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Background
This is a rare benign proliferation of histiocytes, found on the head and face. Although benign, the lesions may persists for months to years. The importance lies with the pathologist who must distinguish this disorder from Langerhans cell histiocytic syndromes. Broadly speaking, the histiocytoses can be divided into Langerhans and non-Langerhans cell histiocytoses. Langerhans cell histiocytoses are all defined by the presence of a unique ultrastructural organelle, the Birbeck granule. This tennis racquet shaped cytoplasmic organelle has an unknown function.
OUTLINE
EPIDEMIOLOGY CHARACTERIZATION INCIDENCE Rare AGE RANGE-MEDIAN Average 29 months
Benign cephalic histiocytosis: case report and literature review.Pena-Penabad C, Unamuno P, Garcia-Silva J, Ludena MD, Armijo M.
Department of Dermatology, University Hospital, Salamanca, Spain.
Pediatr Dermatol 1994 Jun;11(2):164-7 Abstract quote A 3-year-old boy had maculopapules on his face and neck since age 6 months. These were yellow-brown, asymptomatic, and clinically similar to flat warts. Histopathologic study revealed a fibrohistiocytic infiltrate in the superficial dermis.
Ultrastructurally, comma-shaped bodies, desmosome-like junctions, and coated vesicles were seen; there were no lipid droplets or Birbeck granules. With these data, a diagnosis of benign cephalic histiocytosis was made.
Twenty-five cases are reported in the literature: 17 males and 8 females (male:female ratio 2:1). Sixteen patients had lesions on parts of the body other than the head, neck, and shoulders.
GEOGRAPHY
Singapore Med J. 1999 Nov;40(11):697-9. Abstract quote
AIM OF STUDY: Benign cephalic histiocytosis (BCH) is a rare, benign, self-healing papular eruption that affects mainly children. The aim of this study was to characterise our Singapore children affected by this condition and to review some of the recent literature on non-Langerhans cell histiocytoses.
METHODOLOGY: The case records of all cases of BCH seen between January 1990 and December 1996 in our centre were retrieved and analysed. Further details not available in the case records were obtained via a telephone interview with the patients or their parents.
RESULTS: A total of 8 cases were seen. There was no sex preponderance or ethnic predilection. The average age of onset was 29 months. All patients began with small brownish papules on the face and sometimes the upper trunk, and most of the lesions regressed in less than 84 months.
CONCLUSION: BCH is a benign non-Langerhans cell histiocytosis in which spontaneous resolution is the rule. A small skin punch biopsy is simple, diagnostic and helpful in its management. The management of BCE is expectant and reassurance is sufficient. However the regression and resolution of the lesions may take up to several years.
DISEASE ASSOCIATIONS CHARACTERIZATION DIABETES INSIPIDUS
- Benign cephalic histiocytosis with diabetes insipidus.
Weston WL, Travers SH, Mierau GW, Heasley D, Fitzpatrick J.
Departments of Dermatology and Pediatrics, University of Colorado School of Medicine, and Department of Pathology, Children's Hospital, Denver, Denver, Colorado, USA.
Pediatr Dermatol. 2000 Jul-Aug;17(4):296-8. Abstract quote
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).
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. She had infiltration of the pituitary stalk on brain imaging.
Diabetes insipidus has heretofore been associated with LC histiocytosis and xanthoma disseminatum but not benign cephalic histiocytosis.JUVENILE XANTHOGRANULOMA
Benign cephalic histiocytosis progressing into juvenile xanthogranuloma: a non-Langerhans cell histiocytosis transforming under the influence of a virus?Rodriguez-Jurado R, Duran-McKinster C, Ruiz-Maldonado R.
Department of Pathology, National Institute of Pediatrics of Mexico, Mexico City.
Am J Dermatopathol 2000 Feb;22(1):70-4 Abstract quote Benign cephalic histiocytosis (BCH) is best understood as a form of non-Langerhans cell histiocytosis, specifically as an early mononuclear variant of juvenile xanthogranuloma (JXG). However, the progression of BCH into JXG in the same patient has only been reported once before.
We describe the case of a 2-year-old girl with asymptomatic, large, ill-defined infiltrated flat plaques over both cheeks, in addition to isolated papules. A punch biopsy of a plaque revealed dermal infiltration by vacuolated and scalloped histiocytes positive for CD68 KP-1, and that lacked expression of CD1a and S-100 protein, favoring macrophages over Langerhans cells.
Electron microscopy study showed comma-shaped intracytoplasmic bodies in the histiocytic cells leading to the diagnosis of BCH. One year later, after an episode of varicella-zoster infection, the flat plaques over the cheeks became large reddish-yellow nodules, and in a second biopsy appeared to progress to JXG. Virus-related mechanisms of progression are discussed.
PATHOGENESIS CHARACTERIZATION GENERAL
Benign cephalic histiocytosis: a distinct entity or a part of a wide spectrum of histiocytic proliferative disorders of children? A histopathological study.Gianotti R, Alessi E, Caputo R.
1st Clinic of Dermatology, IRCCS Ospedale Maggiore, University of Milan, Italy.
Am J Dermatopathol 1993 Aug;15(4):315-9 Abstract quote When benign cephalic histiocytosis (BCH) was described for the first time in 1971, it was considered a nosologic entity due to its peculiar clinical presentation, but in the last few years its individuality has been questioned because of histopathological features overlapping with other non-X histiocytoses such as generalized eruptive histiocytosis (GEH).
To solve this question, we examined with light microscopy using blinded technique 18 biopsy specimens obtained from 14 patients affected by BCH in comparison with 4 biopsies of GEH, 11 biopsies of papular xanthoma, and 25 biopsies of juvenile xanthogranuloma (JXG) of childhood. In the examined specimens, we identified three distinct patterns of proliferation of histiocytes we named "papillary dermal," "lichenoid," and "diffuse."
With the exception of some peculiar histopathological features of BCH, observed only in the "papillary dermal pattern," a close histological relationship was observed among BCH, GEH, and JXG early nonxanthomatous stage.
Our data suggest that BCH might be a localized form of GEH or an aborted phase of JXG.
GROSS APPEARANCE/
CLINICAL VARIANTSCHARACTERIZATION GENERAL
Benign cephalic histiocytosis: A case report and review.Jih DM, Salcedo SL, Jaworsky C.
Department of Dermatology, Metrohealth Medical Center, Case Western Reserve School of Medicine.
J Am Acad Dermatol 2002 Dec;47(6):908-13 Abstract quote Benign cephalic histiocytosis is a rare non-Langerhans histiocytosis characterized by a self-healing eruption of papules and macules on the head and neck that occurs during infancy or childhood. Histologic and ultrastructural evaluations show a dermal proliferation of histiocytes with intracytoplasmic comma-shaped bodies, coated vesicles, and desmosome-like structures with an absence of Birbeck granules.
We report a case of benign cephalic histiocytosis in a 9-month-old boy who presented with tan papules on his face that spread to his lower extremity and subsequently began to regress at 30 months of age.
We review the features of this rare entity through a literature review and discuss the differential diagnosis.
Benign cephalic histiocytosis.Barsky BL, Lao I, Barsky S, Rhee HL.
Arch Dermatol 1984 May;120(5):650-5 Abstract quote A 2-year-old boy was seen because of an extensive asymptomatic papular eruption on the face, neck, and shoulders of 18-months' duration.
A skin biopsy specimen revealed cellular infiltrates composed predominantly of histiocytes. Electron microscopy of biopsy material disclosed "comma-shaped" inclusion bodies in the cell cytoplasm, consistent with the findings described in benign cephalic histiocytosis.
To the best of our knowledge, this is the first report of this clinical entity in the American literature.
HISTOLOGICAL TYPES CHARACTERIZATION GENERAL
- Benign cephalic histiocytosis: a distinct entity or a part of a wide spectrum of histiocytic proliferative disorders of children? A histopathological study.
Gianotti R, Alessi E, Caputo R.
1st Clinic of Dermatology, IRCCS Ospedale Maggiore, University of Milan, Italy.
Am J Dermatopathol. 1993 Aug;15(4):315-9. Abstract quote
When benign cephalic histiocytosis (BCH) was described for the first time in 1971, it was considered a nosologic entity due to its peculiar clinical presentation, but in the last few years its individuality has been questioned because of histopathological features overlapping with other non-X histiocytoses such as generalized eruptive histiocytosis (GEH).
To solve this question, we examined with light microscopy using blinded technique 18 biopsy specimens obtained from 14 patients affected by BCH in comparison with 4 biopsies of GEH, 11 biopsies of papular xanthoma, and 25 biopsies of juvenile xanthogranuloma (JXG) of childhood. In the examined specimens, we identified three distinct patterns of proliferation of histiocytes we named "papillary dermal," "lichenoid," and "diffuse."
With the exception of some peculiar histopathological features of BCH, observed only in the "papillary dermal pattern," a close histological relationship was observed among BCH, GEH, and JXG early nonxanthomatous stage. Our data suggest that BCH might be a localized form of GEH or an aborted phase of JXG.
Benign cephalic histiocytosis: report of four cases.de Luna ML, Glikin I, Golberg J, Stringa S, Schroh R, Casas J.
Section of Pediatric Dermatology, Hospital Ramos Mejia, Ceni, Argentina.
Pediatr Dermatol 1989 Sep;6(3):198-201 Abstract quote We cared for four patients with benign cephalic histiocytosis, a self-healing non-X, nonlipid cutaneous histiocytosis of children. The age of onset of the disease was 5 to 9 months, with papules and erythematous macules involving the head (mainly the cheeks), and posterior spread to the trunk and limbs in three patients.
Microscopic examination of skin biopsies revealed a histiocytic infiltrate in the superficial dermis that was S100 protein-negative by immunoperoxidase (PAP method). One patient showed comma-shaped bodies and desmosomelike junctions on electron microscopy. No Birbeck's granules were present.
Benign cephalic histiocytosis is a self-limiting condition that requires no treatment.
Benign cephalic histiocytosis.Gianotti F, Caputo R, Ermacora E, Gianni E.
Arch Dermatol 1986 Sep;122(9):1038-43 Abstract quote Benign cephalic histiocytosis is a self-healing non-X, nonlipid cutaneous histiocytosis of children, characterized by a papular eruption on the head.
Mucous membranes and viscera are always spared. In the 13 cases reported herein, the children were otherwise in good general health. The disease appeared during the first three years of life, and spontaneous regression was complete by the age of nine years in the four cases healed to date. The histiocytic infiltrate was localized in the upper and middle dermis and contained no lipids at any stage of evolution.
All the histiocytes contained coated vesicles, and 5% to 30% also contained comma-shaped bodies in their cytoplasm.
VARIANTS XANTHOMATOUS
Multiple eruptive cephalic histiocytomas in a case of T-cell lymphoma. A xanthomatous stage of benign cephalic histiocytosis in an adult patient?Arnold ML, Anton-Lamprecht I.
Department of Dermatology, Ruprecht-Karls University, Heidelberg, Germany.
Am J Dermatopathol 1993 Dec;15(6):581-6 Abstract quote A 67-year-old woman had multiple, pinhead-sized, yellowish papules localized to her face, scalp, and neck. A previously diagnosed T-cell lymphoma of low malignancy had been treated with cytostatic drugs.
Ultrastructurally the dermal infiltrate consisted predominantly of histiocytic cells in various degrees of differentiation. The cells revealed signs of high metabolic activity and contained well-developed mitochondria, rough endoplasmic reticulum, and lysosomes. Groups of worm-like bodies (comma-shape bodies) and great numbers of coated vesicles in various sizes could be regularly found. Microvillous cell processes formed close interdigitations, and desmosome-like structures occurred between adjacent cell membranes. Lipid storage was present in the form of neutral lipid vacuoles, cholesterol clefts, and myelin-like arrangements of phospholipid membranes. Some multinucleated histiocytic cells, mast cells, lymphocytes, and eosinophilic granulocytes were encountered in the infiltrate.
No dermal Langerhans' cells with their characteristic granules were present. Similarities to and differences from benign cephalic histiocytosis, generalized eruptive histiocytoma, and other xanthomatous disorders are discussed.
SPECIAL STAINS/
IMMUNOPEROXIDASE/
OTHERCHARACTERIZATION SPECIAL STAINS IMMUNOPEROXIDASE S100 negative ELECTRON MICROSCOPY
Benign cephalic histiocytosis. A case report and ultrastructural study.Eisenberg EL, Bronson DM, Barsky S.
J Am Acad Dermatol 1985 Feb;12(2 Pt 1):328-31 Abstract quote A 4-year-old Hispanic boy had an asymptomatic eruption of red-brown macules and papules on the face, neck, trunk, and upper extremities. Examination of the skin lesions by light microscopy revealed a diffuse infiltrate of histiocytes in the upper dermis. Examination by electron microscopy showed wormlike inclusion bodies within the histiocytes.
A diagnosis of benign cephalic histiocytosis was made on the basis of these distinct clinical, histologic, and ultrastructural features.
DIFFERENTIAL DIAGNOSIS KEY DIFFERENTIATING FEATURES HISTIOCYTOSES
Indeterminate cell histiocytosis--a clinicopathological entity with features of both X- and non-X histiocytosis.Sidoroff A, Zelger B, Steiner H, Smith N.
Department of Dermatology, University of Innsbruck, Austria.
Br J Dermatol 1996 Mar;134(3):525-32 Abstract quote An otherwise healthy 50-year-old woman presented with a 6-month history of having developed more than 100 generalized, non-confluent, reddish-brown, partially yellow-coloured papules.
A non-epidemotropic, monomorphous infiltrate of vacuolated mononuclear, and occasionally multinuclear, histiocytes, positive for factor XIIIa and macrophage markers HAM56 and KiM1p, was consistent with the clinical impression of generalized eruptive histiocytomas. However, the additional reactivity for S100 protein, in the absence of features of histiocytosis X, suggested a diagnosis of indeterminate cell histiocytosis (ICH).
Further immunohistochemical studies, performed on snap-frozen material, characterized the lesions as being diffusely positive with LN3 (HLA-DR), Leu4 (CD3) and Leu3 (CD4), the infiltrate in the upper dermis as reactive for OKT6 (CD1) and IOT6c (CD1c), and the infiltrate in the lower dermis as reactive for a variety of macrophage markers. Ultrastructural studies showed various non-specific features of histocytic disorders, but no Birbeck granules. Our findings confirm those of previous reports suggesting that ICH is a distinct histiocytic entity, characterized by immunophenotypic features of both X- and non-X histiocytoses. Generalized eruptive histiocytoma seems to be an early indeterminate stage of various non-X histiocytic syndromes including ICH, multicentric reticulohistiocytosis, xanthogranuloma and xanthoma disseminatum.
The distribution pattern of the various X/non-X histiocytic markers suggests dermal arrest of antigen-presenting cells during their physiological trafficking from the skin to the lymph nodes.
JUVENILE XANTHOGRANULOMA
- Is disseminated juvenile xanthogranulomatosis benign cephalic histiocytosis?
Sidwell RU, Francis N, Slater DN, Mayou SC.
Department of Dermatology, Chelsea and Westminster Hospital, Fulham Road, London SW10 9NH, UK.
Pediatr Dermatol. 2005 Jan-Feb;22(1):40-3. Abstract quote
We describe an infant with a congenital form of non-Langerhans cell histiocytosis with clinical and pathologic features of both disseminated juvenile xanthogranulomatosis and benign cephalic histiocytosis.
The findings in this case support the concept of these non-Langerhans cell histiocytoses forming part of a spectrum of disease rather than being separate pathologic entities.
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