Benign Cephalic Histiocytosis Research Articles

The use of topical Rapamycin in successfully treating non-Langerhans cell histiocytosis in a pediatric patient

Non-Langerhans cell histiocytosis (non-LCH) refers to a collection of medical conditions distinguished by the excessive growth of histiocytes in bodily tissues. It is important to note that these conditions do not meet the established diagnostic criteria for Langerhans cell histiocytosis (LCH). Juvenile xanthogranuloma (JXG) and benign cephalic histiocytosis (BCH) represent the prevailing forms of cutaneous non-LCH. We present a case of JXG which responded to topical treatment with a 0.1% topical ointment of Rapamycin. Rapamycin, an immunosuppressive and antineoplastic agent, can thus be a viable alternative non-invasive topical modality for managing JXG. However, it is imperative to conduct prolonged observations to evaluate the efficacy and potential adverse reactions.

Cutaneous mastocytosis in an infant: Case report and clinicopathological correlation

Mastocytosis is a disorder of clonal proliferation of the mast cells, which can be cutaneous or systemic. Abnormal mast cell infiltration comprising multifocal compact clusters or cohesive aggregates can affect one or more organ systems. Cutaneous mastocytosis is a relatively uncommon condition in the pediatric population. We report a case of 9 month infant presented with multiple papular and vesicular skin rashes since 6 months of age. On clinical examination Darier’s sign was negative. The serum tryptase levels were within normal limits. Clinical differential diagnoses were benign cephalic histiocytosis vs cutaneous mastocytosis. Skin biopsy revealed a mononuclear cell infiltrate in the papillary dermis reaching up to the dermo-epidermal junction. Toluidine blue staining highlighted the metachromatic granules. CD117, CD30 IHC stains were positive which confirmed the diagnosis of cutaneous mastocytosis. This case is presented to highlight the histomorphology and the special stains in cases of mastocytosis.

Spontaneous Resolution of Asymptomatic Papules on the Face of a Two-Year-Old

A nine-month old male infant presented with multiple, asymptomatic, and yellow-to-pink lesions on his face. Physical exam revealed multiple 2-5 mm reddish brown papules on the forehead, nose, cheeks, and chin Laboratory findings, developmental milestones, and ophthalmology exam were within normal limits, and biopsy was consistent with benign cephalic histiocytosis. Lesions spontaneously involuted at age 18 months without treatment, and by age two, the lesions had fully resolved without sequelae.

Benign cephalic histiocytosis: an atypical case with plaque-like presentation

Benign cephalic histiocytosis: an atypical case with plaque-like presentation

Childhood Pseudoxanthomatousmastocytosis; Report of two cases

Cutaneous mastocytosis (CM) is a mast cell proliferative disorder that commonly affects pediatric population. It has various clinical forms such as urticaria pigmentosa, solitary mastocytoma, telangiectasia macularis eruptiva perstans, diffuse CM, and erythrodermic mastocytosis. Pseudoxanthomatous mastocytosis is a type of diffuse CM, which is relatively rare. It can mimic xanthoma disseminatum, juvenile xanthogranuloma, and benign cephalic histiocytosis which are also seen commonly in children but have different prognostic behavior. Hence, high degree of clinical suspicion along with histopathology is required for the diagnosis. Herein, we report two cases of childhood pseudoxanthomatous mastocytosis.

Novel Clinical Observations on Benign Cephalic Histiocytosis in a Large Series.

Benign cephalic histiocytosis (BCH) is a rare form of non-Langerhans cell histiocytosis in infants and young children characterized by self-healing macules and papules occurring primarily in the head and neck region. So far there have been nearly 60 reported cases in the English-language literature. In this retrospective study, we evaluated clinical features and follow-up data of 11 patients diagnosed with BCH between 2004 and 2016 in the Department of Dermatovenereology, Istanbul Medical Faculty. There were 5 girls and 6 boys (median age 24 months, range 9-72 months). The median age at the onset of lesions was 8 months (range 3-36 months). The lesions first appeared on the face in 10 patients and on the trunk in 1. Proximal parts of the extremities and trunk were also involved in nine patients (81.8%). Patients were categorized into two groups based on their clinical features; five had 20 to 30 predominantly red-brown dome-shaped papules and six had 50 to hundreds of yellow-brown or predominantly pinkish brown flat papules. Four patients were lost to follow-up. In seven patients with a mean follow-up of 5 years, four had nearly complete resolution and three showed remarkable regression without treatment. With 11 additional cases from a single center, BCH seems to be an underrecognized disease. Its clinical presentation is not uniform. Considering that most of the patients in this series and those previously reported had extracephalic involvement, the term "cephalic" needs to be reevaluated.

Solitary Benign Cephalic Histiocytosis in a Taiwanese Infant

Benign cephalic histiocytosis (BCH) is a rare type of non-Langerhans histiocytosis with features of many small yellow, yellow-red, or yellow-brown papules located on face, head, neck, and shoulders of infants and young children. There would be no associated symptoms. The histopathological images reveal infiltration of histiocytes in dermis; immunohistochemically, the histiocytes are positive for CD68 stain, but negative for S-100 and CD-1a stain. The BCH would regress spontaneously with time, and thus no treatment is needed. However, in some cases, there would be some pigmentation or atropic scar remained. This is such a rare disease, and thus there is no case of BCH been reported in Taiwan. We recently have a chance to observe an 11-year-old boy with a solitary papule of BCH on his right scalp.

Indeterminate cell histiocytosis that presented clinically as benign cephalic histiocytosis

Indeterminate cell histiocytosis (ICH) is a rare, heterogeneous disorder that is characterized by immunophenotypic features of both Langerhans cell histiocytosis (LCH) and non-LCH. We describe a 12-month-old boy with a four-month history of asymptomatic, small, pink-tan papules on his face. Histopathologic evaluation showed a superficial, dermal infiltrate of histiocytes that was positive for S100, CD1a, CD68, and Factor XIIIa. To our knowledge, this represents the first report of the clinical presentation of benign cephalic histiocytosis with immunohistochemical findings of ICH. We review the classification of histiocytic disorders and the clinical and immunohistochemical features of both ICH and benign cephalic histiocytosis.

English

Papular histiocytosis of the head also known as benign cephalic histiocytosis is a rare, self-limiting histiocytosis that typically starts in early childhood. 1 Erythematous macules, papules and nodules develop on cheeks and spread to the forehead, earlobes and neck. Lesions are asymptomatic, gradually become reddish brown and may spread to involve the trunk, shoulders and back region including buttocks. There is no mucous membrane involvement. We report a case of 1 and half year boy, born of non-consanguineous marriage presented with multiple, asymptomatic, erythematous, yellow to red color papules on cheeks and back. Lesion was slowly progressive in nature for the past 7 months. Clinical and histological features were consistent with papular histiocytosis of head.

Eponyms in the dermatology literature linked to ''Bodies'', seen in Skin biopsies

In dermatology practice, it is very common to hear about „Bodies’’, which refer to a pthological structure with a particular features. Most of them are large and can be seen by light microscopy, but there are few very tiny bodies which can only be seen by electron microscopy. Examples for the latter are comma-shaped body, and the worm-shaped body, seen in histiocytoses like benign cephalic histiocytosis (however; they are not specific), and zebra body, seen in mucopolysaccharidoses. Some of the bodies were seen in one disease and they are characteristic for one disease whereas others can be seen in multiple conditions. As an example for the former group, is caterpillar body, which is pale amorphous pink linear structures in the epidermis of porphyria cutanea tarda. Another example is the papillary mesenchymal body which is structure thought to be an abortive attempt of fibroblasts to form mesenchyme necessary for hair induction, reminiscent of early hair germ. They are seen in trichoblastoma and trichoepithelioma. Examples for the bodies which can be seen in multiple conditions include ,asteroid body for example might be seen in several conditions like sarcoidosis and berylliosis. Also, psammoma body is a concentric laminated, calcified bodies seen in papillary thyroid carcinoma, benign nevi, meningiomas, and other conditions. Most of the „bodies’’ are known by a single term. As an exception medlar bodies which are seen in chromoblastomycosis are also called sclerotic bodies and copper penny bodies. Eponyms are very common in the nomenclature of „bodies’’. In Table I [1-21], we are highlighting on Eponyms in the dermatology literature linked to „Bodies’’, seen in skin biopsies. EPONYMS IN THE DERMATOLOGY LITERATURE LINKED TO „BODIES’’, SEEN IN SKIN BIOPSIES

HISTIOCITOSE CEFÁLICA BENIGNA

A histiocitose cefálica benigna faz parte do grupo das histiocitoses de células não-Langerhans. De etio- logia desconhecida, é uma doença autolimitada, que surge na infância e se caracteriza por lesões papulosas que afetam fundamentalmente a face. Descrevemos o caso clínico de uma criança de 10 meses de idade que apresentava desde há 6 meses lesões papu- losas, eritemato-amareladas, assintomáticas e de localização exclusiva à face. O estudo histológico e imuno-histo- químico revelou a presença de um infiltrado difuso de histiócitos em toda a derme, negativo para a proteína S100 e CD1a e positivo para CD68. Perante o diagnóstico de histiocitose cefálica benigna, optou-se por uma atitude expec- tante verificando-se regressão clínica completa após um ano de seguimento. Desde a sua descrição por Gianotti, et al. em 1971, apenas 40 casos foram descritos na literatura.PALAVRAS-CHAVE – Histiocitose cefálica benigna; Histiocitoses de células não-Langerhans; Doença autolimitada.

Benign Cephalic Histiocytosis: Case Report and Review of the Literature

Benign cephalic histiocytosis (BCH) is a rare type of non-Langerhans histiocytic disorder, usually presenting with small, yellow-red or yellow-brown, asymptomatic papules, located mostly on the head and neck of infants and young children. The histopathologic hallmark of BCH is a well-circumscribed histiocytic infiltrate in the superficial to mid-reticular dermis. BCH is a self-healing disorder, with the eruptions regressing spontaneously in most cases; therefore no treatment is required. We present a case of BCH in a 7-month-old boy who was referred to our department with multiple, asymptomatic, yellow-red papules located on his face, and we review 55 cases published in the English-language literature in an effort to better describe and understand this unusual entity.

Benign cephalic histiocytosis

A one and a half year old girl born of a non-consanguineous marriage presented with multiple asymptomatic erythematous to hyperpigmented and skin colored papules on both cheeks slowly increasing in number of 1 year duration. On the basis of clinical, histopathological, and immunohistochemistry findings, a diagnosis of benign cephalic histiocytosis was made.

An Infant Who Has Dome-Shaped Papules

JXG is a benign disorder of early childhood and primarily occurs in infants and children <2 years of age. It is characterized by solitary or multiple yellow-red nodules on the skin and, rarely, in other organs. The clinical diagnosis of solitary cutaneous JXG usually requires confirmation by biopsy. Referral to a dermatologist should be obtained to exclude other disorders if multiple lesions are present. Clinicians should rule out ocular lesions with referral to an ophthalmologist if the patient is <2 years of age and has multiple lesions of JXG. If ophthalmologic consultation reveals no ocular lesions, screening every 6 months for the first 2 years of life is advised.

Caso para diagnóstico

The present paper reports a case of benign cephalic histiocytosis in a 15-month baby boy, who developed multiple papules bilaterally in the malar region with no other associated manifestations. Histopathology revealed a papillary dermal pattern, while immunohistochemistry was negative for S100 and CD1a and positive for CD68. Therefore, diagnosis was established as non-Langerhans cell histiocytosis, based on the clinical, histopathological and immunohistochemical features present.

Benign cephalic histiocytosis with S-100 protein positivity

Benign cephalic histiocytosis with S-100 protein positivity

A case of benign cephalic histiocytosis with clinical and histologic features of juvenile xanthogranuloma on follow-up: Additional support for a unifying concept

A case of benign cephalic histiocytosis with clinical and histologic features of juvenile xanthogranuloma on follow-up: Additional support for a unifying concept

Benign Cephalic Histiocytosis: A Case Report

Histiocytic skin disorders are usually classified as either Langerhans' cell histiocytosis (LCH) or non LCH, based on the pathology. Benign cephalic histiocytosis (BCH) is a rare type of non-Langerhans histiocytitic disorder and is characterized by self-healing multiple small eruptions of yellow to red-brown papules on the face and upper trunk. Histologic features of this disorder show dermal proliferation of histiocytes that have intracytoplasmic comma-shaped bodies, coated vesicles and desmosome-like structures. In this study, we report on a 7-month-old boy who contained small yellow-red papules on his face that spread to his upper trunk. The clinical and histologic features in this patient were consistent with BCH.

A Case of Benign Cephalic Histiocytosis

Benign cephalic histiocytosis (BCH) is a rare non-Langerhans cell histiocytosis of unknown etiology. Clinically, lesions are characterized by small, red-to-yellow papules distributed mainly on the head, face, neck, and shoulders of infants and children. Histopathological specimens show massive histiocytic infiltration of the superficial dermis. Immunohistochemically, they are positive for CD68, but negative for CD1a and S-100. Two cases have been reported so far in the relevant work published in Korean literature. Herein, we report on an additional case of BCH.

Possible Acceleration of Regression by Antibiotic Treatment in Benign Cephalic Histiocytosis with Infiltration of CD11a/CD11c+ Macrophages

© 2010 The Authors. doi: 10.2340/00015555-0872 Journal Compilation © 2010 Acta Dermato-Venereologica. ISSN 0001-5555 Benign cephalic histiocytosis (BCH) is a rare non-Langerhans’ cell histiocytosis (non-LCH) first reported by Gianotti et al. (1). It is characterized by an asymptomatic spontaneous eruption of reddish to brownish macules and papules on the head, mainly on the cheeks, eyebrows, forehead, ears, and neck, which spread later to the trunk and arms (1–3). It occurs within the first 3 years in infants of both sexes, resulting in, usually spontaneous, unscarred regression (2, 3). The aetiology of BCH is unknown; however, some cases report a coincidence with metabolic disorders such as diabetes insipidus and diabetes mellitus (4, 5).