Buschke-Ollendorff syndrome presenting with asymptomatic yellowish papules and leg length discrepancy: A case report.

Buschke-Ollendorff syndrome (BOS) is a rare, usually benign, autosomal dominant genetic disease affecting about 0.005% globally. BOS commonly manifests with asymptomatic connective tissue nevi, sometimes with sclerotic bone lesions like osteopoikilosis or melorheostosis. However, BOS may develop severe, symptomatic complications that require surgical intervention. Here we report a 9-year-8-month girl presenting with multiple nonpruritic, nonpainful skin plaques scattered around the trunk, buttocks, and bilateral legs. She had a history of right varus foot with inadequate plantar flexion. Upon visiting, obvious leg length discrepancy (LLD) was noted. Lesional biopsy revealed increased fibroblasts within dermal collagen bundles. Verhoeff-van Gieson stain revealed scattered foci of thickened elastic fibers between collagen fibers, especially in the mid-dermis. Radiographic examination of the lower extremities showed multiple small, round-to-oval shaped, radiopaque spots on the pelvic bones, femurs, tibiae, and both feet. Hyperostosis along the long axis with "dripping candle wax" appearance was characteristic of osteopoikilosis and melorheostosis. Genetic analysis showed heterozygous point mutation in exon 1 of LEMD3 gene (c.1323C>A, p.Y441X), confirming diagnosis of BOS. Sequential and epiphyseodesis were performed to correct LLD with a favorable outcome at 2-year follow-up. BOS associated with severe bone abnormalities is rare, but orthopedic surgical intervention can provide satisfactory outcome.

Efficacy and safety of picosecond 755-nm alexandrite laser for treatment of nevus of Ota in Taiwanese children: A retrospective study.

OBJECTIVES: To evaluate the efficacy and safety of picosecond 755-nm alexandrite laser in the treatment of nevus of Ota in children. MATERIALS AND METHODS: A retrospective study was conducted by reviewing medical charts and photographs of 86 Taiwanese children with various types of nevus of Ota between January 2017 and September 2020. Picosecond 755-nm alexandrite laser therapy was used to treat pigmentary lesions. Percent clearance of lesions during treatment and the treatment time required to achieve 95%-100% clearance were determined. RESULTS: According to Tanino's classification or Peking University Medical College Hospital (PUMCH) classification of nevus of Ota, most patients belonged to Tanino's Type II (32%) and Type III (38%) or PUMCH Type IIb (33%) and Type IIIb (26%), which indicated that the nevus was mainly distributed in the forehead, upper and lower eyelid, zygomatic, cheek, and temple regions. After treatment with picosecond 755-nm alexandrite laser, 96.5% of the patients achieved 95%-100% clearance with an average of 4.3 treatment sessions. The earlier onset of lesions (before 5 months of age) and the darker Fitzpatrick skin types (type IV vs. type III) significantly increased the number of treatments required to achieve clear response, while sex, age at first treatment, Tanino's classification of nevus, and color of nevus had no significant effect. Posttreatment hypopigmentation or hyperpigmentation was transient and resolved within 6 months. No serious response of the skin was evident. CONCLUSION: Picosecond 755-nm alexandrite laser treatment of nevus of Ota in children was safe and effective. The treatment was well-tolerated, and only a few transient, minor side effects occurred.

Lymphocytic hidradenitis: A distinctive histopathological finding of annular erythema of Sjögren syndrome.

BACKGROUND/OBJECTIVES: Lymphocytic hidradenitis is a non-specific histopathological feature observed in many dermatoses such as lupus erythematosus, morphea or scleroderma. When it occurs it is usually accompanied by the other distinctive histological features of those conditions. Isolated lymphocytic hidradenitis is uncommon and its clinical features and associated underlying medical conditions are still undetermined. METHODS: We performed a retrospective review of patients who clinically presented with annular erythema between 2000 and 2016. Altogether, 30 patients with a histopathological presentation of isolated lymphocytic hidradenitis were identified. Their following characteristics were recorded: clinical features, number and localisation of lesions, serology and other associated medical conditions. RESULTS: Isolated lymphocytic hidradenitis was found most frequently in middle-aged women. Most patients (n = 28, 93%) presented with many annular erythematous patches and plaques with mild pruritus; 22 (73%) had the SS-A antibody and 17 (57%) met the diagnostic criteria of Sjögren syndrome. Among these patients, 11 had primary and six had secondary Sjögren syndrome associated with systemic lupus erythematosus. Altogether 15 (50%) patients tested positive for a high titre of the antinuclear autoantibody. Other underlying diseases identified during the follow-up period include cryoglobulinaemia, angioimmunoblastic T-cell lymphoma, autoimmune hepatitis, hepatitis C infection and toxic thyroid goitre. CONCLUSIONS: Lymphocytic hidradenitis is a microscopic finding associated with annular erythemas of Sjögren syndrome. Systemic survey for sicca symptoms and work up for autoimmune diseases, including antinuclear antibodies, SS-A, SS-B antibodies, cryoglobulin, lymphoma, viral and autoimmune hepatitis should be performed to facilitate the correct diagnosis.

Neoplastic skin lesions of the scalp in children: a retrospective study of 265 cases in Taiwan.

BACKGROUND: The preferential occurrence of certain skin neoplasms on the scalp of children raises concerns from their parents and warrants special diagnostic and therapeutic approaches. OBJECTIVE: To explore the demographic and clinical characteristics of scalp neoplasms in the pediatric population, with attention to malignant tumors and systemic syndromes. METHODS: Scalp neoplasms in patients aged 12 years or younger were retrospectively collected in 1990-2010 from two tertiary referral centers in Taiwan. RESULTS: A total of 267 scalp neoplasms in 265 pediatric patients were recruited. Among the 209 neoplasms with a histopathological diagnosis, nevus sebaceus was the most common (67.9%), followed by melanocytic nevus (6.2%) and juvenile xanthogranuloma (6.2%). Most of the scalp neoplasms (77.9%) were seen at birth or before 1 month of age. Infantile hemangioma was clinically diagnosed without histology in 41.4% of cases. Malignant scalp tumors were identified in two patients (0.95%), with one basal cell carcinoma and one precursor B-cell lymphoblastic lymphoma, respectively. Scalp neoplasms in association with systemic syndromes were found in two cases. One had neurofibromatosis type I with juvenile xanthogranuloma and the other basal cell nevus syndrome with basal cell carcinoma. CONCLUSIONS: Most pediatric scalp neoplasms in our study were hamartomas or teratomas. Malignant scalp tumors and malignant transformation of nevus sebaceus were rare. A detailed medical history taking and complete physical examinations are needed to exclude possible associations with systemic syndromes or malignancies.

A birthmark survey in 500 newborns: clinical observation in two northern Taiwan medical center nurseries.

BACKGROUND: The prevalence of birthmarks in Taiwanese newborns has not been well studied. How gender and maturity of newborns affect presentation and prevalence of vascular birthmarks, in particular, has not been well documented. The aim of this survey was to catalog the above-mentioned problems. METHODS: From May 9 through July 14, 2000, 500 newborns in Linkou and Taipei Chang Gung Memorial Hospital nurseries were examined for the presence of birthmarks. The data were collected and stored in Excel software and analyzed using chi2-test and Fisher's exact test for statistical association. RESULTS: The most common pigmentary birthmark was the Mongolian spot (61.6%), followed by congenital melanocytic nevi (0.6%), café-au-lait spots (0.4%), and nevi depigmentosus (0.4%). Vascular birthmark frequency was: salmon patch (27.8%), Port-Wine stain (0.6%), and hemangioma (0.2%). Other miscellaneous changes included preauricular sinus (0.6%), skin tag (0.6%), and accessory auricle (0.2%). Prevalence of the vascular salmon patch birthmarks in female infants and male infants was 31.0% and 26.6%, respectively, but the difference was not statistically significant (p = 0.51). The prevalence of vascular salmon patch birthmarks in full-term infants was 28.0%, and 25.8% in preterm infants. However, there was also no statistical difference (p = 0.96) between the two. The two nevus depigmentosus cases were isolated types and neither of these were distributed along the Blaschko lines. CONCLUSIONS: The Mongolian spot was the most common birthmark in Taiwanese newborns, followed by the salmon patch. All of the other birthmarks had prevalence of less than 1%. The prevalence of vascular salmon patch birthmarks was noted in 31.0% of female infants and in 26.6% of male infants. The prevalence of vascular salmon patch birthmarks in full-term infants was 28.0% versus 25.8% in preterm infants. However, the findings regarding vascular birthmarks were not statistically significant between the differing gender and maturity groups.

Urachal duct remnant-like umbilical clear cell acanthoma in an infant: an unusual presentation and pitfall in clinical practice.

BACKGROUND: Although an umbilical nodule is common in neonates and young infants, an umbilical nodule of poor therapeutic response will increase the likelihood of other uncommon etiology. Clear cell acanthoma (CCA) has never been described as an oozing umbilical nodule on infants. METHODS: To present a case of CCA which occurred on an 8-month-old female infant presenting with one weeping nodule on the umbilicus since early neonate. The lesion underwent skin biopsy and subsequent hematoxylin-eosin and periodic acid-Schiff staining. RESULT: Three courses of liquid nitrogen cryosurgery were performed after the diagnosis of CCA was confirmed. The lesion resolved rapidly. CONCLUSION: This report presents the first and youngest case of umbilical CCA in the English literature. This case supports the inflammatory dermatosic nature of CCA.

Malignant cutaneous tumors of the scalp: a study of demographic characteristics and histologic distributions of 398 Taiwanese patients.

BACKGROUND: The scalp is a unique anatomic region, in which pilosebaceous follicles are concentrated. OBJECTIVE: We sought to investigate demographic characteristics and histologic distributions of malignant scalp tumors. METHODS: Primary and metastatic scalp malignancies diagnosed histopathologically between 1983 and 2003 were reviewed. Age at diagnosis, sex, and histologic types were analyzed. RESULTS: A total of 398 Taiwanese patients (200 males, 198 females) were selected. Age at diagnosis ranged from 3 to 103 years. Most malignant scalp tumors (69.8%) occurred in those 50 years or older. Basal (41.2%) and squamous (16.6%) cell carcinomas were the most common histologic types. Surprisingly, metastatic tumors (12.8%) came in third, in which lung cancers were the most frequent primary tumor in both male and female patients. LIMITATIONS: In our series, the case number of metastatic scalp malignancies was underestimated because not all patients with metastatic scalp tumors received a scalp skin biopsy. CONCLUSION: Because a wide spectrum of primary and metastatic malignancies can occur on the scalp, scalp inspection should be included in general screening for either skin or internal cancers.

Mixed-type neurothekeoma presenting with an unusual clinical appearance of multiple satellite lesions on the back.

BACKGROUND: Neurothekeoma is a rare cutaneous neoplasm, often occurring as a nondescript cutaneous nodule on the central face, shoulders, and upper extremities. OBJECTIVE: To present a patient with mixed-type neurothekeoma with an unusual clinical presentation. METHODS: We report a 29-year-old female who developed an asymptomatic, red, dermal nodule with satellite papules on the back over a period of 6 months. RESULTS: The coalescing papules on the back were excised, and the histopathology and immunohistochemical study revealed a mixed-type neurothekeoma. CONCLUSION: Neurothekeoma usually presents with a small, solitary, and slow-growing nodule or papule on the upper body. The case is interesting because of the unusual clinical manifestation of one dermal tumor with several satellite lesions.

Childhood neutrophilic eccrine hidradenitis: a clinicopathologic and immunohistochemical study of 10 patients.

BACKGROUND: Neutrophilic eccrine hidradenitis (NEH) is occasionally reported in patients who have not received chemotherapy. OBJECTIVE: The purpose of this study was to describe the clinicopathologic features of NEH occurring in healthy children and to investigate the interleukin (IL)-8 expression in the cutaneous lesions. METHODS: Ten children with characteristic histologic features of NEH were collected from the Chang Gung Memorial Hospital. Their formalin-fixed, paraffin-embedded specimens were examined by immunohistochemical staining for IL-8. RESULTS: The age of first presentation at our clinic ranged from 6 months to 14 months with a median age of 9.1 months. The onset of the disease clustered in the summertime. The most common clinical appearance was multiple erythematous papules and nodules on the limbs. Two of 7 biopsy specimens grew coagulase-negative Staphylococcus . None of patients had underlying systemic disease and all had complete resolution of the lesions within 3 weeks. Immunohistochemical staining for IL-8 was negative in the 10 cases studied. CONCLUSION: Childhood NEH appears as urticaria-like erythematous nodules and plaques on the limbs, trunk, or scalp. This benign and limited disease occurs with a predilection for summer months. In our study, onset was in children less than 15 months of age. IL-8 was not detected in the cutaneous lesions.

Concurrent mycosis fungoides and precursor B cell lymphoblastic lymphoma in a 6-year-old child.

We report a 6-year-old boy with mycosis fungoides on the dorsal surface of his left hand, a diagnosis supported by positive T cell receptor gamma gene rearrangement findings. Precursor B cell lymphoblastic lymphoma of the left orbit developed subsequently. He was treated with chemotherapy and remained under control.

Complications mimicking lupus flare-up in a uremic patient undergoing pegylated liposomal doxorubicin therapy for cervical cancer.

Patients with systemic lupus erythematosus (SLE) have an increased risk for malignancy and end-stage renal disease itself might further augment the risk. Treating uremic patients with cervical cancer by cisplatin-based chemotherapy combined with radiation is hampered by the reduced renal excretion of cisplatin. Doxorubicin, a potential radiosensitizer with an established effect on carcinomas that arise in the ovary, uterine cervix and endometrium, might be applied in these cases. We describe a 36-year-old woman, who had a 9-year history of SLE and was maintained on dialysis, and who developed severe drug reaction manifesting as fever, skin rash and exfoliative dermatitis with positive lupus band test after infusion of pegylated liposomal doxorubicin therapy for advanced cervical cancer. These skin manifestations improved after i.v. methylprednisolone pulse therapy.

Cutaneous lymphocytic vasculitis as the presenting feature of acute lymphoblastic leukemia.

The authors describe a patient with precursor B-cell acute lymphoblastic leukemia who presented with a 3-week history of indurated or ulcerative, purpuric lesions distributed mainly on her legs. A skin biopsy demonstrated a T-cell-mediated lymphocytic vasculitis. After the patient started chemotherapy, the skin lesions abated but she became febrile and a blood culture revealed cryptococci. The cryptococcal infection was successfully treated first with amphotericin B and later with fluconazole. The relationship between lymphocytic vasculitis and acute lymphoblastic leukemia may be an example of paraneoplastic association because both conditions seem to have appeared at about the same time, and both followed a parallel course. Lymphocytic vasculitis may also reflect a new manifestation of host-leukemia interaction.