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Biomedical subjects

W R Heymann

Publications and source records attributed to W R Heymann.

At least 19 recordsLinked to original sources
PubMed1998

Hereditary papulotranslucent acrokeratoderma.

Hereditary papulotranslucent acrokeratoderma (HPA) is a rare autosomal dominant genodermatosis, characterized by persistent, asymptomatic, yellow-white translucent papules and plaques of the hands and feet. A case of HPA is presented and the literature of this variant of punctate keratoderma is reviewed.

Acrodermatitis↗
PubMed1997

Acute lymphoblastic leukemia in a child with the CHIME neuroectodermal dysplasia syndrome.

The "CHIME" syndrome (MIM#280000) is a rare neuroectodermal disorder comprised of Colobomas of the eye, Heart defects, Ichthyosiform dermatosis, Mental retardation, and Ear defects. We report on the sixth child with this syndrome and the first of these to develop acute lymphoblastic leukemia at age 4 1/2 years. Her major problems included a migratory ichthyosiform dermatosis, multiple skin infections and infestations, bilateral retinal coloboma, developmental delay, seizures, infantile macrosomia, facial anomalies, a duplicated renal collecting system, and conductive hearing loss. Histologic examination of the skin demonstrated findings of an epidermal nevus with deep rete pegs, hyperkeratosis, and a markedly increased granular layer. The cause of the CHIME syndrome is unknown, but the disorder is easily recognized because of the striking phenotype. The diagnosis is important to make because of the potential for associated congenital heart disease, neurologic compromise, possible autosomal recessive inheritance, and possible association with malignancy.

Child, Preschool↗
PubMed1997

Acquired angioedema.

Acquired angioedema (AAE) is a rare disorder that has been categorized into two forms, AAE-I and AAE-II. AAE-I is associated with other diseases, most commonly B-cell lymphoproliferative disorders. AAE-II is defined by the presence of an autoantibody directed against the C1-inhibitor molecule. Differentiating AAE-I from AAE-II is vital because different therapeutic interventions are required for each type. This review summarizes the clinical aspects, pathophysiology, and management of AAE compared with the types of hereditary angioedema.

Angioedema↗
PubMed1997

Reticulate hyperpigmentation.

Disorders that are characterized by a reticulate pattern of pigmentation are reviewed. Dyskeratosis congenita (DKC) is the prototype of these. In addition to reticulate hyperpigmentation, mucosal leukoplakia, bone marrow dysfunction, cytogenetic instability, and a predisposition to malignancy are characteristic of DKC. The most common pattern of inheritance is X-linked, with heterozygous females showing variable expression, most likely depending on tissue-specific patterns of random X-inactivation. Other reticulate pigmentary disorders reviewed include the Naegeli-Franceschetti-Jadassohn syndrome, X-linked reticulate pigmentary disorder, dermatopathia pigmentosa reticularis, Dowling-Degos disease, dyschromatosis, confluent and reticulated papillomatosis of Gougerot and Carteaud, reticulate acropigmentation of Kitamura, and Revescz syndrome. Diagnosis, treatment, and sometimes genetic counseling remain problematic for these entities. The pathophysiology of these disorders is unknown, but will certainly be aided greatly by the future identification of the underlying genes.

Bone Marrow Diseases↗
PubMed1997

Submandibular abscess caused by Eikenella corrodens.

Eikenella corrodens is a slow-growing, facultative, anaerobic, gram-negative bacillus that is part of the normal oral flora and is found in dental plaque. It has become increasingly recognized as a pathogen in nonimmunocompromised and immunocompromised hosts. A case of a submandibular abscess due to Eikenella corrodens, which was successfully treated by administration of cefuroxime accompanied by incision and drainage, is presented. Dermatologists need to be aware of this pathogen is the evaluation of suppurative lesions of the head and neck.

Adult↗
PubMed1996

Recurrent toxin-mediated perineal erythema.

BACKGROUND: Important new diseases due to bacterial toxins functioning as superantigens have been described with increasing frequency within recent years. Toxic shock syndrome, recalcitrant erythematous desquamating disorder, streptococcal toxic shock-like syndrome, and, most recently, mucocutaneous lymph node syndrome (Kawasaki disease) have been etiologically linked with certain staphylococcal and streptococcal toxins. We describe two patients with a novel clinical presentation of toxin-mediated disease, which shares certain clinical features with mucocutaneous lymph node syndrome. OBSERVATIONS: Two otherwise healthy young male adults developed recurrent erysipelaslike perineal erythema, which regularly erupted within 1 to 2 days of the onset of acute pharyngitis. Accompanying signs included mucosal changes and acral erythema with desquamation. Throat cultures obtained during the acute episodes yielded toxin-producing Staphylococcus aureus from one patient and toxin-producing Streptococcus pyogenes from the other. CONCLUSION: The recurrent nature, age predilection, and clinical presentation suggest that our patients display a unique clinical syndrome due to toxin-producing bacteria.

Adolescent↗
PubMed1996

Necrobiosis lipoidica treated with topical tretinoin.

Necrobiosis lipoidica is often resistant to therapeutic intervention. Lesions are atrophic and frequently ulcerate. A case is presented in which atrophy was diminished by the application of topical tretinoin. The practical and theoretical use of topical tretinoin in this disorder is reviewed.

Administration, Topical↗
PubMed1994

Acute generalized exanthemic pustulosis.

Acute generalized exanthemic pustulosis often presents dramatically with the abrupt onset of a widespread pustular eruption on an erythematous base, with a frequently positive Nikolsky sign. In most cases, penicillins or macrolides are causally related. The main differential diagnosis of a generalized pustular eruption in a febrile patient is pustular psoriasis, which can be differentiated on both a clinical and histologic basis.

Acute Disease↗
PubMed1992

Cutaneous manifestations of thyroid disease.

Cutaneous manifestations of thyroid disease are protean in nature and affect all age groups. This review focuses on normal thyroid gland physiology, specific cutaneous/thyroid lesions such as the thyroglossal duct cyst and metastatic thyroid malignancies, nonspecific cutaneous alterations of the hyperthyroid and hypothyroid states, and the numerous associations of thyroid disease with other cutaneous and/or systemic disorders.

Humans↗