Necrolytic acral erythema: a rare skin disease associated with hepatitis C virus infection.

Necrolytic acral erythema is a rare skin disease associated with hepatitis C virus infection. We report a case of a 31-year-old woman with hepatitis C virus infection and decreased zinc serum level. Physical examination revealed scaly, lichenified plaques, well-demarcated with an erythematous peripheral rim located on the lower limbs. After blood transfusion and oral zinc supplementation the patient presented an improvement of lesions.

Necrolytic acral erythema: a rare skin disease associated with hepatitis C virus infection

Abstract: Necrolytic acral erythema is a rare skin disease associated with hepatitis C virus infection. We report a case of a 31-year-old woman with hepatitis C virus infection and decreased zinc serum level. Physical examination revealed scaly, lichenified plaques, well-demarcated with an erythematous peripheral rim located on the lower limbs. After blood transfusion and oral zinc supplementation the patient presented an improvement of lesions.

Phototherapy as an effective treatment for Majocchi's disease--case report.

Pigmented purpuric dermatoses are chronic and relapsing disorders characterized by a symmetrical rash of petechial and pigmentary macules, mainly confined to the lower limbs. Purpura annularis telangiectodes of Majocchi is a less common variant of Pigmented purpuric dermatoses characterized by punctate telangiectatic macules progressing to annular, hyperpigmented patches with central clearing and infrequent atrophy. A 12 year-old girl presented with asymptomatic round to oval reddish brown macules, present symmetrically over her lower and upper limbs for 3 years. Few lesions were annular in shape. Biopsy from the lesion was compatible with Pigmented purpuric dermatoses. On the basis of clinical and histopathological findings, a diagnosis of Purpura annularis telangiectodes of Majocchi was made. The patient began phototherapy thrice a week and showed excellent response.

Phototherapy as an effective treatment for Majocchi's disease - Case report

Pigmented purpuric dermatoses are chronic and relapsing disorders characterized by a symmetrical rash of petechial and pigmentary macules, mainly confined to the lower limbs. Purpura annularis telangiectodes of Majocchi is a less common variant of Pigmented purpuric dermatoses characterized by punctate telangiectatic macules progressing to annular, hyperpigmented patches with central clearing and infrequent atrophy. A 12 year-old girl presented with asymptomatic round to oval reddish brown macules, present symmetrically over her lower and upper limbs for 3 years. Few lesions were annular in shape. Biopsy from the lesion was compatible with Pigmented purpuric dermatoses. On the basis of clinical and histopathological findings, a diagnosis of Purpura annularis telangiectodes of Majocchi was made. The patient began phototherapy thrice a week and showed excellent response.

[Scleredema adultorum Buschke and exulcerated necrobiosis lipoidica in diabetes mellitus type 1]. / Scleroedema adultorum Buschke und exulzerierte Necrobiosis lipoidica bei Diabetes mellitus Typ 1.

HISTORY AND ADMISSION FINDINGS: A 53-year-old woman suffering from brown plaques occasionally ulcerating at both lower legs and in the course of disease on the back of the foot presented at our department. Moreover she complained about an increasing sclerosis of her skin at the back over the last 15 years. She had suffered from diabetes mellitus type-1 for more than 40 years. INVESTIGATIONS: Skin biopsies form the ulcerated plaques showed changes typical for necrobiosis lipoidica. Biopsies of the back were diagnosed as scleredema adultorum Buschke. Laboratory tests displayed a normal antinuclear antibody titer. The differential diagnosis of systemic sclerosis could be ruled out. TREATMENT AND COURSE: We repeatedly performed debridement of ulcers, cream-PUVA therapy, applied a vacuum-sealing and topical corticosteroids at the lower legs. Meanwhile she received oral pentoxyfillin. The scleredema was treated with UVA-1 phototherapy but had to be stopped for high photosensitivity. Additionally physiotherapy was prescribed. Nonetheless the course of disease was chronic and therapy-resistant. CONCLUSION: Skin diseases are common in diabetes mellitus. Necrobiosis lipoidica and scleredema adultorum Buschke are rare complications but often refractory to treatment.

Unilateral presentation of pseudo-Kaposi's acroangiodermatitis--a diagnostic and therapeutic challenge.

INTRODUCTION: Acroangiodermatitis is a rare skin disease characterised by hyperplasia of pre-existing vasculature due to venous hypertension from severe chronic venous stasis. Clinical appearance of this condition is often similar to Kaposi sarcoma and is creating serious differential diagnostic difficulties. CASE REPORT: A patient with acroangiodermatitis was presented and the differential diagnosis discussed. Examination of the patella of the affected area showed grayish-blue to brown infiltrates and reduced elasticity, located in the supra- and infrapatellar regions. Clinically, Kaposi's sarcoma was suspected. Histopathologically there were acanthosis and compact hyperkeratosis. The underlying papillary dermis showed fibrosis and edema. A subepidermal lobular vascular proliferation with hemosiderin deposition was also noted. This consisted of multiple newly formed capillaries, featuring small blood vessels with dilated, rounded lumina. Serologies for HIV and Borrelia burgdorferi were negative, as was a HHV-8 PCR in lesional tissue. Doppler analysis of the vessels of the extremities showed chronic venous insufficiency, insufficiency of v. perforantes, insufficiency of the Cockett II-III. No deep thromboses in the area of the shank and thigh were found. Initially, treatment consisted of clindamycin 600 mg 3 times per day, intravenously, during a 2-week period. After that the treatment was continued with prednisolone, 30 mg daily in combination with furosemide 40 mg/day, as well as lymph drainage and adequate compression therapy. The consequent clinical improvement allowed the patient to be discharged from the clinic. CONCLUSION: The most important differential diagnostic marker in distinguishing between acroangiodermatitis and Kaposi sarcoma seems to be the confirmation of the presence of genetic material of HHV-8 in the affected skin areas in patients with Kaposi sarcoma.

Narrowband UVB therapy as an effective treatment for Schamberg's disease.

A 33-year-old man presented with a 3-month history of a widespread pigmented purpuric eruption over his trunk and limbs. The clinical presentation and histology were consistent with a diagnosis of Schamberg's disease. The rash initially cleared following a short course of oral prednisolone at 25 mg daily for 3 weeks, which was weaned over the subsequent 4 weeks. Topical mometasone furoate ointment 0.1% daily was also applied to active areas. The rash recurred when prednisolone was reduced to below 5 mg per day. To prevent a further recurrence with weaning prednisolone, narrowband UVB therapy was commenced three times per week. The patient was continued on UV therapy over the next 5 months. The rash would flare after 2 to 3 weeks without treatment. The frequency of UV therapy was weaned and the patient remained clear on as little as one treatment every 2 weeks. Any further reduction, however, was associated with a recurrence. Narrowband UVB therapy should be considered for difficult or persistent cases of pigmented purpuric eruption.

Livedoid vasculopathy: long-term follow-up results following hyperbaric oxygen therapy.

BACKGROUND: Livedoid vasculopathy, also known as atrophie blanche, is a recurrent painful vasculopathy appearing mostly on the lower limbs. Treatment is challenging and relapses are frequent. OBJECTIVES: To analyse the long-term effect and safety of hyperbaric oxygen (HBO) therapy in treating livedoid vasculopathy. METHODS: Twelve patients with active livedoid vasculopathy were included in this study. All patients underwent HBO therapy five times a week. Each week photographs were taken and the total dose of analgesics was recorded. Side-effects were documented and assessed. Recurrence was defined as the presence of skin ulceration. RESULTS: Of the eight patients who completed the treatment, resumption of ambulation and reduction of analgesics were achieved at an average of 4.9 HBO therapy sessions. Leg ulcers in all eight patients healed completely at a mean of 3.4 weeks (range 2-5 weeks). Six patients suffered relapses of ulceration and responded to additional HBO therapy. No significant side-effects were found. CONCLUSIONS: HBO is a relatively safe, fast and effective method to treat patients with livedoid vasculopathy.

Necrolytic acral erythema: a variant of necrolytic migratory erythema or a distinct entity?

BACKGROUND: Hepatitis C is a major health problem in Egypt. Necrolytic acral erythema (NAE) is a recently described necrolytic erythema that has a distinctive acral distribution and a uniform association with hepatitis C. Some authors believe that NAE is a distinct entity and others consider it as a variant of necrolytic migratory erythema (NME). METHODS: Five patients with clinical features consistent with NAE were included in this study. The patients were subjected to skin biopsy examination, CT scan of the pancreas and a liver biopsy. Liver function tests, serum glucagon, glucose, amino acids and zinc were measured. All patients were tested for hepatitis C by enzyme-linked immunosorbent assay (ELISA) and by polymerase chain reaction (PCR). RESULTS: Three patients presented with early (acute) lesions and two patients with chronic lesions. The distribution of the lesions was almost exclusively on the dorsae of the feet. Histopathological findings were similar to those of other necrolytic erythemas. Hepatitis C virus was uniformly detected in all patients. Serum glucagon was high in two patients, serum glucose was high in four patients, serum amino acids were low in three cases and serum zinc and albumin were low in two cases. Little or no improvement was reported after oral amino acid supplementation, while the response to oral zinc sulfate was moderate to good. CONCLUSION: Necrolytic acral erythema is closely associated with hepatitis C infection. Many findings indicate that NAE seems to be a variant of NME rather than a distinct entity. Hence, an alternative proposed term could be acral NME.

Efficacy of pulsed dye laser treatment of port wine stain malformations of the lower limb.

BACKGROUND: There is relatively little information about treatment of port wine stains (PWS) of the lower limb. Few studies have specifically addressed the efficacy of pulsed dye laser (PDL) treatment of a PWS on the lower limb and there is no information on the relative efficacy at different sites on the lower limb. It has been suggested that treatment is not as successful when compared with the face and postinflammatory hyperpigmentation has been reported to be a significant problem. OBJECTIVES: To review retrospectively patients who attended for PDL treatment of PWS affecting the lower limb and assess the efficacy and adverse effects with particular reference to skin site. METHODS: A retrospective review was carried out of patients attending the Leeds Dermatology Laser Centre for treatment of a PWS on the lower limb with a 585-nm PDL. RESULTS: A total of 52 sites in 39 patients were treated: 17 on the thigh, 31 on the lower leg and four on the foot. The mean number of sessions per patient was 14, with an excellent outcome in seven treatment sites (13.5%), good in 13 (25%), moderate in 21 (40.4%) and poor outcome in 11 (21.1%). Patients were generally pleased with their results with a mean improvement of 7 on a scale of 0-10. Perifollicular persistence of the PWS was observed in six sites (11.5%). Adverse effects occurred in 36 patients (92.3%), most commonly hyperpigmentation (87%). Six patients (15.4%) developed atrophic scarring and four (10.3%) hypopigmentation. Atrophie blanche-like changes were seen in four patients on the lower leg. Hypertrophic scarring was not seen. CONCLUSIONS: Although physician-assessed good or excellent responses of 38.5% are lower than frequently reported for other skin sites and adverse effects may be more frequent, patient satisfaction with treatment was generally high. Patients with PWS on the lower limb merit a trial of PDL treatment.

Treatment of necrobiosis lipoidica with topical psoralen plus ultraviolet A.

BACKGROUND: Necrobiosis lipoidica (NL) is a rare skin disease, mostly seen on the legs and often occurring in patients with diabetes mellitus. The disease belongs to the idiopathic cutaneous palisading granulomatous dermatitides associated with a degeneration of collagen, thus leading to skin atrophy. Application of topical corticosteroids is the most widely used treatment but the results are not always satisfactory and may worsen skin atrophy. Preliminary studies in patients with NL have shown a clinical response with psoralen plus ultraviolet (UV) A (PUVA). Objectives To study the effect of topical PUVA on NL in a multicentre prospective study. METHODS: Thirty patients (27 women and three men) including 13 with insulin-dependent diabetes mellitus, with a diagnosis of NL proven by histopathology, were included. All patients had been unsuccessfully treated with topical and/or intralesional corticosteroids. Patients were treated twice weekly with an aqueous gel containing 0.005% psoralen followed by irradiation with UVA. Clinical photographs were taken for evaluation. In addition, 20-MHz high-frequency ultrasound analysis was performed in 10 patients to evaluate the thickness and density of the dermis during topical PUVA therapy. RESULTS: Five patients (17%) showed complete clearing (healing of ulceration and disappearance of erythema) after a mean of 22 exposures (range 15-30). Eleven patients (37%) showed improvement, defined as healing of ulceration and/or reduction of erythema, after a mean of 23 exposures (range 11-42). Ten patients (33%) showed no effect and four patients (13%) worsened during topical PUVA therapy. The treatment results of the patients who suffered from diabetes mellitus were not different from those who did not have diabetes mellitus. No difference was seen in mean dermal thickness (1666 vs. 1706 micro m) and density (17 vs. 16 units) before and after topical PUVA therapy. Side-effects were seen in 10 patients: hyperpigmentation (n = 4), blistering (n = 4) and bacterial infection (n = 2). CONCLUSIONS: Topical PUVA may be a useful treatment modality for NL in patients not responding to topical or intralesional corticosteroids.

Flegel's disease treated with psoralen ultraviolet A.

Flegel's disease is an uncommon condition which causes asymptomatic keratotic papules on the limbs. It usually develops in the fourth or fifth decade. Therapeutic options are limited to emollients, topical 5-fluorouracil and retinoids, but none of these treatments is consistently helpful. We report a patient with Flegel's disease who responded to psoralen ultraviolet A treatment.

Painful piezogenic pedal papules: response to local electro-acupuncture.

We report the case of a woman who had pain in both heels which was exacerbated by long periods of exercise. On examination, there were small flesh-coloured papules which appeared over the medial and lateral aspects of the heels only on weight bearing. Coincidentally, she was noted to have larger flesh-coloured papules over the anterior surface of the shins. The diagnoses of painful piezogenic pedal papules and bilateral tibialis anterior muscle herniation, respectively, were made. After many attempts to control the pain, a course of electro-acupuncture was commenced. A good subjective clinical response was achieved which has been maintained by fortnightly treatments. We discuss the prevalence, pathogenesis and treatment of painful piezogenic pedal papules. We believe that our patient is the first to have 'herniations' at both heel and shin sites and the first to have successful sustained pain relief for painful piezogenic pedal papules.

Laser treatment for an unusual reactive vascular proliferation.

An unusual case of severe vascular proliferation presenting in the form of haemorrhaging cutaneous lesions on the leg is reported. The similarity between these benign lesions and various malignancies is outlined. The patient healed well when the lesions were excised using a laser under local anaesthetic.

[Mycosis fungoides bullosa]. / Mycosis fungoides bullosa.

We report on a male patient with mycosis fungoides (MF) with blisters mainly occurring on clinically uninvolved skin. This rare association has to be differentiated from other bullous diseases of the skin. The clinical picture has been known since the end of the last century and has been described as mycosis fungoides bullosa. The pathomechanism, however, has not yet been exactly identified. The different clinical and histological features of the disease are discussed.