Childhood Granulomatous Periorificial Dermatitis

Childhood granulomatous periorificial dermatitis (CGPD), also known as facial Afro-Caribbean childhood eruption (FACE), is a distinctive granulomatous form of perioral dermatitis. It is a condition of unknown etiology, characterized by monomorphous, small, papular eruptions around the mouth, nose and eyes that histopathologically show a granulomatous pattern. It affects prepubescent children of both sexes and typically persists for several months but resolved without scarring. We report a 9 year-old girl with multiple, discrete, monomorphic, papular eruptions of 2-months duration on the perioral and periocular areas. Histopathological examination demonstrated upper dermal and perifollicular granulomatous infiltrate.     

Childhood Granulomatous Periorificial Dermatitis with a Good Response to Oral Metronidazole

Childhood granulomatous periorificial dermatitis (CGPD) is a condition included in the spectrum of rosacea and perioral dermatitis that affects prepubescent children. It is characterized by papular eruptions located around the mouth, nose, and eyes that histopathologically show a granulomatous pattern. We report a 9‐year‐old girl with CGPD who required treatment with an oral antibiotic. Oral metronidazole was administered because of its known efficacy in adult rosacea and its safety in children. The patient responded well to this therapy, showing resolution of the lesions.     

Interstitial Granulomatous Dermatitis with Plaques

An 8‐year‐old male presented with several 1–4 cm, indurated, brownish‐red nodules with superficial, punctate hemorrhagic crusts localized to the groin, which developed over several weeks. An initial biopsy was consistent with Langerhans cell histiocytosis. A work up, including a chest and abdominal CT scan demonstrated a large mediastinal mass which was diagnosed as precursor T cell lymphoblastic lymphoma. Treatment with a chemotherapeutic protocol resulted in shrinkage of both the mediastinal and cutaneous lesions. Late in the course of treatment the cutaneous lesions rapidly recurred and spread to various sites. A second skin biopsy showed an extensive diffuse infiltrate of histiocytic‐appearing cells extending from the papillary dermis into the superficial subcutis. The cells had markedly pleomorphic, vesicular nuclei and abundant amphophilic cytoplasm. Numerous mitotic figures were present. The neoplastic cells were S‐100 protein and CD1a positive by immunohistochemistry. The histopathologic diagnosis was Langerhans cell sarcoma. Gene rearrangement studies demonstrated clonal rearrangement of the T cell receptor gamma gene in specimens from both the mediastinal mass and one of the recurrent cutaneous lesions. The sizes of the PCR products were identical demonstrating a clonal relationship between the two neoplasms.     

Recurrent Granulomatous Dermatitis with Eosinophilia

ABSTRACT: A 27-year-old woman developed a chronic, recurrent eruption of the face and upper extremities with the clinical and histopathologic features of recurrent granulomatous dermatitis with eosinophilia (Wells'syndrome). As described in 15 previously reported cases, this disorder is characterized by two clinical phases (ensinophilic cellulitis and granuloniatou plaque phase) and three histopathologic stages. The latter are particularly remarkable for a diffuse dermal and subcutaneous eosinophilia in acute lesions and scattered flame figures in chronic lesions. Distinctive findings in this case were the predominance of facial involvement and the symptomatic response to topical corticosteroids. Although etiology and pathogenesis are unknown, we feel that Wells'syndrome is a unique yet rarely recognized clinicopathologic entity.     

肉芽肿性口周皮炎一例

患者男,35岁,5个月前面部出现红斑及丘疹,逐渐增多,病前曾外用丙酸氯倍他索霜等糖皮质激素。皮损为直径1～3mm大的淡红色坚实小丘疹,主要分布于眶内侧、鼻周及口周等处。病理检查显示,真皮毛囊皮脂腺周围有上皮细胞肉芽肿病变。明确诊断后停用糖皮质激素,局部对症处理,半年后皮损消退。     

Granulomatous Slack Skin in Childhood

Abstract: Granulomatous slack skin is an uncommon cutaneous T- helper cell lymphoma closely related to mycosis fungoides. To the best of our knowledge this disease has not been previously described in children. We report on an 11-year-old boy who presented with painless slack skin masses in the neck, right axilla and arm, anterior wall of the abdomen, both inguinal regions, and the malleolar and dorsal aspects of the feet. The disease started 3 years earlier with erythematous lesions on the neck and wrists. Histologic examination of a specimen from the abdominal mass revealed an extensive lymphoid Infiltrate with scattered multinucleated giant cells extending from the papillary dermis to the subcutis. The lymphoid cells showed the following immunophenotype: CD43+ (MT1), CD45+, CD45RO+, CD20-. The phenotype of the giant cells was lysozyme positive, CD68+ and Mac387–. The tumoral lymphoid cells had clonal rearrangement for the gene of the beta chain of the T-cell receptor (CβTCR). The disease could be controlled with systemic glucocorticoids. Due to the presence of many histiocytes arranged in aggregates in the papillary and mid-dermis, this case was initially considered to be a cutaneous form of histiocytosis. We recommend deep and extensive biopsies in patients with slack skin disease.     

Periauricular Eczematization in Childhood Atopic Dermatitis

Abstract: We investigated the prevalence of periauricular eczematization (PAE) in children with atopic dermatitis (AD) in Korea, and evaluated the clinical significance of the lesion in terms of Its treatment in relation to a possible pathogenetic role for Staphylococcus aureus. Tentatively, PAE was classified as infraauricular and postauricular fissuring, which were observed in 62 (57,9%) and 37 (34.6%) of 107 patients, respectively. Sixty eight patients (64.5%) had infraauricular or postauricular fissures, or both, and this prevalence was significantly (p < 0.01) higher than that in controls (1.3%). Staphylococcus aureus was cultured from oozing material from infraauricular fissures in 12 of 14 patients, and topical application of erythromycin cream to the ear lesions led to faster and more definite improvement. Our study shows that PAE is a characteristic clinical manifestation of AD, probably induced by S. aureus. Topical antibiotics are worth trying for effective management of this disorder.     

A Child with Interstitial Granulomatous Dermatitis and Juvenile Idiopathic Arthritis

We describe a case of interstitial granulomatous dermatitis (IGD) with juvenile idiopathic arthritis (JIA) in an 11‐year‐old girl. She complained of erythematous plaques on her thighs and polyarthritis for 1 year. Histopathologic examination revealed the features of IGD. This case indicates that IGD with arthritis can occur in children and should be considered in the differential diagnosis of cutaneous lesions associated with arthritis in children.     

Steroid‐induced Periorificial Dermatitis in Children—Clinical Features and Response to Azelaic Acid

Abstract: Periorificial dermatitis, a common skin disease in young women, has been occasionally reported in children. This study elaborates the clinical features of periorificial dermatitis in children as well as possible pathogenetic factors and the response to 20% azelaic acid cream. A total of 10 children aged 3 to 12 (mean 7.7) years suffering from nongranulomatous periorificial dermatitis for 3 to 7 (mean 4.9) months were evaluated, and dermatologic examination was carried out. Pretreatment was documented. Skin prick tests with a panel of six common inhalative allergens and patch tests with the European Standard Series were performed. An association between atopy and periorificial dermatitis was evaluated, and patients were screened for skin colonization by fungi, bacteria, and Demodex mites. They were treated with 20% azelaic acid cream, which was topically applied twice daily on all affected areas, until complete resolution was achieved. Treatment period was followed by an individual observation period. Periorificial dermatitis had developed in typical distribution and morphology. In all patients, low‐ to high‐potency topical corticosteroids had been used on the face prior to manifestation. Atopy was found in half of the patients. Allergological, bacteriological, and mycological examinations did not reveal pathologic results. Demodex mites could not be demonstrated by skin surface biopsy. Treatment with 20% azelaic acid cream led to complete resolution of skin lesions after 4 to 8 (mean 5.4) weeks in all patients. Transient exacerbation of skin condition with a peak between the 2nd and 6th day of treatment could be observed in three patients. Side effects of 20% azelaic acid cream were registered in six patients and were predominantly present in the first 2 weeks of treatment. Side effects were minimal and became rarer with ongoing treatment. No recurrences were seen within a follow‐up period of 2 to 8 (mean 4.4) months. Treatment with 20% azelaic acid cream could provide an effective and safe alternative therapeutic option in children with nongranulomatous periorificial dermatitis.     

Anti‐TNF‐Alpha Antibody Induced Interstitial Granulomatous Dermatitis

Cutaneous melanoma is a common and frequently lethal melanocytic neoplasm that can quickly metastasize to regional lymph nodes. Currently, prognosis is determined by measuring tumor thickness but more reliable markers for metastatic spread are urgently needed. We investigated whether the extent of tumor lymphangiogenesis can predict melanoma metastasis to sentinel lymph nodes (SLN). We quantified the extent of tumor lymphangiogenesis, as well as other factors, in excised primary tumors and in SLN biopsy samples from 45 patients with primary cutaneous melanoma. Primary melanomas from patients whose tumors had metastasized to the SLN, along with their metastastic foci, contained prominent hotspots of increased lymphatic vessel density, compared to non‐metastatic tumors. Multivariate risk analysis revealed that the lymphatic vascular area (LVA) of primary melanomas, an index of tumor lymphangiogenesis, was the most sensitive prognostic marker for SLN metastasis, and was even able to more accurately predict which tumors would become metastatic to SLN than measuring tumor thickness. The extent of tumor lymphangiogenesis is a highly sensitive (83%) and specific (89%) prognostic marker of lymph node metastasis. Assessment of lymphangiogenesis in primary melanomas may be a more effective approach than measuring tumor thickness in selecting patients with early metastatic disease for aggressive therapy.     

湿包裹治疗儿童重度特应性皮炎的疗效观察

目的 观察湿包裹(WWT)治疗儿童重度特应性皮炎(AD)的疗效和安全性.方法 2018年9月-2019年9月在南京儿童医院皮肤科门诊诊断为重度AD的患儿(符合Williams诊断标准,SCORAD ＞50分)40例,随机分为WWT组(n =20)和传统治疗组(n=20).WWT组采用0.1％糠酸莫米松乳膏联合2％莫匹罗...     

Palisaded Neutrophilic Granulomatous Dermatitis in a Child with Juvenile Idiopathic Arthritis on Etanercept

Palisaded neutrophilic granulomatous dermatitis (PNGD) is a rare neutrophilic dermatosis that may be associated with significant morbidity because of associated pruritus and pain. It is typically seen in adults with collagen vascular diseases, but may be associated with medications as well. PNGD is rarely reported in children. We describe a girl with polyarticular juvenile idiopathic arthritis who developed PNGD after administration of etanercept and demonstrated rapid and significant improvement upon treatment with topical triamcinolone 0.1% ointment.     

Sporadic Blau Syndrome With Onset of Widespread Granulomatous Dermatitis in the Newborn Period

Abstract:  Blau syndrome is a dominantly inherited, chronic autoinflammatory disorder characterized by the clinical triad of granulomatous dermatitis, symmetric arthritis, and recurrent uveitis with onset below 4 years of age. It is caused by activating mutations in the nucleotide-binding oligomerization domain 2 ( NOD2 ) gene, previously referred to as CARD15 gene. Noncaseating granulomas in affected tissues are the pathologic hallmark of the condition. We report the lifelong severe disease course in a 14-year-old Caucasian boy with sporadic Blau syndrome. Unusually, granulomatous dermatitis started in the first week of life. Whereas skin involvement faded away spontaneously in subsequent years, polyarthritis and anterior uveitis appeared in the second and third year of life respectively. Mutational analysis of the NOD2 gene revealed a missense mutation (R334W) previously detected in other Blau syndrome pedigrees. With this report, we would like to stress the rare possibility of Blau syndrome in generalized papular rashes of infancy and the importance of histopathologic study for clarification. The finding of early-onset widespread granulomatous dermatitis should prompt eye and joint examination in regular intervals and entail mutational analysis of the NOD2 gene.     

A Case of Herpes Zoster Granulomatous Dermatitis: Report of Wolf's Isotopic Response

Wolf''s isotopic response refers to the occurrence of a novel skin disease at the site of a preceding treated or untreated skin disease. Although the most common preceding skin disease was found to be herpes zoster (HZ), HZ-related dermatological phenomena are not well known in the literature. We report a case of HZ granulomatous dermatitis in a 77-year-old female with a previous history of hypertension, diabetes mellitus, chronic kidney disease, and HZ. She presented with a 3-month history of a pruritic skin lesion on her right thigh. The location of the lesion was consistent with a previous HZ site. Histopathological examination revealed lympho- histiocytic infiltration in the superficial dermis, forming a granulomatous structure. Based on clinical and histopathological findings, we made a diagnosis of granulomatous dermatitis at a previous HZ site. We assumed that the lesion arose from an isotopic response of Wolf. The patient was treated with topical steroids for 3 months and showed clearance of the lesion and symptom. We suggest that treatment should be based on the individual disease, which in our case was topical steroid.     

The Course of Life of Patients with Childhood Atopic Dermatitis

Abstract:   Atopic dermatitis mainly covers the period of infancy to adulthood, an important period in the development of an individual. The impairment of quality of life and the psychological wellbeing of children with atopic dermatitis have been well documented but so far no data exist about the impact of atopic dermatitis in childhood on fulfilling age-specific developmental tasks and achieving developmental milestones during this period, referred to as the course of life. The aims of this study were to: (i) assess the course of life and define the disease-related consequences in young adult patients with childhood atopic dermatitis and (ii) determine whether the severity of atopic dermatitis is predictive for the course of life, the disease-related consequences and quality of life later in life. Adult patients who grew up with atopic dermatitis were asked to complete a medical history questionnaire, the Skindex-29, the "course of life" questionnaire and a subjective disease-specific questionnaire. Patients with severe atopic dermatitis in childhood showed a significant delayed social development in their course of life. The results of the disease-specific questionnaire demonstrated remarkable high percentages of psycho-social consequences and physical discomfort caused by atopic dermatitis in childhood. Patients showed a severely negative impact of atopic dermatitis on their current quality of life. This is the first study that applied the "course of life" questionnaire in atopic dermatitis. More insight in the course of life, disease-specific consequences and quality of life of atopic dermatitis is of high importance, especially in case of severe atopic dermatitis.