Atrophoderma (Pasini-Pierini)

A patient with atrophoderma (Pasini-Pierini) was studied. Microscopic examination showed small collections of mononuclear cells around dermal blood vessels. Electron microscopic study demonstrated macrophages and lymphocytes around vessels and between fibers in the dermis; the epidermis, dermis, collagen, and elastic fibers appeared normal. Monoclonal antibody studies of the cells in the perivascular infiltrate demonstrated cells reacting with anti-Leu-1 (pan-T-cell antibody), anti-Leu-3a (the helper/inducer T-cell antibody), and OKM 1 antibody-reacting cells (macrophages). Direct immunofluorescent studies showed IgM and C3 staining in the small blood vessels of the papillary dermis, scattered IgM cytoids at the basement membrane, and focal fibrinogen in the mid-dermis. Mononuclear cells in the perivascular infiltrate, similar in type and percentage concentration, have been demonstrated also in patients with anetoderma, another rare atrophic cutaneous disorder. Macrophages and T lymphocytes around papillary dermal blood vessels may play a role in the pathogenesis of atrophoderma and anetoderma.     

Distribution and expression of type VI collagen in photoaged skin

BACKGROUND: Several of the characteristic clinical features of photoaged skin, including wrinkling, are thought to be dependent on changes in the dermal matrix brought about by chronic sun exposure. Such changes include reductions in collagens I, III and VII, an increase in elastotic material in the reticular dermis and a marked reduction in the microfibrillar glycoprotein fibrillin. OBJECTIVES: To examine whether type VI collagen, a microfibrillar collagen necessary for cell-cell and cell-matrix communication, is affected by the photoageing process. METHODS: Six healthy volunteers with moderate to severe photoageing were enrolled into the study. Immunohistochemistry and in situ hybridization histochemistry were used to examine the levels of type VI collagen in photoprotected and photoaged sites. RESULTS: In photoprotected skin, type VI collagen was concentrated in the papillary dermis immediately below the dermal-epidermal junction, around blood vessels, hair follicles and glandular structures. The distribution of type VI collagen was unchanged in photoaged skin, although we observed an increase in the abundance of the alpha3 chain of collagen VI in the upper papillary dermis, at its junction with the dermal-epidermal junction (P < 0.05). No alterations were observed for any alpha chain at the mRNA level. CONCLUSIONS: These studies suggest that chronic sun exposure (photoageing) has little or no effect on either the distribution, abundance or levels of expression of type VI collagen in human skin. Thus, type VI collagen, unlike other matrix components so far studied, appears to be relatively unaffected by the photoageing process.     

Immunohistochemical demonstration of proteoglycans in the skin of patients with systemic sclerosis

Skin proteoglycan was demonstrated by an immunofluorescent technique using an antibody against bovine cartilage proteoglycan, after the cross-reactivity of human proteoglycan with the antiserum had been confirmed. Normal skin exhibited specific fluorescence mainly in the blood vessels as well as in the subepidermal area. The clinically uninvolved skin of systemic sclerosis (SS) revealed no features different from those of normal skin. However, the vascular proteoglycan deposition of early systemic sclerosis was later replaced by deposition between the collagen fibres, which appeared to progress centrifugally in parallel to the increase in the skin sclerosis, suggesting a vascular initiation of the skin lesion. Sclerotic skin was characterized by random deposition between the collagen fibres. Immunoelectron microscopic studies suggested that the random proteoglycan deposition reflected uncontrolled local accumulation of proteoglycan in the interfibrillar matrix around irregularly arranged collagen fibrils.     

皮肤胶原血管病一例

【摘要】 患者女，22岁，小腿皮肤潮红4年，6个月前渐蔓延至整个下肢、前臂。皮肤科检查：下肢及双前臂可见弥漫潮红及扩张的毛细血管，压之褪色。皮损组织病理：网篮状角化过度，基底层色素增加，真皮浅层散在淋巴细胞浸润，未见明显红细胞溢出；PAS染色显示，血管周围可见增厚的均一化物质沉积；免疫组化显示，血管壁增厚，Ⅳ型胶原阳性。诊断：皮肤胶原血管病。     

脂水肿性脱发1例

患者女,62岁,头皮肿胀且局部横沟伴压痛半年。皮损组织病理示:表皮大致正常,真皮浅中部血管周围少量淋巴单核细胞浸润,胶原间纤维母细胞及胶原增生,真皮中下部毛囊周围见成熟脂肪细胞增生;黏蛋白染色示:毛囊周围及毛乳头见少量黏蛋白沉积。综合临床和病理表现,该患者诊断为脂水肿性脱发,脂水肿性头皮。     

Annular atrophic plaques of the skin (Christianson's disease)

A 69-year-old Caucasian man presented to the Gainesville Veterans Administration Medical Center for evaluation of several asymptomatic enlarging lesions on the face and forearms that had been present for 10 to 15 years. They were initially small but had progressively enlarged, especially during the previous 5 years. He reported having sustained a concussive grenade blast injury of the left temple and right forearm during the Korean Conflict in 1951. The injured areas healed uneventfully in 2–3 weeks. The patient was otherwise healthy, and the review of systems was noncontributory. Laboratory work-up (complete blood count, chemical profile, erythrocyte sedimentation rate, rheumatoid factor, hepatitis profile, antinuclear antibodies, urinalysis, and chest X-ray) was within normal limits. Lyme antibody titers were negative. Physical examination revealed similar-appearing lesions located variously on both temples, left preauricular and infraauricular areas, and the right forearm (Fig. 1). The lesions were large (ranging from 4 to 12 cm in diameter), centrally atrophic patches with ivory-colored thickened edges, and had the distinct appearance of healed skin grafts. Blood vessels were easily seen through the atrophic skin. All lesions were located on sun-exposed areas (predominantly on the patient's left side). No similar lesions elsewhere on the body or skin graft donor sites were found. The patient was treated with doxycycline 100 mg by mouth twice a day for 21 days to no avail, Intralesional steroids and clobetasol propionate ointment under occlusion were used without any change in the lesions. At the submission of this paper, the lesions remain stable. Skin biopsies were taken from the edge and center of the lesions, Histologic examination (Fig. 2) of the center of a lesion showed a flattened epidermis without interface changes. Sclerosis of superficial and mid, but not deep, dermal collagen was present, particularly within the specialized connective tissue surrounding eccrine structures. A focus of lymphocytic inflammation with rare plasma cells and edema was present at the dermal-subcutaneous interface. Histologic examination of the edge of the lesion showed a flattened epidermis overlying a perivascular and periadnexal lymphohistiocytic inflammatory infiltrate. The papillary dermis was sclerotic centrally, and sclerosis was present around one eccrine unit. Increased interstitial mucin was noted. Direct immunofluorescence using antibodies to IgG, IgM, IgA, and C3 was negative, A silver stain for borrelia organisms was negative. Electron microscopy of a biopsy from the advancing border of a lesion revealed foci of complete cytoiysis of the basilar epidermis above an interrupted basal lamina (Fig, 3a). Cellular debris was evident even within the subajacent papillary dermis (Fig. 3b). Abundant colloid-like material associated with occasional 8–10-nm straight tubules was also present within the upper papillary dermis. A solitary lymphocyte lay closely apposed to a basilar keratinocyte having large cytoplasmic vacuoles. Electron microscopy of a biopsy from the center of the same lesion revealed a flattened basilar epidermis and focal reduplication and interruption of the basal lamina (Fig. 3c). Abundantly interspersed among the collagen bundles of the upper and lower papillary dermis were aggregates that appeared amorphous at low magnification. Upon higher magnification, however, the aggregates could be seen to be comprised of straight and wavy tubules embedded in a finely granular matrix (Fig. 3d). A dermal blood vessel was surrounded by several basal laminae, just outside of which were presumptive lymphocytes with cerebritorm nuclei and histiocytes. No dermal elastic fibers could be identified. Collagen fibrils appeared normal.     

Secondary anetoderma overlying pilomatrixomas

Five pilomatrixomas with anetodermic cutaneous changes are presented. Four large tumors revealed soft, atrophic, pink translucent skin covering a firm subcutaneous mass, and their appearance was similar to that of a keloid or hypertrophic scar. One small tumor revealed soft, slightly atrophic, reddened or purplish skin covering a firm subcutaneous mass: its clinical appearance was similar to that of a hematoma or minor infection. Histopathological examination of the skin showed atrophic and edematous changes in the dermis associated with diminished fragmented collagen and absent elastic tissue. It is supposed that the dermal atrophic appearance bears a relation to the loss of elastic fibers and the dermal edema is related to the leakage of lymphatic fluid. Both phenomena may be caused by continuous pressure from the outside, as these anetodermic pilomatrixomas were located where they were apt to be exposed to mechanical irritation.     

Degos病1例

患者女,24岁。躯干及四肢泛发萎缩性丘疹1年。皮损组织病理示:表皮局限性坏死,真皮浅层胶原纤维素样坏死,真皮深层血管壁增厚,管壁内有嗜中性白细胞、淋巴细胞和组织细胞浸润,管腔内血栓形成。血管周围慢性炎症细胞浸润。诊断为Degos病。     

Vascular Ehlers-Danlos syndrome: a case with fatal outcome

A 13-year-old boy, born prematurely and hypotonic, from non-consanguineous healthy parents, was referred to our department because of easy bruising. A slightly extensible, thin and translucent skin, associated with dysmorphic facies, acrogeria, multiple ecchymoses, hypermobility of the small joints, dorsal kyphosis, genu valgum, flat feet, elongated upper limbs, and low muscle tone were all evident. A history of learning disability and bilateral inguinal hernia was present. Blood and imaging studies were unremarkable. A skin biopsy disclosed an unremarkable dermis; electron microscopy showed abnormalities in the diameter, contour, and shape of collagen fibrils/fibers. Genetic analysis revealed heterozygosity for a novel mutation in COL3A1 gene (c.3527G>A), confirming the diagnosis of vascular Ehlers-Danlos syndrome (VEDS). The patient died at 15 years of age because of aortic dissection. Vascular Ehlers-Danlos syndrome is a rare, life-threatening, autosomal dominant variant of EDS, resulting from mutations in COL3A1 gene. Affected individuals are prone to serious and potentially fatal complications, especially vascular, intestinal, and uterine ruptures. Delay in diagnosis is common, even when the clinical presentation is typical. Therefore, dermatologists should be familiar with VEDS features because the skin findings may be the first signs. Early diagnosis will improve management of visceral complications and allow early genetic counseling.     

The subbasement membrane distribution of type IV collagen in normal human skin

Samples of normal human skin were obtained from 48 sites in 26 subjects ranging in age from 2 to 85 years. The samples were examined by indirect immunofluorescence and immunoelectron microscopy using anti-human type IV collagen antibodies produced by immunizing rabbits with type IV collagen extracted from human placenta. Fluorescence was observed as granular or fine fibrous patterns, not only in the basement membrane at the dermo-epidermal junction, around the vessels, and the accessory organs of the skin, but also in the dermal regions in the vicinity of the basement membranes. This suggests the presence of type IV collagen in the dermis deep to the basement membrane. Ultrastructurally, the extrabasal lamina distribution of type IV collagen was noted as a partial distribution around the fibroblasts that existed close to the basal lamina. These findings are considered to be important in examining the function of this collagen in the dermis and the dynamics and metabolism of the basement membrane under normal and abnormal conditions.     

Increased expression of lysyl oxidase in skin with scleroderma

Summary Lysyl oxidase initiates cross-linkage of collagen and elastin by catalysing the formation of a lysine-derived aldehyde. In order to study cross-linking in scleroderma, we used monoclonal antibodies to lysyl oxidase to determine the localization of this enzyme in systemic and localized scleroderma, and compared the distributions obtained with that in normal skin. Using an indirect immunofluorescent antibody method and an avidin-biotinylated enzyme complex method. 11 cases of diffuse type of systemic scleroderma and seven cases of localized scleroderma were studied. In the oedematous stage of systemic scleroderma, intracellular and extracellular lysyl oxidase were remarkably increased in the dermis, particularly in groups around blood vessels. In the sclerotic stage of systemic scleroderma, lysyl oxidase was detected intracellularly in fibroblasts and extracellularly among collagen bundles between the lower dermis and the subcutaneous fat tissue. In localized scleroderma, a marked increase in lysyl oxidase was observed in mononudear cells and libroblasts near blood vessels in the lower dermis and in the subcutaneous fat tissue, in addition to the extracellular deposits between collagen bundles. The increase in lysyl oxidase in localized scleroderma was much more common than in the oedematous stage of systemic scleroderma. These findings indicated that intracellular and extracellular expression of lysyl oxidase expression was greater in sclerodermatous skin than in normal skin.     

Lipodermatosclerosis: a postphiebitic syndrome

A 30-year-old businessman, weighing 94 kg, presented with pigmentation and skin changes extending from the calf to the dorsum of the left foot. One year ago, the patient noticed erythema around the medial malleolus and edema of the ankle, following trauma while starting a bike. The patient was afebrile during the event. He gave no history suggestive of muscle weakness, cramping calf pains at rest, intermittent claudication while walking, paresthesia, or increased sensitivity to cold. No history suggestive of systemic involvement was obtained. On examination, the affected part showed pigmentary color changes varying from brown to bluish black (diffuse around the ankle and mottled towards the knee). The overlying skin was warm, indurated, brawny, and stony hard. Along with patchy hair loss, there was a painless decrease in the mobility of the left ankle. The circumference of the left calf and ankle measured approximately 2.5 cm less than the corresponding right side. The skin on the thigh above was hypertrophied and pachydermatous. There was no evidence of varicose veins. The blood count, urine analysis, erythrocyte sedimentation rate, bleeding, clotting, and prothrombin time were all within normal limits. Blood sugar levels were moderately elevated. Antinuclear-antibodies were absent. Histopathology of the skin biopsy showed uniform thinning of the epidermis, with focal areas of increased pigmentation in the stratum basale. A sparse perivascular lymphocytic infiltrate was seen around the superficial and deep vessels of the retioular dermis. The collagen bundles were closely packed, homogeneous, thickened, and deeply stained. The subcutaneous tissue was replaced with collagen fibers, thus resembling a scleroderma-like picture. Color Doppler showed evidence of segmental thrombosis in the left posterior tibial and peroneal veins. Before the dermatologic referral, the patient had been treated with intravenous heparin followed by tablet aspirin. We advised dietary restrictions for weight reduction and control of diabetes. In addition, we recommended vitamin supplements and compression stockings. The patient was taught limb care with special emphasis on leg elevation and exercise (to hasten the development of collaterals).     

Nevus lipomatosus cutaneous superficialis with perifollicular fibrosis

Nevus lipomatosus cutaneous superficialis (NLCS) is a rare hamartomatous skin lesion histopathologically characterized by the presence of mature fat tissue even within the upper dermis. Clinically, two types of NLCS can be distinguished; a multiple type and a solitary type. We here report a 10-month-old girl showing multiple type NLCS as a collection of a nodule and papules on her right abdomen. Histological examination revealed that the lesion was composed of a lobular proliferation of fat tissue throughout the dermis and immature hair follicle-like structures with perifollicular fibrosis. Histological alterations of the dermal connective tissue components were also seen, including thickening of collagen bundles and increased numbers of both fibroblasts and blood vessels. This is the first reported case of NLCS with perifollicular fibrosis.     

ATYPICAL CUTANEOUS HEMANGIOMA—A SKIN SIGN OF AORTITIS SYNDROME?

A 52-year-old man had an atypical cutaneous hemangioma on the right side of his neck. He had suffered from hypertension since he was 20 years old with marked differences in blood pressure in each arm. He had no other clinical symptoms. Angiography revealed that the patient had a typical aortitis syndrome which included obstruction of the left common carotid artery, stenosis of the left subclavicular artery, stenosis of both renal arteries and an irregular shadow of the abdominal aorta. On the area of the skin lesion there were a slightly dilated feeding artery and an obscure net work of arteries. No sign of direct A-V shunt was seen. This hemangioma could be pure capillary malformation in the angiographic classification by Merland et al. Renin activity and angiotensin I were high. Histopathological examination of the skin lesion revealed dilatation and enlargement of small veins in the upper dermis and increased arteries and veins of medium size from the deep dermis to subcutaneous fat tissue. This atypical cutaneous hemangioma is thought to be an important skin marker for this syndrome.     

PROGRESSIVE SYSTEMIC SCLEROSIS ASSOCIATED WITH CUTANEOUS AMYLOIDOSIS

A 43-year-old woman presented with a 3-year history of Raynaud's phenomenon and a six-month history of numbness in both arms. Sclerosis was noted on the entire body surface. The skin of the face was smooth and the lips were constricted (Fig. 1). The fingers and hands were atrophic and sclerotic, and full extension of the fingers and metacarpal joints was impossible (Fig. 2). There was pigmentation on the dorsal aspect of the hands. From the nape to the upper back, pruritic wavy, rippled or reticular pigmented macules in addition to sclerosis were noted (Fig. 3). Other parts of her skin did not show such a wavy pigmentation. Physical examination revealed no specific findings in the lung, heart, and abdomen. Neurologic examination was unremarkable. Motor function including muscle tonus was normal.
Laboratory studies disclosed that the complete blood count and tests of hepatic and renal function were within normal limits. Antinuclear antibody was I:80 and showed a speckled pattern, antitopoisomerase 1 antibody, anticen-tromere antibody, anti-Sm antibody and anti-RNP antibody were all negative, and the CH50 was 31.5 units/ml; C3 was 59.4 mg/dL; and C4, 17 mg/dL. Radiologically the chest and esophagus were normal.
Pulmonary function and electro-cardiogram were also normal. Histologic examination of a skin biopsy obtained from the upper back revealed that the collagen bundles throughout the dermis were thickened, homogenous, and closely packed. In the upper dermis, a small number of inflammatory cells around blood vessels was observed. Eosinophilic homogeneous masses were seen in the papillary dermis and upper dermis (Fig. 4). These homogeneous masses were positive to Dylan' (Fig. 5), Congo red, and thioflavin T staining. Therefore, the diagnosis of cutaneous macular amyloidosis was made.     

A Case of Acquired Smooth Muscle Hamartoma on the Sole

A smooth muscle hamartoma is a benign proliferation of smooth muscle bundles within the dermis. It arises from smooth muscle cells that are located in arrector pili muscles, dartos muscles, vascular smooth muscles, muscularis mammillae and the areolae. Acquired smooth muscle hamartoma (ASMH) is rare, with only 10 such cases having been reported in the English medical literature to date. Most of these cases of ASMH were shown to have originated from arrector pili and dartos muscles. Only one case was reported to have originated from vascular smooth muscle cells. A 21 year-old woman presented with a tender pigmented nodule, with numbness, on the sole of her foot, and this lesion had developed over the previous 18 months. The lesion showed no hyperpigmentation or hypertrichosis, and the biopsies demonstrated increased smooth muscle bundles in the dermis, and especially around the blood vessels. Moreover, the specimens stained positive with Masson trichrome stain and α-smooth-muscle actin antibodies, thus supporting our diagnosis of ASMH of the foot sole. Herein, we report on a rare case of ASMH on the foot sole, and this lesion originated from vascular smooth muscle cells. This type of case has not been previously described in the English medical literature.     

The patch stage of mycosis fungoides. Criteria for histologic diagnosis

It has long been claimed that a specific histologic diagnosis of mycosis fungoides cannot be made in the premycotic" or "eczematous" (patch) stage of the disease. Indeed, the histologic features of the premycotic lesions were constantly said to be those of "chronic non-specific dermatitis." We studied 46 biopsy specimens of patch lesions from patients in whom mycosis fungoides was unequivocally established by clinical events (i.e., concurrence or later development of typical plaque and/or nodular lesions) and indubitable histologic findings. We divided patch lesions into early nonatrophic patches and late atrophic ones. The early patches are considered to be evolving lesions of mycosis fungoides, whereas late patches represent resolving plaques of the disease. On the basis of this study, we concluded that histologic diagnosis can be made with near certainty in patch lesions of the disease. We found that the critical feature for histologic diagnosis of early and late patch lesions of mycosis fungoides is the presence of an increased number of mononuclear cells distributed singly or in small collections within an epidermis devoid of spongiotic microvesiculation. Other important features are lacunae surrounding intraepidermal mononuclear cells which gives them the appearance of "haloed cells." A sparse infiltrate of mononuclear cells is present around the blood vessels of the superficial, and sometimes the deep, vascular plexus. Atypical mononuclear cells are not necessary for the diagnosis of early patch lesions of mycosis fungoides, but they are found commonly in late patch lesions. Late atrophic patches show a thinned epidermis, loss of the usual configuration between rete ridges and dermal papillae, and coarse collagen throughout a thickened papillary dermis.     

反应性结节性增生1例

报告1例反应性结节性增生。患者男,57岁。左手无名指结节3年,无自觉症状。给予手术切除治疗。组织病理检查：真皮内有大量交叉和波浪状排列的成纤维细胞和胶原纤维,其周围无包膜,与周围组织分界不明显;边缘血管周围可见少量单一核细胞浸润。     

Atrophoderma of moulin with preceding inflammation

A 16-year-old Vietnamese man presented to the Dermatology Clinic with a 10-year history of bizarre brown patches, which initially started as red asymptomatic "bumps" on the trunk, upper and lower extremities, and face. His past medical history was significant for hypothyroidism and idiopathic urticaria. He was on Eltroxin for hypothyroidism. The family history was noncontributory. Physical examination revealed two types of lesion: erythematous, well-circumscribed papules in a linear configuration along with linear hyperpigmented atrophic patches following Blaschko's lines were noted on the lower extremities (Fig. 1), right upper extremity, right flank (Fig. 2), and right jawline. Initial biopsies taken from the papular lesions on the right thigh and right elbow revealed the following changes. The first biopsy showed a slightly thinned epidermis with prominent dilated blood vessels in the superficial dermis. There also appeared to be a slight increase in the amount of collagen in the deep dermis. The findings were reported as in keeping with "epithelial atrophy." The second biopsy from the lesion on the right elbow revealed an acanthotic epidermis. The granular layer was absent in several areas and there was marked overlying parakeratosis. In the dermis, there was a heavy perivascular lymphocytic infiltrate. The appearances were consistent with a psoriasiform dermatitis (Fig. 3). A biopsy taken from the left thigh approximately 18 months later showed slight irregular acanthosis with dermal edema, dilated blood vessels, and a patchy lymphocytic infiltrate. The appearances were compatible with mild inflammation.