Adult Henoch-Schönlein purpura with fatal complications.
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Biomedical subjects
Publications and source records attributed to S S Raimer.
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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.
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.
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.
Herpes gestationis (pemphigoid gestationis) is a rare autoimmune disease that appears during pregnancy or in the immediate postpartum period. We report the cases of a mother and neonate with immunofluorescence confirmed herpes gestationis both of whom had extensive cutaneous involvement.
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First described by Shelley and Wood in 1977, mid-dermal elastolysis (MDE) is a rare acquired disorder in which there is a bandlike absence of elastic tissue limited to the mid-dermis. In their patient, MDE developed in an area previously involved with recurrent episodes of urticaria. We describe a 15-year-old white girl with well-circumscribed, minimally palpable yellow-white plaques and wrinkling diagnosed histologically as MDE in areas clinically diagnosed 5 years previously as granuloma annulare. As in the first described patient, five years elapsed between clearance of the original skin lesions and the clinical appearance of MDE. To our knowledge, we report the first adolescent case of MDE localized to previous sites of lesions clinically consistent with granuloma annulare and propose that MDE represents an abnormal end-stage reaction to multiple processes.
The clinical and pathologic findings of Kikuchi's histiocytic necrotizing lymphadenitis may mimic those of malignant lymphoma. We describe a 6-year-old boy with generalized lymphadenopathy, spiking fever, chills, myalgias, malaise, and erythematous, crusted papules. Although cutaneous manifestations have been noted in 16% to 40% of patients with histiocytic necrotizing lymphadenitis, only three publications described skin lesions. The skin lesions and affected lymph nodes revealed histiocytic aggregates, atypical lymphoid cells, karyorrhectic debris, and patchy necrosis. Spontaneous resolution occurred in 2 months. Results of serologic studies, Epstein-Barr virus (EBV) latent membrane protein immunoperoxidase staining, EBER-1 RNA in-situ hybridization, and EBV EBNA-1 DNA polymerase chain reaction implicate EBV as the causative agent.
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We describe 5 new cases of striated muscle hamartoma (SMH) and review the 7 cases previously described in the literature. Eleven of the 12 patients had single lesions centrally located on the chin (4), near the ala of the nose (3), on the upper chest (3), and on the upper lip (1). Histologically all of the lesions were characterized by a central core containing bundles and individual fibers of skeletal muscle as well as other mesenchymal elements. The muscle fibers penetrated the dermis among numerous folliculo-sebaceous structures. Of the 12 cases of SMH, 3 had other associated congenital anomalies including a cleft lip in 1 patient and a thyroglossal duct sinus in another with single lesions, and preauricular sinuses, low-set ears and bilateral sclerocorneas in the case of multiple SMH. The relationship of SMH with oculocerebrocutaneous syndrome is discussed, and we conclude that at least some of the skin appendages associated with that syndrome are SMH.
Reviewed herein are the major types of vasculitis affecting children, including Henoch-Schönlein purpura, necrotizing vasculitis of small vessels, Kawasaki's disease, and, less commonly, classic polyarteritis nodosa, Wegener's granulomatosis, Churg Strauss syndrome, and lymphomatoid granulomatosis. The clinical features, diagnosis, treatment, and suspected etiologies and pathogenic mechanisms for each entity are discussed.
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A procedure for transplanting cultured human keratinocytes has been developed and used successfully to treat patients with persistent leg ulcers. Shave excisions of donor skin of 1 cm2 or less were expanded in culture in 1 week to cover wounds as large as 90 cm2. The uniqueness of this system is the use of a biochemically modified surgical dressing that permits the transplantation of undifferentiated cells. Several hours after inoculation, trypsinized single cells become attached to the dressing and are ready for grafting. Seven of eight persistent stasis ulcers completely reepithelialized after application of the modified surgical dressing with undifferentiated keratinocytes.
A 4-month-old male infant had a 2-month history of an exfoliative erythroderma and alopecia. Recurrent mucosal infections, diffuse lymphadenopathy, hepatosplenomegaly, lymphocytosis and eosinophilia, anemia, and failure to thrive later developed. Investigation revealed a combined immunodeficiency with T cells of an unusual phenotype in his peripheral blood, skin, and lymph nodes. Our patient's clinical manifestations most closely resemble Omenn's syndrome, a rare form of autosomal recessive combined immunodeficiency.