Actinic prurigo, tropical (South-East Asian) variant.

Actinic prurigo is a chronic familial photodermatitis found predominantly among the Amerindians. It has been reported from North and South America, Britain and Japan. We report a case of actinic prurigo seen in Singapore. A 20-year-old Malay female presented with a persistent pruriginous eruption in the sun-exposed parts and on her abdomen. She also had lower lip cheilitis and thinning of the outer eyebrows, features often seen in actinic prurigo. The minimal erythema dose to ultraviolet A (UVA) and UVB were persistently lowered. We propose that this condition be called actinic prurigo, tropical (South-East Asian) variant.     

A case of actinic prurigo showing hypersensitivity of skin fibroblasts to ultraviolet A (UVA)

We here report a patient with actinic prurigo. He had had erythematous papulovesicular eruptions on the sun-exposed sites from fall to early summer for 4 years. The lesions healed leaving atrophic scars. The histology showed epidermal necrosis and dermal dense perivascular lymphohistiocytic infiltration and edema. His minimal erythema doses to ultraviolet B (UVB) and UVA were normal and lowered, respectively. Skin lesions were produced by repeated irradiation with UVA plus UVB, but not with UVA alone. Then he was diagnosed as having actinic prurigo. Skin fibroblasts from the patient were hypersensitive to UVA. We believe that the hypersensitivity relates to the pathomechanisms of the photosensitivity in the case. UVA sensitivity of fibroblasts may be useful for differentiating actinic prurigo, hydroa vacciniforme, and other similar photosensitive disorders.     

Terapia UVB de banda estrecha en prurigo nodular

—Introduction. Narrow-band UVB therapy, considered a first-choice treatment in psoriasis, has also been proposed as an effective alternative in other inflammatory dermatoses. The clinical experience obtained in a group of patients affected with prurigo nodularis treated with narrow-band UVB therapy is presented.Material and methodAn assessment was made of the clinical and symptomatic response, with no control group, of a series of 15 patients affected with prurigo nodularis who received a total of 22 cycles of narrow-band UVB therapy administered in a full-body cabinet.ResultsAfter an average number of 32 sessions and an average cumulative dose of 26.2 J/cm2, there was evidence of clinical and symptomatic improvement greater than 50 % in 73% of the therapeutic cycles completed (16/22), with 23% (5/22) considered to be in full remission.Discussion and conclusionsThe data provided suggests that narrow-band UVB therapy may be a well tolerated, moderately effective therapeutic alternative in prurigo nodularis.     

Treatment of actinic prurigo with PUVA: mechanism of action

Five patients with actinic prurigo were treated twice weekly with PUVA. One area on the back was shielded from UVA throughout the 15-week treatment period. Before PUVA, all patients had increased erythemal sensitivity to UVA and showed abnormal augmentation of UVB erythema by topical indomethacin. After PUVA, all patients were free of photosensitive symptoms and skin that had been exposed to UVA showed normal erythemal responses. By contrast, the areas of skin that had been protected from UVA showed erythemal responses that were unchanged from pre-PUVA values. Augmentation of UVB erythema by topical indomethacin persisted, both on UVA exposed and UVA protected skin. These results show that, although PUVA is an effective treatment in actinic prurigo, it does not alter the underlying mechanism of photosensitivity. The protective effect is local and is due presumably to an increase in melanin pigmentation and epidermal thickness.     

Aktinische Prurigo

The non-Native American type of actinic prurigo belongs to the group of rare idiopathic photodermatoses and therefore is often diagnosed with delay. The typical clinical and epidemiological features of actinic prurigo are described in a 10 year old girl. Detailed phototesting showed urticarial early onset and prurigo-like late onset reactions towards long-wave UVA. Repetitive photoprovocation with UVB induced delayed development of papules. HLA typing showed the typical association with HLA-DR4, in particular DRB1*0407. Treatment is usually extremely difficult and unrewarding. In this patient, the course was considerably improved by more intense physical photoprotection.     

Prurigo nodularis: a review

Prurigo nodularis is a chronic condition characterized by a papulonodular pruriginous eruption of unknown aetiology. This condition is a difficult disease to treat and causes frustration to both the patient and the treating doctor. A variety of systemic conditions have been reported to be associated with prurigo nodularis. The mechanism by which these disorders may trigger prurigo nodularis is unknown. Nerve growth factor has been implicated in the pathogenesis of prurigo nodularis. Calcitonin gene-related peptide and substance P immunoreactive nerves are markedly increased in prurigo nodularis when compared with normal skin. These neuropeptides may mediate the cutaneous neurogenic inflammation and pruritus in prurigo nodularis. Topical or intralesional glucocorticoids are the treatment of choice. Other topical treatments such as topical vitamin D3, and topical capsaicin have also been reported to be effective. Oral treatments such as cyclosporin and thalidomide have been shown to improve both appearance of the skin and pruritus. We review the clinical features, associations, pathology, pathogenesis and treatment of prurigo nodularis.     

UVB-308-nm- (NUVB-)Therapie mittels Excimer-Laser bei atopischer Dermatitis und weiteren inflammatorischen Dermatosen

UVB 311 nm phototherapy (NUVB) is used successfully in the treatment of psoriasis or atopic dermatitis and is often choosen as “first-line” therapy. In a prospective study we investigated the efficacy of the targeted UVB 308 phototherapy by excimer laser in the treatment of 57 patients with localized, mild therapy-resistant atopic dermatitis. We achieved a complete remission (CR) in nearly 85% and a partial remission (PR) in 15% after 10 treatments during 6–8 weeks in all patients. A mean reduction in patient-reported pruritus was one hallmark after 3–4 treatments. 48 other patients with lichen simplex chronicus Vidal, nodular prurigo, granuloma anulare, alopezia areata (AA) and lichen ruber planus were also treated by targeted NUVB. We found higher rates of CR and PR only in patients with AA and in patients with oral Lichen ruber mucosae. These results and the systematically reviewing of the current medical literature shows that targeted NUVB by excimer laser cannot be successfully transfered for all inflammatory skin diseases. In particular, Excimer UVB treatment expands the therapeutic options in patients with localized and therapy-resistant atopic dermatitis enormously.     

沙利度胺联合糠酸莫米松乳膏封包及紫外线照射治疗结节性痒疹的疗效观察

目的 观察沙利度胺联合糠酸莫米松乳膏封包和紫外线照射治疗结节性痒疹的疗效。 方法 采用非随机同期对照研究方法,结节性痒疹患者80例,分别入选对照组（23例）、UVA1组（32例）、UVB组（25例）,3组患者均口服沙利度胺75 mg/d每晚1次,0.1%糠酸莫米松乳膏每晚1次均匀涂抹于整个患侧肢体或躯干部位并用保鲜膜封包;UVA1组和UVB组同时分别给予UVA1和窄谱UVB照射。在治疗前、治疗后30天分别对患者病情严重程度评分。应用秩和检验比较组间皮损临床疗效与瘙痒疗效,同时对外周血嗜酸粒细胞绝对计数与瘙痒视觉评分进行关联性分析。结果 ①治疗30 d后,皮损改善的临床疗效：对照组、UVA1组、UVB组显效分别为5例（21.74%）、13例（43.33%）、9例（37.5%）,有效分别为7例（30.43%）、12例（40%）、7例（29.17%）,对照组疗效显著低于UVA1组（Z = 8.21,P ＜ 0.01）和UVB组（Z = 5.22,P ＜ 0.01）,UVA1组和UVB组疗效接近（Z = 0.50,P ＞ 0.05）;②瘙痒改善的临床疗效：治疗30 d后,对照组、UVA1组、UVB组显效分别为7例（30.43%）、18例（60.00%）、14例（58.33%）,对照组疗效低于UVA1组（Z = 4.50,P ＜ 0.01）和UVB组（Z = 4.50,P ＜ 0.01）,UVA1组与UVB组疗效接近（Z = 0.35,P ＞ 0.05）;③对患者的嗜酸粒细胞计数与瘙痒评分进行相关分析,r = 0.53,P ＜ 0.01。结论 沙利度胺联合糠酸莫米松乳膏封包,及联合紫外线照射治疗结节性痒疹均有显著疗效,且联合UVA1、UVB的疗效优于沙利度胺联合糠酸莫米松乳膏封包。     

Photosensitivity disorders: cause,effect and management

Abnormal photosensitivity syndromes form a significant and common group of skin diseases. They include primary (idiopathic) photodermatoses such as polymorphic light eruption (PLE), chronic actinic dermatitis (CAD), actinic prurigo, hydroa vacciniforme and solar urticaria, in addition to drug- and chemical-induced photosensitivity and photo-exacerbated dermatoses. They can be extremely disabling and difficult to diagnose. PLE, characterized by a recurrent pruritic papulo-vesicular eruption of affected skin within hours of sun exposure, is best managed by restriction of ultraviolet radiation (UVR) exposure and the use of high sun protection factor (SPF) sunscreens. If these measures are insufficient, prophylactic phototherapy with PUVA, broadband UVB or narrowband UVB (TL-01) for several weeks during spring may be necessary. CAD manifests as a dermatitis of chronically sun-exposed skin. Again, UVR exposure needs to be restricted; cyclosporine, azathioprine or PUVA may also be necessary. Actinic prurigo is characterized by the presence of excoriated papules and nodules on the face and limbs, most prominent and numerous distally. Actinic prurigo is managed again by restriction of UVR and the use of high SPF sunscreens; PUVA or broadband UVB therapy, or low doses of thalidomide may be necessary. Hydroa vacciniforme causes crops of discrete erythematous macules, 2 to 3mm in size, that evolve into blisters within a couple of days of sun exposure. Treatment for this rare disease is difficult; absorbent sunscreens and restricted UVR exposure may help. Solar urticaria is characterized by acute erythema and urticarial wealing after exposure to UVR. Treatment options for solar urticaria include non-sedating antihistamines such as fexofenadine and cetirizine; other options include absorbent sunscreens, restriction of UVR at the relevant wavelength, maintenance of a non-responsive state with natural or artificial light exposure and plasmapheresis. Industrial, cosmetic and therapeutic agents can induce exogenous drug- or chemical-induced photosensitivity. The clinical pattern is highly varied, depending on the agent; treatment is based on removal of the photosensitizer along with restriction of UVR exposure. Predominantly non-photosensitive dermatoses may also be exacerbated or precipitated by UVR; exposure to UVR should be reduced and sunscreens should be advocated, along with appropriate treatment of the underlying disease.     

Adult-onset actinic prurigo in Thailand

BACKGROUND: Actinic prurigo (AP) is one of the rare idiopathic photodermatosis. It is said to be a familial disease and is usually seen in certain specific geographical areas. The adult-onset type of AP is reported less frequently in the Asian population and has never been reported in Thailand. METHODS: The study population comprised 30 patients. Demographic data were collected. Photo-tests and photo provocation tests for UVA, UVB and visible light were carried out on non-exposed skin. The other investigations included antinuclear antibody, anti-HIV antibody and urine porphyrin level. Histopathology studies were also carried out. RESULTS: There were 18 males and 12 females. The mean age of onset was 36.86 years. The duration of disease was from 1 month to 20 years. Forearms (27 patients) were the most frequently affected site. Other screening tests showed negative results. Five patients had abnormal MED to UVA and one patient had abnormal MED to UVA as well as UVB. Photo provocation tests showed positive responses to both UVA and UVB in 12 cases (40%), a positive response to UVA in 11 cases (37%), a positive response to UVB alone in four cases (13.3%) and a normal response in three patients (10%). None of the patients had a positive response to visible light. Skin biopsies were performed on nodular lesions in 23 cases. Histopathology from these 23 cases showed hyperkeratosis ortho- or parakeratosis and acanthosis in 20 of the 23 cases. CONCLUSIONS: Adult-onset AP in our country may have different geographic and racial distribution from previous reports or may be the tropical variant as described by Tham et al. It may not be an uncommon disease in our country, if there is increased awareness of this disease. Only 16.6% of patients had reduced MED. Photo provocation tests were positive in 90% of cases. Most of the positive wavelengths were UVA or both UVA and UVB. Therefore, photo provocation tests should be performed in cases suspected of AP. The prognosis for AP is not good, despite combinations of treatment. The disorder may run a chronic course. This may be because of our sunny climate and the sun-exposed occupations of patients.     

Nodular prurigo associated with Hodgkin's disease

We report a case of prurigo nodularis of Hyde as the presenting sign of Hodgkin's disease in a 22-year-old girl. Though pruritus is a common symptom of Hodgkin's disease, prurigo nodularis has rarely been reported as a Hodgkin's symptom.     

Prurigo

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.     

Vesiculous prurigo pigmentosa in a 13-year-old girl: good response to isotretinoin

Prurigo pigmentosa is a rare inflammatory disease of unknown origin, first reported from Japan, with only 33 cases described in non-Japanese patients. We describe a 13-year-old girl with a pruriginous symmetrical eruption of papules and vesicles affecting her back, neck and chest of 1 month duration. She remembered a similar, but lighter eruption, 2 months before. As the initial diagnosis was of a vesiculobullous form of Darier disease, treatment with isotretinoin 40 mg/day was started with good response. Histological study showed a superficial perivascular and interstitial dermatitis composed predominantly of lymphocytes. The epidermis was spongiotic, with exocytosis of lymphocytes and some neutrophils and necrotic keratinocytes. All these findings were consistent with prurigo pigmentosa. The lesions resolved leaving a light brown reticulate hyperpigmentation. Prurigo pigmentosa has never been reported in prepubescent patients, the vesiculobullous forms are unusual, and the only treatments used previously are sulphonamides, tetracyclines and macrolides. We report a 13-year-old Caucasian girl with vesiculobullous prurigo pigmentosa successfully treated with isotretinoin.     

Diagnosis and treatment of actinic prurigo

Actinic prurigo (AP) is an idiopathic photodermatosis that affects mainly the mestizo population in Latin America. It has an early onset, a slight predominance in women, and affects the sun-exposed areas of the skin, causing erythematous papules and lichenified plaques secondary to intense and chronic pruritus. Lesions can be induced by both ultraviolet A (UVA) and ultraviolet B (UVB). An association with several human leukocyte antigen (HLA) alleles has been reported. AP is unique among all photodermatoses in its remarkable response to thalidomide. In the past the microscopic features of AP have been considered as nonspecific; however, the constant finding of dense lymphocytic inflammatory infiltrates and the immunogenetic features of AP support the existence of an immunologic mechanism in its pathogenesis.     

Papular dermatitis (subacute prurigo, “itchy red bump” disease): Pilot study of phototherapy

Background: Some patients with a subacute or chronic pruritic erythematous papular eruption are refractory to treatment. We previously described a number of these patients with papular dermatitis or subacute prurigo. Objective: The purpose of this study was to examine the effectiveness of different types of phototherapy for treatment of papular dermatitis. Methods: We reviewed the medical records of 11 patients who were diagnosed with papular dermatitis and who underwent phototherapy within the last 5 years. Results: Eleven patients had a total of 17 phototherapy courses: psoralen–UVA (PUVA; 9), UVA/UVB light (3), and UVB alone (5). Within the PUVA treatment group, three of nine patients experienced total clearing, and six of nine patients experienced partial improvement. Although patients in all groups relapsed with time, overall the PUVA-treated patients had the best response rate and the best chance of the condition remaining clear after treatment was stopped. Conclusion: PUVA may be an effective treatment for papular dermatitis. The frequency of relapse indicates that maintenance treatments may be necessary for long-term control of the disease. (J Am Acad Dermatol 1998;38:929-33.)     

Narrow-band UVB (TL-01) phototherapy: an effective preventative treatment for the photodermatoses

Summary Twenty patients with photodermatoses [actinic prurigo n=6), hydroa vacciniforme (n=4). idiopathic solar urticaria (n=1), amiodarone-induced photosensitivity (n=1) and a range of cutaneous porphyrias (n=8)] were treated with a ‘hardening’ course of narrow-band ultraviolet B (TL-01) phototherapy in springtime. The response to phototherapy was monitored subjectively, by interviewing patients after the summer, and objectively by monochromator phototesting, before and after phototherapy. Fifteen patients reported that treatment was worthwhile. Monochromator phototesting after phototherapy revealed a fourfold increase in the minimal erythema dose in those with abnormal photosensitivity to ultraviolet A wavebands. Adverse effects included erythema (seven patients), pruritus (five) and provocation of the eruption (four). We now routinely consider narrow-band UVB phototherapy for problem photodermatoses.     

UVB 308‐nm excimer light and bath PUVA: combination therapy is very effective in the treatment of prurigo nodularis

Background Prurigo nodularis (PN) is a chronic inflammatory skin disease with nodular itching lesions. UV therapy – both PUVA and NUVB – are known to clear up PN temporarily due to the antipruritic effect of UV light. However, relapse after treatment is common in PN, which means that either long‐term therapy is necessary or the treatment protocols have to be optimized to minimize side‐effects. Objective The aim of this study was to evaluate the effect that combining bath PUVA and targeted UVB 308 nm excimer radiation has on recalcitrant nodular prurigo. Methods In a prospective trial, 22 patients with PN were treated with either PUVA alone or with a combination of PUVA and excimer UVB. The end point was complete or almost complete remission of PN. Results Adding a 308‐nm excimer UVB to the treatment of the pruritic nodules sped up the healing process; 30% less PUVA radiation was needed. Conclusion The combination of PUVA and excimer UVB in PN appears to be very efficacious. Reducing psoralen UVA doses by 30% offered long‐term benefits in phototherapy of chronic recalcitrant diseases like PN.     

结节性类天疱疮1例附文献复习

结节性类天疱疮是大疱性类天疱疮的一个特殊亚型,临床罕见.其特征为出现结节性痒疹样皮损,可有水疱病史,免疫病理符合大疱性类天疱疮的改变.作者近期遇诊1例,现作报道如下,并复习文献报道,综述该病的诱因、发病机制、临床表现、诊断及治疗等研究进展,以供临床参考.     

Grouping prurigo: report of cases

Grouping prurigo was reported as a new clinical entity by Ofuji et al. in 1977 and 1988. No further cases have been reported since. Herein we report four cases of grouping prurigo and discuss the possible relationship between this condition and other diseases in the prurigo group, including chronic pruritic papular dermatitis in adult men, which was reported by Chang et al. in 1999. Since grouping prurigo and chronic pruritic papular dermatitis in adult men share approximately the same characteristics in both clinical and histopathological findings, we concluded that these two disorders may be the same entity, characterized by grouped pruritic solid papules on the trunk.     

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.