A Case of Eczematid‐Like Purpura of Doucas and Kapetanakis in a Child

Eczematid‐like purpura of Doucas and Kapetanakis is a subtype of the pigmented purpuric dermatoses, a group of uncommon dermatoses of unclear etiology characterized by purpura, petechiae, and hyperpigmentation. The Doucas and Kapetanakis subtype is rare in children, and its subtle findings can initially be overlooked and mistaken for other, more common dermatologic disorders in this age group. We present a case eczematid‐like purpura of Doucas and Kapetanakis in an 11‐year‐old boy initially treated as eczema.     

Nerve alterations showing autophagy in 2 patients with lichen aureus

Lichen aureus is a rare, chronic, persistent purpuric dermatosis clinically characterized by striking yellow‐ to bronze‐colored lesions. Histologically, lichen aureus differs from other pigmented purpuric dermatoses in containing dense, band‐like infiltrates closely associated with the epidermis. This report describes 2 patients with lichen aureus, a 20‐year‐old woman with a lesion on her right arm and a 51‐year‐old man with a lesion on the right side of his groin. Skin biopsy specimens revealed almost identical findings in both patients, including dense band‐like infiltrates containing lymphocytes, histiocytes with hemosiderin deposits scattered extravasated red blood cells and nerve alterations at the dermo‐epidermal interface. The nerves within the lesions were filled with granules, which stained positive with antibody to microtubule‐associated protein 1A/1B‐light chain 3, suggesting autophagy within the nerves. These altered nerves were present only in areas of band‐like dermal lymphocytic infiltration. Electron microscopy of the lesions showed the accumulation of autophagosomes in Schwann cells.     

窄谱中波紫外线照射治疗色素性紫癜性皮病疗效观察

目的观察窄谱中波紫外线(NB-UVB)治疗色素性紫癜性皮病的临床疗效。方法将入选患者随机分为两组,治疗组11例,用NB-UVB治疗仪治疗,初始剂量0.30J/cm2,每次递增0.06～0.10J/cm2,最大剂量2.00J/cm2,隔日1次,共30次,随访3个月。对照组10例,口服复方芦丁2片,3次/d;维生素E丸100mg,3次/d;外用糠酸莫米松软膏1次/d,连续治疗2个月。结果治疗组有效率90.91%,对照组为40.00%,两组比较差异有统计学意义(P<0.05)。结论 NB-UVB治疗色素性紫癜性皮病疗效好,不良反应少。     

Phototherapy in Childhood

Abstract: Background: Little data exist about the efficacy of phototherapy in childhood dermatoses. Objective: To report our experience with pediatric patients treated with phototherapy. Methods: The study included children ≤17 years of age, who were treated in our phototherapy unit between 1985 and 2005. Data were retrospectively collected. Results: The study included 113 patients (50 boys, 63 girls) with a median of age of 13 years (range: 3–17 yrs). Narrow‐band ultraviolet‐B (34.6%), and ultraviolet‐B (33.1%) were the most common treatments administered to the patients. Indications for phototherapy were psoriasis in 53.5%, vitiligo in 20.5%, pityriasis lichenoides in 14.2%, alopecia areata in 7.9%. Response was achieved in 92.9% of the psoriasis patients treated with narrow‐band ultraviolet‐B, in 83.3% treated with psoralen plus ultraviolet‐A, and in 93.3% with ultraviolet‐B. All the pityriasis lichenoides chronica patients who received narrow‐band ultraviolet‐B responded to the treatment and seven of nine treated with ultraviolet‐B had a response. Response was achieved in 57% of the vitiligo patients treated with psoralen plus ultraviolet‐A and in 50% of vitiligo patients treated with narrow‐band ultraviolet‐B. Of the 10 alopecia areata patients, two responded to psoralen plus ultraviolet‐A. Among all the patients, erythema was the most common adverse effect. Conclusion: Phototherapy is a well‐tolerated treatment for childhood dermatoses, and is especially efficacious in psoriasis and pityriasis lichenoides chronica patients.     

Localized pagetoid reticulosis (Woringer–Kolopp disease) completely response to NB‐UVB combined with IFN

A cutaneous lymphoproliferative disorder, localized pagetoid reticulosis (LPR), is rare but distinctive in its clinical and histopathological manifestations. Without an evidence‐based medicine treatment guideline because of extremely low incidence, LPR poses a therapeutic challenge. We describe a 67‐year‐old Chinese woman with LRP who showed complete response to narrow‐band ultraviolet B combined with intramuscular interferon alpha‐2b.     

A case of generalized lichen nitidus successfully treated with narrow‐band ultraviolet B treatment

Lichen nitidus (LN) is a rare skin disorder presenting with multiple, small and bright papules located on the chest, abdomen, penis glans and upper extremities. It usually presents with limited involvement; however, it can present as generalized involvement. There is no consensus on treatment. Corticosteroid, astemizole, phototherapy has been used; however, the results are controversial. A 15‐year‐old male with clinical and histopathological diagnosis of LN was treated with narrow‐band ultraviolet B (NB‐UVB). The lesions completely regressed with post‐inflammatory hypopigmentation on the second month of the therapy (25 sessions). We believe that NB‐UVB is an effective treatment on generalized LN.     

Successful treatment with narrow‐band UVB therapy for a case of generalized Hailey–Hailey disease with a novel splice‐site mutation in ATP2C1 gene

Hailey–Hailey disease (HHD) is a rare autosomal dominant disorder characterized by development of recurrent blisters, erosions, and crustations in the intertriginous areas. The treatment of HHD is often challenging, and various methods have been tried. We report here a case of a 45‐year‐old woman with a generalized form of HHD that was dramatically improved and well controlled by narrow‐band ultraviolet B phototherapy.     

Treatment of pigmented purpuric dermatoses with Narrow‐band UVB: a report of six cases

Background Pigmented purpuric dermatoses (PPD) are a group of chronic disorders that pose a therapeutic challenge. Objective In this study, we evaluated the effectiveness of narrow band ultraviolet B (NB UVB) in the treatment of PPD. Patients and methods Six patients of PPD were treated with NB UVB: one patient had Majocchi's purpura and five had Schamberg's disease. NB UVB was given on three weekly basis till clearance, then maintenance treatments were scheduled as twice weekly for 3 weeks and once weekly for another 3 weeks. The patients were followed up to 1 year. Results Successful treatment was achieved in all treatment patients after 24–28 NB UVB treatments and maintenance of nine treatments. Only two patients showed flare of their lesions after stoppage of NB UVB and efficiently controlled with further 14 NB UVB treatments. Conclusion NB UVB is effective in the treatment of PPD and should be considered as a treatment option for PPD.     

Relapsing Henoch-Schönlein purpura in an adult patient associated with hepatitis B virus infection

Henoch‐Schölein purpura is usually a disease of children presenting with arthralgia, abdominal pain, renal involvement, and palpable purpura. Viral and bacterial infections may have a role in its etiology. We present a 32‐year‐old male patient with recurrent Henoch‐Schölein purpura in association with a chronic hepatitis B infection of ten years duration. The patient had received lamuvudine and interferon‐α for the treatment of hepatitis B infection for a year. The skin lesions disappeared with the treatment of the hepatitis B infection. Four months after discontinuation of the therapy, the purpuric papules reappeared with reactivation of the hepatitis B infection. Although rarely reported, hepatitis B virus infection should be considered in patients with Henoch‐Schölein purpura.     

Granulomatous pigmented purpuric dermatosis

The granulomatous variant of the pigmented purpuric dermatoses (PPDs) is a rare and infrequently described condition, with a total of 16 cases published to date. We report a case of granulomatous PPD in a 59‐year‐old white woman who demonstrated involvement of the arms, legs, chest and back with concurrent hyperlipidaemia. Histopathological examination revealed a lymphohistiocytic infiltrate obscuring the dermoepidermal junction, and loose granuloma formation in the superficial dermis, with extravasated erythrocytes. Other conditions within the differential diagnosis such as atypical infection, papular sarcoidosis and generalized granuloma annulare were excluded on clinical and histological grounds. Our patient represents the ninth patient reported to have granulomatous PPD with coexisting hyperlipidaemia, and the fifth patient with granulomatous PPD and a lichenoid infiltrate.     

Successful treatment of generalized childhood Schamberg's disease with narrowband ultraviolet B therapy

Pigmented purpuric dermatoses are a group of dermatoses characterized by petechia and hyperpigmented macules that occur predominantly on the lower extremities. Although it is most commonly seen in adults, this disease can also affect children. Phototherapy has been shown to be effective in some cases in the literature. Here, we present two cases of Schamberg's disease that improved rapidly with narrowband ultraviolet B (UVB) therapy. To our knowledge, they were the first pediatric cases of successful narrowband UVB therapy in this disease.     

Idiopathic guttate hypomelanosis‐like lesions in patients with mycosis fungoides: a new adverse effect of phototherapy

Background Idiopathic guttate hypomelanosis (IGH) is a common pigmentary disorder, the aetiology and pathogenesis of which are largely unknown. The appearance of IGH‐like lesions during phototherapy has been reported previously in only one patient. Objective To describe the clinical and histological features of phototherapy‐induced IGH‐like lesions, their relation to ultraviolet dosimetry and the course of this eruption in patients with mycosis fungoides (MF). Methods The files of all patients with MF who underwent phototherapy in our centre from 1992 to 2008 were searched to identify those in whom IGH‐like lesions appeared during treatment. Results Among 87 patients with early‐stage MF who underwent phototherapy, seven acquired IGH‐like lesions during monotherapy with narrow‐band ultraviolet B (NB‐UVB; four patients) or psoralen and ultraviolet A (PUVA; three patients). All but one had a light complexion. The lesions appeared in areas exposed to ultraviolet light, and not exclusively on the skin previously involved by the disease. The mean number of exposures until appearance of the lesions was 92 for NB‐UVB and 137 for PUVA. Biopsy study showed a decreased number of melanocytes. Phototherapy was discontinued in four patients, of whom three showed a partial or complete disappearance of the IGH‐lesions. The other three patients are still receiving phototherapy, with no change in their IGH‐like lesions. Conclusions Phototherapy may induce an eruption bearing similar clinical and histopathological features to IGH. The eruption is rare, appears to emerge only after prolonged therapy and seems to be reversible upon discontinuation of phototherapy. IGH‐like eruption should be added to the list of side‐effects of phototherapy.     

Generalized Pigmented Purpuric Dermatosis in a Boy Treated with Narrow‐Band‐Ultraviolet B Therapy

Abstract: A 14‐year‐old boy presented with extensive capillaritis. Narrow‐band ultraviolet B was administered on a thrice‐weekly schedule followed by once‐a‐week maintenance. A favorable response to treatment was seen without any side effects.     

Report of eight infants with acute infantile hemorrhagic edema and review of the literature

Acute infantile hemorrhagic edema (AIHE) is a cutaneous leukocytoclastic vasculitis, clinically characterized by the symptom triad of fever, large purpuric skin lesions, and edema. The clinical picture has a violent onset, a short benign course, and spontaneous complete recovery. In this article, we present eight patients who were admitted with rashes on the skin and edema on the eyelids and extremities, and were diagnosed with AIHE according to their clinical and histopathological features (immunohistological study was also performed in three of them). Our purpose was to emphasize that, aside from Henoch‐Schönlein purpura, meningococcemia, septicemia, and purpura fulminans, AIHE benign disorder should also be considered in the differential diagnosis to determine the clinical course and treatment protocol in patients with purpuric rashes.     

Narrow‐band ultraviolet B as a potential alternative treatment for resistant psychogenic excoriation: an open‐label study

Narrow‐band ultraviolet therapy has been used successfully for the treatment of inflammatory skin disorders and generalized pruritus. We have prospectively evaluated seven consecutive patients with resistant psychogenic excoriation (PE) treated with narrow‐band ultraviolet B (NB‐UVB). Approximately 70% of all patients showed improvement in their condition. NB‐UVB therapy was well tolerated, with no serious side effects. We may conclude that, when treating a patient with PE, NB‐UVB in combination with other approaches may provide extra benefit in resistant cases.     

Neue und etablierte Indikationen der UV‐B‐311‐nm‐Phototherapie

Phototherapy with ultraviolet (UV) irradiation of wavelengths between 280 and 320 nm (UV‐B) is a safe and effective treatment for a variety of inflammatory skin diseases. In addition to standard broad band UVB, narrow band phototherapy with fluorescent bulbs emitting near monochromatic UV between 310 – 315 nm has become an important treatment for diseases such as psoriasis, atopic dermatitis or vitiligo. Other diseases respond favorably to narrow band UV‐B phototherapy, the number of potential indications for such phototherapy is continuously growing. The differential effects of narrow band UV‐B phototherapy in comparison to other UV phototherapies, as well as new and established indications for this treatment modality are reviewed.     

Progressive macular hypomelanosis, excellent response with narrow-band ultraviolet B phototherapy

Progressive macular hypomelanosis (PMH) is an acquired disorder of skin pigmentation, which is mostly under‐diagnosed. It is characterized by nummular hypopigmented lesions appearing on the trunk in young persons. Several treatment options are available, although topical clindamycin and benzoyl peroxide have been used traditionally. However, good results have recently been achieved using narrow‐band ultraviolet B (NBUVB) phototherapy. We present the case of a 13‐year‐old girl with hypopigmented lesions on the trunk and limbs that had progressed over 1 year and that were diagnosed as PMH. The patient was initially treated with topical clindamycin and benzoyl peroxide. However, little improvement was seen and treatment was then started with NBUVB phototherapy. After 25 sessions, with a total cumulative dose of 18 J/cm², the patient showed almost total repigmentation of the lesions. The treatment of PMH is often difficult, and very little is currently known about the treatment response in this disorder, as most reports have very small series of patients with a short disease progression time. NBUVB phototherapy has been shown to be effective, as seen in our patient, although in many cases, there is recurrence after the cessation of treatment.     

Pediatric CD8+/CD56+ mycosis fungoides with cytotoxic marker expression: A variant with indolent course

Mycosis fungoides is predominantly a disease of older patients, but occasionally occurs in children. We report a rare case of CD8+/CD56+ mycosis fungoides with cytotoxic marker (perforin, TIA‐1, and granzyme B) expression in a 10‐year‐old boy. Disease presented with three asymptomatic, slowly progressive erythematous and scaling plaques, surrounded by hypochromic alone in the left tight and lower trunk. UVB narrow band associated with topical corticosteroids resulted in complete remission in about 2 months, and no recurrence at 2‐year follow‐up. Three similar cases have been retrieved in children through PubMed search, showing similar clinical presentation with erythematous scaling lesions, good response to skin‐directed treatments and a favorable prognosis.     

Pediatric Mycosis Fungoides in Singapore: A Series of 46 Children

Few studies have evaluated Asian children with mycosis fungoides (MF). We report a series of patients from a tertiary dermatologic institution in Singapore. A retrospective review was performed of patients younger than 16 years old diagnosed with MF between 2000 and 2008 at the National Skin Centre, Singapore. Forty‐six patients were identified. At initial presentation, a provisional diagnosis of MF was made in 19 patients (41.3%), pityriasis lichenoides chronica (PLC) in 11 (23.9%) and postinflammatory hypopigmentation due to eczema or other causes in 11 (23.9%). After skin biopsy, the hypopigmented variant of MF was diagnosed in 42 patients (91.3%). There was one case each of PLC‐like MF, pigmented purpuric dermatosis‐like MF, classic MF, and solitary MF. Pityriasis lichenoides coexisted in three cases (6.5%). All except one patient presented with the early patch‐plaque stage of disease (stage IA/B). The disease did not progress in any of our patients after a mean follow‐up of 71.0 ± 52.5 months. Twenty‐seven patients (58.7%) had complete disease clearance after a mean duration of 27.1 ± 28.1 months; 15 (49.7%) of 32 patients who received narrowband ultraviolet B treatment had complete clearance within an average of 8.9 ± 5.3 months, but 7 patients relapsed within 14.9 ± 14.8 months. One patient with solitary MF failed multiple treatment modalities before eventually achieving disease clearance with photodynamic therapy. Hypopigmented MF is the most common MF variant in Asian children. The diagnostic difficulty is in differentiating this from PLC, which may coexist with MF. Long‐term prognosis is generally favorable.     

A rare case of purpuric rash caused by Saprochaete clavata in a pediatric patient with acute leukemia

We present a 13‐year‐old boy who developed invasive infection with Saprochaete clavata after induction chemotherapy for B‐cell acute lymphoblastic leukemia, complicated by fungemia, septic shock and acute renal, and liver failure. He developed purpuric papulonodules over bilateral upper and lower limbs, which also grew S clavata on fungal culture.