I-Hsin Shih

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.

Myoepitheliomas of the Skin and Soft Tissue-A Clinicopathologic Study of Three Cases

Myoepithelioma of the skin and soft tissue is a newly recognized entity with characteristic histopathologic and immunohistochemical features, which should be differentiated from a variety of tumors. Myoepitheliomas are in the same pathologic spectrum of mixed tumors and parachordomas. Tumors comprised mostly of myoepithelial cells without obvious epithelial differentiation are designated myoepitheliomas. Since the entity has not been well documented in Taiwan, the clinicopathologic features of three cases of cutaneous and soft tissue myoepitheliomas are reported.

Juvenile Pityriasis Rubra Pilaris in Chang Gung Memorial Hospital, Taipei and Linkou: A Retrospective Study in the Past Ten Years

Pityriasis rubra pilaris (PRP) is an uncommon dermatosis in childhood. Only a few long-term studies to evaluate the course and prognosis of PRP in children have been done. In this study, we retrospectively reviewed the clinical manifestation, family history, course of the disease and response to treatment in 18 patients with PRP ages 18 years old or younger. These patients were seen at Chang Gung Memorial Hospital, Taipei and Linkou, between 1997 and 2006. Of the 18 patients, 11 were male and 7 were female. The most common sites of involvement were the palms and soles, knees, elbows, and buttock. According to Griffiths classification, 4 patients were classified as type III PRP, and the other 14 patients were classified as type IV PRP. In our studies, we suggested that palmoplantar involvement could be a diagnostic feature in juvenile PRP and buttocks could be another common site of involvement in type IV PRP. All patients were treated in an outpatient setting with topical agents. In addition to topical treatment, a few patients took acitretin for short durations. In our studies, there is no conclusion on the treatment of choice for juvenile PRP, but generally speaking, the prognosis of juvenile PRP is good.

Congenital Malignant Rhabdoid Tumor of the Skin-Report of a Case and Review of the Literature

Malignant rhabdoid tumor is a well-established clinicopathologic entity occurring classically in the kidney and central nervous system in children. Cutaneous origin has rarely been reported. We herein report a male newborn infant presented with an erythematous and ulcerated mass on his lower back at birth and was found to be a malignant rhabdoid tumor, diagnosed by histopathologic and immunohistochemical studies. In spite of aggressive treatment with chemotherapy and surgery, cerebral and pulmonary metastases developed later. The patient finally died at 10 months of age. This rare case is reported and literatures are reviewed.

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

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.

Hyperimmunoglobulin E syndrome presenting as eosinophilic pustular folliculitis: a case report

Hyperimmunoglobulin E syndrome (HIES) is a rare primary immunodeficiency disorder characterized by high serum IgE levels, eosinophilia, eczema, and recurrent cutaneous and sino-pulmonary infections. It was first described as Job’s syndrome in 1966 in two girls with recurrent skin abscesses, pneumonia, and chronic eczema. In 1972, hyperimmunoglobulin E syndrome (HIES) was named after the associated feature of greatly elevated serum IgE. Three genetic mutations of HIES have been found successively since 2006: tyrosinase kinase 2 (TYK2), signal transducer and activator of transcription 3 (STAT3), and dedicator of cytokinesis 8 (DOCK8). Skin findings of HIES similar to atopic dermatitis or eczema were described in previous literatures. Herein is a case of STAT3-mutated HIES manifesting with eosinophilic pustular folliculitis (EPF)-like symptoms. The cutaneous manifestations of HIES have also been reviewed.

Exanthem of measles and clues to its diagnosis: experience of a nosocomial outbreak in Taiwan

Taiwan has been experiencing a resurgence of measles since the beginning of 2009. We retrospectively reviewed the clinical presentations of eight confirmed measles cases during a nosocomial outbreak, in order to identify diagnostic clues. The exanthem was polymorphous and non-specific in terms of its morphology, distribution, sequence of appearance, and symptoms. The presence of cough and coryza as well as abnormal results of routine laboratory studies were also non-specific and unhelpful for the differential diagnosis of measles from other infectious diseases, adverse drug reactions, or autoimmune diseases. In our experience, the combination of exudative conjunctivitis with febrile exanthem is the most distinguishing feature of measles. Serologic studies can confirm the diagnosis within a few hours. Further measles outbreaks are possible in the near future as a result of increased international travel, immigration of foreign workers, and overseas marriages. Dermatologists must be proficient in the diagnosis of measles in order to prevent the potential morbidity and mortality associated with future outbreaks.

Congenital Self-Healing Reticulohistiocytosis Presenting as Hypopigmented Macules and Papules in a Neonate

Congenital self-healing reticulohistiocytosis, also known as Hashimoto-Pritzker disease) is a rare variant of Langerhans cell histiocytosis. It is characterized by developing multiple or single red-brown papules, nodules or vesicles at birth or first few weeks of life and involutes spontaneously without systemic involvement of other organs. We report a rare case of female neonate presented with multiple white macules and papules on trunk. Langerhans cell histiocytosis is diagnosed by histological examination and immunohistochemical study. We remind dermatologists to be aware of this rare presentation and should put it into differential diagnosis lists when facing hypopigmented lesions in a neonate.

Thalidomide-induced severe facial erythema in a patient with hepatocellular carcinoma

Thalidomide (a-phthalimidoglutarimide) has become an increasingly prescribed medication for various disorders thought to have an autoimmune or inflammatory basis. With the most notable dangerous adverse effects, such as teratogenicity, peripheral neuropathy and somnolence (1–7), cutaneous adverse effects reported include erythema multiforme, toxic epidermal necrolysis, erythroderma, toxic pustuloderma, morbilliform rash and nonspecific dermatitis (8). Herein, we report on a case of severe facial erythema developed while taking thalidomide.

Multicentric Infantile Myofibromatosis-A Case Report

We present a case of multicentric infantile myofibromatosis in a 3-month-old male infant who had multiple papular lesions on his extremities and trunk and a slowly growing and bulging mass on his left occipital area since birth. His general physical condition was good and psychomotor development was normal. The diagnosis was established by histopathological and immunohistochemical studies along with clinical manifestations and imaging findings. The patient underwent left occipital craniectomy and the defect was repaired with bone cement. He had been doing well with spontaneous regression of one of the skin lesions on his face 6 months after the operation.