Chii-Shuenn Yang

Paraneoplastic pemphigus as the first manifestation of follicular dendritic cell sarcoma

Paraneoplastic pemphigus (PNP) is a life-threatening autoimmune bullous disease characterized by severe intractable mucositis and polymorphous skin eruptions [1]. The condition is associated mostly with hematological malignancies and less commonly with solid non-hematological neoplasms [1–3]. In about one-third of patients, there is no known underlying malignancy at the time of initial mucocutaneous Clinical Letter eruption [3]. Therefore, PNP can be a warning sign and an obligatory paraneoplastic marker for occult malignancy [4]. We describe a patient with PNP as the first manifestation of follicular dendritic cell sarcoma (FDCS). A 54-year-old woman previously in good health presented with painful erosions on the lips and oral mucosa for one month (Figure 1a), followed by erythematous to violaceous papules, vesicles and plaques on the face, trunk, limbs (Figure 1b) and palms (Figure 1c) for one week. Histopathology of the skin lesion revealed vacuolar interface change with necrotic keratinocytes and subepidermal bullous formation (Figure 1d). Direct immunofluorescence (DIF) was negative for IgG and complement. The clinicopathological features were compatible with erythema multiforme (EM). After initiation of systemic corticosteroids therapy, the cutaneous lesions showed slight improvement but the mucosal involvement persisted. The skin eruption became more

Flare-up of incontinentia pigmenti in association with Behçet disease.

A 16-year-old girl, who was diagnosed as having incontinentia pigmenti (IP) during infancy, presented with diffuse hyperpigmented patches along the lines of Blaschko over most of her body below the neck. She had no associated ocular or neurological abnormalities, but dental X-ray revealed congenital absence of one lower lateral and two upper lateral incisors. At the age of 7, she suffered from recurrent genital ulcers, oral aphthosis, and facial pustules. Pathergy test was positive. Behçet disease was diagnosed. After systemic corticosteroids were administered, her condition improved and remained stable for years. She experienced occasional flares of oral aphthous ulcers, but no recurrence of genital ulcers developed. Her mother and older sister had been diagnosed with IP, and her mother also had Behçet disease. She recently presented with itching, stinging erythematous patches with vesiculobullous lesions on previous hyperpigmented patches over her left calf. Genital ulcers and oral aphthous ulcers recurred simultaneously. There were no other infectious symptoms and signs over the upper respiratory tract and genitourinary tract. Routine laboratory tests revealed mild leukocytosis and elevated erythrocyte sedimentation Clinical Letter rate (113 mm/hr.). Blood chemistry, urinalysis, and antinuclear antibodies were within normal limits. The erythematous lesions disappeared quickly and turned to hyperkeratotic verrucous papules and vesicles (Figure 1a, b) within one week. Virus cultures for varicella-zoster virus and herpes simplex virus of lesional skin and bacterial cultures of blood and urine were negative. Histological examination showed marked hyperkeratosis, acanthosis, papillomatosis, and focal dyskeratosis of the epidermis (Figure 2a). The dyskeratotic cells were arranged in a whorled configuration (Figure 2b). The skin lesions resolved in ten days. The oral aphthous ulcers and genital ulcers healed two weeks later. Recurrent inflammation of incontinentia pigmenti associated with flare-up of Behçet disease was diagnosed. Incontinentia pigmenti is an X-linked dominant multisystemic syndrome with cutaneous, neurologic, dental, and ophthalmologic manifestations. Classically, there are four stages of cutaneous lesions: vesicular, verrucous, hyperpigmented, and hypopigmented. In recent studies, mutation in the gene NEMO (nuclear factor κB [NF-κB] essential modulator), also known as “the inhibitor of kappa light polypeptide gene enhancer in B-cells, kinase gamma”, on the X chromosome at Xq28, has been related to the development of incontinentia pigmenti [1, 2]. NF-κB may protect keratinocytes from tumor necrosis factor-α (TNF-α)-induced apoptosis [1, 2]. Epidermal cells with the mutant NEMO gene cannot activate NF-κB, so they will lose the ability to inhibit TNFα-induced apoptosis, giving rise to cutaneous lesions associated with IP [1, 2]. Most patients with IP remain stable after the initial inflammatory stage. However, some patients experienced episodes of late reactivation of the inflammatory cutaneous

Sweet's syndrome associated with Mycobacterium kansasii infection in an immunocompetent woman.

Six months prior to referral to our department, a 48-year-old otherwise healthy Chinese woman underwent a routine physical examination. Chest X-rays revealed mediastinal lymphadenopathy, and subsequent culture of esophageal endoscopic ultrasound-guided lymph node biopsy yielded Mycobacterium (M.) kansasii three months later. The patient was given antitubercular drugs (ATD), including rifampicin and ethambutol, along with clarithromycin at the pulmonology clinic. Due to a high-grade fever of 39°C lasting for two weeks and multiple painful skin eruptions on the face and all four extremities, the patient was subsequently admitted to our department. On physical examination, there were multiple painful, tender and edematous erythematous papules and nodules, coalescing into plaques, with a transparent, vesicle-like appearance (Figure 1a). Some lesions had ulcerated showing pyoderma gangrenosum-like features with pustulation (Figure 1b). Systemic and pelvic examinations did not reveal any abnormality, especially no lymphadenopathy or hepatosplenomegaly. Laboratory investigations showed a white blood cell count of 16 800/mm3 with 88.3 % polymorphonuclear cells, Clinical Letter hemoglobin 10.3 g/dl, platelet count 248 000/mm3, C-reactive protein 16.6 mg/dL, and a erythrocyte sedimentation rate of 113 mm in the first hour. Serum chemistry tests, urinalysis, and serologic thyroid, hepatic, and renal function tests were all within normal limits. The patient was serologically HIV negative. Histology of a skin lesion of the left anterior leg showed marked edematous changes in the papillary dermis and dense diffuse neutrophilic infiltrations throughout the upper dermis (Figure 2a, b). Bacterial, fungal, and mycobacterial cultures of lesional tissue were all negative. A bone marrow biopsy only showed mild hypocellularity without any obvious hematologic abnormalities. Polymerase chain reaction of a follow-up mediastinal lymph node biopsy revealed M. kansasii, resulting in the eventual diagnosis of Sweet’s syndrome associated with M. kansasii. Treatment with ATD, including rifampicin and ethambutol, along with clarithromycin was continued as recommended by the pulmonologist. Fever and skin lesions promptly subsided following treatment with 20 mg of prednisolone daily (0.4 mg/kg), allowing for a gradual prednisolone taper. However, upon reducing prednisolone to 5 mg daily two months later, fever accompanied by numerous tender indurated, erythematous pustular papules and nodules – similar to previous cutaneous lesions – recurred on the patient’s face and all four extremities. Repeated skin biopsy showed pathologic changes similar to previous findings. The prednisolone dose was again increased to 20 mg daily, leading to a quick resolution of both fever and skin lesions. Prednisolone at 10 mg every day was maintained for another 12 months until the ATD regimen was completed. There was no further relapse of cutaneous lesions. Sweet’s syndrome, also known as acute febrile neutrophilic dermatosis, was first described by Dr. Robert Douglas Sweet in 1964 [1]. There are three variants of Sweet’s

Sweet‐Syndrom in Verbindung mit einer Infektion mit Mycobakterium kansasii bei einer immunkompetenten Frau

Eine 48-jährige, ansonsten gesunde Frau chinesischer Herkunft unterzog sich sechs Monate vor Überweisung in unsere Abteilung einer Routineuntersuchung. Die Röntgenthoraxaufnahmen zeigten eine mediastenale Lymphadenopathie, und die darauffolgende Kultur der endoskopischen ultraschall-geführten ösophagealen Lymphknotenbiopsie ergab drei Monate später Mycobacterium (M.) kansasii. Der Patientin wurden in der Pulmologie Antituberkulotika, darunter Rifampicin und Ethambutol, zusammen mit Clarithromycin, verabreicht. Aufgrund von hohem, zwei Wochen lang anhaltendem Fieber von 39°C und multiplen schmerzhaften Hauterosionen im Gesicht und an allen vier Extremitäten wurde die Patientin in unserer Abteilung stationär aufgenommen. Eine Untersuchung ergab multiple schmerzhafte, empfindliche und ödematöse gerötete Papeln und Knötchen mit transparentem, bläschenartigem Erscheinungsbild, die sich zu Plaques vereinigten (Abbildung 1a). Einige Läsionen waren Clinical Letter ulzeriert und zeigten Pyoderma gangrenosum-artige Eigenschaften mit Vereiterungen (Abbildung 1b). Systemische Untersuchungen und Untersuchungen des Beckens zeigten keine Abnormitäten, insbesondere keine Lymphadenopathie oder Hepatosplenomegalie. Laboruntersuchungen ergaben eine Leukozytenanzahl von 16800/mm3 mit 88,3 % polymorphkernigen Zellen, einen Hämoglobinwert von 10,3 g/dl, eine Thrombozytenzahl von 248000/mm3, einen Wert an C-reaktivem Protein von 16,6 mg/dl, und eine Blutsenkungsreaktion von 113 mm in der ersten Stunde. Blutchemieuntersuchungen, Urinanalyse, und serologische Schilddrüsen-, Leber-, und Nierenfunktionsuntersuchungen waren alle im normalen Rahmen. Die Patientin war serologisch HIV-negativ. Die Histologie einer Hautläsion an der Vorderseite des linken Beins zeigte deutliche ödematöse Veränderungen an der papillären Dermis und dichte diffuse neutrophile Infiltrationen über die gesamte obere Dermis (Abbildung 2a, b). Bakterien-, Pilz-, und Mykobakterienkulturen des erkrankten Gewebes waren durchweg negativ. Eine Knochenmarkbiopsie zeigte nur eine leichte Hypozellularität ohne offensichtliche hämatologische Abnormitäten. Die Polymerase-Kettenreaktion einer nachfolgenden Biopsie der mediastinalen Lymphknoten ergab M. kansasii, was in einer Diagnose des Sweet-Syndroms in Verbindung mit M. kansasii resultierte. Die Behandlung mit ATD, darunter Rifampicin und Ethambutol, verbunden mit Clarithromycin, wurde, wie vom Pneumologen empfohlen, fortgeführt. Infolge der Behandlung mit 20 mg Prednisolon täglich ließen Fieber und Hautläsionen umgehend nach, was das langsame Ausschleichen von Prednisolon ermöglichte. Nach der Reduktion von Prednisolon auf 5 mg täglich zwei Monate später kehrte jedoch das Fieber, verbunden mit zahlreichen empfindlichen verhärteten, geröteten pustulösen Papeln und Knötchen – ähnlich