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Benign Cephalic Histiocytosis


Presentation

  • 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.[ncbi.nlm.nih.gov]
  • Benign cephalic histiocytosis not to be confused with "Neonatal cephalic pustulosis" is a rare skin condition affecting boys and girls equally, characterized by skin lesions that initially present on the head in all cases, often the cheeks, eyelids, forehead[en.wikipedia.org]
Asymptomatic
  • 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.[ncbi.nlm.nih.gov]
Pathologist
  • The excised specimen was submitted to the pathologist, which revealed ill-defined histiocytes infiltrated in superficial and deep dermis. The histiocytes revealed vesicular nuclei with fine chromatin pattern and abundant clear cytoplasm.[omicsonline.org]
Weakness
  • However, recent reports have suggestedthat focal or weak S-100 immunopositivity can beseen.3 Similarly, Tomaszewski and Lupton described7 of 29 xanthogranulomas and reticulohistiocytomasthat expressed S-100 protein yet lacked expressionof CD1a.4 These[docslide.net]
Falling
  • Cutis . 2015 June;95(6):E15–E17 Benign cephalic histiocytosis (BCH) falls into the group of non–Langerhans cell histiocytosis (non-LCH), which is characterized by a benign course and tendency toward spontaneous remission.[mdedge.com]
Sore Throat
  • Other symptoms include cough, sore throat, difficulty swallowing, weight loss and tiredness, in cases that present in extranodal sites outside of the head and neck region, organ specific symptoms are observed.[wikivisually.com]
Papule
  • Benign cephalic histiocytosis is a rare skin condition consisting of small tan papules on the face and upper trunk that is believed not to be associated with internal organ involvement. The infiltrating histiocytes are not Langerhans' cells (LCs).[ncbi.nlm.nih.gov]
  • The differentials include Langerhans cell histiocytosis (red-brown papules that show erosion, hemorrhage and crusting), juvenile xanthogranuloma (orange/yellow papules often with mucosal/ocular involvement), urticaria pigmentosa (yellowish papules turning[indianpediatrics.net]
Darier's Sign
  • Mucosa, palms, and soles were spared and Darier's sign was negative. There was no hepatosplenomegaly and lymphadenopathy. Skeletal, neurodevelopmental, and ophthalmological examination were normal.[ijpd.in]
  • Darier sign was negative. The boy was otherwise healthy and developmentally normal. All laboratory tests were within reference range and his family history was uneventful. Figure 1.[mdedge.com]
Facial Papule
Shagreen Patch
Skin Papule
  • We report a 5-year-old girl who presented with diabetes insipidus 1 year after developing multiple small brown asymptomatic skin papules. Histologic examination revealed a non-LC histiocytic proliferation in the dermis without epidermal invasion.[ncbi.nlm.nih.gov]
Stroke
  • Langerhans cell histiocytosis (red-brown papules that show erosion, hemorrhage and crusting), juvenile xanthogranuloma (orange/yellow papules often with mucosal/ocular involvement), urticaria pigmentosa (yellowish papules turning into weals on firm stroking[indianpediatrics.net]

Workup

  • Progress logically from the histologic pattern, through the appropriate workup, around the pitfalls, to the best diagnosis.[books.google.de]
  • An initial laboratory workup revealed anemia, thrombocytopenia, and hepatosplenomegaly. A working diagnosis of Langerhans cell histiocytosis was reached that was later confirmed by a skin biopsy.[scidocpublishers.wordpress.com]
Lymphocytic Infiltrate
  • infiltrates approximating the epidermal undersurface), and Spitz nevi (clusters of melanocytes with amphophilic cytoplasm and often prominent nucleoli at the dermal-epidermal junction, in the dermis or both) from BCH.[dermatologyadvisor.com]

Treatment

  • Benign cephalic histiocytosis is a self-limiting condition that requires no treatment.[ncbi.nlm.nih.gov]
  • The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.[orpha.net]
  • Although treatment is not usually indicated, a recent case report demonstrated accelerated regression over 3 months with use of antibiotic treatment that was required due to co-existing immmunoglobulin A deficiency and bacterial superinfection.[dermatologyadvisor.com]

Prognosis

  • The severity and prognosis depend on the type and extent of organ involvement. The disease classically presents with three syndromes namely Eosinophilic granuloma, Letterer-Siwe disease, and Hand-Schuller-Christian disease.[scidocpublishers.wordpress.com]
  • […] in cases with pulmonary hypertension Worse prognosis for those with baseline poor respiratory function and those with concurrent neoplasms Worse prognosis with extremes of age, prolonged constitutional symptoms, multiorgan involvement, extensive cysts[pathologyoutlines.com]
  • , interdigitating dendritic cells, plasmacytoid dendritic cells, microglia, Kupffer cells, and alveolar macrophages CLINICAL ISSUES Epidemiology Age Depends on type of histiocytosis Treatment Benign tumors that do not require treatment in most cases Prognosis[basicmedicalkey.com]
  • The prognosis is quite good even if multisystem involvement occurs. When serious complications occur they are usually related to any underlying blood malignancies rather than the histiocytosis itself.[dermnetnz.org]
  • Prognosis Patients with only skin or soft tissue involvement have a high survival rate, and lesions can spontaneously disappear over time in a majority of cases.[atlasgeneticsoncology.org]

Etiology

  • Joo EJ Park, Hee Jin HJ Cho, Kwang Ho KH Kim, Kwang Joong KJ Kim Indexed on: 27 Oct '11 Published on: 27 Oct '11 Published in: Annals of dermatology Abstract Benign cephalic histiocytosis (BCH) is a rare non-Langerhans cell histiocytosis of unknown etiology[sparrho.com]
  • Katie Morrison, MD, University of Texas Houston, Houston, TX, United StatesThe etiology of histiocytic and nonhistiocytic disorders is poorly understood.[docslide.com.br]
  • Etiology Pathophysiology The etiology of BCH is unknown, but it is theorized to exist on a spectrum with other non-Langerhans cell histiocytoses, including generalized eruptive histiocytosis (GEH) and JXG.[dermatologyadvisor.com]
  • Etiology of disease is still unknown. Some investigators believe that it is a variant of JXG or GEH or perhaps they all belong within the spectrum of one single disease.[ijdvl.com]
  • In any case, the pathogenesis and etiology of BCH are still unclear, and thus more researches are needed.[omicsonline.org]

Epidemiology

  • […] or dendritic cell lineage and functional variants Includes macrophages, Langerhans cells, interstitial dendritic cells, interdigitating dendritic cells, plasmacytoid dendritic cells, microglia, Kupffer cells, and alveolar macrophages CLINICAL ISSUES Epidemiology[basicmedicalkey.com]
  • Epidemiology Preferentially affects children, usually occurs by 10 years of age, with one-half of reported cases occurring in the first year of life. Twenty percent of the cases occur in adolescents and young adults.[atlasgeneticsoncology.org]
  • Langerhans cell granulomatosis, histiocytosis X ("H-X") Multisystem diseases: Letterer-Siwi disease, Hand-Schüller-Christian disease, Hashimoto-Pritzker syndrome, where lung involvement is histologically indistinguishable from cellular phase of PLCH Epidemiology[pathologyoutlines.com]
  • Histiocytic disorders of children and adults: introduction to the problem, overview, historical perspective and epidemiology. In: Weitzman S, Egeler RM, editors. Histiocytic disorders of children and adults.[link.springer.com]
Sex distribution
Age distribution

Pathophysiology

  • Etiology Pathophysiology The etiology of BCH is unknown, but it is theorized to exist on a spectrum with other non-Langerhans cell histiocytoses, including generalized eruptive histiocytosis (GEH) and JXG.[dermatologyadvisor.com]
  • - 50 years More common in Caucasian than African Americans or Asians Sites 50% of cases only involve lung 20% of those with multicentric disease (i.e. bone, skin, lymph nodes, spleen, CNS, pituitary, rarely thyroid and thymus) have lung involvement Pathophysiology[pathologyoutlines.com]
  • Histiocytic disorders: recent insights into pathophysiology and practical guidelines. Biol Blood Marrow Transplant. 2010;16(1 Suppl):S82–9. PubMed Google Scholar 10. Favara BE, Feller AC, Pauli M, Jaffe ES, Weiss LM, Arico M, et al.[link.springer.com]

Prevention

  • The information presented here is not intended to diagnose, treat, cure or prevent any disease. Read disclaimer.[gulfdoctor.net]
  • Hematology – Hematology, also spelled haematology, is the branch of medicine concerned with the study, diagnosis, treatment, and prevention of diseases related to blood.[wikivisually.com]
  • ,To improve curability and prevent reactivation and permanent consequences, early definitive diagnosis and optimal therapeutic strategies need to be determined prospectively.[ci.nii.ac.jp]
  • There is no known prevention of this disease. Langerhans cell histiocytosis; Erdheim-Chester disease Diamond EL, Durham BH, Haroche J, et al. Diverse and targetable kinase alterations drive histiocytic neoplasms. Cancer Discov . 2016; 6(2):154-165.[medlineplus.gov]

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