Case Report: Pemphigoid Nodularis-Five Patients With Many Years of Follow-Up and Review of the Literature.

Pemphigoid nodularis is a rare form of pemphigoid that joins the clinical picture of prurigo nodularis and the immunological features of bullous pemphigoid, which is therapeutically challenging. Here, we analyze five female patients with a long-lasting course of nodular pemphigoid in terms of clinical and immunological characteristics and therapy. All the patients fulfilled clinical and immunological criteria of nodular pemphigoid. We applied numerous techniques allowing the proper diagnosis: direct and indirect immunofluorescence, salt split skin, ELISA, BIOCHIP, and fluorescence overlay antigen mapping using laser scanning confocal microscopy. Our study showed that 4 of 5 patients fulfilled the clinical and immunological criteria of nodular bullous pemphigoid. Two out of 4 patients presented exclusively nodular lesions; in the other two patients, blisters and erythematous lesions preceded prurigo-like lesions by a few years. The remaining patient had clinical and immunological criteria of nodular mucous membrane pemphigoid, presenting oral erosions, scarring conjunctivitis, and numerous disseminated nodules on the skin. All the patients were treated with multiple medicines; however, it was observed that the use of clobetasol propionate on the entire body plus antidepressants best controlled the disease. Pemphigoid nodularis mainly occurs in elderly women. In cases with coexisting psychological problems, antidepressants should be considered as an important complementary therapy to the basic one with clobetasol propionate.

Thalidomide for the treatment of chronic refractory prurigo nodularis.

Prurigo nodularis (PN) is a highly pruritic skin condition that is caused by chronic scratching. It occurs in patients with chronic itch and is characterized by multiple hyperkeratotic papules and nodules. The pathogenesis of PN is unclear, but involves a complex interplay of numerous pathways including neurogenic and inflammatory factors. As such, PN is very difficult to treat and patients are often refractory to multiple medications before finding a treatment that is effective. We present a woman with a 20-year history of exuberant prurigo nodularis who failed multiple therapies, including dapsone, azathioprine, mycophenolic acid, prednisone, topical steroids, and phototherapy. She only obtained significant relief of chronic pruritus and lesion flattening with thalidomide 100mg daily. Thalidomide is an antipruritic and anti-inflammatory agent that has shown to be very effective in treating a variety of dermatologic conditions. However, its use today is limited by concerns for its teratogenic and neuropathic side effects. With strict adherence to medication protocols, these adverse effects can be minimized. As such, thalidomide should be considered for patients with refractory dermatologic conditions.

Generalized eruptive keratoacanthoma with vitiligo followed by the development of prurigo nodularis: A case report and published work review.

Herein, we report a unique case of generalized eruptive keratoacanthoma (GEKA) in a 47-year-old Chinese man presenting with extensive pruritic papules and nodules accompanied by oral lesions. He also had a 2-year history of vitiligo and long-term experience of working outdoors. Biopsies were consistent with keratoacanthoma . Interestingly, prurigo nodularis (PN) was found in histopathology at 1-year follow up. To our knowledge, this is the first report describing a case of GEKA with oral lesions complicated with vitiligo and developed with PN.

Prurigo nodularis: an update on etiopathogenesis and therapy.

Prurigo nodularis (PN) is a chronic, highly pruritic condition characterized by the presence of hyperkeratotic, excoriated, pruritic papules and nodules, with a tendency to symmetrical distribution. No reliable data exist about incidence and prevalence of PN in the general population, but it seems to be more frequent and more intense in females. PN may be associated with many dermatological and non-dermatological comorbidities, including psychiatric disease. Recent findings suggest a neuropathic origin of PN, with alterations in the dermal and epidermal small diameter nerve fibers. PN may have a tremendous impact on the quality of life, and few effective treatment options are available. Few randomized controlled trials (RCT) on the therapy of PN are available, demonstrating the efficacy of phototherapy alone or with psoralen, and of topical calcipotriol and topical steroids in occlusive medications. Thalidomide may be effective, but no RCT are available and its use is impractical due to the unfavorable safety profile. Gabapentin, pregabalin and the neurokinin receptor 1 antagonist, aprepitant, seem also to be effective in the therapy of PN, but RCTs are still lacking.

Excimer laser vs. clobetasol propionate 0·05% ointment in prurigo form of atopic dermatitis: a randomized controlled trial, a pilot.

BACKGROUND: Recent findings have established the 308-nm xenon chloride excimer laser (EL) as a new option in the area of ultraviolet (UV) B phototherapy. As this laser enables high radiant exposure of narrowband UVB and precise targeting of affected skin, it appears to be a promising treatment for the prurigo form of atopic dermatitis (AD). OBJECTIVES: To investigate the efficacy and safety of the EL compared with clobetasol propionate (CP) in the prurigo form of AD. METHODS: In a prospective randomized within-patient controlled study, 13 patients with a prurigo form of AD were randomized to receive EL on one side and topical CP on the other side. Laser treatment was performed twice a week for 10 weeks. Clinical responses were evaluated using Physician Assessment of Individual Signs, Physician Global Assessment, Patient Global Assessment and photographic documentation. Histopathological changes were evaluated and duration of remission was monitored during a 6-month follow-up period. RESULTS: Both treatments resulted in a significant improvement of all outcome measures after 10 weeks of treatment. During follow up, the EL showed more improvement compared with CP. Histopathology demonstrated marked decrease of epidermal thickness and inflammatory infiltrate at the EL-treated sites. No significant side-effects occurred. CONCLUSIONS: This study suggests that the EL can safely and effectively be used in the treatment of the prurigo form of AD. For the long term, the EL might be a good alternative to topical corticosteroids and an option in case of therapy-resistant patients.

Therapeutic hotline: Treatment of prurigo nodularis and lichen simplex chronicus with gabapentin.

Psychocutaneous conditions are frequently encountered in dermatology practice. Prurigo nodularis and lichen simplex chronicus are two frustrating conditions that are classified in this category. They are often refractory to classical treatment with topical corticosteroids and antihistamines. Severe, generalized exacerbations require systemic therapy. Phototherapy, erythromycine, retinoids, cyclosporine, azathiopurine, naltrexone, and psychopharmacologic agents (pimozide, selective serotonin reuptake inhibitor antidepressants) were tried with some success. Here five cases with lichen simplex chronicus and four cases with prurigo nodularis, who responded well to gabapentin, are presented.

UVB de banda angosta para el prurigo nodular de difícil tratamiento / Narrow-band ultraviolet B in recalcitrant nodular prurigo

Introducción. La luz UVB de banda angosta (nb-UVB) ha surgido como nueva propuesta para el tratamiento del prurigo nodular. Objetivo: Describir nuestra experiencia en el uso de nb-UVB para el prurigo nodular de difícil manejo. Método: Análisis retrospectivo de cinco pacientes con prurigo nodular tratados con luz nb-UVB asociado a terapia estándar. Resultados: Se obtuvo respuesta favorable en los cinco pacientes, utilizando 23,4 mas 5,6 sesiones con una dosis acumulada de 44,5 J/cm2. El promedio de remisión fue de 4,2 mas 1,6 meses. Conclusión: La terapia nb-UVB parece constituir una alternativa eficaz para el tratamiento del prurigo nodular.

Introduction. Narrow band ultraviolet B (nb-UVB) has emerged as a new proposal for treatment of nodular prurigo. Objective. To describe our experience with nb-UVB in recalcitrant nodular prurigo. Method. Retrospective analysis of five patients treated with nb-UVB associated to standard therapy Results: We obtained a favorable response in all patients with 23.4 mas: 56 sessions, using a mean accumulative dose of 44.5 J/cm2. The mean duration of remission was 42 mas: 1.6 months. Conclusion. NS-UVB phototherapy appears to be an effective treatment for recalcitrant nodular prurigo.

Narrow-band ultraviolet B phototherapy in patients with recalcitrant nodular prurigo.

Management of nodular prurigo has been less than satisfactory. Conventional therapies such as systemic antihistamines and topical steroids have not been particularly successful. The effects of narrow-band ultraviolet B (NB-UVB) phototherapy in the treatment of various inflammatory dermatoses have been proven, however, no data exist on the efficacy and the duration of remission in NB-UVB monotherapy for nodular prurigo. The aim of this study was to evaluate the effect of NB-UVB phototherapy on recalcitrant nodular prurigo. NB-UVB phototherapy was performed once a week on 10 patients with recalcitrant nodular prurigo. The initial dose was 0.4 J/cm(2), and the dose was increased by 0.1 J/cm(2) for each treatment. The treatment was performed until the eruption was almost clear. In each patient, a mean cumulative dose of 23.88 J/cm(2) was applied over a mean of 24.3 irradiations. The mean maximum daily dose of ultraviolet B was 1.2 +/- 0.4 J/cm(2). NB-UVB phototherapy notably improved the eruption of nodular prurigo in all patients. Follow up at 1 year revealed that only one patient had relapsed. The remaining nine patients continued to derive long-term benefits. NB-UVB phototherapy appears to be an effective treatment for recalcitrant nodular prurigo, offering long-term benefits in the majority of those treated.

A randomised controlled trial on photo(chemo)therapy of subacute prurigo.

BACKGROUND: Psoralen ultraviolet A (PUVA) is the standard photo(chemo)therapeutic regimen for patients suffering from subacute prurigo (SP). HYPOTHESIS: Regarding efficacy, bath PUVA is not superior to medium-dose ultraviolet-A1 (MD-UVA1) and narrowband ultraviolet-B (NB-UVB), which may be considered the new photo(chemo)therapeutic options for SP. METHODS: We performed a prospective randomised, controlled, three-arm photo(chemo)therapeutic study. Patients suffering from histopathologically proven SP with a clinical score (PIP score; papules, infiltration and pruritus) of at least 5 points were enrolled into the study. Treatment with bath PUVA was performed 4 times weekly and MD-UVA1 and NB-UVB 5 times weekly. Photo(chemo)therapy was administered over a 4-week period. Outcome measure was the severity of SP investigated by means of the PIP score after 4 weeks of therapy. RESULTS: In total, 33 patients with SP were randomly allocated to photo(chemo)therapy. Bath PUVA (n = 9), MD-UVA1 (n = 11) and NB-UVB (n = 13) resulted in a significant reduction of the baseline PIP score as assessed on the basis of intention-to-treat (ITT) analysis (P = 0.003). However, ITT analysis revealed significantly higher PIP score reduction in patients who were treated with bath PUVA and MD-UVA1 compared with NB-UVB (P < 0.01, 95% CI 1.1-3.63 and P < 0.05, 95% CI 0.42-2.70, respectively). CONCLUSIONS: Photo(chemo)therapy, including bath PUVA, MD-UVA1 and NB-UVB, appears to be an effective and safe treatment option for patients suffering from SP. UVA1 and particularly PUVA seem superior to NB-UVB in the management of SP.

Prurigo: diagnosis and management.

Prurigo is a condition of nodular cutaneous lesions that itch (pruire) intensely. Although the acute form can be caused by insect stings, most of the subacute and chronic forms appear to be idiopathic. Toxic agents deposited in the skin by exogenous factors such as parasites, bacteria, or topically or orally administered drugs can induce itch. In susceptible individuals, physical mechanisms such as UV light can induce changes in epidermal innervation that result both in itch generally and in prurigo lesions. Prurigo is sometimes associated with atopy, pregnancy, internal diseases, malabsorption, or malignancy. Some forms of prurigo may be secondary to scratching. Emotional factors can also influence the perception of itch and induce prurigo by provoking scratching. These are the various specialized forms of prurigo, and there are certain others, such as prurigo pigmentosa, that have some ethnic preference. Topical treatments by corticosteroids, coal tar, bath photochemotherapy, UVB, cryotherapy, or capsaicin, as well as systemic regimens involving use of psoralen + UVA (PUVA), erythromycin, arotinoid acid, cyclosporine, chloroquine, dapsone, minocycline, naltrexone, azathioprine or thalidomide are used for the treatment of this condition. Psychotherapeutic agents to treat problems of mood that deteriorate prurigo are also prescribed. Combined sequential treatments for generalized, therapy-resistant cases need to be tailored to the exacerbations that occur and to provide maintenance treatment in order to enable the patient to withstand the intolerable itch.

Treatment of prurigo nodularis with topical capsaicin.

BACKGROUND: Prurigo nodularis is an eruption of lichenified or excoriated nodules caused by intractable pruritus that is difficult to treat. Therefore the antipruritic efficacy of capsaicin seemed to be of particular interest because this alkaloid, extractable from red pepper, interferes with the perception of pruritus and pain by depletion of neuropeptides in small sensory cutaneous nerves. OBJECTIVE: The aim of this concentration- and regimen-ranging study was to evaluate the efficacy, safety, and practicability of capsaicin in the topical treatment of prurigo nodularis in a large series of patients. METHODS: A total of 33 patients with prurigo nodularis of various origins were selected to receive capsaicin (0.025% to 0.3%) 4 to 6 times daily for 2 weeks up to 10 months. The consecutive follow-up period was up to 6 months. In 7 patients, skin biopsy specimens were taken before, during, and after therapy and investigated histologically, immunohistochemically, and ultrastructurally. RESULTS: All 33 patients could be evaluated for efficacy. After cessation of the symptoms of neurogenic inflammation, such as burning sensations or erythema, all of them experienced a complete elimination of pruritus within 12 days. In addition, capsaicin largely contributed to the gradual healing of the skin lesions. After discontinuation of the therapy, pruritus returned in 16 of 33 patients within 2 months. At the ultrastructural level, no degenerative changes of cutaneous nerves could be found during or after capsaicin therapy. Depletion of substance P was demonstrated by confocal laser scanning microscopy thus confirming the specific effect of capsaicin in vivo. CONCLUSION: Topical treatment of prurigo nodularis with capsaicin proved to be an effective and safe regimen resulting in clearing of the skin lesions.

Use of topical cyclosporin for conjunctival manifestations of actinic prurigo.

PURPOSE: To report the successful treatment of conjunctival manifestations of actinic prurigo with topical cyclosporin. METHODS: A 12-year-old female with extensive limbal lesions and chronic conjunctivitis secondary to actinic prurigo was treated with topical cyclosporin, and a rapid clinical response was observed. RESULTS: The patient had the limbal lesions surgically excised. The lesions completely recurred over the first 2 postoperative months. Treatment with topical steroids and vitamin A ointment was unsuccessful. The patient was then treated with cyclosporine 2% drops and a dramatic improvement occurred. Over a 3-month period, the lesions completely resolved. CONCLUSION: Cyclosporine eyedrops may be an effective treatment for the conjunctival manifestations of actinic prurigo.

[Ofuji's papulo-erythroderma: treatment with cyclosporin A]. / Papulo-érythrodermie d'Ofuji: traitement par ciclosporine-A.

BACKGROUND: Papuloerythroderma of Ofuji is an uncommon condition characterized by diffuse pruriginous eruptions of infiltrating papulae. The large folds are not involved. The eruptions are associated with lymphopenia and eosinophilia. Very few cases have been reported in the literature and because of their heterogeneous nature, there is some debate as to whether this is a single clinical entity or a particular presentation of erythrodermia; Immunomodulation is recommended. CASE REPORT: A 73 year-old man of asian origin living in France presented chronic pruriginous erythrodermia with flat purplish-brown confluent papulae which did not involve the large folds. Eosophilia and lymphopenia were present. The biopsy specimen evidenced dermal infiltration with CD4+CD45+RO+ T cells, eosinophils and DC1a+ dendritic cells. Further explorations did not reveal any sign favoring lymphoma, carcinoma or underlying infection. Dermocorticoids, PUVA-therapy and interferon-alpha were ineffective. Considerable clinical improvement was obtained with cyclosporine A. DISCUSSION: We used ciclosporine A in our patient after repeated failure of other therapies reported to be effective in this dermatosis. Despite the favorable outcome, this therapeutic approach should be used with prudence.

A case of prurigo pigmentosa considered to be contact allergy to chromium in an acupuncture needle.

A 53-year-old male developed prurigo pigmentosa on his back, after undergoing acupuncture for 3 years. The eruptions were ceased on discontinuing the therapy but recurred with its resumption. The acupuncture needle contained 18.12% chromium. Erythema was induced by patch testing with potassium dichromate, and a flare-up was observed in the area of the patch test on resumption of acupuncture. We consider that the eruptions were induced by contact allergy to the chromium component of the acupuncture needles.

The evolution of perforating folliculitis in patients with chronic renal failure.

The evolution of perforating folliculitis in six patients with chronic renal failure was investigated with special attention to clinical and histopathologic changes in early, evolving, and mature lesions. Different and distinct histologic features at each stage were found. The earliest lesions, follicular pustules, evolved into perforating folliculitis that eventuated in prurigo nodularis. A combined treatment consisting of an anti-staphylococcal antibiotic by mouth, phototherapy, and application of a topical corticosteroid lotion proved helpful in controlling the generalized pruritus and the evolution of the lesions in these cases.

Prurigo nodularis associated with malabsorption.

The case of a 72-year-old female with a syndrome of malabsorption associated with prurigo nodularis is reported. The patient had been suffering from these disorders for 16 years. The pathological alteration of her malabsorption syndrome was an idiopathic sprue. 5 months after treatment with gluten-free diet supplemented with vitamins and iron, the disappearance of the clinical and analytical alterations was complete.