Two cases of multiple eccrine spiradenoma with linear or localized formation

We report two rare cases of recurrent, multiple eccrine spiradenoma. Both cases presented with extensive lesions comprised of multiple red papules of various sizes and a soft blue-red nodule. The first case was a 30-year-old woman. Her lesions followed a linear arrangement on her chin, and extended down the right side of the neck with spontaneous pain. The second case was a 57-year-old woman with tumors in a localized group on the left occipital region without pain. A search of the literature revealed only 15 reported cases of linear/zosteriform/nevoid multiple eccrine spiradenoma. Both cases were treated by surgical excision. Most of the red papules displayed typical histological features including two cell types: large clear cells with low-density cytoplasm; and small dark cells with high-density cytoplasm. The large soft tumors exhibited a variable histological appearance. In the first case, the cystic tumors displayed an homogeneous structure comprised of eosinophilic material. In the second case, the cystic tumors included abundant interstitial tissue.     

毛囊黏蛋白病一例

患者女,60岁,因面部结节、斑块1年余就诊.患者以面部结节和浸润性斑块为皮损表现.皮肤科检查:前额见皮色和淡红色结节,左下颏见浸润性斑块,上覆黄色痂皮.组织病理检查示,真皮及皮下血管、附属器周围以淋巴细胞为主的炎症细胞浸润,毛囊上皮网状变性,并形成囊腔,经阿新蓝染色见囊腔中大量黏蛋白沉积.治疗方面给予阿维A和泼尼松口服,局部外用莫米松乳膏联合异维A酸凝胶等治疗,2个月后痊愈.     

Congenital blaschkoid eccrine spiradenoma on the face

Eccrine spiradenoma is an uncommon tumour of the sweat gland(s) and generally occurs as a solitary small painful nodule. Very rarely, it may present as multiple tumours arranged in a linear/blaschkoid pattern. An 8-year-old girl had many papulonodular lesions, arranged in a linear pattern on the left half of the face, involving the forehead and lateral side of the nose. The lesions were along Blaschko's lines. Lesions were reported to be present at birth; no family member had similar lesion(s), either in solitary or multiple form. Histopathologic examination of a biopsy specimen revealed characteristic features of eccrine spiradenoma. The case is reported because of the rare occurrence of this presentation.     

MULTIPLE ANGIOKERATOMAS WITH ZOSTERIFORM DISTRIBUTION IN TWO SISTERS

Case 1 : At the age of 12 years a 28 year-old woman noticed multiple reddish papular lesions on the left buttock and thigh. The lesions gradually spread to involve her left thigh towards the knee. The patient had no systemic symptoms, Examination of the skin revealed 23 well-circumscribed, purplish verrucous lesions each of 2–3 mm diameter, located over the left buttock and extending in a linear fashion to the popliteal flexure (Fig. 1). General examination and routine laboratory studies were normal.
Case 2 : A 27-year-old woman, sister of the previous patient, noticed at the age of 10 years a steadily increasing number of purple lesions on her left buttock that gradually extended to the left posterior thigh. The lesions were asymptomatic, although the patient noticed occasional bleeding with trauma. The lesions of the thigh had disappeared spontaneously without scarring during the previous 4 years. When we first examined this patient, at the age of 27, she had eight reddish papular hyperkeratotic lesions, 2–3 mm in size, with a linear zosteriform distribution over the left buttock. She was healthy and symptom free. Routine laboratory studies were normal.
An excision biopsy was taken from each sister. The histopathologic examination revealed similar findings in both lesions. Hyperkeratosis with slight acanthosis and papillomatosis was present. Greatly dilated capillaries were seen in the upper and middermis. The capillary walls were lined by flattened endothelium. In one area of the lesion from the second patient there was intravascular thombosis (Fig. 2).     

Seborrheic keratosis that follows Blaschko's lines

A 66-year-old Japanese man had skin lesions on the left side of his trunk and the left upper extremity for approximately 10 years. The skin lesions were asymptomatic but increased gradually, which brought the patient to our hospital. On the initial examination, we noticed 5 mm x 5 mm to 15 mm x 5 mm, round or oval, light to dark brown keratotic papules on the left side of chest and abdomen and the left upper extremity. The papules were aligned in an S-shaped line on the trunk and in a straight line on the upper extremity. Clinical and histopathological findings led to a diagnosis of seborrheic keratosis that followed Blaschko's lines. To our knowledge, no such a case has been reported previously. Our case supports the hypothesis that seborrheic keratosis can be associated with genetic mosaicism.     

TUBERCULOSIS VERRUCOSA CUTIS ASSOCIATED WITH LUPUS VULGARIS

A 68-year-old peasant had an 8-year history of tuberculosis verrucosa cutis. After an injury the patient developed a pustule, which extended slowly and formed a plaque. New lesions appeared and increased in number and size. He was treated with various antibiotics without any significant effect. Three months previously a dull-red plaque developed on the chin without any complaints from the patient. The patient denied any history of tuberculous infection. The examination of the left foot revealed a verrucous plaque over the medial surface. There were firm, raised, and verru-cous papules, which exuded yellow pus. Similar lesions were present on the edge of the sole. The plaque was re-stricted by a margin of crusted papules. The affected nails were thick, keratotic, and damaged (Fig. 1). On the skin of the chin a single lesion was present, 1 × 2 cm in size, flat, irregular, dull-red in color and firm, without any secretion or accompanying symptoms (Fig. 2). Histology from the lesion of the foot revealed: necrosis in the superficial stratum corneum with numerous spongio-form pustules around and beneath the necrotic areas; marked acanthosis reaching pseudocarcinomatous hyperplasia and papillomatosis; and pronounced mononuclear infiltrate without tuberculid structures. Ziehl-Neelsen staining for mycobacteria was negative (Fig. 3). Histology from the lesion of the skin revealed: crusted parakeratosis and acanthosis and pronounced band-like infiltrate in the upper and middermis with well-formed tuberculoid structures and numerous giant cells. In the deep dermis the infiltrate was predominantly perivascular and periadnexial with tuberculoid structures in some areas. The collagen bundles were hyalinized (Fig. 4). The fluorescent staining with auramine revealed mycobacteria. (Fig. 5). The routine laboratory findings were within the normal limits except for a mild elevated ESR of 45 mm. Mantoux-test with 10 Ul of PPD showed induration with purple red surface (Fig. 6). The patient was x-rayed, but another tuberculous focus could not be detected. Treatment with isoniazid and rifampin was applied with rapid results (Fig. 7).     

Multiple Segmental Eccrine Spiradenoma with a Zosteriform Pattern: A Case Report and Literature Review

Eccrine spiradenoma usually occurs as solitary tender nodules. However, it rarely occurs as multifocal localized tumors or has zosteriform distribution. We report the case of a 32-year-old woman with a 20-year history of itchy lesions on the left side of the back and forearm with a zosteriform distribution. Before disease onset, almost no patients have inducing factors, but our patient received an injection beforehand; however, whether this was coincidental or causative remains unknown. The lesions became very itchy after perspiration or eating spicy food, which has never been reported. A literature search revealed 22 cases of multiple segmental eccrine spiradenoma; we summarized the clinical characteristic in order to aid diagnosis and treatment selection.     

Case of the Brooke-Spiegler syndrome

A 36-year-old woman presented with lesions on her scalp, face and trunk. Histopathological examination of these lesions demonstrated facial trichoepithelioma, and scalp cylindroma. A solitary nodule on the trunk had features of cylindroma, spiradenoma and trichoepithelioma, a previously unreported occurrence. Based on the clinical picture, the diagnosis of Brooke-Spiegler syndrome was established. Genetic studies confirmed the diagnosis, demonstrating a splice site mutation, designated 1518+2T>C, on the CYLD1 gene of chromosome 16q12-q13.     

Cutaneous and paranasal aspergillosis in an immunocompetent patient

A 26-year-old Libyan woman presented with asymptomatic nodulo-ulcerative skin lesions present for 1 year. Three years prior to presentation, she had experienced a nasal discharge followed by the development of a nodule in the nasal cavity and a plaque on the hard palate. These lesions had gradually increased in size and ulcerated, resulting in perforation of the nasal septum and palate. Two years later, the patient noticed the appearance of skin lesions: a nodule on the right thumb and numerous nodulo-ulcerative lesions on the extremities. General physical examination was normal with no significant lymphadenopathy. Examination of the oral cavity revealed perforation of the distal nasal septum, with a perforated nodular plaque involving the entire palate, associated with subluxation of the upper incisors (Fig. 1a). On skin examination, multiple firm nodules and nodulo-ulcerative lesions with a central eschar and raised margins were observed. The lesions ranged in size from 0.5 to 5 cm and were distributed on the right hand and fingers, left upper arm (Fig. 1b), left calf, and right thigh. Routine laboratory investigations (liver function tests, serum calcium, electrolytes, lipid profile, urine and stool culture studies) were normal. Immunoelectrophoresis disclosed normal levels of immunoglobulins IgG, IgA, and IgM. Serologic studies for human immunodeficiency virus (HIV) and syphilis, and a tuberculin test, were all negative. A Giemsa-stained tissue smear was negative for Leishmania tropica organisms. Radiological studies disclosed a slight haziness of the maxillary sinuses with perforation of the nasal septum. A chest X-ray was normal. Histopathologic examination of biopsies taken from both the palate and from ulcerated and nonulcerated skin lesions was performed, and all showed similar findings. The biopsy of a nonulcerated skin lesion showed pseudoepitheliomatous epidermal hyperplasia with neutrophilic microabscesses (Fig. 2a). A dermal diffuse and nodular granulomatous mixed infiltrate of lymphocytes, histiocytes, giant cells, numerous eosinophils, and neutrophilic microabscesses was seen in all tissues examined. Septate hyphae were present both within giant cells and free in the dermis (Fig. 2b). The hyphae were branching at a 45 degrees angle and were positive on periodic acid-Schiff and Grocott methenamine silver stains (Fig. 2c). Fungal culture studies of material taken from an ulcerated skin lesion grew Aspergillus flavus. Blood cultures were negative for Aspergillus sp. or other microorganisms. The patient was treated with intravenous amphotericin B, but the medication was discontinued due to her intolerance to the drug. She was subsequently lost to follow-up.     

Alopecia areata associated with regression of cutaneous melanoma

A 41-year-old white female with a past medical history of hypothyroidism and alopecia universalis presented on January 24, 2002 with a recently changing mole. She indicated changes in size and color of the superior aspect of a mole that had been present for more than 8 years. She had approximately 20 lifetime peeling sunburns due to being a lifeguard. No family or previous personal history of skin cancers, including melanoma or atypical nevi, was reported. Her history of alopecia universalis began 12 years previously and has partially resolved with remaining patchy alopecia of the scalp and eyebrows. On diagnosis of alopecia universalis, she was initially treated with oral prednisone for 1 year and topical minoxidil for 3 months. Currently, she is not being treated for this condition. She denied other previous skin conditions. She had a surgical history of tonsillectomy at the age of 7 years. Her current medication includes levothyroxine (0.015 microg) for hypothyroidism diagnosed 12 years previously. She reported no known drug allergies. During the initial physical examination, she presented with phototype II skin with two adjacent pigmented lesions on her left foot within a 1.3 cm square. The first lesion on the left posterior distal heel was an irregular, brown-black, 0.5 x 0.6 cm macule. The second lesion, on the left posterior proximal heel, was an irregular, brown, speckled, 0.3 x 0.4 cm macule (Fig. 1). The patient had ophiasis of the scalp and total alopecia of the bilateral eyebrows. In keeping with the patient's wishes, alopecia lesions were not biopsied and clinical photographs of the alopecia are not included in this article. Two 3 mm punch biopsies were performed within each lesion. The left posterior proximal lesion showed malignant melanoma, with a Breslow depth of 0.4 mm, anatomic level II, marked lymphocytic response and partial regression (Fig. 2). The left posterior distal lesion showed malignant melanoma in situ, arising in a lentiginous compound nevus, with architectural disorder and cytological atypia. These two lesions were concluded to be one lesion with clinical regression. She underwent local excision with 1-cm margins and sentinel lymph node biopsy owing to the presence of regression, which showed no evidence of metastatic melanoma. Lactate dehydrogenase and chest X-ray were within normal limits. The alopecia areas were not biopsied previously or at that time.     

Multiple linear eccrine spiradenomas with eyelid involvement

Eccrine spiradenoma is an uncommon tumour of the sweat gland and usually occurs as a solitary small painful nodule. Very rarely, it may present as multiple tumours arranged in a linear/zosteriform fashion. We present the case of a 23-year-old female who had multiple spiradenomas arranged linearly from the midline of forehead to the left corner of the mouth and a few lesions also on the margin of the left eyelid. The lesions were probably along the Blaschko's lines. Tumours were excised surgically in stages. Literature search revealed only 13 reported cases of linear/zosteriform/naevoid multiple spiradenomas and only four cases of solitary spiradenoma of the eyelid. The review of reported cases suggests that arrangement of tumours in a linear/zosteriform pattern is an important diagnostic clue for multiple (naevoid) spiradenomas.     

Preliminary communication: imiquimod in mixed capillary/lymphatic malformation

The present authors reported a 14-year-old white boy who visited the present authors' dermatology department in January 2004. Physical examination revealed multiple translucent and hemorrhagic vesicles and skin-colored nodules on the chin. The lesion had grown slowly in size over the previous 7 years. The objective of this study is to estimate the exact mechanism of action of topical imiquimod on mixed capillary/lymphatic malformation. After 4 weeks of therapy the lesions were less protuberant. At the follow-up examination after a further 2 months of therapy, there was partial clinical regression of the capillary component with a return to normal skin color. One month after termination of therapy the lesions had completely regressed and there was no evidence of recurrence of the hemangiomatous section. The present authors' case suggests the efficacy of the use of topical imiquimod and this therapeutic modality may be of particular benefit in superficial type of capillary/lymphatic malformation, in which the destructive intervention may be undesirable.     

Multiple eccrine spiradenomas in a linear nevoid pattern

A 16-year-old boy presenting with multiple slightly painful, bluish red papules and plaques on the left thorax is reported. The skin lesions were arranged in a linear and arched pattern following the lines of Blaschko. Clinical examination led to the suspicion of multiple leiomyomas, syringomas or glomus tumours. Histological examination revealed that the lesions were typical benign eccrine spiradenomas.     

Papular umbilicated granuloma annulare in association with Alagille syndrome

A 10-year-old white boy with Alagille syndrome was referred to the dermatology clinic for evaluation of an eruption on the lower extremities. The lesions were mildly pruritic, gradual in onset, and increased in number over several months. The patient reported no insect bites, no unusual contacts, no history of chronic dermatologic disorders, and no recent febrile illness or systemic complaints. He had Alagille syndrome with cholestatic liver disease, hyperlipidemia, pulmonary artery stenosis, retinal pigment abnormalities, and myopia. His medications included rifampin and phenobarbital. There was no family history of Alagille syndrome; however, the patient's father had retinal cone abnormalities and a half-sister had died of Aicardi syndrome.
Physical examination revealed typical facies with hypertelorism, deep set eyes, and a sharply pointed chin. Dermatologic examination showed firm umbilicated papules symmetrically distributed on the dorsa of both feet and ankles (Fig. 1).
The differential diagnosis included xanthomas, prurigo papules, and molluscum contagiosum. A skin biopsy revealed palisading necrobiotic granulomas consistent with granuloma annulare (Fig. 2).     

Progressive facial hemiatrophy (Parry-Romberg syndrome)

A 48-year-old white woman presented with a 2-year history of progressive facial hemiatrophy involving the right side of the chin and tongue, associated with mastigatory spasm. Neurological examination showed no abnormalities. Computed tomography and magnetic resonance imaging of the brain and electrophysiological investigations were normal. Histopathological examination of a skin biopsy specimen from the chin revealed atrophy of the subcutaneous fat with homogenization of dermal collagen fibres. Phenytoin 100 mg t.d.s. relieved the mastigatory spasm.     

Melanoma in private practice: Do dermatologists make a difference?

Malignant melanoma is a major contributor to Australian morbidity and mortality. In this era of resource rationalisation, we seek to address the issue of whether routine full-skin examination by a dermatologist, rather than focussed examination of flagged lesions, will increase melanoma diagnosis. A retrospective chart review was undertaken between 1 July 2007 and 30 June 2008 in a private dermatology group practice in order to ascertain the number and characteristics of incidentally detected melanomas on routine skin examination. A total of 94 melanomas were detected during this 12-month period. Of these, 57 (60.6%) were incidentally detected by the dermatologist, 41 (71.9%) were in situ melanomas and 16 (28.1%) were invasive melanoma. Of the invasive lesions, 15 (94%) were 'thin' (less than 1.0 mm Breslow thickness). The majority of melanomas were found in men, and were distributed in areas of high cumulative sun exposure. Nine (9.6%) lesions were clinically misdiagnosed by the dermatologists and picked up on histopathology. This audit reaffirms the usefulness of routine full-skin examination by dermatologists in detecting de novo melanoma as part of the global strategy in reducing the burden of melanoma in Australia.     

Nail abnormalities and scleroderma-like lesions on the face associated with systemic amyloidosis

INTRODUCTION: Mucocutaneous involvement in systemic amyloidosis occurs in 29 to 40 p. 100 of cases. Nail abnormalities are infrequent in AL amyloidosis. We report an original case of AL amyloidosis associated with cutaneous and integument alterations and scleroderma-like infiltration of the face. CASE REPORT: A 73 year-old woman was hospitalized because of weight loss and asthenia. She had been treated 4 years earlier with chemotherapy for a IgG-type multiple myeloma with complete resolution of the underlying monoclonal gammapathy. Cutaneous examination showed nail dystrophy of all fingernails associated with scleroderma-like skin changes on the chin and lips. Histopathologic study of a chin biopsy confirmed the presence of amyloid deposits in the dermis. Laboratory data were normal, without signs of recurrence of multiple myeloma. DISCUSSION: We report an original case of a patient who developed two unusual cutaneous manifestations associated with AL amyloidosis. Moreover, there was no correlation between the severity of the cutaneous lesions and the extent of the underlying hematological disease.     

Screening for skin cancer in The Netherlands.

In the summer of 1989 a screening campaign for skin cancer was organized along part of the beach in the western region of The Netherlands, using a mobile trailer. On 4 consecutive Saturdays, 3,069 individuals were examined. A total of 65 individuals with a suspected lesion were found and referred to their general practitioner. The compliance with referral was 80%. Histological reports, obtained from 46 suspected lesions, showed: 6 melanomas (all with a thickness less than 1 mm), 2 squamous cell carcinomas, 23 basal cell carcinomas, 5 dysplastic naevi and 10 benign skin lesions. The positive predictive value of the visual examination appeared to be 83%. The campaign attracted much publicity. The effects of this publicity were measured by a questionnaire sent to all general practitioners and dermatologists in the region. It appeared that during the campaign and in the ensuing 2 months there had been an increase in the number of diagnoses of benign skin lesions and a moderate increase in the diagnoses of malignant lesions.     

Agminated histiocytomas in a 23-year-old patient

A 23-year-old patient presented with a group of 17 reddish-brown papules and nodules on the left shoulder lasting for 4 years. The histopathologic examination after a punch biopsy was repetitively consistent with the diagnosis of fibrous histiocytoma (FH). We use the term agminate histiocytomas for the first time to stress the presence of grouped lesions in one skin segment. Intralesional corticosteroids and cryotherapy were partially successful in this patient.     

Skin vascular lesions: A new therapeutic option with sequential laser‐assisted technique

Treatment of skin unaesthetic vascular lesions is still suboptimal. In this article, we present a multicenter study evaluating the efficacy and safety of the sequential 755 nm Alexandrite and 1064 nm ND:YAG lasers for the treatment of acquired and congenital vascular lesions of the skin. A total of 171 patients were included in the study (141 female and 30 male), median age 38, skin type from I to IV. Numbers of patients categorized for treated disease: 22 spider angiomas, 67 facial telangiectasia/spider veins, 58 leg telangiectasia/reticular leg veins, 24 port wine stains (PWS). Of those lesions 30.7% was on legs, 63.7% on face (6.7% on forehead, 10.1% on temples, 21.7% on cheeks, 25.2% on nose, 1.7% on chin) and 5.6% on chest. Spider angiomas required from 1 to 3 treatments. Facial telangiectasias had been treated from 1 to 4 times. Leg telangiectasias needed from 2 to 6 treatment sessions. Regarding PWS, it takes from 6 to 9 laser treatment to reach the clearance of the lesion. All the vascular disorders treated showed marked improvement while side effects were limited to low pain sensation and transitory fenomena. This sequential treatment is an effective, safe, and new approach for unaesthetic skin vascular lesions.