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.

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 patient's phaeohyphomycosis responded to surgical excision and multi-drug therapy, and the patient underwent prolonged therapy with trimethoprim/sulfamethoxazole for treatment of the nocardiosis.

A case of Bloom syndrome with conjunctival telangiectasia.

Bloom syndrome is a rare genodermatosis characterized by photosensitivity, telangiectasias, growth retardation and malignancies. Eye findings have rarely been mentioned in case reports of this syndrome. We report a child with Bloom syndrome who had pronounced bulbar conjunctival telangiectasia originally diagnosed as episcleritis.

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.

Bullous mastocytosis in an infant associated with the use of a nonprescription cough suppressant.

Bullous mastocytosis is an unusual expression of mastocytosis typically seen in young children, and many causes of the acute mast cell degranulation with bulla formation have been identified. We report a 6-month-old boy with urticaria pigmentosa and an extensive bullous eruption associated with the ingestion of a nonprescription cough suppressant containing dextromethorphan. The pathogenesis of mastocytosis and the care of patients with this disease are discussed.

Developmental neural abnormalities and seizures in epidermal nevus syndrome.

The epidermal nevus syndrome (ENS) is an unusual neurocutaneous disorder consisting of the combination of an epidermal nevus and a central nervous system (CNS), ophthalmological, and/or skeletal abnormality. The study reports four new patients with ENS. Each had a confirmatory biopsy of the epidermal nevus, abnormal neurological examination findings, and documented CNS anatomical studies by imaging or autopsy.

Disseminated Mycobacterium scrofulaceum infection: a potentially treatable complication of AIDS.

Disseminated Mycobacterium scrofulaceum infection has rarely been reported (only 8 cases to date), and no case of infection associated with AIDS has been reported in detail. We report a case of disseminated M. scrofulaceum infection in an AIDS patient that presented as chronic ulcerative and nodular skin lesions with probable cavitary lung involvement.

Alopecia areata in childhood.

Aproximately 1% of the population will have had alopecia areata by the age of 50 and the peak incidence occurs in children and young adults. All body hair may be affected including lashes and brows. Alopecia areata is a systemic disease with frequent involvement of nails or eyes.

Incontinentia pigmenti: three cases with unusual features.

Three patients with incontinentia pigmenti are described who illustrate some of the unusual features of this uncommon genodermatosis. One child with skin, ophthalmologic, and dental findings had atrophic, hypopigmented streaks on her legs by the age of 2 1/2 years that were consistent with the fourth stage of incontinentia pigmenti. This child's mother, who also had incontinentia pigmenti, had identical atrophic streaks on the legs, as well as irregular axillary pigmentation, scarring alopecia, and dental abnormalities.

Neurologic disease in a child with hepatoerythropoietic porphyria.

Hepatoerythropoietic porphyria (HEP) is a rare, autosomal recessive disorder due to deficient uroporphyrinogen decarboxylase enzyme activity. Patients exhibit photosensitivity, red urine, hypertrichosis, and characteristic serum and urine porphyrin profiles. Two siblings had the classic clinical and biochemical findings of HEP.

Vesiculopustular diseases of neonates and infants.

Vesiculopustular diseases of neonates and infants can be divided into infectious and noninfectious categories. Recent clinical and scientific literature has focused mainly on the infectious diseases and on epidermolysis bullosa. This review covers the following disorders: neonatal sepsis, staphylococcal scaled skin syndrome, neonatal herpes simplex, neonatal varicella, congenital syphilis, scabies, mastocytosis, incontinentia pigmenti, eosinophilic pustular folliculitis, and epidermolysis bullosa.

Familial lichen sclerosus et atrophicus in childhood.

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.

A black girl was born with flexion contractures and experienced pain on movement by 1 week of age. She subsequently developed perioral papules, gingival hyperplasia, perianal nodules, torticollis, diarrhea, rectal prolapse, and inability to open her mouth. Her skin became increasingly sclerodermatous, and velvety, hyperpigmented plaques arose over bony prominences.

La (SS-B)-positive neonatal lupus erythematosus: report of a case with unusual features.

Neonatal lupus erythematosus is most often associated with autoantibodies against Ro and La antigens. Rarely, neonatal lupus erythematosus occurs in the absence of Ro antibody. We present a case of La antibody-positive neonatal lupus erythematosus with unusual features.

Musculoaponeurotic fibromatosis (extraabdominal desmoid tumor) in a child with idiopathic multicentric osteolysis.

The fibromatoses are a group of benign proliferations of fibrous tissue with clinical behavior ranging from that of truly malignant tumors to that of benign reactive fibrous proliferations. Some of the superficial fibromatoses are fairly common, but the deep ones, also known as desmoid tumors or musculoaponeurotic fibromatoses, are rare. Idiopathic multicentric osteolysis is a rare skeletal disorder of childhood that causes progressive destruction of bones and renal failure.

Demonstration of silicon in sites of connective-tissue disease in patients with silicone-gel breast implants.

Background And Design: Silica, Silastic, and silicone (any organic compound in which silicon replaces carbon) have been associated with a number of connective-tissue diseases, most commonly systemic sclerosis (scleroderma). Silicone is known to leak from breast implants and spread to surrounding tissues, including lymph nodes, but silicone's role in the origin and pathogenesis of the inflammation and fibrosis related to such conditions remains controversial. Synovial tissue, alveolar macrophages, and skin, each from three different patients with silicone-gel implants, plus the breast implant capsules from each of the three patients, were examined by light microscopy, transmission electron microscopy, and electron probe microanalysis for the presence of silicon-containing material.