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Background: Oral lichen planus (OLP) is a chronic inflammatory disorder that can cause local irritation and discomfort with attendant poor dentition and nutrition. Although a range of therapeutic options is available, data on the long-term efficacy of treatments for this chronic disease are limited. To identify agents that might be effective in OLP treatment over a longer term, and to explore their sequential use in treatment-refractory patients, we studied patients who received multiple OLP therapies and who were followed up for an average of more than 2 years., Observations: We performed a retrospective medical record review of 50 patients with histologically confirmed OLP. Patients were treated according to a therapeutic ladder of sequential treatments, beginning with topical corticosteroids and progressing through topical immunomodulators, systemic retinoids, methotrexate, and thalidomide. The best responses were observed in previously untreated patients. Most patients eventually achieved a substantial response with limited toxic effects., Conclusions: Our results identify low-dose methotrexate as an agent with substantial activity in OLP. We also demonstrate that a laddered therapeutic approach to patients with this disease can achieve substantial lesion regression even in heavily pretreated and treatment-refractory patients.

Background: Thalidomide is an anti-inflammatory agent and an immunomodulator that inhibits the production of tumor necrosis factor alpha. It has shown promise as a treatment option for the cutaneous manifestations of lupus erythematosus (LE)., Objective: To assess the degree of clinical response per subtype of cutaneous lupus, the duration of therapy before documented clinical improvement, and the incidence of adverse effects, including peripheral neuropathy, with low-dose thalidomide therapy at 100 mg daily in the treatment of refractory cutaneous lesions of LE., Methods: This retrospective medical record review of patients with refractory cutaneous manifestations of LE is one of the largest modern series in the literature. There were 29 patients seen at the Department of Dermatology, Wake Forest University School of Medicine (Winston-Salem, NC), who were unresponsive to conventional agents including antimalarial agents, and who started treatment between 1998 and 2000. Twenty-three patients who took the drug for 1 month or more were included in the analysis. Clinical responses were assessed by the investigators based on statements of improvement listed in the clinic notes and were categorized as "no response," "partial response," and "complete response." Partial response was classified as either 75% or greater or less than 75% improvement. The incidence of adverse effects including peripheral neuropathy was determined., Results: Of the 23 patients, 17 (74%) demonstrated complete resolution of the cutaneous manifestations of LE, whereas 3 patients (13%) demonstrated 75% or greater partial improvement; 3 patients (13%) had less than 75% partial clinical improvement; and 21 patients (91%) who demonstrated a complete or partial response did so within 8 weeks of initiating thalidomide therapy., Conclusions: Based on the results of this case series, we believe that thalidomide should be given prime consideration as a treatment for antimalarial drug-resistant interface lesions of LE. The design of prospective, randomized, double-blind, placebo-controlled trials for this indication is warranted.

Background and Methods: It is well documented that the cutaneous lesions of dermatomyositis may precede clinical myositis or may occur in the absence of any muscle disease detectable by current diagnostic criteria. In this single-blind study, we used magnetic resonance imaging (MRI) and ultrasound to evaluate five patients who presented with classical clinicopathologic dermatomyositis, but with normal levels of serum muscle enzymes. This patients group has not been previously studied with these techniques. Patients who served as positive and negative control subjects were also examined., Results: Ultrasonography revealed hyperechogenicity, and MRI revealed high signals on T2-weighted images in several muscle groups of the patient with active myositis (positive control). Increased echogenicity was also noted in the deltoid region of one patient who had previously had a normal muscle biopsy finding. In the same patient, MRI revealed inflammatory changes in the lumbar paraspinal muscles. Another patient, with all previous study results being normal, had MRI evidence of T2 high signals in the gluteus minimus., Conclusions: Noninvasive examinations such as MRI and ultrasound are beneficial as adjunctive means of examination in the evaluation of patients with dermatomyositis sine myositis or dermatomyositis. Future studies may suggest additional uses for these tests, including serial evaluation of patients, noninvasive confirmation of diagnosis in pediatric patients, or in directing muscle biopsy, thus increasing sensitivity. Ultrasound appears to be the more cost-effective and simple test; but MRI, although more expensive, may be more sensitive and specific.

Background: Lymphangioma circumscriptum is characterized clinically by grouped vesicles, some of which may be filled with serosanguineous fluid. The depth and extent of involvement of lymphangioma circumscriptum cannot be adequately estimated from the cutaneous examination., Observation: We describe two patients with lymphangioma circumscriptum evaluated by magnetic resonance imaging. Magnetic resonance imaging accurately demonstrated the true extent of involvement., Conclusions: With magnetic resonance imaging to delineate the entire lesion of lymphangioma circumscriptum, one can (1) provide education to the family regarding the extent of involvement and (2) prevent incomplete invasive surgical procedures from being performed.

Background: Granuloma annulare is a common skin condition usually presenting as annular plaques composed of intradermal papules. Variants such as disseminated, subcutaneous, and perforating have been described. In this article, the clinical and histologic features of a distinct papular umbilicated form of granuloma annulare are described., Observations: Four boys aged 5 to 9 years presented with papular, umbilicated, flesh-colored papules limited to the dorsa of the hands and fingers. Histologically there were unusually distributed but typical features of granuloma annulare, including well-demarcated areas of necrobiosis of collagen, localized beneath areas of epidermal thinning and parakeratosis. There was no perforation., Conclusions: Papular umbilicated granuloma annulare appears to be a distinct variant that may be difficult to diagnose because of its unique clinical and histologic features.

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.

A 36-year-old woman with a 1 1/2-year history of systemic lupus erythematosus was first seen in October 1984 with a six-month history of several ulcerated and scarred lesions on the lower extremities. A biopsy specimen showed a granulomatous infiltrate of deep dermis and subcutaneous tissue. Over the next three weeks, the patient developed a violaceous, warm, indurated, tender, fluctuant lesion involving most of the left buttock. A culture showed a rapidly growing atypical acid-fast bacteria, which was later identified as Mycobacterium fortuitum. The patient was treated with surgical drainage of the left hip abscess, followed by a three-week treatment with doxycycline hyclate and amikacin sulfate. She was discharged while receiving oral doxycycline and ethambutol hydrochloride.

A 42-year-old man had idiopathic lipemic tears and massive seborrhea. Recent investigations have shown that cholesterol and other lipids occasionally found in tears are produced by meibomian rather than lacrimal glands. The finding in this patient of hypersecretion by both sebaceous glands and meibomian glands, a sebaceous gland variant, suggests that a common mechanism may regulate secretory control of both of these glands.

Nocardiosis is an increasing clinical problem, especially in immunocompromised patients. The offending species is almost always Nocardia asteroides. Cryptococcosis is also an increasing problem in the immunosuppressed. We describe a patient with probable disseminated cryptococcosis followed by disseminated Nocardia brasiliensis infection. Only eight patients with disseminated N brasiliensis infection have been described, to our knowledge, in the United States.

The physical urticarias represent a unique subgroup of chronic urticarias. Investigators from the dermatology, allergy, immunology, pharmacology, and other fields are challenged by these experimentally reproducible forms of urticaria. Clinicians are excited by these diagnosable entities among the sometimes frustrating chronic urticarias. This review is an attempt to update the reader, in a categoric manner, on the physical urticarias. The following format will be used to review each of the physical urticarias--clinical features, diagnostic tests, pathomechanism, course, and therapy.

A 46-year-old man was diagnosed as having pyoderma gangrenosum after special stains and cultures from a skin biopsy specimen were reported as negative. Cutaneous sporotrichosis is usually diagnosed with relative ease on the basis of clinicopathologic features and prompt growth of the fungus in culture, although organisms are difficult to detect in tissue even with special stains. Identification of Sporothrix schenckii was delayed for three months in this patient because of unusual growth characteristics noted in the culture. The report of this patient's clinical course illustrates both the need to frequently reassess the diagnosis of pyoderma gangrenosum in treatment-resistant patients and the fact that S schenckii may be difficult to diagnose clinically and mycologically if the growth characteristics of the fungus are unusual.

Although necrobiosis is not a precise term, it primarily refers to collagen changes found in association with the palisading granulomas seen in granuloma annulare, necrobiosis lipoidica diabeticorum (NLD), and rheumatoid nodules. While the pathogenesis of each of these conditions remains unknown, immune complex vasculitis and delayed hypersensitivity mechanisms have been postulated. Two patients are described, having "classic" rheumatoid arthritis with chronic, superficial, ulcerating, discrete lesions on their lower legs that histologically were interpreted as NLD. While the argument for a chance association of NLD and rheumatoid arthritis could be advanced, the occurrence of identical lesions in two patients with high-titer rheumatoid factor, rheumatoid nodules, and some clinical and microscopic evidence of a low-grade rheumatoid vasculitis suggests a new subset of patients with necrobiotic palisading granulomas.

Two patients, a father and son, with pachyonychia congenita were treated with orally administered isotretinoin because the extreme deformity and discomfort associated with their massive keratoderma interfered with their work and school, respectively. While clinical benefits could not be sustained, electron microscopic findings compatible with suppression of abnormal keratinization were observed. In addition, skin biopsy samples were analyzed by sodium dodecyl sulfate-polyacrylamide gel electrophoresis, and the gels were then subjected to a lectin overlay technique with concanavalin A labeled with iodine 125. The distribution of specific glycoproteins was found to be different for lesional as against normal epidermis. The procedure was repeated after oral treatment with isotretinoin. The labeled glycoprotein pattern of the lesional epidermis was clearly distinguishable from both the pretreatment lesional and the normal epidermis; it was mostly intermediate between the two. The normal epidermis was virtually unaffected by the retinoid treatment.

Eight of ten patients with chronic or subacute cutaneous lupus erythematosus completed 16 weeks of oral isotretinoin therapy (80 mg/day). All eight patients noted an excellent clinical response without significant side effects. (Two patients did not return to initial two-week follow-up.) Peripheral blood B- and T-cell counts were unaffected by therapy. Therapy was associated with resolution of routine histopathologic abnormalities, conversion of abnormal lesional direct immunofluorescence microscopy to normal, normalization of the epidermis on electron microscopy, and reduction of all T cells near the dermoepidermal junction without change in ratio of T-helper/inducer cells to T-suppressor/cytotoxic cells. Isotretinoin is a clinically effective short-term therapy for chronic or possibly for subacute cutaneous lupus erythematosus. The primary mechanism of action remains unestablished.