Dermatologic Entities Research Articles

PsEma—A Hitherto Unnamed Dermatologic Entity With Clinical Features of Both Psoriasis and Eczema

Recent advances in molecular biology have helped establish differences between psoriasis and a group of inflammatory skin disorders commonly referred to as eczema. The authors have observed significant overlap between these two conditions such that a distinction between them may not always be made, even with histologic examination of skin biopsy specimens. To determine how frequently psoriasis patients present features of both psoriasis and eczema. The authors conducted a prospective analysis of 100 consecutive psoriasis patients in their clinic. The authors found that 20% could be diagnosed as "intermediate," having lesions with characteristics of both psoriasis and eczema, or a personal history of both. The authors suggest naming this category of inflammatory dermatosis "PsEma"--an overlap condition in which the clinical, histologic, molecular, biologic, and therapeutic responses show characteristics of both psoriasis and eczema.

Cutaneous adiaspiromycosis: A distinct dermatologic entity associated with Chrysosporium species

Primary cutaneous infection with Chrysosporium, a saprophytic fungus commonly found in soil, is believed to be very rare, with only two previously reported cases. We present a case of localized cutaneous Chrysosporium in an immunocompromised heart transplant patient. Considering that the histology of the skin in this case is superimposable on that seen in pulmonary Chrysosporium known as adiaspiromycosis, we regard the cutaneous variant in the absence of pulmonary disease as a distinct dermatologic entity. The low frequency of reports of primary cutaneous Chrysosporium infection suggests either underreporting of this diagnosis in the literature, or misidentification of this fungus as another more common mycotic species sharing morphologic similarities. By amplifying our understanding of Chrysosporium infection in the skin, this disorder will be easier to identify and treat.

Dermatologic diseases of the breast and nipple

Breast and nipple skin is commonly affected by various inflammatory and neoplastic processes. Despite this fact, many physicians are unaware of the spectrum of diseases that can involve this area. Because breast and nipple skin represents a cosmetically, sexually, and functionally important entity to most patients, awareness of these disease entities is invaluable. This article reviews the normal anatomy of the breast, cutaneous manifestations of neoplastic processes that can present in these areas, and common inflammatory diseases of the breast and nipple skin. (J Am Acad Dermatol 2000;43:733-51.) Learning objective: At the conclusion of this learning activity, participants should be familiar with (1) the normal anatomy of the breast, (2) common benign and malignant tumors of the breast, and (3) the spectrum and differential diagnosis of inflammatory and infectious processes involving the breast. Better understanding of dermatologic entities affecting the breast may aid in improved diagnosis of these entities as well as ensuring appropriate intervention.

Investigation of skin disease and urticaria in the horse

THE veterinary surgeon is commonly presented with horses with skin disease, the management of which requires a thorough clinical examination and diagnostic work-up. This article outlines the general approach to...

Warfarin induced skin necrosis.

Warfarin induced skin necrosis is a rare complication with a prevalence of 0.01-0.1 per cent. It was first described in 1943. A literature review was undertaken using Medline; all relevant papers on this rare compli-cation of warfarin therapy were used. There are several adverse skin manifestations associated with the use of oral anticoagulants, ranging from ecchymoses and purpura, haemorrhagic necrosis, maculopapular vesicular urticarial eruptions to purple toes. This article concentrates mainly on warfarin induced skin necrosis. The syndrome typically occurs during the first few days of warfarin therapy, often in association with the administration of a large initial loading dose of the drug. Although the precise nature of the disease is still unknown, advances in knowledge about protein C, protein S and antithrombin III anticoagulant pathways have led to a better understanding of the mechanisms involved in pathogenesis. Differential diagnosis between warfarin induced skin necrosis and necrotizing fasciitis, venous gangrene and other causes of skin necrosis may be difficult; the disease may also be confused with other dermatological entities. Warfarin induced skin necrosis, while rare, is an important complication. All surgeons should be aware of its existence.

The skin immune system: lupus erythematosus as a paradigm.

Lupus erythematosus (LE) was first described as a clinical dermatological entity in 1851. The possibility of serious systemic manifestations became recognized by 1872 as the result of the work of M. Kaposi. Since then, it took a long time before LE was recognized to be an immunological disease. Recognition of antinuclear antibodies and their deposition at the basal membrane region in skin resulted in two concepts of LE pathogenesis. In one, antibody complexing and complement activation with generation of the membrane attack complex (C5-C9) is thought to be the origin of the chronic inflammatory reaction. In the other, antibody deposition enables antibody dependent cellular cytotoxicity, leading to hydropic degeneration of the basal epidermal layer and subsequent chronic inflammation. Norris postulated in 1993, that the epidermis acts as a pro-inflammatory organ, in which an UVB-induced increase in cytokine production is followed by increased expression of adhesion molecules on keratinocytes as well as dermal endothelial cells. Translocation of certain antigens (i.e. Ro & La) to which circulating auto-antibodies exist in LE, enables recognition by adhesion molecule directed skin-invading T cells with subsequent cytotoxic effector activity. A persistent chronic inflammatory reaction then ensues. A similar development in knowledge may be seen in the history of immunodermatology. Originally, lupus erythematosus could not be recognized as an immune disease, since concepts of immunology were virtually non-existent when LE was first described. Immunology, In the first half of this century, was mainly antibody-oriented and thus came the concept of (S)LE as an antibody-mediated disease.(ABSTRACT TRUNCATED AT 250 WORDS)

Historical approach to scleroderma

S cleroderma seems to be a comparatively easy dermatologic entity to trace through medical history; yet, it differs little from a multitude of other diseases for which the nomenclature and the classification have been repeatedly muddled.’ The current terminology, including morphea, scleroderma, and progressive systemic sclerosis, can sometimes lead to confusion (Table 1).2 The condition under consideration has clinical manifestations that can be recorded by patients and nonprofessional observers.3 Physicians in ancient times viewed patients who may have had the characteristic hardening of the skin, but there are often not sufficient details to confirm the diagnosis. Hippocrates, for example, records patients in whom “the skin is stretched, and parched and hard, the disease terminates without sweat.” 4 More information can be found in several reviews of the history of scleroderma.2,5-* Scattered reports from the seventeenth and early eighteenth centuries are suggestive of sclerodenna but illustrate the existing confusion. A 1731 presentation before the Royal Society in London may have been scleroderma or more possibly scleredema:

Disseminated Hypopigmented Keratoses

We present two cases of asymptomatic, widespread keratotic eruptions in young female patients. Clinically, the lesions are well-demarcated, small, hypopigmented, flat-topped papules occurring on the trunk and extremities in a uniform distribution. Skin biopsy specimens from one patient revealed hyperorthokeratosis, papillomatosis, and a normal amount of melanin. We suggest that this is a newly recognized dermatologic entity that may be descriptively termed disseminated hypopigmented keratoses. Disseminated hypopigmented keratoses may be distinguished by clinical and histologic criteria from similar keratotic eruptions. Since the lesions of disseminated hypopigmented keratoses are both inconspicuous and asymptomatic, it is likely that the disorder is more prevalent than our two cases would suggest.

The development of 20th century dermatologic drugs

The purpose of medicine is to cure or to alleviate suffering. Until this century, the essence of dermatology resided in the realm of morphology. Early contributors largely confined their activities to the classification of skin diseases and to the elaboration of clinical dermatologic entities based on morphologic features. Today, professional colleagues and patients alike find it convenient to consult physicians who are not only able to recognize but also effectively treat many skin diseases. In the last 50 years, there have been significant scientific discoveries in the field of therapeutics that have revolutionized the practice of dermatology. This article primarily concerns itself with the development of therapeutic agents in which dermatologists were involved. Most of these advances resulted from academic and clinical research, some from old-fashioned armchair research from untrained investigators who thought a problem through and came up with logical answers. Other advances resulted from hunch, chance, or serendipity.

Colour Atlas of Dermatology

This volume is intended primarily for senior medical students, general practitioners, and residents in dermatology as well as internal medicine. It will be extremely valuable for the dermatology teacher in a clinical practice who can quickly turn to appropriate photographs demonstrating the teaching points of the patient he is interviewing in the clinic.The book has 20 clinical chapters, a chapter on therapeutics, a glossary, and an index. The clinical chapters contain excellent photographs of Indian patients. Although the background skin in the cases is intermediate in color, one can easily imagine the same features in both blacks and whites. All important dermatological entities are covered. There is a brief telegraphic description of the disease and a summary outline of therapy for each disorder.I liked this approach very much. A minor disturbance for me was the fact that most of the clinical photographs are placed on a horizontal axis, whereas the text follows a vertical axis.

Acanthosis palmaris in a patient with bullous pemphigoid

Acanthosis palmaris, a recently described dermatologic entity, is often associated with internal malignancy. We report the development, disappearance, and reappearance of this condition in a patient with bullous pemphigoid. Relevant screening tests did not reveal any internal malignant neoplasm. The significance of the appearance and disappearance of the condition is discussed.

Reticular erythematous mucinosis syndrome in a patient with polyarthritis

A patient with seronegative oligoarthritis who developed the reticular erythematous mucinosis (REM) syndrome is described. This syndrome is considered to be a dermatological entity unrelated to systemic disorders. Aggravation of the rash by exposure to sunlight and a good response to anti-malarial agents suggest a relationship with rheumatological disorders, e.g. rheumatoid arthritis and systemic lupus erythematosus. Dermatologists consulted by a patient with the REM syndrome should be aware of the possibility of an associated rheumatological disease.

The increasing role of Microsporum canis in the variety of dermatophytic manifestations reported from Israel.

During a period of 8 years 300 cases of dermatophytoses involving both hairy areas and the glabrous skin were found to be caused by M. canis. There was scalp involvement in 60%, including 8 infants and 27 adults; most of the adults presented Kerion-like lesions and presented various clinical aspects such as seborrhea capitis, folliculitis and discoid lupus erythematosus. In the 21 patients showing invasion of the beard the clinical manifestations included superficial erythemato-squamous patches with hyperemic slightly elevated margins, folliculitis or abscess-like lesions and Kerion-like lesions. Among the lesions found on the glabrous skin there were unusual aspects of tinea faciei in 19 adults, mimicking lymphocytic infiltration, granuloma faciale or discoid lupus erythematosus. Some of the cases of tinea corporis found in 70 patients also had lesions simulating various other dermatological entities, including erythema multiforme, psoriasiform eruption, pityriasis rosea and seborrheic dermatitis. The hands were invaded in 5 adults patients, with involvement of the finger nails in one. Repeated mycologic examinations were necessary to establish the true etiology in many of these cases.

Idiopathic Deciduous Skin

ABSTRACT:Five patients are described with more or less continuous peeling of the skin aggravating in summer months. No family members are affected and consanguinity among parents of affected individuals is not seen. Characteristically the palms, soles, mucous membrane, and scalp are spared from shedding. Generally the patients look dirty and occasional erythema and vesiculation occur. Both sexes are affected equally. The disease does not start at birth and the onset may be delayed. Histopathologic examination shows subcorneal reparation of bulla formation without acantholysis. The stratum corneum appears very loose and shows little hyperkeratosis (or‐thokeratosis). Treatment is ineffective except mild keratolytics give cosmetic improvement. Etiology remains unknown. An autoimmune mechanism may be implicated. The authors propose the name ‘idinpathic deciduous skin’ for this distinct new dermatologic entity.

Kyrle's Disease in Patients With Chronic Renal Failure

• Hyperkeratosis follicularis et parafollicularis in cutem penetrans, or Kyrle's disease, is a rare dermatologic entity with distinctive clinical and histopathologic features. Over a two-year period, nine cases of Kyrle's disease were observed among 200 patients receiving hemodialysis for chronic renal failure. This observation suggests an association between Kyrle's disease and renal disease that, to our knowledge, has not previously been reported. (Arch Dermatol1982;118:85-88)

Keratosis Circumscripta

45 cases of a dermatosis believed to be the same as those originally described by Schrank in 1963 among Nigerian children of Yoruba ethnic origin and named keratosis circumscripta by him are reviewed. All evidences point towards the fact that the dermatosis observed by us as well as those originally described by Schrank are a variant of psoriasis possibly modified by environmental factors. It has therefore been suggested that this dermatosis should not be regarded as a new dermatological entity, and that the original name should be modified. To reflect the original work done by Schrank as well as the true clinical picture of this dermatosis, the nomenclature psoriasis circumscripta with palmoplantar keratosis is suggested.

Gardner-Diamond Syndrome With Multiple Glomus Tumors

The case of a patient with two unusual dermatologic entities, multiple glomus tumors and the painful purpura of Gardner-Diamond syndrome, is reported. The diagnosis of multiple glomus tumors was confirmed by biopsy, and the Gardner-Diamond syndrome was confirmed by reproduction of the painful purpura with the intradermal injection of the patient's sonicated erythrocytes.

Bitemporal Aplasia Cutis Congenita

Aplasia cutis congenita is an uncommon entity and is very rarely found on the face. A girl had an unusual bilateral, symmetrically located aplasia cutis congenita of the temples, with a peculiar facies and a constellation of other cutaneous findings. Aplasia cutis congenita is a rare dermatologic entity presenting in the newborn infant as localized areas of skin deficiency. 1,2 Most often the affected area lies in the midline and posteriorly on the scalp and measures 1 to 2 cm in diameter. The defect is most often solitary, but multiple lesions may occur, which are usually symmetrically located. Aplasia cutis presents at birth as sharply marginated, raw, granulating wounds and may range in shape from circular to elongate, stellate, and triangular. 3 These defects may reach enormous sizes, sometimes covering most of the scalp, and they are apt to be large when located on the trunk or limbs. 4 The

Linear Sebaceous Nevus Syndrome-Reply

We attempted to clarify existing nomenclature utilized in describing linear epidermal nevi and Dr. Lantis' letter illustrates the lack of uniformity in classification of the dermatological entities. The references quoted by Dr. Lantis were reviewed by us and only because of space limitations, were not included in the reference section. Biopsies were performed in all three cases and histologic findings of hyperkeratosis and parakeratosis were common to all. We wish to emphasize that the linear sebaceous nevus syndrome is representative of one of several types of linear epidermal nevi. Classification of neurocutaneous syndromes into groups and subgroups is far less important than attempting to delineate the pathogenesis of these entities. Furthermore, we do not believe that the nomenclature referred to in the article will mislead or create confusion, particularly among pediatricians. Dr. Lantis appears to be engaged in a battle of semantics and has completely overlooked the authors' intent to

Lymphomatoid papulosis. An additional case of a new disease.

A papulonodular eruption which is clinically benign but histologically malignant occurred in a 52-year-old man. During the last five years this patient has shown a continual appearance and disappearance of asymptomatic reddish papules which leave a brownish pigmentation or a small, shallow scar after a duration of two to four weeks. Some of the papules develop a central necrosis with a small ulcer and others show a small hemorrhage in the center, with a black crust. Whereas the clinical course is benign and self-limited, the histologic picture is suggestive of a malignant disease. The similarity to other described cases suggests the existence of a new dermatological entity.