Multiple keratoacanthomas in a young woman: report of a case emphasizing medical management and a review of the spectrum of multiple keratoacanthomas

A 27-year-old white woman was referred for consultation with regard to the presence of extensive multiple keratotic lesions. She began to develop these lesions at the age of 9 years, with healing of the lesions resulting in scar formation. A biopsy was performed at the age of 16 years, but the patient was unsure of the results. Since then, she had not had any treatment or biopsies, and stated that she had not suffered from any health problems during the intervening period. She was most concerned about the tumors on her heels and soles, which caused difficulty with ambulation. The family history was negative for skin diseases, including melanoma, nonmelanoma skin cancer, psoriasis, and eczema, and positive for Type II diabetes mellitus. A relative reported that the patient's grandfather had similar lesions, but the patient's parents and siblings were healthy. She was married and had one child, a 9-year-old daughter. Her child had no skin lesions. The patient's only medication was Ortho-Tricyclene birth control pills. She had no known drug allergies. Physical examination revealed the presence of multiple lesions on her body (Fig. 1). Her left superior helix contained a well-demarcated, dome-shaped nodule with a rolled, mildly erythematous border with a central hyperkeratotic plug. A similar lesion was present in the scaphoid fossa of the left ear and smaller lesions were scattered on her face. Numerous lesions were present on the arms and legs bilaterally, with the majority of lesions being located on the anterior lower legs. There were also lesions present on the palms and soles. The lesions ranged in size from 5 mm to 3 cm, the largest being a verrucous exophytic nodule on the anterior aspect of her left leg. Overall, there appeared to be two distinct types of lesion. One type appeared round, oval, and symmetric with a central keratotic plug, similar to that on the ear. The other type was larger, more exophytic, and verrucous, including the lesions on the volar surfaces. Also present were numerous, irregularly shaped atrophic scars where previous lesions had healed spontaneously. There were no oral lesions or lesions on her fingernails or toenails, and her teeth and hair were normal. A biopsy was obtained from an early lesion on the right dorsal forearm. Histology revealed an exo-/endophytic growth having a central crater containing keratinous material (Fig. 2). The crater was surrounded by markedly hyperplastic squamous epithelium with large squamous epithelial cells having abundant glassy cytoplasm. Some cells were dyskeratotic. Within the dermis was a dense, chiefly mononuclear inflammatory infiltrate. A buttress of epidermis surrounded the crater. The clinical and pathologic data were consistent with keratoacanthomas. Initial laboratory screenings revealed elevated triglycerides and total cholesterol, 537 mg/dL (normal, < 150 mg/dL) and 225 mg/dL (normal, < 200 mg/dL), respectively, with all other laboratory results within normal limits. In anticipation of starting oral retinoid therapy for her multiple keratoacanthomas, she was referred to her primary care physician for control of hyperlipidemia. After her lipids had been controlled, she was placed on isotretinoin (Accutane) 40 mg/day. There was some interval improvement with regression of some lesions leaving atrophic scars. She was also started on topical application of tazarotene (Tazorac) for all nonresolving lesions. Possible side-effects from the isotretinoin occurred, including dry mouth and eyes. After 8 months of isotretinoin, the patient was switched to acitretin (Soriatane) 25 mg to determine whether it might have a more beneficial effect on the resistant lesions. Many of the larger lesions regressed leaving atrophic scars. The dose of acitretin was subsequently increased to 35 mg because the lesions on her heel and the ball of her foot persisted. Almost all of the lesions resolved, except those on her feet, which are slowly regressing. Currently, the patient is on a regimen of acitretin 25 mg once a day with tazarotene 0.1% gel applied directly to the few residual keratoacanthomas on her feet, which are slowly improving.     

Epidermodysplasia verruciformis: association with isolated IgM deficiency and response to treatment with acitretin

We describe a 25-year-old woman, who had extensive, large viral warts consistent with epidermodysplasia verruciformis (EV) since she was 6-year-old. Laboratory studies revealed an isolated IgM-deficiency, but the patient demonstrated no other abnormalities. She was treated with oral acitretin (0.5-1 mg/kg/day) for six months and her skin lesions improved slightly. However, after discontinuing the treatment, the lesions came back but she declined further treatment.     

Lymphomatoid papulosis and FK 506

A 53-year-old woman underwent an orthotopic liver transplant in Pittsburgh in October 1990. She had been suffering from chronic hepatitis C that had evolved into cirrhosis with ascites, jaundice, and encephalopathy. In April 1991 she required reconstruction of the biliary anastomosis because of stricture, and in October 1991 she underwent a liver biopsy because of mild elevation of alkaline phosphatase. The biopsy revealed mild chronic rejection and changes consistent with viral hepatitis. She was on oral FK 506, 4 mg b.i.d. In November 1992 she developed recurrent self-healing erythematous, papulonodular lesions on the chest and shoulders (Pig. 1). The lesions were purplish red, mildly pruritic, and undergoing vesiculation on the top. The lesions ranged from 3 to 5 mm in size, and a few showed ulceration and necrosis and were healing with hypopigmented scars. The lesions gradually increased in number and size. A trephine biopsy specimen was obtained from the lesional skin. The tissue was prepared for light microscopic study by fixing in 10% formaldehyde solution and staining with hematoxylin and eosin. Immunohistochemistry was carried out for lymphoid markers. In February 1993, a papulonodular lesion near the left axilla enlarged to a size of 10 × 10 cm; it was ulcerated and necrotic. A biopsy was taken and sent for histopathology and immunohistochemistry. The patient continued on FK 506, 4 mg b.i.d. Many of the early lesions had become purpuric, eroded, and healed with scarring; however, fresh lesions continued to appear. A decision about modification of the patient's immunosuppressive medication was left to the Transplant Team; however, there was concern that her lymphomatoid papulosis may be a side-effect of her PK 506 treatment. Laboratory investigation revealed a normal peripheral blood film. Liver enzymes and a renal profile were within normal limits. The results of a bone marrow study, liver and spleen scan, and endoscopic examination of the gastrointestinal tract were normal. A skin biopsy revealed a nodular and wedge-shaped dermal infiltrate involving the entire dermis. The pleomorphic epidermotropic infiltrate in the upper one-third of the dermis was composed of many round or oval cells with hyperchromatic nuclei. A few nuclei were indented or kidney shaped. Mild mitotic activity was seen. The larger immunoblast-like cells were mixed with normal lymphoctyes and histiocytes. The capillaries had thickened walls with prominent endothelial cells. The second biopsy from the ulcerated axillary lesion showed a dense superficial and deep wedge-shaped infiltrate, abnormal pleomorphic cells, and a few large lymphoid cells (Fig. 2) infiltrating the dermis and extending to the subcutaneous tissue. There were scanty histiocytes, neutrophils, and a few eosinophils. Some of the large abnormal cells had kidney shaped nuclei but most had irregularly shaped nuclei with abundant cytoplasm (Fig. 3). There were many abnormal mitoses, and there were also numerous extravasated red blood cells and edema in the dermis. The epidermis was necrotic and ulcerated. The results of immunohistochemical tests showed a strong reaction for LCA and CD8, but a negative reaction for CD20. The patient's general condition and laboratory tests of hepatic and renal functions remained normal. The results of all hematologic studies, including bone marrow smear, lymph node biopsy, and liver-spleen scan, were normal. The patient continued on FK 506, 4 mg b.i.d. Serum levels of FK 506 which were never communicated to us were sent for the first time in May 1993 and showed a serum level of 39 ng/mL (normal 0.5–2 ng/mL)¹ with an instruction to repeat the test. The dose of FK 506 was immediately reduced to 4 mg/day. The patient revisited the clinic for routine check-up on June 26, 1993. The axillary lesion had disappeared leaving a hyperpigmented scar, the small lesions of lymphatoid papulosis continued to come and go.     

口服他克莫司联合糖皮质激素治疗难治性寻常型天疱疮一例

报道口服他克莫司联合糖皮质激素治疗难治性寻常型天疱疮1例。患者,男,55岁,确诊为天疱疮后给予甲泼尼龙64 mg每日1次,口服他克莫司胶囊1 mg每日2次以及其他对症治疗,治疗5周后,原有黏膜及皮肤糜烂面愈合出院。出院时患者泼尼松60 mg分2次口服,他克莫司胶囊1 mg每日2次。8个月后Dsg1抗体、Dsg3抗体转阴,泼尼松减量至15 mg每日1次,他克莫司1 mg每日1次,患者病情稳定,现仍在随访中。     

Leg ulcers and hydroxyurea: report of three cases with essential thrombocythemia

CASE 1: A 65-year-old woman with essential thrombocythemia (ET) had been taking oral hydroxyurea (HU), 1,000 mg daily, for 7 years. Six months ago, she developed an ulcer on the outer part of her left ankle, which healed spontaneously within 2 months. She presented with a new, tender, shallow ulcer, 2 cm x 2 cm in size, at the same site. Doppler examination revealed thrombosis of the left common femoral vein and a calcified atheroma plaque of the left common femoral artery. The dosage of HU was decreased to 500 mg daily when the platelet counts were found to be within normal levels. The ulcer completely healed within 2 months with occlusive wound dressings, and has not recurred within the follow-up period of 1 year. CASE 2: A 56-year-old women presented with multiple, painful, leg ulcers of 1 year duration. She had been diagnosed as having ET and had been on HU therapy, 1,500 mg/day, for the past 5 years. Interferon-alpha-2b was started 3 months ago, in addition to HU, which was tapered to 1,000 mg daily. She had suffered from hypertension for 20 years treated with nifedipine and enalapril, and had recently been diagnosed with diabetes mellitus which was controlled by diet. Examination revealed three ulcers located on the lateral aspects of both ankles and right distal toe. Arterial and venous Doppler examinations were within normal limits. Histopathology of the ulcer revealed nonspecific changes with a mixed inflammatory cell infiltrate around dermal vessels. The ulcers completely healed within 10 weeks with topical hydrocolloid dressings. After healing, she was lost to follow-up. A year later, it was learned that she had developed a new ulcer at her right heel, 3 months after her last visit (by phone call). This ulcer persisted for 8 months until HU was withdrawn. CASE 3: A 64-year-old woman with ET presented with a painful leg ulcer of 6 months' duration. She had been taking oral HU for 5 years. She had a 20-year history of hypertension treated with lisinopril. Examination revealed a punched-out ulcer of 2 cm x 2 cm over the right lateral malleolus. Doppler examination of the veins revealed insufficiency of the right greater saphenous and femoral veins. Angiography showed multiple stenoses of the right popliteal and femoral arteries. As her platelet count remained high, HU was continued. During the follow-up period of 13 months, the ulcer showed only partial improvement with local wound care.     

Churg‐Strauss syndrome presenting as scar reactivation: histopathologic features and an illustration of ‘locus minoris resistentiae’

We report a 33‐year‐old female with cutaneous involvement by Churg‐Strauss syndrome confined to surgical scars that were obtained 13 years before. She presented to the emergency department with 2‐day history of fever, night sweats, right‐sided weakness, hoarseness and worsening asthma symptoms. She was found to have an eosinophilia and two sub‐5‐mm pulmonary nodules. The patient also reported that the scars on her right thumb, inner wrist and back had been swollen, red and painful for 2 days. Examination revealed tender, erythematous, well‐healed edematous scars studded with small skin colored papules. She had no clinical findings that were classic for cutaneous vasculitis. A skin biopsy of a scar revealed perivascular and palisading granulomatous inflammation consisting of histiocytes and neutrophils with leukocytoclasia. Focal vascular injury was identified. Scattered tissue eosinophils were seen. Special stains were negative for infection. Thereafter, she was started on intravenous steroids, at which point the fever, pulmonary and cutaneous symptoms subsided. Although scar sarcoidosis is a well‐described phenomenon, granulomatous inflammation and vasculitis seen in Churg‐Strauss syndrome exclusively manifesting in well‐healed surgical scars highlights the unique features seen in this case and draws attention to the concept of locus minoris resistentiae. This case also highlights how a skin biopsy in the setting of suspected systemic vasculitis can confirm the presence of vasculitis and/or granulomatous inflammation and obviate the need for more invasive, higher risk procedures such as lung biopsy.     

Partial lipodystrophy in a patient with systemic lupus erythematosus

A 54-year-old woman developed partial lipodystrophy on the left side of her face. She had been suffering from systemic lupus erythematosus (SLE) since 1985 when she was 45 years old, and she had been treated with 30 mg/day of oral prednisolone as an initial dose. Partial lipodystrophy appeared on her left lower jaw in 1994 when the SLE was inactive, and the dose of prednisolone was reduced to 5 mg/ 3 days. Gradually, the lipodystrophy spread toward her left cheek and her left forehead without any preceding skin symptoms. Histological examination showed a loss of fat tissue and mild lymphocytic infiltrations mainly around cutaneous appendages and vessels in the dermis and subcutaneous tissue. The dose of prednisolone was increased to 10 mg/day and the lesions stopped spreading. Such partial lipodystrophy is distinct from lipoatrophy of lupus profundus.     

Wegener肉芽肿1例

报告1例Wegener肉芽肿病。患者女，29岁。主要表现为肺部炎症，抗生素治疗无效，鼻黏膜糜烂，舌部溃疡，双下肢紫癜和坏死性结痂。组织病理检查：鼻黏膜糜烂为非特异性炎症：小腿皮疹为坏死性血管火和肉芽肿性血管炎改变。实验室检查：抗中性粒细胞胞质抗体（cANCA）和蛋白酶3（PR3）-ANCA均阳性，肾功能不全。给予甲泼泥龙80mg/d和环磷酰胺100mg/d治疗，2周后小腿皮疹消退，但肾功能改善不明显，转入肾脏科继续治疗。     

Healing of Granulomatous Skin Changes in Ataxia‐Telangiectasia After Treatment with Intravenous Immunoglobulin and Topical Mometasone 0.1% Ointment

Ataxia‐telangiectasia (AT) is a rare autosomal recessive disorder characterized by faulty DNA damage repair. The disease affects multiple systems and is noted to be particularly difficult to diagnose in children because of the wide spectrum of clinical presentations. We present an unusual case of a child in whom the primary cutaneous manifestation of AT was noninfectious cutaneous caseating granulomas. A 3‐year‐old girl presented to the emergency department with ataxia, poor growth, and multiple ulcerated plaques on both upper extremities that had been present for 2 years. She had two prolonged hospitalizations and underwent extensive examination to identify an etiology for the skin lesions. She was diagnosed with AT after immunology examinaton and genetic testing. Outpatient intravenous immunoglobulin (IVIG) therapy was initiated and she was prescribed twice‐daily mometasone 0.01% ointment under occlusion. After 6 weeks on this regimen her lesions had completely healed. Twenty‐two cases of AT have been reported in which patients presented with cutaneous granulomas. This report demonstrates the first reported case in which the granulomatous skin lesions of AT healed after aggressive application of topical steroids with concurrent IVIG therapy, without oral steroids. A brief review of cutaneous granulomas in the setting of immunodeficiency is also presented.     

A case of sarcoidosis involving the tongue.

Sarcoidosis is a systemic granulomatous disorder which commonly affects the skin. Involvement of the tongue is rare; a review of the previous literature over the last 30 years revealed only six cases of sarcoidosis affecting the tongue. We studied a case of sarcoidosis involving the tongue in a 32-year-old Japanese woman with characteristic clinical and pathological findings. She visited our department with a complaint of a tongue lesion of which she had been aware for a month. A diagnosis of sarcoidosis was made for the lesion by clinical and pathological examinations. Oral involvement by sarcoidosis is rare, however this disorder should be considered as a possible cause of intraoral granulomatous lesions.     

Case of isolated benign primary cutaneous plasmacytosis in a child

A collection of plasma cells in the skin can represent a broad spectrum of disease entities. Secondary syphilis, primary cutaneous plasmacytoma, primary cutaneous plasmacytosis, cutaneous lymphoid hyperplasia and nodular amyloidosis are considered possible differential diagnoses. We present a case of a 7-year-old girl with an erythematous scaly plaque on her right buttock that had been present for approximately 5 years. Prior to her visit to our department she had been treated at a local dermatology clinic with topical methylprednisolone acetate and topical calcitriol without significant improvement. Histopathological examination revealed psoriasiform hyperplasia, hyperkeratosis, parakeratosis and a band-like or dense perivascular infiltration of plasma cells with a few lymphocytes and histiocytes. Other laboratory tests were within the reference ranges. At our department, the patient was given oral prednisolone along with an intralesional injection of triamcinolone and application of topical methylprednisolone acetate and tacrolimus hydrate to the affected area. The lesion improved significantly but recurred 3 months later. We present a rare case of isolated benign primary cutaneous plasmacytosis in a female pre-adolescent child.     

Benign familial Degos disease worsening during immunosuppression

We describe a 61-year-old woman with skin lesions consistent with those found in Degos disease, both in clinical and in histological appearance. She had had several of these lesions for many years, as had her mother, sister and niece. In 1991, she underwent cadaveric renal transplantation and was treated with immunosuppression: prednisolone, azathioprine and cyclosporin. At that time, she developed many more characteristic skin lesions, and these were slightly larger and more noticeable than those she had had previously. She and the other affected family members appear to fit into the more benign subgroup of Degos disease, and it seems that her immunosuppression aggravated her cutaneous disease.     

Primary cutaneous cryptococcosis in a patient with systemic immunosuppression after liver transplantation

We report a 36-year-old woman who slowly developed an ulceration on the left thigh 2 years after transplantation for Budd-Chiari syndrome. At this time point, the patient was treated with prednisone, tacrolimus and azathioprine for immunosuppression and with phenprocoumon and low-dose aspirin for anticoagulation in the presence of polycythemia vera. A biopsy of the skin lesion was obtained and revealed encapsulated yeast that was identified by microbiological and serological methods as Cryptococcus neoformans serotype D. The patient had no signs of systemic infection and a therapy with fluconazole (200 mg/day) was started. The lesion healed within 8 weeks and fluconazole was stopped after 3 months. Due to interactions between fluconazole, tacrolimus and phenprocoumon, the latter drugs were decreased to prevent toxicity. So far, 1 month after stopping fluconazole, no recurrence of skin lesions has been observed.     

Stevensjohnson Syndrome Caused by a Health Drink (Eberu®) Containing Ophiopogonis Tuber

Stevens-Johnson syndrome is considered to be a severe type of erythema exsudativum multiforme. It is characterized by erythema with bullous and eroded lesions of skin and mucous membranes. We report a case of Stevenjohnson syndrome following consumption of a health drink containing ophiopogonis tuber. A 66-year-old female took an O.T.C. health drink for fever. The next morning, she noted erythema and swelling of her face, neck, and chest. She started to develop bullous and eroded lesions on the skin of her entire body and the mucous membranes of her oral cavity, conjunctiva, and cornea, and she became feverish. She had high degrees of corneal erosion and liver dysfunction. Skin biopsy showed diffuse necrosis of the epidermis. After admission to the hospital, steroid pulse therapy (1000 mg/day of methylprednisolone sodium succinate) was continued for 5 days. The health drink induced a positive drug lymphocyte stimulation test (DLST) and patch test. A challenge test was done with a one hundredth dose, and it was positive. We did patch tests with all components of the drink and found that Mai-Meu-Dong-Tang (ophiopogonis) alone was positive at 72 hours. There is no previous report of Stevens-Johnson syndrome caused by a health drink or Mai-Meu-Dong-Tang. Even though it is a health drink, we should be aware of the possibility of a severe reaction.     

Squamous cell carcinoma in a patient with Netherton's syndrome

A 29-year-old white woman with a history of Netherton's syndrome presented with two squamous cell carcinomas on the right dorsal hand and the left upper arm. She reported a 2-year history of these lesions, which were originally treated as warts. She denied excessive sun exposure, immunosuppressive therapy, or a previous history of skin cancer. Her past medical history included acute renal failure, multiple urinary tract infections, meningitis, and recurrent otitis media as a child. In addition, she had an ovarian abscess at 4 years of age with resulting salpingo-oophorectomy. She also reported a history of severe myopia, glaucoma, and multiple ocular infections with a resulting corneal scar. In addition to atopic dermatitis, she had a 10-year history of psoriasis. Her medications included topical steroids and emollients for atopic dermatitis and psoriasis, in addition to Timolol ophthalmic drops for glaucoma. Her family history was significant for a 22-year-old sister with Netherton's syndrome (Fig. 1). She denied any history of skin cancer in her sister or other members of her family. On physical examination, she had an exfoliative erythroderma, madarosis, and diffuse patchy alopecia. In the bilateral axilla, she had well-defined pink scaly plaques which were confirmed as psoriasis by biopsy. On the right dorsal hand, she had a 1.5 x 1.0 cm pink verrucous plaque (Fig. 2). On the left upper arm, she had a 1.5 x 0.8 cm pink scaly plaque. Biopsies of both sites confirmed squamous cell carcinomas. Both lesions were completely excised with 4 mm margins.     

Wells' syndrome: vesiculobullous presentation and possible role of ectoparasites

A 21-year-old woman with recurrent skin lesions present since December 1992 was seen at the University of Alberta hospitals in 1994. The skin lesions consisted of pruritic, painful, erythematous, “beefy” plaques on the trunk and extremities and also of vesicles, bullae, and pustules on the ankles and hands. She experienced numerous flare-ups of her eruption that required hospital admission on five separate occasions. Each episode was associated with sweats, chills, and dizziness. Headaches, arthralgias, myalgias, and generalized fatigue occurred on occasion. Previous skin biopsies suggested Wells' syndrome. Detailed investigations did not reveal any underlying etiology. The patient was a single woman who had recently moved from New Brunswick to live with her aunt and two cousins in Edmonton and to find employment. She had previously been a healthy young woman until her episodes first began in December 1992. There was a personal history of childhood atopic dermatitis, but she denied any other manifestations of atopy. Her mother had recently passed away at the age of 37 from Hodgkin's lymphoma, otherwise the family history was unremarkable. At the time of this admission she was not taking any medications; however, during the previous months in New Brunswick she had been using numerous medications including prednisone, dapsone, colchicine, and an oral contraceptive for a functional ovarian cyst. Prednisone, up to 60 mg/day, had initially appeared beneficial for her skin lesions, but later her symptoms became resistant. In addition, she suffered from many side-effects induced by chronic corticosteroid usage, inciuding biurred vision, weight gain, striae, and irreguiar menstruai periods. She had not been on corticosteroids for the past 6 weeks, and none of the other medications were of benefit. In fact, she had suffered a severe idiosyncratic reaction to dapsone consisting of high fever, abdominai pain, and abnormal liver parameters. There was no significant travel history and she denied any drug abuse or HIV risk factors, the patient was an animal lover and often played for hours at a time with her aunt's dog and two cats. By coincidence, in New Brunswick she had also owned a dog and two cats. A review of systems was unremarkable. On physical examination, she was a moderately obese young woman who appeared cushingoid, but otherwise well. She was afebrile. Examination of the skin revealed multiple vesicles, bullae, and pustules on her hands and fingers, involving both dorsal and palmar surfaces and extending to her wrists (Fig. 1). Some of the lesions had broken open forming overlying crusts. A few bullae had become hemorrhagic. There was an obvious foul-smelling odor emanating from the lesions. Multiple small excoriations were present bilaterally on the shins and two erythematous, edematous papules were seen on the abdomen. A live flea was found in her suprapubic area. No other fleas were found. The rest of the general examination was unremarkable. Pertinent laboratory investigations revealed an absolute eosinophilia of 2700/mm3 (24% eosinophilia) with a white blood count (WBC) of 11,400/mm”. Swabs of hand lesions were culture-positive for multiple aerobes and anaerobes: 3+ growth of Staphylococcus aureus, 3+ growth of Haemophilus parainfluenza, 1 + growth of Coxiella oxytoca, 2+ growth of Streptococcus viridans, and 4-i- growth of mixed anaerobes. Virologic tests were negative. Examination of a skin biopsy specimen (Fig. 2) revealed multiple flame figures in the dermis with surrounding and interspersed eosinophils. Numerous eosinophils, / lymphocytes, and histiocytes were also present around small blood vessels. There were degenerative changes of the upper epidermis with spongiosis, suggestive of early intraepidermal vesicle formation. Direct immunofluorescence on skin specimens was negative. Skin prick testing with flea antigen was positive for immediate hypersensitivity, but negative for a delayed reaction after 48 h. On further questioning, the patient admitted that she had played with a cat just prior to her admission, which, unbeknownst to her at the time, was flea-infested. Her aunt strongly denied any fleas in the home environment, despite her pets. No other household members complained (5f insect bites or skin symptoms. The patient's skin improved remarkably with hospitalization and treatment with antibiotics; no new lesions developed while in hospital and she was discharged 12 days later. She continued to experience recurrent, sporadic eruptions that resolved without corticosteroids, but was later lost to follow-up when she returned home to New Brunswick.     

Recurrent sarcoidosis on a scar associated with vitiligo

We report a case of systemic sarcoidosis, diagnosed 13 years ago by transbronchial biopsy. Our patient had a spontaneous resolution of hilar and mediastinal lymphadenopathy 6 months after diagnosis. She presented with a skin rash on her thyroidectomy scar after a period of 4 years of complete remission. The patient complained that her thyroidectomy incision scar rose up, reddened and hardened. Biopsy of the skin lesion revealed non-caseating epithelioid granulomas with histiocytes and multinucleated giant cells. She was successfully treated with oral prednisone. Scar sarcoidosis recurred twice in a 7-year follow-up period without accompanying systemic sarcoidosis. Vitiligo appeared with the last relapse of scar sarcoidosis. The case we present is of interest due to the recurrent attacks of scar sarcoidosis in a patient during the remission period of systemic sarcoidosis. Another aspect of this case is the association of the disease with vitiligo without any sign of an autoimmune disease.     

FK506 inhibits tumour necrosis factor-alpha secretion in human keratinocytes via regulation of nuclear factor-kappaB

BACKGROUND: Tacrolimus (FK506) ointment has been used for treatment of inflammatory dermatoses with remarkable success. Our previous studies have indicated that direct modulation of tacrolimus on keratinocytes (KCs) may have an impact on its therapeutic effect. The use of monoclonal antibody specific for tumour necrosis factor (TNF)-alpha has shown efficacy in treating both psoriasis and Crohn disease. Topical tacrolimus has also been shown to be effective for treating cutaneous manifestations of both diseases. OBJECTIVES: To explore the effects of FK506 on human KCs in terms of TNF-alpha secretion and to investigate the regulatory pathway involved. METHODS: Ultraviolet (UV) B-irradiated cultured KCs were treated with various concentrations of FK506. At indicated time points after UVB irradiation we determined: (i) the TNF-alpha concentrations present in the culture supernatants; (ii) the activation and translocation of nuclear factor (NF)-kappaB in the cell nucleus; and (iii) the protein expressions of IkappaB kinase (IKK) and IkappaB in the cell lysates. In addition, a mouse model was used to corroborate our in vitro findings in vivo. More specifically, topical tacrolimus was applied on to mouse skin unilaterally after UVB irradiation. The effects of FK506 on nuclear NF-kappaB expression of UVB-irradiated mouse skin were determined. RESULTS: Our results showed that FK506 dose-dependently downregulated the secretion of TNF-alpha from UVB-irradiated KCs. The activation and translocation of NF-kappaB in UVB-irradiated KCs were also dose-dependently suppressed by FK506. The degradation of IkappaB induced by UVB was also inhibited by FK506, while no change in IKK expression was noted regardless of UVB and FK506 treatment. Murine skin biopsies showed that nuclear NF-kappaB expression induced by UVB was inhibited by topical tacrolimus treatment. CONCLUSIONS: Our results indicate that FK506 inhibits TNF-alpha secretion in human KCs via direct regulation of NF-kappaB. This modulatory effect of FK506 on KCs offers a possible mechanism for how topical tacrolimus regulates cutaneous inflammatory conditions.     

Malignant histiocytosis with panniculitis--a case report

We report a case of malignant histiocytosis which began with the skin lesions of panniculitis. A 32-year-old woman presented with recurrent erythematous plaques, subcutaneous nodules, and ulcers on the trunk and the extremities and intermittent fever for 7 months. The cutaneous lesions consisted of erythematous and brownish irregular-shaped patches and tender cutaneous nodules 0.5-1.0 cm in diameter. Central necrosis and shallow ulcers were seen in the lesions. The patient also suffered from general fatigue, arthralgia, and weight loss. She was anemic and thrombocytopenic and had progressive impairment of liver function with coagulation defect. Histopathological study of skin lesions showed lobular panniculitis without vasculitis in the subcutaneous fat tissue. In the panniculitis lesion, moderate mixed cell infiltration consisting of lymphocytes and histiocytes was observed. Bone marrow aspiration revealed an increase in the number of histiocytes, mostly immature with active phagocytosis of erythroid cells, myeloid cells, and platelets. She was diagnosed as having malignant histiocytosis and treated with cyclophosphamide, vincristine, and prednisolone which she responded well; her fever subsided and the lesions healed with hyperpigmentation. In this patient, benign histiocytes with hemophagocytosis without immature forms were found in the skin lesions. According to our knowledge, this is the first Thai report of malignant histiocytosis with clinical features of panniculitis.     

Maffucci's syndrome with extensive gastrointestinal involvement

A 10-year-old girl with cutaneous, oral and gastrointestinal vascular lesions was referred for consideration of laser treatment of her skin lesions. She was noted to have multiple venous malformations predominantly affecting the hands and feet, some of which had been present from birth. Her right hand was deformed by multiple venous malformations, and X-rays of this hand revealed enchondromata within metacarpal and phalangeal bones. She was known to have extensive involvement of her gastrointestinal tract by venous malformations, resulting in refractory iron-deficiency anaemia. In view of the multiple cutaneous and gastrointestinal vascular lesions, a diagnosis of blue rubber bleb naevus syndrome had been made many years earlier. However, after recognition of the characteristic enchondromata, this diagnosis has been revised to Maffucci's syndrome. In addition to her ongoing dermatological and paediatric follow up, she has now been referred to the orthopaedic surgeons for surveillance of her skeletal lesions.