Evan W. Piette

Response to Antimalarial Agents in Cutaneous Lupus Erythematosus: A Prospective Analysis

OBJECTIVE To demonstrate response to antimalarial agents in patients with cutaneous lupus erythematosus (CLE) using activity scores from the Cutaneous Lupus Erythematosus Disease Area and Severity Index, a validated outcome measure. DESIGN Prospective, longitudinal cohort study. SETTING University cutaneous autoimmune disease clinic. PARTICIPANTS A total of 128 patients with CLE who presented from January 2007 to July 2010 and had at least 2 visits with activity scores. INTERVENTION Administration of antimalarial agents. MAIN OUTCOME MEASURES Response was defined by a 4-point or 20% decrease in activity score. Response to initiation was determined by the difference between the scores before treatment and at the first visit at least 2 months after treatment. Response to continuation was determined by the difference between the scores at the first visit and the most recent visit while undergoing treatment. RESULTS Of 11 patients who initiated treatment with hydroxychloroquine, 55% were responders (n = 6), showing a decrease in median (interquartile range [IQR]) activity score from 8.0 (3.5-13.0) to 3.0 (1.8-7.3) (P = .03). Of 15 patients for whom hydroxychloroquine failed, 67% were responders to initiation of hydroxychloroquine-quinacrine therapy (n = 10), showing a decrease in median (IQR) activity score from 6.0 (4.8-8.3) to 3.0 (0.75-5.0) (P = .004). Nine of 21 patients who continued hydroxychloroquine treatment (43%), and 9 of 21 patients who continued hydroxychloroquine-quinacrine (43%) were responders, showing a decrease in median (IQR) activity score from 6.0 (1.5-9.5) to 1.0 (0.0-4.5) (P = .01) and 8.5 (4.25-17.5) to 5.0 (0.5-11.5) (P = .01), respectively. CONCLUSIONS The use of quinacrine with hydroxychloroquine is associated with response in patients for whom hydroxychloroquine monotherapy fails. Further reduction in disease activity can be associated with continuation of treatment with antimalarial agents.

Characterization of clinical photosensitivity in cutaneous lupus erythematosus

BACKGROUND Photosensitivity (PS) in lupus erythematosus (LE) is frequently determined by patient report. OBJECTIVE We sought to characterize self-reported PS in cutaneous LE (CLE). METHODS The PS survey was used to classify subject responses into 5 phenotypes: direct sun-induced CLE flare (directCLE); general exacerbation of CLE (genCLE); polymorphic light eruption-like reactions (genSkin); general pruritus/paresthesias (genRxn); and sun-induced systemic symptoms (genSys). In all, 91 subjects with CLE alone or with CLE and systemic LE were interviewed. RESULTS In all, 81% ascribed to 1 or more PS phenotypes. CLE-specific reactions (direct sun-induced CLE flare or general exacerbation of CLE) were reported by 86% of photosensitive subjects. Higher CLE disease activity (measured by CLE Disease Area and Severity Index activity scores) was suggestive of direct sun-induced CLE flare reactions (P = .09). In all, 60% of photosensitive subjects described CLE-nonspecific reactions: polymorphic light eruption-like rash and general pruritus/paresthesias. These phenotypes often co-occurred with CLE-specific reactions and were predicted by more systemic disease activity as measured by Physicians Global Assessment (PGA) scores in regression analyses (genSkin, P = .02) and (genRxn, P = .05). In all, 36% of subjects reported systemic reactions and higher PGA scores were predictive of the sun-induced systemic symptoms phenotype (P = .02); a diagnosis of systemic LE was not (P = .14). LIMITATIONS PS was inferred from patient report and not directly observed. CONCLUSIONS Characterization of self-reported PS in LE reveals that patients experience combinations of CLE-specific, CLE-nonspecific, and systemic reactions to sunlight. Sun-induced CLE flares are associated with more active CLE disease. Polymorphic light eruption-like, generalized pruritus/paresthesias, and systemic reactions are associated with more active systemic disease. Recognition of PS phenotypes will permit improved definitions of clinical PS and allow for more precise investigation into its pathophysiology.

Dapsone in the management of autoimmune bullous diseases.

Dapsone is used in the treatment of autoimmune bullous diseases (AIBD), a group of disorders resulting from autoimmunity directed against basement membrane and/or intercellular adhesion molecules on cutaneous and mucosal surfaces. This review summarizes the limited published data evaluating dapsone as a therapy for AIBD.

Dapsone in the Management of Autoimmune Bullous Diseases

Dapsone is used in the treatment of autoimmune bullous diseases (AIBD), a group of disorders resulting from autoimmunity directed against basement membrane and/or intercellular adhesion molecules on cutaneous and mucosal surfaces. This review summarizes the limited published data evaluating dapsone as a therapy for AIBD.

Purpuric and cream-colored plaques in an immunocompromised person: A case of disseminated trichosporonosis

Trichosporon species have become increasingly recognized as opportunistic pathogens capable of causing disseminated infections. Cutaneous lesions are fairly common and may serve as a diagnostic clue to invasive Trichosporon infection. We report a case of disseminated trichosporonosis in an immunocompromised patient who presented with purpuric and cream-colored plaques.

Stenotrophomonas maltophilia: an emerging multidrug-resistant opportunistic pathogen in the immunocompromised host

Stenotrophomonas maltophilia is a multidrug-resistant opportunistic pathogen with increasing prevalence and high morbidity and mortality. In addition to its classic association with pulmonary infections, S. maltophilia can cause skin and soft tissue infections with varying clinical presentations. We describe the case of a man in his 30s with B-cell acute lymphoblastic leukaemia who presented with a solitary patch of faint but tender purpura found to have rapidly progressive S. maltophilia infection diagnosed on skin biopsy. S. maltophilia infection should be considered in the cutaneous evaluation of the immunocompromised host.

Ulcerated Plaque With Lymphocutaneous Spread

Awomaninher50sadmittedforbacterialpneumoniawasnotedtohave anulceratedplaqueontheforearm.Thepatienthadbeendischargedapproximately2weeksprior,afteranadmissionforcongestiveheart failure. A few days after that discharge, the patient noticedapainfululcerationontherightdistal forearm,at thesitewhereaperipheral intravenous catheter had been inserted during her initial hospitalization. On physicalexamination,thepatienthadanulceratedplaqueontherightforearm with 2 tender, 1to 2-cm red nodules in the right antecubital fossa (Figure,A).Onfurtherquestioning,thepatientnotedthatbetweenherhospitalizationsshehadgonehomeandcleanedherfishtank,playedwithher petcat,andgardenedinher indoorcactusgarden.Shesaidshehadnotrecentlytraveled,andwasnottakinganyimmunosuppressivemedications. Askinbiopsyandculturewereperformedfromtheforearmlesion(Figure, B andC). Quiz at jamadermatology.com A B

A Crusted Rash in a Patient With AIDS

A 39-year-old man with a recent diagnosis of AIDS (CD4 cell count, 13 cells/μL) was admitted to the hospital for diarrhea and dehydration and was noted to have an intensely pruritic rash with diffuse crust and scale. Dolutegravir and emtricitabine/tenofovir were continued, and diphenhydramine was prescribed for pruritus. On day 3 of hospitalization, the dermatology service was consulted. The patient had a history of mild psoriasis, which significantly worsened 1 year prior to presentation, necessitating treatment with systemic agents and ultimately leading to a diagnosis of human immunodeficiency virus (HIV) infection. Physical examination was remarkable for severe cachexia and diffuse plaques of thick, tan scale (Figure 1). Hyperkeratotic areas were verrucous and fissured over bony prominences, including the elbows, knees, ribs, and clavicles. Linear excoriations were noted diffusely. Unroofed crust revealed a smooth, red, moist undersurface.

An unusual case of lichen planus

dysplasia and is characterized by hypohidrosis, hypotrichosis, hypodontia, periorbital hyperpigmentation, and sculpted noses. The most frequently affected structure is the hair on the scalp and on other parts of the body. Scalp hair is usually sparse, fragile, and dry. Hair-shaft abnormalities are of great concern to these patients, but no effective treatments are available. Eyebrow tattoos or wigs can improve quality of life in severe cases. A favorable outcome after treatment of congenital alopecia with topical tretinoin and minoxidil was reported in a patient with hidrotic ectodermal dysplasia. After 6 months of combined treatment, the density of hair on the scalp increased without side effects. By contrast, our patient did not have any vellus hair on the scalp at the start of the treatment, and treatment with minoxidil alone, without tretinoin, produced a positive cosmetic outcome. Avoiding tretinoin is advantageous because it can cause skin irritation and a burning sensation. Systemic effects, such as hypotension and palpitation, can occur during the use of topical minoxidil. These side effects are very rare and mostly not drug-related. Minoxidil has a mitogenic effect on epidermal cells, increases the duration of anagen hair, and enlarges miniaturized follicles. This may explain the hair growth in our patient: in ectodermal dysplasia there may be a decrease in the maturation of the hair follicles rather than a complete absence. This case study strongly suggests that topical minoxidil alone canbeused as the treatment of choice for congenital alopecia in ectodermal dysplasia.