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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↗
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↗
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↗
PubMed2002

Evaluation of the role of contact sensitization and photosensitivity in the pathogenesis of poikiloderma of Civatte.

BACKGROUND: Poikiloderma of the face and neck (Civatte) is a rather common, indolent, chronic dermatosis, most often affecting menopausal females. Cumulative excessive sun exposure, a phototoxic or a photoallergic reaction, hormonal changes of menopause and genetic factors have all been incriminated in its obscure aetiopathogenesis. OBJECTIVE: To evaluate the role of contact sensitization and photosensitivity in the pathogenesis of poikiloderma of Civatte (PC). METHODS: Thirty-two patients (24 females and eight males, age range 38-74 years) with PC were patch tested with the European standard series and the fragrance series, and were photopatch tested with the photoallergens series. Additionally, photo-testing with a monochromator was performed. RESULTS: Thirteen of 32 patients (40.62%) had one or more positive reactions to allergens of the standard series. Eight patients (25%) had positive reactions to fragrance mix and/or Balsam of Peru, which are included in the standard series, or to allergens of the fragrance series. Nickel sulphate was the single most common cause of contact sensitization (18.75%) among our patients. Ninety-seven subjects, who were patch tested with the standard series for suspected allergic contact dermatitis of the face and/or neck, served as age, sex and site controls. Of these, nine (9.27%) had one or more positive reactions to fragrance compounds. Statistical analysis showed a statistically significant difference in the frequency of positive reactions to fragrances between the PC group and the control group (chi2 value = 3.91, P < 0.05). In contrast, none of the PC patients had a positive photopatch test for the allergens included in the photoallergens series. The estimated minimal erythemal dose for the PC group was in all cases within normal limits for all wavelengths of ultraviolet (UV) radiation examined. CONCLUSIONS: Contact sensitization, mostly to perfume ingredients, may develop in PC, possibly playing a pathogenetic part, at least in a subset of patients. Despite negative results of photopatch testing, an allergic photo-contact reaction cannot be definitely excluded. PC seems not to be a photosensitivity disorder of the type of chronic actinic dermatitis. UV radiation-induced dermal connective tissue changes are the predominant histological feature of PC, leading to telangiectasia due to loss of vascular support. Reticular pigmentation may result from a delayed hypersensitivity reaction to perfume and/or cosmetic ingredients. Patch testing with the standard series and avoidance of documented allergens may be of value in patients with PC.

Adult↗
PubMed1987

Epidermal dysplasia and skeletal deformity in congenital poikiloderma (Rothmund-Thomson syndrome).

Two sisters with congenital poikiloderma (Rothmund-Thomson syndrome) are described. One sister developed numerous keratotic lesions on the skin at an early age; these showed histological, ultrastructural and autoradiographic features of dysplastic change. The second sister had severe skeletal involvement in addition to the cutaneous poikiloderma, but no keratotic lesions. The clinical features of these cases demonstrate the variation in phenotypic expression of this disorder within a single family.

Bone Diseases↗