Poikiloderma of Civatte: a clinical and epidemiological study

BACKGROUND: Although a common dermatosis, idiopathic poikiloderma of the face and neck has not been studied in depth for decades. OBJECTIVES: To reassess the clinical and epidemiological characteristics of poikiloderma of Civatte (PC). MATERIAL AND METHODS: Fifty consecutive patients with PC. Evaluation included history taking and physical examination. Epidemiological and clinical parameters were recorded and analysed. The literature from 1923 until today, was reviewed thoroughly. RESULTS: The frequency of PC among dermatologic patients was estimated to be 1.4%. There were 34 females (68%) and 16 males in the present study. The mean age at diagnosis was 47.8 years for females and 61.7 years for males. The majority (88%) had skin phototype II or III. Among females, 26 were at their peri-menopausal stage, including three cases of iatrogenic menopause. Four patients reported that other blood-related family members also had PC. The v and the sides of the neck and the upper chest were most often affected in a symmetric distribution. The face (preauricular and parotid region) was involved in 19 patients (38%). The erythemato-telangiectatic clinical type predominated (58%), followed by the mixed (22%) and the pigmented type (20%). Almost half of the patients (46%) were symptomatic (itching, burning and 'flushing'). The mean duration from onset to diagnosis was 6.2 years according to the patients' report. The course was usually slowly progressive (82%) and irreversible. CONCLUSIONS: PC shows characteristic features, supporting the theory that it represents a distinct entity. It is rather common in Greece. Although menopausal women predominated in our cohort, men were not uncommonly affected and were diagnosed at an older age. Based on the predominating clinical feature, PC can be classified into three clinical forms. Symmetry and sparing of the anatomically shaded areas of the neck are highly characteristic for PC. Face involvement was not as common and as severe as it had been considered in the past. Recognition of clinical type is important for the selection of the most appropriate treatment, which, despite the advent of novel modalities, remains problematic.     

Civatte皮肤异色病

患者女,37 岁,戏剧演员. 主诉:面颈部褐色色素沉着斑21年,加重2年. 现病史:患者自16 岁化彩妆后双颊部开始出现淡褐色斑,无自觉症状.近2 年皮损逐渐蔓延至颧部、额部及颈部,并融合呈网状,颜色渐深.同时下腹部、腹股沟也有类似皮损,但未呈网状,于2009年11 月来我科就诊.     

Civatte皮肤异色病1例

患者女,50岁。面部皮肤褐色斑片40余年。查体见面颈部弥漫分布黑褐色斑片,两颊呈网状分布,伴有白色斑点、毛细血管扩张和轻度萎缩。皮肤组织病理示:表皮变薄,基底细胞液化变性,真皮浅层血管周围炎细胞浸润,见明显噬色素细胞。诊断:Civatte皮肤异色病。     

Civatte皮肤异色病1例

患者女,40岁。面部色素斑9年,其上可见浅表性淡白色斑点,日晒或受热时加重,无明显自觉症状。结合组织病理诊断为:Civatte皮肤异色病。给予防光剂治疗。     

Androgenetic alopecia in males: a histopathological and ultrastructural study

Background  Androgenetic alopecia is a common cosmetic hair disorder, resulting from interplay of genetic, endocrine, and aging factors leading to a patterned follicular miniaturization. Microinflammation seems to be a potential active player in this process.
Aims  To study the histopathological and ultrastructural changes occurring in male androgenetic alopecia (AGA).
Patients/methods  Fifty-five subjects were included in this study (40 with AGA and 15 as normal age-matched controls). Skin biopsies from frontal bald area and occipital hairy area were subjected to histopathological examination, immunohistochemical staining for collagen I and ultrastructural study.
Results  The frontal bald area of patients showed highly significant increase in telogen hairs and decrease in anagen/telogen ratio and terminal/vellus hair ratio ( P  <   0.001). Perifollicular inflammation was almost a constant feature in early cases and showed a significant inverse correlation with perifollicular fibrosis ( P  =   0.048), which was more marked with thickening of the follicular sheath in advanced cases.
Conclusion  Follicular microinflammation plays an integral role in the pathogenesis of AGA in early cases. Over time, thickening of perifollicular sheath takes place due to increased deposition of collagen, resulting in marked perifollicular fibrosis, and sometimes ends by complete destruction of the affected follicles in advanced cases.     

PDT and BF‐200 ALA: The therapy option for the treatment of Poikiloderma of Civatte

Poikiloderma is a chronic skin condition affecting middle‐age men and women that comprises cutaneous atrophy, telangiectasias, and changes in the pigmentation of the skin usually of mottled appearance (both hyper‐ and hypopigmentation). There is no specific medical treatment for Poikiloderma of Civatte (PC), but the use of photoprotector/sunscreen + SPF 50 to avoid prolonged sun exposure is highly recommended in these patients. Some authors recommend the use of topical corticoids in early stages; however, the results have not been satisfactory. Multiple topical (retinoids, dimethyl sulfoxide, or calcineurin inhibitors), systemic (cyclophosphamide), and physical (dermabrasion, phototherapy, and fractional photothermolysis) treatments have been described with unequal and inconsistent responses or unsatisfactory. This report underlies the combination of BF‐200 ALA and photodynamic therapy (PDT) with positive results at the clinical level: significant improvement of pigmentary changes and telangiectasias after two PDT sessions.     

Digital papular calcific elastosis: a histopathological, histochemical and ultrastructural study of 20 patients

In 1960, 5 patients were reported with a condition termed "degenerative collagenous plaques of the hands". Ultraviolet light and chronic pressure seem to be the inciting factors. Clinically, it is characterized by keratotic, translucent papules, in linear array, on the radial border of the hands. Histology shows hyperkeratosis and dermal elastosis. The current study documented the histopathological, histochemical and ultrastructural features of 20 patients with this condition and considered the differential diagnoses. The findings were in accordance with those of previous studies, but peculiar, basophilic elastotic masses (BEMs), were a prominent finding. Ultrastructurally, elastic tissue appeared in the form of degenerated non-fragmented and fragmented elastic fibres, degenerated fibres showing foci of increased density, with or without a fibrillar component and as amorphous, structureless material. BEMs arose almost exclusively from degenerated elastic tissue and contained calcium. Disintegrated collagen may be incorporated in the amorphous material and, subsequently, form a minor part of BEMs. Solar elastosis, and its variants, show some similarity to degenerative collagenous plaques of the hands, but lack the constant presence of these calcified angular elastotic masses. BEMs show resemblance to elastic globes, and elastotic bodies found in elastotic nodules of the anthelix and cartilage-hair hypoplasia. In the clinical setting of "degenerative collagenous plaques of the hands", BEMs seem to be unique and due to their extensive presence serve as a non-specific marker for this condition. Digital papular calcific elastosis therefore seems to be more appropriate nomenclature.     

Eccrine tubular adenoma--a histopathological, histochemical, and ultrastructural study

A case of eccrine tubular adenoma on the dorsum of the right foot is presented. Histopathologically, in the central nodule of the tumor, the whole dermis was involved and the tumor islands were connected to the epidermis; in the rest of the lesion, tumor islands were observed in the upper dermis. The tumor islands were composed of cystic or alveolar structures and cell masses possessing multiple lumina. Tubules were surrounded by two or more layers of epithelium forming papillations projecting into the lumen. There were only a few basaloid islands and no sclerotic strands. The tumor cells were well differentiated. Acid mucopolysaccharides were seen in the stroma. Histochemically, phosphorylase and acid phosphatase reacted moderately. Succinic dehydrogenase gave a weak reaction and β-glucuronidase was negative in tumor cells. Ultrastructurally, intracellular ducts with numerous microvilli, periluminal filamentous zones, and many multivesicular dense bodies surrounded by a limiting membrane were observed. Keratohyalin granules were absent. Based on these findings, the tumor reported here was considered to be an adenoma differentiating toward the eccrine duct. Some aspects resembled tubular apocrine adenoma, syringoma and basal cell tumor with eccrine differentiation, but they were most similar to papillary eccrine adenoma.     

Hairy leucoplakia—a clinical, histopathological and ultrastructural study in 33 patients

Thirty-three cases of hairy leucoplakia (HL) in HIV-positive patients were studied. The most frequent clinical presentation was the development of corrugated plaques on the lateral sides of the tongue without spiculations on the surface. In all biopsies, herpetic-type viral inclusions were found. In 12 out of 14 cases in whom an electromicroscopic study was carried out, only viral particles of the herpes group were found. Human papillomavirus (HPV) was not demonstrated, although by means of immunohistochemistry, positive labelling for HPV was found in 95% of the cases. The aetiopathological significance of these findings remains to be evaluated.     

Intrinsic aging vs. photoaging: a comparative histopathological,immunohistochemical, and ultrastructural study of skin

Cutaneous aging is a complex biological phenomenon affecting the different constituents of the skin. To compare the effects of intrinsic and extrinsic aging processes, a total of 83 biopsies were collected from sun-exposed and protected skin of healthy volunteers representing decades from the 1st to the 9th (6-84 years of age). Routine histopathology coupled with computer-assisted image analysis was used to assess epidermal changes. Immunoperoxidase techniques with antibodies against type I and type III collagens and elastin were used to quantitatively evaluate changes in collagen and elastic fibers and their ultrastructure was examined by transmission electron microscopy. Epidermal thickness was found to be constant in different decades in both sun-exposed and protected skin; however, it was significantly greater in sun-exposed skin (P = 0.0001). In protected skin, type I and III collagen staining was altered only after the 8th decade, while in sun-exposed skin the relative staining intensity significantly decreased from 82.5% and 80.4% in the 1st decade to 53.2% and 44.1% in the 9th decade, respectively (P = 0.0004 and 0.0008). In facial skin the collagen fiber architecture appeared disorganized after the 4th decade. The staining intensity of elastin in protected skin significantly decreased from 49.2% in the 1st decade to 30.4% in the 9th decade (P = 0.05), whereas in sun-exposed skin the intensity gradually increased from 56.5% in the 1st decade to 75.2% in the 9th decade (P = 0.001). The accumulated elastin in facial skin was morphologically abnormal and appeared to occupy the areas of lost collagen. Collectively, the aging processes, whether intrinsic or extrinsic, have both quantitative and qualitative effects on collagen and elastic fibers in the skin.     

Post-kala-azar dermal leishmaniasis: a histopathological study

BACKGROUND: Post-kala-azar dermal leishmaniasis follows an attack of visceral leishmaniasis and is caused by the same organism, i.e. Leishmania donovani. METHODS: In the present study, biopsy specimens from hypopigmented macules, nodules or plaques of 25 patients clinically diagnosed as PKDL were evaluated for epidermal and dermal changes and for the presence or absence of Leishmania donovani bodies (LDBs). RESULTS: The hypopigmented macules showed a patchy perivascular and periappendageal infiltrate with no demonstrable LDBs in any of the biopsies. In the nodular and plaque lesions, the infiltrate was diffuse, beneath an atrophic epidermis (74%) and follicular plugging (95.6%) was seen in most biopsies. The infiltrate consisted of lymphocytes, histiocytes and plasma cells in decreasing order of presence. LDBs could be demonstrated in only 10 (43.5%) biopsy specimens from nodular and plaque lesions and were never numerous. CONCLUSIONS: Histopathological features of PKDL are elucidated and discussed.     

Clinical, histopathological and ultrastructural characteristics of naevus depigmentosus

Background.  Naevus depigmentosus (ND) is a congenital, nonfamilial, well-circumscribed, uniformly hypopigmented macule, the relative size and distribution of which is stable throughout life. The aetiopathogenesis of ND is not yet fully understood, and reports about the clinical and histopathological characteristics of ND are few.
Objective.  To investigate the clinical and histopathological characteristics of ND, and to make it easier to diagnose ND clinically.
Methods.  A clinical survey on 38 patients with ND was performed according to the diagnostic criteria proposed by Coupe. Wood's lamp examination was used to distinguish the different appearance of ND and vitiligo. Skin-biopsy specimens were stained with haematoxylin and eosin, silver, antibodies to S-100 protein, tyrosinase-related protein-1 and tyrosinase, then used for ultrastructural study. Melanocytes were also cultured.
Results.  Leucoderma was present at birth in 13 patients (34.2%), and appeared during the first 3 years of life in 15 patients. The trunk was the most commonly affected site, and the lesions usually had serrated, irregular borders. Under Wood's lamp, lesions had an off-white accentuation without fluorescence. Immunohistochemistry showed that the melanin content of ND lesions was decreased compared with perilesional normal skin, but there was no change in the number of melanocytes. Ultrastructural study showed that some aggregated melanosomes were present in the affected keratinocytes.
Conclusion.  As a result of the above findings, we suggest changes to Coupe's criteria for ND.     

Solitary epidermolytic acanthoma: a clinical and histopathological study

Background Epidermolytic acanthoma (EA) is an uncommon cutaneous entity that typically presents as a solitary lesion, or, less commonly, as multiple or disseminated discrete lesions. It usually appears at or after middle‐age, and has been reported in various locations including the face, trunk, extremities and genitalia. Histopathologically, EA shows epidermolytic hyperkeratosis (EHK) involving either the entire thickness of the epidermis or just the granular and upper spinous layers. Objective and methods To describe the clinical and microscopic features of EA, we retrospectively reviewed all cases diagnosed as EA at the Skin Pathology Laboratory at Boston University between 1999 and 2009. Results Solitary EA is more common in men (65%) and usually presents as a hyperkeratotic papule on the trunk (45%) or extremities (25%). Histopathologically, all cases of solitary EA showed the classical features of hyperkeratosis, acanthosis and EHK. Three architectural patterns were observed on scanning magnification: papillomatous (55%), cup‐shaped (40%) and acanthotic (15%). Additional common features encountered included focal parakeratosis (85%), and a sparse to mild superficial perivascular lymphocytic infiltrate (90%). Conclusion This large case series of solitary EA reviews the clinical features of this entity and describes several new histological variants.     

Atrophoderma of Pasini and Pierini: a clinical and histopathological study

Background: Idiopathic atrophoderma of Pasini and Pierini (IAPP) usually manifests as one or multiple depressed and hyperpigmented patches, with a predilection to the trunk. No diagnostic changes are usually seen on histology. Elastic stains often reveal no abnormalities. Objective: To review our cases of IAPP, describe their clinical and histological findings and compare them with the literature. Methods: Retrospective review of IAPP cases who presented to our institution between 1994 and 2006. Results: From a total of 16 patients, only 19% displayed hyperpigmented lesions, while 81% had either hypopigmented (9/16) or skin‐colored (4/16) lesions. The sites predominantly affected were the lower extremities (62.5%), followed by the upper extremities and trunk. Only in one patient was IAPP co‐existent with morphea. Histology revealed no diagnostic abnormalities; however, elastic stains showed a spectrum of changes ranging from normal to severe diminution and fragmentation of elastic fiber network. Conclusions: Our study shows several new aspects of IAPP. Clinically, the lesions were most commonly hypopigmented and involved predominantly the extremities. Histologically, IAPP exhibited a spectrum of alterations in elastic fibers. The most prevalent form of IAPP in our country seems to be unassociated with morphea.