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At least 19 recordsLinked to original sources
PubMed1978

Congenital poikiloderma with features of hereditary acrokeratotic poikiloderma.

An adolescent boy with a bizarre progressive poikiloderma had episodic acral blistering and the development of palmar and plantar keratosis suggestive of hereditary acrokeratotic poikiloderma. Growth and development had been normal. Vesicobullous lesions were said to be present at birth and during early infancy. No family members were similarly affected. Results of laboratory studies were normal. Histological sections of skin demonstrated epidermal atrophy in some areas, increased epidermal melanin fibrosis of the papillary dermis, and modest capillary proliferation.

Adolescent↗
PubMed2000

Treatment of poikiloderma of Civatte with an intense pulsed light source.

BACKGROUND: Effective treatment of poikiloderma of Civatte combines elimination of both the vascular and pigmented components simultaneously. A broad spectrum, noncoherent, intense pulsed light (IPL) source delivers multiple wavelengths with software controlled pulse durations and sequencing, which permits treatment of both vascular and pigmented lesions simultaneously. OBJECTIVE: To determine response and side effects of poikiloderma of Civatte of the neck and chest when treated by IPL. METHODS: One hundred and thirty-five patients randomly selected with typical changes of poikiloderma of Civatte on the neck and/or upper chest were treated with one to five treatments using IPL. RESULTS: Clearance of more than 75% of telangiectasias and hyperpigmentation comprising poikiloderma was observed. The incidence of side effects was 5%, including pigment changes. In many cases, improved skin texture was noted both by physician and patient. CONCLUSION: IPL is an effective mode of therapy for poikiloderma of Civatte. It offers a reduction of pigment and telangiectasias with a low risk profile. Additional benefits include subjective changes of improvement in skin texture.

Adult↗
PubMed2006

Treatment of poikiloderma of Civatte with the pulsed dye laser: a series of patients with severe depigmentation.

BACKGROUND: Pulsed dye laser (PDL) treatment is widely used for poikiloderma of Civatte. Some adverse events in small numbers of patients have been reported. Guidelines for treatment of poikiloderma of Civatte do not exist. OBJECTIVE: To report the occurrence of persistent depigmentation as a late adverse event in a series of patients with poikiloderma of Civatte after treatment with PDL. METHODS: Eight patients (seven women and one man, mean age 48 years) with poikiloderma of Civatte were treated with PDL using a 585-nm wavelength and a fixed pulse duration of 450 micros. In all patients one or two test PDL patches were performed and reviewed after 3 months. All of the patients tolerated the testing without complications. Subsequent treatments were undertaken at intervals of 3 months. RESULTS: All patients were treated with fluences between 3.5 and 7 J/cm2, using a 7- or 10-mm spot size. All patients had a good result with respect to clearing of the vascular component. Nevertheless, six of them, treated with 5-7 J/cm2, reported severe depigmentation 4-11 months after treatment. Two patients treated with lower fluences (3.5-5.5 J/cm2) did not report this depigmentation. CONCLUSIONS: Great care is needed when PDL treatment is used for poikiloderma of Civatte. Pigment changes have been incidentally mentioned as late complications but have not been well documented as the late depigmentation has been in this series. It is advisable to use fluences as low as possible, and not exceeding an upper limit of 5 J/cm2, on a 10-mm spot size. More research is needed to define an optimal pulse duration.

Adult↗
PubMed2005

Clericuzio type poikiloderma with neutropenia is distinct from Rothmund-Thomson syndrome.

Two siblings from a consanguineous family presented with a poikiloderma of limbs and face, plantar keratoderma, and toenail pachyonychia. Neutropenia and neutrophil dysfunction with impairment of the respiratory burst and bacterial killing resulted in frequent respiratory tract infections. A bronchocentric granulomatous pneumonia was a fatal complication. The clinical presentation is consistent with Clericuzio type poikiloderma with neutropenia. Literature review identified several additional probable patients. Genetic linkage analysis excluded the locus of the RECQL4 gene, mutations in which have been described in some patients with the Rothmund-Thomson poikiloderma syndrome. This report confirms the clinical and genetic identity of the Clericuzio type of poikiloderma with neutropenia syndrome.

Abnormalities, Multiple↗
PubMed2001

Treatment of poikiloderma of Civatte on the neck with an intense pulsed light source.

Effective treatment of poikiloderma of Civatte is difficult. The ideal treatment combines elimination of both the vascular and pigmented components simultaneously. Treatment with a broad-spectrum noncoherent intense pulsed light source delivers multiple wavelengths with software-controlled pulse durations and sequencing that permit treatment of both vascular and pigmented lesions simultaneously. The objective of this study was to determine the response and side effects of treating this condition with intense pulsed light. In the study, 66 patients with typical changes of poikiloderma of Civatte on the neck were treated with intense pulsed light at various settings every 4 weeks until the desired improvement occurred. A 50 to 75 percent improvement in the extent of telangiectasias and hyperpigmentation was observed after an average of 2.8 treatments. The incidence of hypopigmentation was 5 percent. It was concluded that intense pulsed light is an effective mode of therapy for poikiloderma of Civatte. It seems to offer a reduction in both pigmentation and telangiectasia-associated erythema, with minimal side effects.

Adult↗
PubMed1998

Poikiloderma-like cutaneous amyloidosis in an ethnic Chinese girl.

Primary cutaneous amyloidosis is the deposition of amyloid in the skin without involvement of internal organs. It is easily diagnosed when presented in its typical manifestation. Atypical or rare clinical presentations can pose diagnostic difficulties. Poikiloderma-like cutaneous amyloidosis (PCA), a rare variant of primary cutaneous amyloidosis, was first reported in the literature in 1936 (1). It is characterised by: 1) poikilodermatous skin lesions; 2) lichenoid papules; 3) cutaneous amyloid deposit in the pigmented and lichenoid lesions; 4) light sensitivity; 5) short stature; and 6) other features such as blister formation or palmoplantar keratosis. Ogino coined the term PCA syndrome when these unusual features present early in life (2). We report a 26-year-old Chinese woman who presented with poikilodermatous skin lesions and was misdiagnosed as poikiloderma atrophica vasculare (PAV) on the basis of clinical appearance without any histological proof. The diagnosis of PCA was made after skin biopsy which showed amyloid deposits in the skin. This condition can easily be confused with other true poikiloderma skin diseases. Histology is important in confirming the diagnosis.

Adult↗
PubMed2006

Poikiloderma, tendon contracture and pulmonary fibrosis: a new autosomal dominant syndrome?

Members of two generations of a South African family have a unique syndrome comprising poikiloderma, tendon contractures and progressive pulmonary fibrosis. The condition is clinically important as the skin changes, which involve the face, have considerable cosmetic impact, while lung involvement is potentially lethal in adulthood. Skin manifestations which facilitate diagnosis include facial telangiectasia, mottled hypo- and hyperpigmentation, papules and epidermal atrophy. The scalp, facial and body hair are fine and scanty. The tendon contractures lead to progressive digital flexion deformities and abnormalities of the ankles and feet, with disturbance of gait. Pulmonary involvement manifests as progressive dyspnoea. Pedigree data are compatible with an autosomal dominant mode of transmission. Poikiloderma of Weary is characterized by linear sclerotic and fibrous bands and not tendon contractures and is not associated with potentially lethal pulmonary fibrosis. Rather than name this disorder a variant of Weary syndrome, it might be prudent to use as an umbrella title one composed by Weary himself: 'hereditary sclerosing poikiloderma' (HSP), under which variants such as HSP Weary type, HSP with cardiac involvement (aortic stenosis described as inconsistently associated with Weary syndrome) and HSP with tendon/pulmonary involvement (current family) may be classified. The manifestations in this family differ from other poikilodermata and, to the best of our knowledge, have not been previously documented.

Adult↗
PubMed1989

Poikiloderma of Theresa Kindler: report of a case with ultrastructural study, and review of the literature.

Kindler's syndrome occurred in a 13-year-old boy with parental consanguinity. The patient had acral congenital blistering and photosensitivity, both regressive, at 12 years of age, together with diffuse progressive poikiloderma, marked cutaneous atrophy, mild hyperkeratosis of the palms, gingival hypertrophy and fragility, and anal stenosis. The ultrastructural study of bullae induced by rubbing showed clefts in three levels of the dermoepidermal junction zone. A review of 14 cases in the literature consistent with Kindler's syndrome showed this to be a definite clinical entity among the other bullous hereditary poikilodermas. In two cases, ultrastructural studies showed intraepidermal, junctional, and dermal cleavage. This syndrome must be differentiated from congenital epidermolysis bullosa, Weary's syndrome, and the other bullous hereditary poikilodermas.

Adolescent↗
PubMed1999

Congenital poikiloderma with unusual hypopigmentation and acral blistering at birth.

Congenital poikiloderma is an uncommon hereditary disorder. It has been reported in association with various syndrome. No case has previously been reported from this environment. We report a case of congenital poikiloderma in a two and a half year old female Nigerian associated with unusual generalised hypopigmentation and acral blisters at birth. The child subsequently developed macular hyperpigmentation on an erythematous background and atrophy of the skin. Although she had some features which were suggestive of Rothmund-Thomson syndrome (RTS), the presence of hypopigmentation at birth, along with acral blistering, was noted to be peculiar to this child. We, therefore, feel that this case presents a distinct variant of congenital poikiloderma that has not been described previously.

Blister↗
PubMed1989

[Congenital poikiloderma].

Poikiloderma is characterized by the syntopy of diffuse atrophy, leukomelanodermia and teleangiectasia. We differentiate between symptomatic, idiopathic and congenital forms. Congenital poikiloderma shows 3 decisive features: early appearance, familial occurrence, and various associated congenital malfomations. On account of distinctive features - such as the consanguinity of the parents, cataracts, leukoplakia, bullas, and verrucous keratoses - we can distinguish between 5 biotypes of congenital poikiloderma, which are named after their first observers: Rothmund's, Thomson's, Zinsser's, Brain's and Dowling's syndrome.

Diagnosis, Differential↗
PubMed1984

[Thomson-type congenital poikiloderma with major bone dysplasias].

The authors report one case of Thomson type congenital poikiloderma with major bone dysplasias. Acroplasia of all extremities with agenesis of tibia and radius, and a peculiar aspect of the face are in contrast with the scarcity of cutaneous symptoms, the absence of consanguinuity, cataract, photosensitivity. The position of Thomson type congenital poikiloderma among the congenital poikilodermas is reviewed.

Bone Diseases, Developmental↗
PubMed1991

Calcinosis cutis, osteoma cutis, poikiloderma and skeletal abnormalities (COPS syndrome)--a new entity?

A 4-year-old boy with subcutaneous tumours is described. These tumours were calcified and had secondary osteoma formation. In addition the patient showed poikiloderma on the face and less prominently on arms and legs. X-ray films of the distal metaphyses of the radius, ulna and tibia revealed irregular mineralisation. Repeated laboratory tests revealed no abnormalities of fat, bone and mineral metabolism. This patient showed a unique combination of symptoms. We propose to call this syndrome: COPS-syndrome (Calcinosis cutis, Osteoma cutis, Poikiloderma and Skeletal abnormalities).

Bone Neoplasms↗
PubMed1998

[Congenital poikiloderma with verruciform hyperkeratoses (Dowling type)].

A male patient with congenital poikiloderma (type Dowling) developed Bowen's disease and initial squamous cell carcinoma. As the patient suffered from hypertriglyceridaemia (Frederickson type IV) and a long term tumor prophylaxis with retinoids was not appropriate, we attempted to treat the hyperkeratotic plaques with dermabrasion. After a period of six month we found complete healing of several plaques and only slight keratoses left in a few others. Long term follow up results are not yet available. Through this case report, the heterogenous clinical picture of congenital poikiloderma with warty hyperkeratoses and its high risk of malignancies is discussed.

Bowen's Disease↗
PubMed2000

Subacute cutaneous lupus erythematosus presenting with generalized poikiloderma.

A 64-year-old woman experienced progressive generalized poikiloderma after an episode of sunburn 4 years earlier. The diagnosis of subacute cutaneous lupus erythematosus (SCLE) was confirmed by the presence of anti-Ro/SS-A and antinuclear antibodies, the histology, and the direct immunofluorescent findings (ie, positive lupus band test and "dust-like" epidermal IgG staining pattern). Poikiloderma has not been previously reported in the spectrum of SCLE. As a major pathomechanism of SCLE, photosensitivity might explain this uncommon clinical manifestation of the disease.

Dermatitis, Photoallergic↗