Therapeutic evaluation of the oral retinoid Ro 10-9359 in several non-psoriatic dermatoses

Forty-five patients suffering from various genodermatoses and erythematous, scaling, non-psoriatic dermatoses were treated orally with the aromatic derivative of retinoic acid, Ro 10-9359 (Tigason). In the genodermatoses the best results were obtained in ichthyosis, keratodermias and Darier's disease (95.6% good to excellent). Among the erythematous scaling diseases, treatment was effective in lichen planus, parapsoriasis and pityriasis rubra pilaris (53.3% good to excellent results). In comparison with therapies previously employed Ro 10-9359 was more effective. No serious side-effects were noted.     

Pityriasis rubra pilaris in Singapore

Fourteen patients with pityriasis rubra pilaris seen between 1981 and 1989 were evaluated retrospectively. There were nine male and five female patients. There was a bimodal age of onset in early childhood and in adulthood. All the patients had either follicular hyperkeratosis or hyperkeratosis of the palms and soles. Nine patients had erythroderma. The classical forms, either in the children or in the adult patients, were more likely to recover. Several treatment modalities were used and these included steroids, Vitamin A, etretinate, retinoic acid and ultraviolet light (UBV). Nine patients had complete recovery. Four of these patients received only steroids, two cleared while on vitamin A, two cleared while on etretinate and one cleared with methotrexate.     

The effect of 13-cis retinoic acid on epidermal lysosomal hydrolase activity in Darier's disease and pityriasis rubra pilaris

Five patients with Darier's disease and 6 patients with pityriasis rubra pilaris were treated with 13-cis-retinoic acid. Extracts of separated epidermis were assayed for extractable protein, lactic dehydrogenase, Cathepsin D, beta glucuronidase and neutral proteinase before beginning therapy and 2, 4 and 8 weeks after therapy had begun. The epidermal extracts from patients with pityriasis rubra pilaris before beginning therapy were similar to extracts from normal control patients. During the course of therapy with 13-cis-retinoic acid, protein extractability, lactic dehydrogenase and neutral proteinase did not change; there was a highly significant decrease in the specific activity of the lysosomal hydrolases Cathepsin D and beta glucuronidase. A similar but less dramatic fall was noted in the Darier's patients taking 13-cis-retinoic acid. Darier's patients also had a decrease in neutral proteinase activity before beginning therapy; the specific activity of this enzyme increased during the course of therapy. 13-cis-retinoic acid does not induce clinical remission by increasing the intracellular concentration of lysosomal enzymes in epidermis in vivo.     

非典型幼年型毛发红糠疹1例

患儿男,13个月。双掌跖弥漫性红斑、角化过度10个月;面颈部、腋下、腹股沟、臀部红斑、鳞屑,伴瘙痒9个月。家族中有类似疾病患者。右臀部皮损组织病理示:表皮角化过度伴灶性角化不全,在水平方向和垂直方向可见交替的角化不全和角化过度;颗粒层及棘层肥厚,表皮突增宽;真皮乳头血管扩张,浅层血管周围少量淋巴细胞浸润。诊断:非典型幼年型毛发红糠疹。     

Pityriasis rubra pilaris and vitiligo in Down''s Syndrome

A 30-year-old woman with Down's syndrome is described associating circumscribed juvenile pityriasis rubra pilaris and vitiligo. This appears to be the first reported case of pityriasis rubra pilaris in Down's syndrome.     

司库奇尤单抗成功治疗毛发红糠疹一例并文献复习

毛发红糠疹常规治疗包括系统应用维A酸类药物、甲氨蝶呤、环孢素,外用糖皮质激素以及物理治疗等,但效果并不理想。近年来,国外有应用司库奇尤单抗治疗毛发红糠疹的病例报道,且取得了较好的疗效,但国内未见相关报道。本文报道司库奇尤单抗成功治疗毛发红糠疹一例并对相关文献进行复习。     

Response of juvenile circumscribed pityriasis rubra pilaris to topical tazarotene treatment

Abstract:  We report juvenile circumscribed pityriasis rubra pilaris (type IV) in a 12-year-old Afghani girl in whom local therapy with tazarotene led to rapid, sustained remission. Tazarotene might be considered a useful treatment for circumscribed pityriasis rubra pilaris.     

Topical treatment of pityriasis rubra pilaris with calcipotriol

Summary From a clinical, histological and therapeutic point of view, psoriasis and pityriasis rubra pilaris share important characteristics. Recently, calcipotriol has been shown to be an effective treatment in psoriasis, and we report three patients with pityriasis rubra pilaris who showed a favourable response to topical therapy with calcipotriol. In one case, analysis of markers for epidermal growth, differentiation and inflammation revealed reduction of suprabasal expression of keratin 16. and the number of T lymphocytes, monocytes and macrophages. It is of interest that a reduction of the recruitment of cycling epidermal cells, which is a consistent response pattern during treatment of psoriasis, was not observed during treatment of pityriasis rubra pilaris.     

Exacerbation of pityriasis rubra pilaris under efalizumab therapy

A 60-year-old woman, diagnosed as having psoriasis vulgaris in 2004 and unresponsive to standard therapies, received weekly subcutaneous injections with efalizumab. Within 9 weeks of treatment a massive aggravation of skin lesions occurred with widespread orange-tinged erythroderma, islands of normal skin on the back and the inner side of the forearms and palmoplantar hyperkeratosis. A biopsy confirmed the clinical diagnosis of pityriasis rubra pilaris. After discontinuation of efalizumab and treatment with oral corticosteroids, acitretin (30 mg/day) and PUVA therapy, the skin lesions continuously improved. Efalizumab, a fully humanized monoclonal antibody against CD11a, inhibits various T cell processes important in the pathogenesis of psoriasis. Efalizumab has been approved for the treatment of moderate to severe psoriasis, but there are no reports in the literature on the use of efalizumab for pityriasis rubra pilaris.     

Further experience with toxic vitamin A therapy in pityriasis rubra pilaris

A patient with short-duration pityriasis rubra pilaris was successfully treated with high-dose, toxic vitamin A (retinol), but the disease subsequently recurred in new areas. Serum levels of vitamin A were highest four hours after treatment and returned to the base level within twelve hours. Fasting blood levels of vitamin A during treatment increased to five times the pretreatment level. Ultrastructural changes in the keratinocytes were notable vacuolation, granularity of the cytoplasm, and a decrease in tonofilament masses, all indications of the cellular effect of the vitamin A. We believe that patients with long-duration pityriasis rubra pilaris should be considered for this treatment.     

Histochemical applications of skin surface biopsy

SUMMARY.— The method of skin surface biopsy in which a cyanoacrylate adhesive is used to remove a coherent strip of stratum corneum, has been used to investigate aspects of epidermal function. The Sudan dyes were employed to demonstrate sebum in the hair follicle openings. Although increased quantities of Sudanophilic material were detected with increasing time intervals before sampling, it proved impractical to measure sebum secretion quantitatively by this method. Hydrolase activity in and around hair follicle orifices of the back, forehead and forearm were demonstrated, using enzyme histochemical methods for non-specific esterase, acid phosphatase, leucine aminopeptidase and β-glucuronidase activities. Specimens from the forehead and back were found to have stronger activities than those from the forearm. Apart from the vicinity of the follicular openings, non-specific esterase activity was not normally found in the superficial stratum corneum, although scattered areas of activity were present in the deeper parts of the horny layer. Strong, non-specific esterase activity was detected in skin surface biopsies from lesions of pityriasis lichenoides and, to a lesser extent, those of psoriasis. Mitochondrial enzyme activities were retained in parakeratotic scale from patients with psoriasis, pityriasis rubra pilaris and areas made parakeratotic after repeated removal of the stratum corneum, although not from the lesions of pityriasis lichenoides. The method also proved suitable for the demonstration of melanin granules, blood pigment and sweat gland activity.     

SYSTEMIC LUPUS ERYTHEMATOSUS: A REPORT OF FORTY-FIVE CASES WITH UNUSUAL CLINICAL AND IMMUNOLOGICAL FEATURES

SUMMARY.— Forty five cases of systemic lupus erythematosus in Hong Kong are reviewed.
Of common occurrence were hyperkeratotic follicular papules, which in some cases resembled pityriasis rubra pilaris and keratosis pilaris, hyperkeratosis of the palms and soles, and psoriasiform lesions. Less frequent cutaneous manifestations included chilblains, livedo reticularis, and scleroderma-like changes. Biological false positive serological tests for syphilis were not encountered.
The possible significance of these deviations from the familiar clinical and immunological picture is discussed.     

Pityriasis rubra pilaris

Pityriasis rubra pilaris is a chronic, papulosquamous dermatosis of unclear etiology. The case of a 61-year-old man with pityriasis rubra pilaris is presented. The clinical forms, histopathologic features, and treatment options of pityriasis rubra pilaris are reviewed.     

毛发红糠疹的治疗进展

毛发红糠疹病因及发病机制不明,通常对传统治疗抵抗。由于缺乏高质量的证据,目前尚无治疗指南,维A酸类药物被普遍作为一线治疗,近年来生物制剂被证明在严重难治性病例中有快速、显著的疗效。本文就PRP治疗方法进行综述。     

CORNEOCYTES IN SCALY PARAKERATOTIC DISEASES

Background and Objectives. The stratum corneum of some of the scaly (parakeratotic) diseases was examined with light and scanning electron microscopy (SEM) with the purpose to reveal the importance of this layer in the diagnosis of some of the diseases associated with the formation of scales. Materials and Methods. Two biopsies of the skin surface were taken: one, obtained from 80 patients with various parakeratotic scaly diseases and from 25 control subjects, was processed for light microscopy; the other biopsy for SEM was taken from 10 control subjects and 25 patients. The diagnoses of these patients were: psoriasis (5 patients), erythrodermic psoriasis (2 subjects), parapsoriasis (5 patients), pityriasis rubra pilaris (5 subjects), pityriasis rosea (3 subjects), and seborrheic dermatitis (5 subjects). Results. The light microscopic studies showed that normal corneocytes are of polygonal shape with their largest diameter measuring 42 μm; these cells lacked nuclei. All parakeratotic cells appeared bizarre in shape, smaller than normal, and the cells contained a nucleus. With SEM, normal cells appeared relatively regular in size and shape, trabeculated, and had a flat surface. Cells examined in all the diseases revealed various sizes, outlines, and trabeculae. Specific surface patterns (print) of diseased cells were: “fish-scale” in psoriasis; “marbled” in parapsoriasis, “rocky stone” in pityriasis rubra pilaris; “heart-shaped” in seborrheic dermatitis, and semi-crystalloid in pityriasis rosea. Conclusions. Parakeratosis is characterized not only by the retention of the nucleus in keratinocytes, but is also characterized by a cell of smaller size. The specific print of a disease helps in the diagnosis. The print will change with different stages of a disease.     

Pathogenesis of the permeability barrier abnormality in epidermolytic hyperkeratosis

Epidermolytic hyperkeratosis is a dominantly inherited ichthyosis, frequently associated with mutations in keratin 1 or 10 that result in disruption of the keratin filament cytoskeleton leading to keratinocyte fragility. In addition to blistering and a severe disorder of cornification, patients typically display an abnormality in permeability barrier function. The nature and pathogenesis of the barrier abnormality in epidermolytic hyperkeratosis are unknown, however. We assessed here, first, baseline transepidermal water loss and barrier recovery kinetics in patients with epidermolytic hyperkeratosis. Whereas baseline transepidermal water loss rates were elevated by approximately 3-fold, recovery rates were faster in epidermolytic hyperkeratosis than in age-matched controls. Electron microscopy showed no defect in either the cornified envelope or the adjacent cornified-bound lipid envelope, i.e., a corneocyte scaffold abnormality does not explain the barrier abnormality. Using the water-soluble tracer, colloidal lanthanum, there was no evidence of tracer accumulation in corneocytes, despite the fragility of nucleated keratinocytes. Instead, tracer, which was excluded in normal skin, moved through the extracellular stratum corneum domains. Increasing intercellular permeability correlated with decreased quantities and defective organization of extracellular lamellar bilayers. The decreased lamellar material, in turn, could be attributed to incompletely secreted lamellar bodies within granular cells, demonstrable not only by several morphologic findings, but also by decreased delivery of a lamellar body content marker, acid lipase, to the stratum corneum interstices. Yet, after acute barrier disruption a rapid release of preformed lamellar body contents was observed together with increased organelle contents in the extracellular spaces, accounting for the accelerated recovery kinetics in epidermolytic hyperkeratosis. Accelerated recovery, in turn, correlated with a restoration in calcium in outer stratum granulosum cells in epidermolytic hyperkeratosis after barrier disruption. Thus, the baseline permeability barrier abnormality in epidermolytic hyperkeratosis can be attributed to abnormal lamellar body secretion, rather than to corneocyte fragility or an abnormal cornified envelope/cornified-bound lipid envelope scaffold, a defect that can be overcome by external applications of stimuli for barrier repair.     

Pityriasis Rubra Pilaris Responsive to Ascorbic Acid

A case of pityriasis rubra pilaris with concomitant ascorbic acid deficiency is presented in an elderly Negro woman. Vitamin shortage was incidental but treatment with vitamin C yielded favourable results; spontaneous resolution seemed unlikely. Addition of vitamin A in less than optimal dose checked a mild cutaneous relapse and enhanced further the involuting process. Conjoint administration of vitamins A and C may prove ideal in management of pityriasis rubra pilaris. The therapeutic value of ascorbic add in this dermatosis warrants further study.     

Ultrastructure of pityriasis rubra pilaris with observations during retinoid (etretinate) treatment

The light and electron microscopic structure of pityriasis rubra pilaris (PRP) is described in five patients. Hyperkeratosis, hypergranulosis, keratotic plugs in the follicular openings, acanthosis and focal parakeratosis were observed. A moderate perivascular infiltrate was seen in the upper dermis. Electron microscopy revealed moderately activated keratinocytes, a decreased number of tonofilaments and desmosomes, enlarged intercellular spaces, parakeratosis with lipid-like vacuoles and a large number of keratinosomes. Lymphoid cells were present in the epidermis in moderate numbers. At the dermo-epidermal junction, the basal lamina was focally split, containing gaps. Etretinate therapy produced moderate to marked clinical improvement. The histological picture improved but the typical signs of PRP, including follicular plugging, persisted. Ultrastructurally the cellular activity and the amount of hyperkeratosis and parakeratosis decreased, while increases in keratinosomes, intercellular substance, microvilli and desmosomes were observed during treatment.     

Pityriasis rubra pilaris and focal acantholytic dyskeratosis

A 65-year-old man presented with a widespread erythematous maculopapular eruption. Skin biopsy showed spongiosis and focal acantholytic dyskeratosis consistent with Grover's disease. Clinically the eruption evolved to erythroderma with typical features of pityriasis rubra pilaris. On review of the histology, changes consistent with this diagnosis were also present in addition to the acantholytic dyskeratosis.     

Dermatomyositis with a pityriasis rubra pilaris-like eruption: an uncommon cutaneous manifestation in dermatomyositis

A pityriasis rubra pilaris-like eruption has been described in patients with dermatomyositis. We describe an 11-year-old girl with dermatomyositis who had additional clinical findings of pityriasis rubra pilaris. Over a year, she developed muscle weakness, increasing fatigue, and a markedly elevated creatinine kinase level in addition to her cutaneous eruption and was seen in our clinic for these complaints. A year earlier, when a generalized, scaly erythematous eruption had appeared, she had been diagnosed as pityriasis rubra pilaris clinically and histopathologically. Dermatologic examination found scaling erythematous plaques involving the trunk and upper and lower extremities. Islands of unaffected skin were intermingled with erythematous plaques that were characteristic of pityriasis rubra pilaris. A skin biopsy specimen showed the findings of dermatomyositis and that diagnosis was made. The laboratory findings, electromyographic pattern, and muscle biopsy were also consistent with dermatomyositis. Her presentation is interesting, as she had been diagnosed as pityriasis rubra pilaris both clinically and histopathologically 1 year earlier and, although the cutaneous lesions had not changed, a diagnosis of dermatomyositis was made a year later.