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Alopecia and periorificial dermatitis in biotin-responsive multiple carboxylase deficiency.

Three siblings with infantile-onset biotin-responsive multiple carboxylase deficiency are described. Recognition of the characteristic dermatologic manifestations, alopecia and periorificial dermatitis, should result in early diagnosis and institution of potentially lifesaving therapy with biotin. Other metabolic disorders may present a similar clinical picture. Immunologic dysfunction and/or aberration in lipid or branched chain amino acid metabolism may be the common pathophysiologic link in some or all of these disorders.

Alopecia↗

Self-healing juvenile cutaneous mucinosis.

A case of self-healing juvenile cutaneous mucinosis is presented. It demonstrates typical features of the disease: early age of onset, infiltrated plaque lesions of the head and torso, nodules of the face and periarticular regions, rapid onset accompanied by inflammatory phenomena, and spontaneous resolution over a few months. Skin biopsy showed the deposition of the mucin, hyaluronic acid, mainly in the upper reticular dermis, and a mild increase in fibroblasts and mast cells. Results of studies of B and T lymphocytes were normal. Although three cases have been reported in Europe, to our knowledge this is the first reported case in the English-language literature.

Adolescent↗

Glucagonoma syndrome: in vitro evidence that glucagon increases epidermal arachidonic acid.

A 63-year-old white woman with perioral dermatitis, a sore tongue, and an erythematous dermatosis in the inframammary and perineal regions underwent surgical removal of a pancreatic glucagonoma. The patient's plasma and pooled normal human plasma containing Sigma glucagon were fed to human keratinocyte cultures and increased arachidonic acid levels by 300% and 200%, respectively, when compared to pooled normal human plasma with no added commercial glucagon. These experiments suggest that glucagon may increase inflammatory mediators such as arachidonic acid and its metabolites in the epidermis, causing the skin lesions seen in the glucagonoma syndrome.

Adenoma, Islet Cell↗

Metastatic Crohn's disease.

Cutaneous granuloma formation distant from the gastrointestinal tract in patients with Crohn's disease of the bowel has been called metastatic Crohn's disease. We report two patients with this entity, including the first to present with an erysipelas-like eruption of the face. A review of the world's literature reveals that all patients with metastatic Crohn's disease have had gastrointestinal disease involving the colon or rectum. Clinical features of the nine previously reported cases are reviewed.

Adult↗

Acquired, bilateral nevus of Ota-like macules.

Blue-brown macules of the face occurring on both sides of the forehead, temple, eyelids, malar area, alae of the nose, and root of the nose are often observed in middle-aged Japanese women. These lesions histologically are a form of dermal melanocytosis as shown by electron microscopic examination. They differ clinically from nevus of Ota. The differential diagnosis includes nevus of Ota, Riehl's melanosis (female facial melanosis), and melasma. The differences between them are discussed.

Adult↗

Cutaneous nocardiosis. Case reports and review.

Two cases of cutaneous nocardial infection are reported. The Nocardia species are gram-positive, partially acid-fast bacteria. Cutaneous involvement may develop as one of four types: (1) mycetoma, (2) lymphocutaneous (sporotrichoid) infection, (3) superficial skin infection, or (4) systemic disease with cutaneous involvement. A review of each of these types of infection is included, as well as potential clues that may suggest the diagnosis of nocardiosis.

Abscess↗

Large keratoacanthomas in difficult locations treated with intralesional 5-fluorouracil.

Keratoacanthoma is a benign tumor; however, it may cause massive destruction of normal tissue during its rapid growth phase. Intralesional 5-fluorouracil (5-FU) has been shown to be an effective treatment for keratoacanthoma. We have successfully treated five patients with large keratoacanthomas in difficult locations. There have been no recurrences during follow-up periods ranging from 18 to 45 months. Intralesional 5-FU may be ineffective for keratoacanthomas that are not rapidly proliferating.

Adult↗

Periocular dermatitis akin to the perioral variety.

A persistent periocular dermatitis resulting from the prolonged use of topical fluorinated corticosteroids that resembled the perioral variety was observed in three patients. All topical therapy was ineffective until systemic tetracycline was administered.

Drug Eruptions↗

Acute surgical complications: cause, prevention, and treatment.

The acute complications that may result in failure of the surgical procedure are discussed. These include bleeding, hematoma, infection, dehiscence, and necrosis. The background of each is covered in detail and an attempt is made to show the interrelationships among them. The purpose of this communication is to aid in the recognition, treatment, and prevention of these acute surgical complications.

Dermatitis, Contact↗