Atypical acral persistent papular mucinosis.

A 70-year-old woman presented with a 10-year history of cutaneous lesions on her calves that worsened with solar exposure. Her past medical history was irrelevant. Physical examination revealed 10–15 erythematous, firm nodules with a diameter of 2–5 mm, which were slightly painful on palpation. The lesions were located on both calves, although some elements extended to the posterior side of the thighs ( Fig. 1 ). General physical examination did not show any abnormalities. A biopsy specimen from one of the elements showed a normal epidermis and, in the upper dermis, a slight inflammatory infiltrate of mononuclear cells, with the presence of splits between collagen fibers ( Fig. 2 ). Alcian blue stain at pH 2.5 revealed the existence of mucinous material in these spaces ( Fig. 3 ). An electron microscopic study did not show any abnormalities.

Figure 1 Open in figure viewer PowerPoint Clinical appearance of the lesions     

单侧性真皮中层弹性组织溶解症一例

患者女,38岁,颈右侧黄色丘疹伴带状萎缩20年。皮损缓慢发展,无明显症状。否认有长期日晒及家族史。皮肤科检查：颈右前侧皮肤见沿皮纹方向、大小不一的黄色丘疹、斑块;颈右后侧见多数以毛囊为中心的针帽大丘疹;整个颈右侧皮肤呈境界较清楚的带状萎缩斑,表面为细皱纹样改变,似老年人皮肤外观。颈部左侧皮肤大致正常,黄色丘疹处组织病理检查：表皮大致正常;真皮浅层血管周稀疏淋巴组织细胞及少量噬黑素细胞浸润,未见日光性变性改变;真皮中层胶原纤维增粗,排列紧密,未见明显炎细胞浸润。弹力纤维染色示真皮网状层弹性组织几乎完全缺失,剩余的少量弹性纤维肿胀、断裂明显。Von Kossa染色阴性。诊断：单侧性真皮中层弹性组织溶解症。     

会阴部单纯性获得性淋巴管瘤1例

患者女,56岁,外阴部丘疹、水疱10余年。体检:外阴部可见密集片状分布的粟粒大正常皮色丘疹和水疱。行皮损部分切除术,切除物组织病理示:表皮肥厚,真皮见囊性扩张淋巴管,有含淋巴细胞的腔隙。诊断:单纯性淋巴管瘤。     

Cutaneous collagenous vasculopathy in a pediatric patient

We report the case of a 13-year-old female who presented with punctate, erythematous macules coalescing into patches on the upper extremities and left thigh. A skin biopsy demonstrated dilated capillary-sized blood vessels in the papillary dermis consistent with a diagnosis of cutaneous collagenous vasculopathy (CCV). To our knowledge, this is the youngest patient to present with CCV and will represent the third pediatric case in the literature.     

SOLITARY TRUNCAL POROKERATOTIC ECCRINE OSTIAL AND DERMAL DUCT NEVUS IN A SIXTY-YEAR-OLD MAN

A sixty-year-old white man presented with a 2 × 1.5 cm lesion on his right buttock. The lesion had been present for several years, but had not been present when the patient was a child. No other family members reported having similar lesions. His past medical history was unremarkable. By history, the lesion was slowly growing, but was neither painful nor pruritic. On examination, a well defined pink nodule was noted on his right buttock. The surface of the lesion had a crateriform appearance with verrucous edges. A shave biopsy was performed.
Histopathology : The specimen was cut into four pieces and sections stained with hematoxylin and eosin were examined. A low power view (Fig. 1) shows an epidermis with considerable hyperkeratosis and several focal areas of parakeratosis that fill epidermal dilated eccrine ducts. Higher magnification (Fig. 2) reveals, that these foci of parakeratosis resemble the cornoid lamellae seen in porokeratosis of Mibelli. Figure 2 also reveals that the process originates within the eccrine duct. Higher magnification demonstrates the porokeratotic column arising from dyskeratotic keratinocytes that lack a granular layer (Fig. 3). An inflammatory infiltrate consisting of lymphocytes and histiocytes is present in the underlying dermis with accentuation directly under the cornoid lamellae.     

Lupus erythematosus profundus with unusual skin manifestation: subcutaneous nodules coexisting with eyelid plaques

A 71-year-old Japanese woman presented with erythematous plaques on the eyelids and subcutaneous indurations or nodules with or without overlying erythema on the hands, thigh, and leg. She also had oral ulcers, arthralgia and a low grade fever. Laboratory tests revealed an elevated titer of antinuclear antibody, an increased erythrocyte sedimentation rate and anemia. Skin biopsy specimens from the hand and thigh showed lymphocytic perivascular and periappendageal infiltrates and vacuolar alterations at the basement membrane zone of the skin appendages. Moreover, there was a dense lymphocytic infiltrate deep in the dermis with extension into the subcutaneous fat, which was compatible with the diagnosis of lupus erythematosus profundus. Although the biopsy specimen from the eyelid lesion did not contain the subcutaneous fat, the changes in the dermis were essentially the same as those of the hand and thigh. The eruption as well as the other symptoms promptly responded to oral prednisolone.     

Familial anetoderma

A 31-year-old Caucasian male presented with a history of erythematous, saccular outpouchings of the skin on his back, shoulders, and upper extremities (Fig. 1). The patient reported that his mother and aunt had a similar skin disorder, which initially began with inflammation, but healed leaving lax skin in its wake. He did not recall the name of the skin condition. Physical examination revealed large confluent zones of sac-like protrusions of erythematous skin on the back and shoulders. Histopathologic examination of the excisional biopsy revealed a relatively unremarkable epidermis. Perivascular lymphocytes were present in small numbers in the papillary dermis. Adnexal structures and deep dermis remained intact. The acid-orcein-Giemsa stain highlighted the absence of elastic fibers within the mid and lower reticular dermis (Fig. 2a,b), consistent with anetoderma.     

CIGARETTE PAPER ATROPHY

A 40-year-old West African woman was referred to me with a 12-month history of an asymptomatic patch over the right loin that had been gradually increasing in size. The patient was otherwise in good health, and the family history was negative. The lesion was linear, 7 cm long and 4 cm wide, porcelain-white, well-demarcated, and had a raised hyperpigmented border. The overlying skin was atrophic and wrinkled (Fig. 1). The patch also showed a central depression and a few dilated pilosebaceous duct orifices. She had no lesions elsewhere on her body. Laboratory studies showed no particular abnormalities. Histopathologic examination showed thinning of the epidermis, hyperkeratosis, and prominent follicular plugs. There was a band of hyalinized collagen below the epidermis and a zone of lymphocytic infiltrate below the zone of hyalinization. The above histopathologic features were highly diagnostic of lichen sclerosis et atrophiens (LSA).     

Eosinophilic annular erythema

Eosinophilic annular erythema is a rare benign recurrent disease, originally described in children, characterized by the recurrent appearance of persistent non-pruritic, urticarial annular lesions. Histologically a perivascular infiltrate composed of lymphocytes and abundant eosinophils in the dermis is exhibited. We report the case of a 15-year-old boy who presented with a 4-year history of recurrent flares of erythematous annular plaques on the trunk and extremities. The lesions resolved spontaneously after 3-5 weeks with no accompanying signs. A biopsy showed a mainly perivascular lymphocytic infiltrate with numerous eosinophils in the dermis.     

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.     

A Case of Generalized Lichen Sclerosus et Atrophicus

A 62-year-old female, with previous history of asthma and hypertension, presented with generalized hyperpigmented skin lesion, found a year ago. Physical examination revealed brown colored lichenified and sclerotic patches on the lower abdomen and flexural areas of extremities. Punch biopsy was performed and histopathological examination revealed hyperkeratosis, follicular plugging and thinning in epidermis. In dermoepidermal junction, cleft like space separating atrophic epidermis and dermis was seen. Also, lichenoid lymphocytic infiltration was observed in mid-dermis. Based on clinical and histopathological findings, a diagnosis of generlaized lichen sclerosus et atrophicus (LSA) was made. Other laboratory examinations were unremarkable. As there is no standard treatment for LSA, the patient received various treatments including topical steroid, tacrolimus and narrow-band ultraviolet B therapy. The skin lesion has softened and its color improved after treatment. LSA is defined as infrequent chronic inflammatory dermatosis with anogenital and extragenital manifestations. Generalized type is rare and genital involvement is the most frequent and often the only site of involvement. We report this case as it is an uncommon type of LSA with generalized hyperpigmented and sclerotic skin lesion in a postmenopausal female patient.     

Pagetoid Bowen''s Disease on the Breast

A 75-Year-old while woman was referred to Memorial Sloan-Kettering Cancer Center for evaluation of a slowly enlarging, asymptomatic plaque that had been present on the left breast for several years. She had developed hypothyroidism and hypertension. There was no personal or family history of skin cancer or broast disease. Results of mammography, performed 1 month earlier, were normal. Physical examination revealed an elderly, moderately obose white woman. Examination of the breasts did not reveal any masses, and there were no palpable axillary lymph nodes.
On the left breast, tbere was a well circumscribed, orangered plaque, 3 cm in diameter, with a peripheral collarette scale. The lesion was located 2 cm from the areola, in the medio-inferior quadrant of the breast (Fig. 1). A punch biopsy of the lesion showed atypical cells in the epidermis characterized by abundant pale cytoplasm (Fig. 2). Result of PAS stain for glycogen was only weakly positive, and results of all stains for mucin were negative. A lichenoid lymphohistiocytic infiltrate was seen in the dermis. A complete excision of the lesion was performed, revealing Bowen's disease. The specimen contained areas of pagetoid cells (Fig. 2) as well as more typical areas of Bowen's disease (Fig. 3).     

Lichen sclerosus vulvae

Vulvar lichen sclerosus (LS) is a chronic progressive skin disease of unclear etiology. It is often overlooked in early stages, but progresses to destructive atrophy and is associated with an increased risk of vulvar squamous cell carcinoma. The classical symptoms are pruritus and pain, but they are often not distinctive, so that unclear vulvar problems often lead to a biopsy. The histological picture of early LS is quite different from that of late LS with an atrophic epidermis, markedly sclerotic dermis and stiff dilated vessels. The epidermis in early LS is usually normal with only minor irregularities in the rete pattern. The basement membrane is normal or focally widened, while the edematous dermis has only scattered ectatic vessels. The often dense lichenoid and intraepidermal infiltrate explains the spongiosis and vacuolization of the basal layer keratinocytes. Very early cases may only have a sparse lymphocytic infiltrate and hyper-/parakeratosis of the follicular ostia. Early topical therapy can dampen the progression to atrophic, irreversible LS.     

Omenn's syndrome

A 7-month-old boy presented with a 6-month history of a skin eruption of the scalp and the diaper area (Fig. 1) resembling seborrheic dermatitis. Since the age of 3 months, he had developed high grade fever, diffuse pruritic skin lesions, stomatitis (Fig. 2), chronic diarrhea, diffuse lymphadenomegaly, and failure to thrive. The parents were nonconsanguineous and no similar disease had ever been reported in the family. Physical examination showed alopecia, generalized lymphadenopathy, and hepatosplenomegaly. The patient's skin was dry with generalized erythematous, scaling pruritic eruptions and small violaceous nodules on the extremities. Laboratory investigations revealed: slight normochromic anemia, leukocytosis (18,000/mm³) with lymphocytosis (42%), and mild eosinophilia (12%); decreased levels of all classes of serum immunoglobulins were observed with normal IgE levels. Examination of phagocytosis, opsonization, and granulocyte chemotaxis revealed no abnormalities. The number of circulating T cells was within normal limits, whereas that of B cells was low. Recall antigens and T- and B-lymphocyte proliferative response to mitogens and Candida albicans proteins resulted in poor stimulation. Chromosomal analysis of mononuclear ceils from the peripheral blood and skin fibroblasts showed a normal male Kariotype, and HLA typing did not reveal any maternal chimerism. A skin biopsy specimen showed satellite cell necrosis of Keratinocytes and a dense lymphohistiocytic and eosinophilic perivascular infiltrate in the dermis. A specimen from a lymph node showed a picture consistent with dermatopathic iymphadenopathy, with an increase of cells with histiocytic appearance and a dense infiltrate of eosinophils. On electron microscopic examination, no cells with Birbeck's granulations were found in the skin infiltrate and lymph nodes. A bone marrow biopsy was normal. One month later the patient developed generalized exfoliative erythroderma with recurrent cytomegalovirus infections and staphylococcal septicemia. Despite antimicrobial and prednisone therapy, the patient died of toxic shock at the age of 9 months. An autopsy showed diffuse lymphohistiocytic infiltration involving the skin and lymph nodes and extreme lymphocytic depletion in the thymus. Meningitis and pneumonia were also present.     

Two Japanese cases of lichen planus pigmentosus-inversus

Case 1 was a 51-year-old Japanese woman. She presented with an asymptomatic brown macule located on the right axilla of 2 months' duration. The smooth macule was 2 cm in diameter with a sharp demarcation (Fig. 1A). Case 2 was a 62-year-old Japanese man. He presented with asymptomatic, symmetric, gray-brown macules located on the groin, axillae, and popliteal region of 6 months' duration. The smooth macules were several millimeters to centimeters in diameter and sharply demarcated (Fig. 1B). Oral or nail lesions, previous inflammatory processes in affected areas, and internal malignancies were absent. A causal relationship with drugs, recent sun exposure, or trauma could not be identified. Findings for work-up, including blood cell count, fasting blood sugar levels, liver function, serum electrolyte levels, serum electrophoresis, urinalysis, antinuclear antibodies, and serological examinations for human hepatitis viruses and syphilis, were within normal limits or negative. The lesions gradually disappeared without medication within 6 months. Biopsy specimens showed a lymphocytic infiltrate with basal vacuolar changes and prominent melanin incontinence in the upper dermis (Fig. 2A). The band-like lymphocytic infiltrate was moderate in Case 1 and mild in Case 2. Immunohistochemistry showed infiltrative CD8(+) T lymphocytes with keratinocytic damage, indicating cytotoxic injury of the keratinocytes (Fig. 2B). Both the epidermis and the upper dermis contained CD1a(+) cells (Fig. 2C). The keratinocytes focally and weakly expressed HLA-DR (Fig. 2D). These findings were identical in samples from both patients.     

Annular lichen planus involving the esophagus: a rare diagnosis

A 49‐year‐old woman presented with a 2‐year history of a sore mouth, annular, atrophic patches, and chronic ulcerative vulvovaginitis. For 6 years, she had suffered from dysphagia to solids which was increasing in severity. There was no past history of heartburn or other symptoms of esophageal reflux. Mucocutaneous examination showed white erosive patches on the buccal mucosa, cicatricial alopecia, dystrophic nails, and annular atrophic pigmented plaques localized on the trunk ( Fig. 1 ). Genital examination showed atrophic and sclerotic vulvovaginal lesions with synechia. Cutaneous biopsy showed an atrophic epidermis, a dense lymphocytic infiltrate in the upper dermis with degeneration of the basal epithelium, and Civatte bodies. Serologic tests for hepatitis B and C were negative. A diagnosis of cutaneous annular lichen planus with nail, scalp, oral, and genital involvement was made.

Figure 1 Open in figure viewer PowerPoint Annular, atrophic, and pigmented plaques localized on the back     

过敏性紫癜合并急性播散性脑脊髓炎

报告1例过敏性紫癜并发急性播散性脑脊髓炎。患者女,31岁。躯干及四肢反复瘀点和瘀斑2余年。体格检查:双侧眼球外展受限,颈软,肌力和肌张力均正常,脑膜刺激征(-),双侧Chaddock征(+),右侧抓握反射(+)。皮肤科检查:四肢、腹部、腰部及背部多发对称性分布的紫红色斑疹及斑丘疹,部分融合成片,皮损压之不褪色。皮损组织病理检查:表皮大致正常;真皮浅中层细血管周围及胶原束间小片状淋巴细胞浸润,伴稍多中性粒细胞及核尘,部分血管管壁模糊,可见红细胞漏出。头颅磁共振成像(MRI)示两侧大脑半球、基底节区及右侧桥臂多发脱髓鞘病变。诊断:过敏性紫癜合并急性播散性脑脊髓炎。     

Ecthyma gangrenosum without pseudomonas septicemia in a kidney transplant recipient

We report a case of ecthyma gangrenosum (EG) without septicemia in a renal transplant recipient who presented with a 1-month history of painful ulcers, vesicles and bullae on the face and extremities. Histopathological findings revealed subepidermal bullae covered by a necrotic epidermis containing an infiltrate of a moderate number of lymphocytes, neutrophils and necrotic collagen. Many dilated and congested capillaries were also present due to thrombi beneath the bullae, with alteration of collagen fibers through the superficial to middle dermis with some infiltrate. A culture from the ulcers revealed the presence of Pseudomonas aeruginosa and methicillin-resistant Staphylococcus aureus, whereas the results of repeated blood cultures were negative. The ulcers were completely cured by early appropriate i.v. antibiotic therapy with granulocyte colony-stimulating factor, without progression to EG with septicemia. An immunocompromised state due to immunosuppressive drugs, in addition to diabetes mellitus, hypogammaglobulinemia and hypoproteinemia, may have caused the EG and herpes zoster may have exacerbated the condition.     

Acne in Becker''s nevus

An 18‐year‐old man presented with a progressively enlarging, asymptomatic, well‐demarcated, irregular, hyperpigmented patch, 13 cm × 11 cm, on the anterior aspect of the chest below the left clavicular region, with a few small macules at the periphery, of 2 years duration. After 18 months, multiple, discrete, erythematous papules and comedones developed over the patch ( Fig. 1 ). A few melanocytic nevi were present in and around the patch. A clinical diagnosis of Becker's nevus with acne vulgaris was made. A skin biopsy from the patch showed acanthosis, elongated rete ridges, increased pigmentation in the basal layer, and a mild perivascular lymphocytic infiltrate in the upper and lower dermis with few melanophages in the dermis. Histopathology of the comedone revealed slight acanthosis, irregular elongation of the rete ridges, and hyperpigmentation of the basal layer of the epidermis. Multiple melanophages were seen in the upper dermis. In the mid‐dermis, a ruptured closed comedone was seen. The follicular lumen contained a plug of loosely arranged keratinized cells, sebaceous material, and bacterial colonies. Aggregates of neutrophils were found at the site of rupture of the follicular wall. There was a mild perivascular lymphocytic infiltrate in the upper and lower dermis ( Fig. 2 ). These features confirmed the diagnosis of Becker's nevus with acne vulgaris. The acne was treated with 0.05% tretinoin and 2% erythromycin creams. After 2 months of treatment, all the acne lesions subsided. On further follow‐up, the patient had relapse of the acne lesions over the patch with the discontinuation of treatment. A few new lesions also appeared on the face. The treatment was restarted, but the patient was lost to follow‐up.

Figure 1 Open in figure viewer PowerPoint Multiple, discrete, erythematous papules and comedones over Becker's nevus with a few melanocytic nevi in and around the lesion     

Epstein-Barr virus-associated lymphoproliferative eruption with progression to large granular lymphocytic leukaemia

A 12-year-old Korean girl gave a 9-year history of recurrent necrotizing papules and vesicles on the face, scalp and extremities. Skin biopsy specimens showed an atypical lymphoreticular infiltrate with vasculitis in the dermis and subcutis. In situ hybridization demonstrated latent infection by Epstein-Barr virus (EBV) of the lymphoid cells in the dermis. The disease was diagnosed as an EBV-associated lymphoproliferative skin eruption presenting as recurrent necrotic papulovesicles. The patient subsequently developed large granular lymphocytic leukaemia of natural killer cell origin. Our observations suggest that a patient with an EBV-associated lymphoproliferative skin eruption presenting with recurrent necrotic papulovesicles might progress to develop leukaemia as well as lymphoma.