|Comments from Faculty and Members
David Elpern MD, Dermatologist, Williamstown, MA, USA on August 26, 2011
Terima kasih for this instructive case. In the United States we would probably biopsy this patient for confirmation. I have not seen a similar patient. We do see JXG but this is much less common. We have some pediatric derms on VGRD and hopefully one or two will comment.
The following looks like a good accessible reference. Dr. Yoon Cohen of VGRD is at Case and perhaps she will run this case by the case report's authors:
J Am Acad Dermatol. 2002 Dec;47(6):908-13. Benign cephalic histiocytosis: a case report and review. Jih DM, Salcedo SL, Jaworsky C.
Source Department of Dermatology, Metrohealth Medical Center, Case Western Reserve School of Medicine, Cleveland, Ohio 44109-1998, USA.
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 b
Stelios Minas MD, Dermatologist, Limassol, Cyprus on August 27, 2011
I think its a typical case of mastocytosis, infantile.
Carlos Garcia MD, Associate Professor, University of Oklahoma, Oklahoma City, Oklahoma, USA on August 27, 2011
1) Do you routinely do skin biopsy to confirm its diagnosis in your setting? Yes
2) Any practical cut off age do you quote to parents? No. It may take several years to resolve.
3) Do you send to opthalmology for assessment? Only if the lesions are disseminated and I am considering multiple JXG.
4) Any other differential diagnosis? Yes. Spitz nevi, JXG, Langerhan's cell histiocytosis, urticaria pigmentosa, lichenoid sarcoidosis
Julianne Mann MD, Fellow, Pediatric Dermatology, Department of Dermatology, Oregon Health and Science University, Portland, Oregon USA on August 28, 2011
I've been taught that these infants all need a biopsy to confirm the diagnosis of benign cephalic histiocytosis, and they need regular clinical follow-up to make sure that they do not evolve into xanthoma disseminatum or multiple JXGs, as this transformation has been reported in the literature. We send children with multiple JXGs to ophthalmology, but not those with benign cephalic histiocytosis. I've been taught that the typical disease course is 4-5 years, sometimes up to 8 years.
Jonathan Karnes MD, Assistant Professor, Family Medicine, Clinical Director, CMA Skin CARE Center, University of Texas Health Sciences, San Antonio, TX, USA on September 3, 2011
I diagnosed a case of benign cephalic histiocytosis earlier this year by clinical and biopsy. A review of the literature at the time and a consultation with pediatric dermatology suggested a non-urgent exam and regular follow up. I counseled the parents to expect the lesions for several years. We tried topical rapamycin solution (preparation for oral use, used topically) on half of the lesions which had a modest effect after 2 months.
Abdullah Mancy MD, Al-Ramadi Teaching Hospital Iraq on September 10, 2011
We know that BCH is one of a spectrum of Non-X histiocytosis which is located at the beginning of the spectrum which depend on morphology of histiocyte., It is self-limiting in aboute 2-8 years but it can evolve to JXG If think of JXG ,will ask an ophthalologist opinion , main differential is JXG .
Yoon Cohen, DO, Intern, Traditional – Dermatology, University Hospitals Richmond Medical Center, Cleveland, OH, USA on September 12, 2011
I've learned to perform a biopsy to make a diagnosis for benign cephalic histiocytosis (BCH) as it offers specific features without presence of Touton cells, foamy cells and multinucleate giant cells (which are usually present in juvenile xanthogranuloma, JXG), and occasionally even immunostains. Most children with BCH have a self-limited course without internal organ involvement. Some of literatures report that resolution with flattening and hyperpigmentation of the papules occur after a mean of 26 months from onset. However, there has been a report of an association with diabetes insipidus, so clinical monitoring is usually recommended.
If suspected of JXG, ophthalmology follow up should be considered, but it is usually not necessary with BCH.
Differential diagnosis would include JXG, generalized eruptive histiocytomam (although it is more common in adults), of course other langergans cell histiocytosis (LCHs have different immunohistochemical profile as well as a different histological findings such as birbeck granules), so differentiation of those diseases is possible although there are recent reports that these disorders may rather be a continuous spectrum of disease categorized as non-Langerhans cell histiocytoses.
Of course, a combination of thorough history,clinical, histopathological, and immunohistochemical evaluations will yield the most correct diagnosis. Although sometimes there is no correct or definite diagnosis. Thank you for letting me participate the discussion.