Taxane associated subacute cutaneous lupus erythematosus.

Numerous medications have been associated with the development of subacute cutaneous lupus erythematosus. A mechanism explaining how unrelated drug classes can lead to subacute cutaneous lupus erythematosus has remained elusive, suggesting that there may be multiple etiologic pathways. Taxanes (docetaxel, paclitaxel, and cabazitaxel) inhibit cell mitosis through microtubule stabilization and their use has uncommonly been associated with subacute cutaneous lupus erythematosus. Recently the antigen recognized by anti-Ro/SS-A antibody (Ro52) has been localized to the cytoplasmic microtubule network. A case report of docetaxel exacerbated subacute cutaneous lupus erythematosus is presented and literature review performed, revealing 11 additional cases of taxane associated subacute cutaneous lupus erythematosus. Taxanes are proposed to exacerbate or induce subacute cutaneous lupus erythematosus in immunogenetically predisposed patients by stabilizing microtubules and affecting Ro/SS-A antigen (Ro52) expression. This may be an under recognized adverse drug reaction because taxanes are used for a defined period and the cutaneous eruption tends to spontaneously improve. Studies analyzing how particular drug classes affect Ro/SS-A antigen expression may be useful in identifying mechanisms of action in drug-induced subacute cutaneous lupus erythematosus.

Sarcoidosis.

UNLABELLED: Sarcoidosis is a systemic noncaseating granulomatous disorder of unknown origin. The cutaneous manifestations of sarcoidosis often enable the dermatologist to be the first physician to make the diagnosis. This article reviews essential sarcoidosis pathophysiology, clinical polymorphisms, systemic evaluation, and treatment modalities for cutaneous sarcoidosis to further enhance the dermatologist's understanding of this disease entity. LEARNING OBJECTIVE: At the conclusion of this learning activity, participants should be familiar with the theories of the pathogenesis of sarcoidosis, its cutaneous manifestations, its various syndromes and associations, and its presentation in children. Participants should also be more knowledgeable about diagnostic evaluation, measurement of disease progression, treatment modalities, and the prognosis and mortality data of sarcoidosis.

Cutaneous manifestations of endocrine disorders.

Endocrine disorders such as diabetes or Cushing's syndrome often have cutaneous findings, including changes to the skin, hair, and nails. In this review, the major causes, clinical manifestations, laboratory workup, and treatment of the most important endocrine diseases are discussed.

Scurvy in an unrepentant carnivore.

For centuries, scurvy, or vitamin C deficiency, decimated crews of sailing ships on long sea voyages and populations deprived of fresh fruits and vegetables during times of war or famine. Today, scurvy is extremely rare in the United States, and its classic findings of perifollicular petechiae, edema and purpura of the lower extremities, corkscrew hairs, and hemorrhagic gingivitis may go unrecognized. We report the case of a man from rural Appalachia who developed typical signs and symptoms of scurvy on two separate occasions, approximately 2 years apart. Both times, the patient underwent an extensive work-up and was diagnosed with numerous other conditions before his vitamin C deficiency was recognized. We discuss the clinical presentation, pathophysiology, diagnosis, and treatment of scurvy, with attention to specific findings that should alert the clinician to this diagnosis.

Lipedematous alopecia: a clinicopathologic, histologic and ultrastructural study.

Lipedematous alopecia is a rare condition of unknown etiology characterized by a thick, boggy scalp with varying degrees of hair loss that occurs in adult black females, with no clearly associated medical or physiologic conditions. The fundamental pathologic finding consists of an approximate doubling in scalp thickness resulting from expansion of the subcutaneous fat layer in the absence of adipose tissue hypertrophy or hyperplasia. Observations by light and electron microscopy detailed in this report suggest that this alteration principally manifests by localized edema with disruption and degeneration of adipose tissue. Some diminution in the number of follicles as well as focal bulb atrophy is noted. Aberrant mucin deposition such as that seen in myxedema or other cutaneous mucinoses is not a feature. The histologic findings bear some resemblance to those seen in lipedema of the legs, a relatively common but infrequently diagnosed condition. We present a case of lipedematous alopecia with emphasis on histologic and ultrastructural features. The etiology is unknown.

Unilateral laterothoracic exanthem in a child with acute lymphoblastic leukemia.

Unilateral laterothoracic exanthem (ULE) is a rare childhood condition of unknown etiology characterized by a morbilliform rash that usually begins in the axilla and spreads centrifugally. The condition is usually self-limited, does not require specific treatment, and disappears within 4 to 6 weeks. Pruritus may be relieved by oral antihistamines and topical emollients and/or bath oils. We describe ULE in a 6-year-old girl with acute lymphoblastic leukemia, the first report of ULE in a patient with malignancy.

Nonpigmented dysplastic melanocytic nevi.

BACKGROUND: Dysplastic melanocytic nevi (DMN) are thought to represent a clinical and histologic bridge between common pigmented nevi and superficial spreading malignant melanoma. The following clinical criteria for DMN were established to aid in the proper identification of these lesions: irregular perimeter, size exceeding 5 mm in diameter, background erythema, and variegated color (shades of browns, tans, blacks, and reds). Histologic features include basilar melanocytic proliferation with nuclear atypia, a patchy lymphocytic infiltrate with concentric eosinophilic fibroplasia, and lamellar fibroplasia. To our knowledge, there have been no previously reported cases of uniformly nonpigmented DMN. OBSERVATIONS: A 31-year-old brown-haired, browneyed white woman with no personal or family history of either DMN or melanoma presented for evaluation of numerous, discrete, nonindurated, 2- to 5-mm-diameter, nonpigmented macules and slightly elevated papules that had appeared in a truncal distribution over the course of several years. Microscopic examination of these lesions showed lentiginous epidermal hyperplasia and disordered proliferation with variable cellular atypia of intraepidermal melanocytes. CONCLUSIONS: Nonpigmented, nonindurated, macular or slightly elevated papular lesions may represent nevi with features of dysplasia. In light of the significant risk of malignant melanoma that is associated with pigmented varieties of dysplastic nevi, it is essential that clinicians consider nonpigmented DMN in the differential diagnosis of entities that present as hypopigmented macules.

Sporotrichoid cutaneous leishmaniasis in a traveler.

A 19-year-old construction worker from Virginia who had traveled in Bolivia had sporotrichoid lesions on the left arm. Only after unsuccessful therapy for sporotrichosis was a diagnosis of cutaneous leishmaniasis considered. Biopsies revealed necrotizing granulomatous changes, and culture of the biopsy specimens grew Leishmania (Viannia) braziliensis. The sporotrichoid pattern seen in this patient is a rare but recognized presentation of cutaneous leishmaniasis, more commonly seen in American cutaneous leishmaniasis than in Old World cutaneous leishmaniasis. This case illustrates the necessity of careful and early consideration of tropical infections in the differential diagnosis of disease in a traveler.

Rubinstein-Taybi syndrome with multiple flamboyant keloids.

A single case of Rubinstein-Taybi syndrome associated with numerous giant keloids is reported. The patient manifested all the major features of Rubinstein-Taybi syndrome. The cause of this multisystem developmental disorder is unknown.

Erythrokeratodermia variabilis present at birth: case report and review of the literature.

A healthy boy had the distinctive lesions of erythrokeratodermia variabilis (EKV) at birth. Twenty-eight patients described in the literature had EKV that presented in childhood. Nine of the 28 were said to have had a rash since birth, but none were distinctive of EKV. To our knowledge this is the first well-documented case describing a child born with the skin manifestations of EKV. We conclude that patients with EKV are infrequently born with a rash, and that only very rarely when the rash is present is it suggestive of the disorder.

Venous disease: how to heal and prevent chronic leg ulcers.

Venous disease accounts for up to 90% of all cases of chronic leg ulcers. Patients with venous disease have relatively unhealthy, ischemic tissue in the lower legs, and slight trauma often initiates an ulcer. Symptoms include leg swelling, which is often unilateral and persistent, and a dull ache that worsens with standing, walking, or sitting with the legs down. Approximately 50% of patients have a history of deep vein thrombosis. A careful history and examination can rule out other causes and guide appropriate therapy as well as prophylaxis against progressive disease. Elevation, compression, occlusion, and debridement are the mainstays of treatment. Ulcer healing is gradual, often requiring weeks to months of therapy. Preventing recurrence requires careful attention to compression; surgery may be indicated.

Plantar warts in the athlete.

Plantar warts are thick, endophytic, hyperkeratotic lesions caused by human papilloma virus. Because they are frequently mistaken for calluses, they are often misdiagnosed in athletes. The diagnosis of a plantar wart is made by paring down the hypertrophic epithelium until multiple "seeds" are detected in the dermis representing the thrombosed vessels supplying the wart. A patch system containing 40% salicylic acid in the rubber-based vehicle is applied to the debrided site every 48 hours until healing occurs.

Papular mucinosis associated with acquired immunodeficiency syndrome.

Papular mucinosis, also known as lichen myxedematosus, is a rare, idiopathic disorder in which focal deposits of mucin accumulate in the dermis. It has been previously reported in two patients with human immunodeficiency virus infection; we describe a third such case and note the presence of a granuloma adjacent to the mucin deposit. We also comment on other clinical manifestations of cutaneous mucin deposition and granuloma formation in human immunodeficiency virus infection.

Epidermal naevi and bullous aplasia cutis congenita in a neonate.

The case of a neonate with cutaneous lesions consistent with epidermal naevi is presented. In addition to typical epidermal naevi, this baby had an unusual, bullous form of aplasia cutis congenita. Although aplasia cutis has been described as bullous and has been found in association with the epidermal naevus syndrome, both of these occurrences are rare in medical publications. This case illustrates an unusual presentation of epidermal naevi with bullous aplasia cutis congenita and raises difficult diagnostic and counselling issues.

Generalized eruptive keratoacanthoma of Grzybowski.

A patient with generalized eruptive keratoacanthoma of Grzybowski is described. Our findings, along with those of the 21 reported cases from the world literature, are summarized. This rare variant of keratoacanthoma most commonly affects patients in the fifth to seventh decades of life and appears as a generalized eruption of hundreds to thousands of follicular papules. These small papules often have a keratotic center and demonstrate the microscopic features of keratoacanthoma. Marked facial involvement is characteristic and can lead to masked facies with ectropion. Severe pruritus, mucosal lesions, and koebnerization are features of the disease. The course is chronic and the response to therapy is poor, although a few patients have improved with the use of systemic retinoids.

Sclerosing panniculitis. A clinicopathologic assessment.

Six patients with well-circumscribed, indurated, inflammatory plaques of the lower extremity are described. Incisional biopsy specimens in each patient revealed fat necrosis, sclerosis, and a lobular panniculitis. These patients are similar to those described under the designations hypodermitis sclerodermaformis or lipodermatosclerosis. The name sclerosing panniculitis is proposed to unify these conditions. The pathogenesis is unknown, although a role for venous stasis or previous thrombophlebitis has been proposed. Systemic or local anti-inflammatory therapies have not been clinically effective.