Eleanor E. Sahn

Scleroderma Following Augmentation Mammoplasty: Report of a Case and Review of the Literature

A 46-year-old woman developed localized scleroderma after surgical manipulation of her silicone gel-filled breast prostheses. She developed firm, shiny plaques on her legs that progressed to involve the thighs. Histopathologic examination of a deep-skin biopsy specimen confirmed the diagnosis of scleroderma. On surgical removal of the silicone implants, and their replacement with saline-filled implants, the scleroderma gradually resolved. Histopathologic examination of the removed implant capsules revealed evidence of silicone leakage. All new female patients with scleroderma should be questioned and examined regarding augmentation mammoplasty. Until prospective studies are completed on the possible association between scleroderma and silicone breast implants, it would seem prudent to use the saline-filled, elastomeric envelope-type breast implant for augmentation mammoplasty rather than the silicone gel-filled implant.

Genital cutaneous Crohn disease : Two cases with unusual clinical and histopathologic features in young men

Cutaneous Crohn disease, sometimes called metastatic Crohn disease or Crohn disease with cutaneous involvement, is a rare complication of Crohn disease in which granulomatous lesions involve skin separated from gastrointestinal lesions by normal tissue. We report two cases of cutaneous Crohn disease presenting in young males with erythematous, nontender swelling of the scrotum. One of the young males presented erythematous, nontender swelling of the penis as well. In one case, cutaneous Crohn disease represented the primary presentation. The original biopsy in this case showed unusual areas of degeneration of dermal connective tissue forming cystic cavities. The diagnostic biopsies in both cases showed sarcoidal granulomas with an associated superficial and deep perivascular mixed infiltrate including eosinophils. On endoscopy, both patients showed lesions of active Crohn disease in the colon. Because changes that would suggest cutaneous Crohn disease may not be present on the initial biopsy, unusual presentations and negative cultures may warrant a second biopsy. A high index of suspicion and open communication with the clinician are essential to diagnose this disease.

Pyoderma gangrenosum in pediatric acquired immunodeficiency syndrome

We describe two children with human immunodeficiency virus infection in whom pyoderma gangrenosum developed. Although pyoderma gangrenosum most commonly occurs in children with inflammatory bowel disease, it has also been described in patients with a variety of immunodeficiencies. In such patients a vigorous search to exclude a treatable infection should be made before the lesions are treated as pyoderma gangrenosum.

Multiple rhabdomyomatous mesenchymal hamartomas of skin.

A case of multiple rhabdomyomatous mesenchymal hamartomas is presented. The patient is a black male infant, the product of an uncomplicated term gestation and delivery. At birth, there were numerous polyps distributed over the periorbital and periauricular areas bilaterally. Some appeared fingerlike with constrictions below their tips. Others were branched or globular in shape. These projections showed spontaneous and independent movement, particularly during feedings. On histopathologic examination, the polyps were covered by squamous epithelium and contained normal follicular units. Bundles of skeletal muscle were present in the reticular dermis, extending into the subcutis. Regular cross-striations were seen in these muscle fibers. In some specimens, the muscle bundles formed a solid, central core. Skeletal muscle histochemical stains confirmed the presence of both types 1 and 2 muscle fibers. Electron microscopy revealed a normal skeletal muscle banding pattern. This case is the first report of multiple rhabdomyomatous mesenchymal hamartomas of skin. Functional skeletal muscle with spontaneous movement is part of the clinical picture.

Demodicidosis in a child with leukemia

A 2-year-old girl developed acute lymphoblastic leukemia at the age of 9 months. She was treated successfully with chemotherapy but developed a pruritic, papulopustular facial eruption that was caused by Demodex folliculorum. The eruption cleared after treatment with one overnight application of 5% permethrin cream. Demodicidosis should be included in the differential diagnosis of facial eruptions in children who undergo chemotherapy and in those with congenital or acquired immunodeficiency.

Persistent Erythema Infectiosum-Like Rash as a Prodrome of Acute Lymphocytic Leukemia

Abstract: Lichen sclerosus et atrophicus (LSA) is uncommonly reported in family members and is seen occasionally in young children. We report genital LSA in two young sisters who were diagnosed a year apart. The English language literature on familial LSA in childhood is reviewed and discussed.

Infantile Systemic Hyalinosis in a Black Infant

Abstract: A black girl was born with flexion contractures and experlenced pain on movement by 1 week of age. She subsequently developed perioral papules, gingival hyperplasla, perianal nodules, torticollis, dlarhea, rectal prolapse, and inability to open her mouth. Her skin became increasingly sclerodermatous, and velvety, hyperpigmented plaques arose over bony prominences. A skin biopsy specimen snowed hyallne material in the papillary dermis with tack of elastic fibers. Ultrastructural examination revealed fibrilIogranular material around fibroblasts and blood vessels, This child had the clinical, histologic, and ultrastructural features of Infantile systemic hyalinosis. This disorder has not been described in a black infant. Previous case reports of infantile systemic hyalinosis are reviewed and unusual features of our case are discussed.

MULTIPLE CUTANEOUS GRANULAR CELL TUMORS IN A CHILD WITH POSSIBLE NEUROFIBROMATOSIS

A 4-year-old girl with possible neurofibromatosis I had multiple subcutaneous nodules. On histopathologic examination, these nodules were diagnosed as multiple cutaneous granular cell tumors. An association between these tumors and neurofibromatosis I has been suggested because of their common neuroectodermal origin. We review cases of multiple cutaneous granular cell tumors in association with neurofibromatosis I in childhood.

D-penicillamine-induced elastosis perforans serpiginosa in a child with juvenile rheumatoid arthritis. Report of a case and review of the literature.

Elastosis perforans serpiginosa is a rare complication of D-penicillamine therapy. It has been reported to occur in Wilsons disease and cystinuria, usually after many years of high-dose therapy. We report a case of D-penicillamine-induced elastosis perforans serpiginosa with unique clinical features occurring in a 10-year-old child with juvenile rheumatoid arthritis who received only 71 gm of the drug over 9 months. The case is also unusual because of the short course and low cumulative dose of drug received and because of the calcification of elastic fibers. The calcification of elastic fibers suggests that this case may represent an unusual variant of elastosis perforans serpiginosa or an overlap with pseudoxanthoma elasticum. All reported cases of D-penicillamine-induced elastosis perforans serpiginosa are reviewed, and histopathologic and electron microscopic findings are presented. The theoretic mechanisms of action of D-penicillamine on elastic tissue synthesis and morphology are discussed.

Subcutaneous phaeohyphomycosis and nocardiosis in a kidney transplant patient

Phaeohyphomycosis and nocardiosis are uncommon infections that are more frequently reported in immunocompromised patients. To our knowledge, this is the first report of subcutaneous phaeohyphomycosis caused by Exophiala jeanselmei in association with systemic infection with Nocardia asteroides. The patients phaeohyphomycosis responded to surgical excision and multi-drug therapy, and the patient underwent prolonged therapy with trimethoprim/sulfamethoxazole for treatment of the nocardiosis.