ultrastructural study.

Electron microscopic examination in a case of papular xanthoma revealed the presence of myelinlike laminated bodies in the cytoplasm of the foam cells. To our knowledge, similar bodies have been described in large numbers in only two cases of congenital self-healing histiocytosis and one case of generalized eruptive histiocytoma. The presence of laminated bodies may be a morphologic characteristic of papular xanthoma. However, this possibility should be confirmed by identification of the same inclusions in other cases of this disease.

Systemic lupus erythematosus occurring in a patient with epidermolysis bullosa acquisita.

A dystrophic bullous eruption that met all the criteria for epidermolysis bullosa acquisita (EBA) developed in a 19-year-old woman five years before the onset of clinical and serologic evidence of systemic lupus erythematosus (SLE). Electron microscopic studies of skin lesions both before and after the development of SLE were consistent with the previously reported electron microscopic findings in patients with EBA. Direct immunofluorescence microscopic studies done on bullae before and after the diagnosis of SLE showed linear depositions of immunoglobulin and complement; indirect immunofluorescence microscopic study findings consistently showed no abnormalities. These findings have been noted in other cases of EBA and may implicate autoimmune, immunologic factors in the pathogenesis of the disease process. To our knowledge, the finding of SLE in association with EBA has not been previously reported.

Cutaneous manifestations of tumoral calcinosis.

We have followed up a large family in which seven members have tumoral calcinosis. One girl had the skin lesions of localized calcinosis cutis apart from the typical subcutaneous deposits of calcium. Like most persons with tumoral calcinosis, our patient had normal serum calcium concentrations; however, the serum phosphorus levels were greatly elevated. The familial occurrence and elevated serum phosphorus levels suggest the possibility of some as yet undefined, heritable metabolic defect as the underlying cause. The occurrence of tumoral calcinosis with localized calcinosis cutis is a rare association, and there has been only one other reported case to our knowledge. This report describes our patient and offers a brief discussion of tumoral calcinosis. The therapeutic response to the phosphate depletion regimen and topical steroids was disappointing in our case.

Epidermolysis bullosa letalis associated with congenital pyloric atresia.

A 32-week-old (gestational age) female infant with epidermolysis bullosa letalis (EBL) (confirmed by light and electron microscopy) had a gastric-outlet obstruction on routine roentgenographic examination. An autopsy showed a fibrous cord connecting the stomach and first part of the duodenum in the area of the pylorus. A review of the literature indicated 12 additional cases of epidermolysis bullosa (EBL where type was confirmed) associated with pyloric atresia. The possibility of coexistent pyloric atresia should be considered in a newborn who has suspected EBL.

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.

Cutaneous manifestations of acrodynia (pink disease).

A 14-month-old girl who presented with multiple systemic complaints was found to have gingivitis, peeling of her palms and soles, and a peculiar acral eruption. A diagnosis of acrodynia, or pink disease, was confirmed by elevated levels of mercury in the urine. The many cutaneous manifestations of this once common disease are discussed.

New and emerging therapies in pediatric dermatology.

Many of the dermatologic conditions for which children seek medical attention are caused by infectious organisms. Several medications have recently become available or are on the horizon for the treatment of pediatric skin infections and infestations. Treatment of tinea capitis with fluconazole, itraconazole, and terbinafine, antibiotic therapy for staphylococcal skin infections, cidofovir for the treatment of verrucae vulgaris and molluscum contagiosum and ivermectin for scabies and head lice are discussed.

Pigmented purpuric eruptions.

The pigmented purpuras comprise a group of dermatoses that are most commonly located on the lower extremities and share related clinical and histopathologic appearances. Included is a discussion of the following entities: progressive pigmentary disease of the skin (Schamberg's disease), purpura annularis telangiectodes (Majocchi's disease), pigmented purpuric lichenoid dermatosis (Gougerot and Blum's disease), and lichen aureus.

Histiocytic syndromes.

The histiocytic syndromes are currently divided into two major categories: Langerhans cell histiocytosis and non-Langerhans cell histiocytosis. The disease entities recognized under these categories are discussed. The discussion includes clinical features, histopathology, and treatment.

Dye rashes.

Physicians may administer intravenous dyes to patients, most commonly to delineate vascular or urinary anatomy, without an appreciation of the potential hazards associated with these compounds. We report two cases in which skin eruptions followed the intravenous administration of the dyes fluorescein and methylene blue; these eruptions were the same colors as the dyes. In our first patient, urticaria, which was yellowish in color and fluorescent under a Wood's lamp, occurred after the administration of fluorescein. In the second patient, painful blue macules appeared randomly on the forearm within 15 seconds after methylene blue was injected into a free-flowing intravenous cannula on the dorsal aspect of the hand.

Metastatic calcinosis cutis.

Calcinosis cutis may be associated with metastatic calcification, dystrophic calcification, tumoral calcinosis, or may be deemed idiopathic. The association of cutaneous calcification with metastatic or tumoral calcinosis is quite rare. A case of metastatic calcinosis cutis in a woman with chronic renal failure and secondary hyperparathyroidism is presented. Other causes of calcinosis cutis are discussed briefly.

Toxic shock syndrome. Possible confusion with Kawasaki's disease.

Toxic shock syndrome (TSS) is a recently recognized condition associated with toxin-producing strains of Staphylococcus aureus. Patients affected with this syndrome are frequently young and have multisystemic complaints such as fever, headache, edema, myalgia, scarlatiniform rash, conjunctival injection, confusion, diarrhea, oliguria, hypotension and shock, This is followed by desquamation of the skin, especially the palms and soles. The majority of cases reported have been in menstruating women who used vaginal tampons regularly. Because similarities exist between toxic shock syndrome and Kawasaki's disease (mucocutaneous lymph node syndrome), as well as other conditions, proper diagnosis and management are of the utmost importance.

Managing pediatric atopic dermatitis.

Although atopic dermatitis is a very common inflammatory skin condition in children and results in many pediatric healthcare visits, its exact cause is unknown. No single laboratory test can reliably diagnose atopic dermatitis, but a relatively simple set of diagnostic criteria was recently validated for use by practicing physicians. Because existing remedies for atopic dermatitis do not cure the disorder, a program of disease control and management should be pursued. Patients and their caregivers should be advised that current therapies are primarily preventive and palliative. However, a comprehensive plan that includes routine general skin care, medical management of symptoms, identification and avoidance of aggravating factors (including psychological factors), and attention to quality-of-life issues can reduce the occurrence of skin flares. Successful treatment of acute flare-ups can be achieved with appropriate use of topical corticosteroids, but occasionally children afflicted with severe atopic dermatitis require more intensive therapies (e.g., ultraviolet light exposure systemic corticosteroids, and cyclosporine) that need close physician monitoring. Physicians must remain mindful of the psychological and quality-of-life burdens imposed on children with atopic dermatitis and their families and tailor treatments to the needs of each individual patient.