Dr. Anthony Perri, M.D.

Histiocytoses - Indeterminate Cell Histiocytosis

Dr. Perri — Sat, 12 May 2012 14:22:17 +0000

Indeterminate Cell Histiocytosis is a rare type of Non-X Histiocytosis that I occasionally encounter in both my The Woodlands dermatology and Conroe dermatology offices. Indeterminate cells are the precursors of Langerhans cells but lack the distinctive intracellular Birbeck granules. Indeterminate Cell Histiocytosis is most common in males and occurs equally across all ages. Clinically, Indeterminate Cell Histiocytosis appears as yellow/red/brown papules and nodules on the skin. Rarely, does Indeterminate Cell Histiocytosis involve internal organs and the bones. In severe cases, chemotherapy has been successfully used as a treatment.

Indeterminate Cell Histiocytosis (

http://archderm.ama-assn.org/content/vol147/issue8/images/small/dlv15006f1.gif)

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Histiocytoses - Multicentric Reticulohistiocytosis

Dr. Perri — Sun, 29 Apr 2012 14:50:54 +0000

Multicentric Reticulohistiocytosis is a rare Non-X Histiocytosis that I occasionally encounter in both my The Woodlands dermatology and Conroe dermatology offices. Multicentric Reticulocytosis usually appears after age 50 and is most common in women. Patients present with reddish brown papules typically on the fingers around the nailfold and the clinical appearance has been termed "coral beads." The skin can also be involved on the extremities (especially over joints), face, ears, neck and trunk. Multicentric Reticulohistiocytosis is a systemic disease and osteoarthritis is a hallmark finding. This arthritis can be very destructive and disabling. When the arthritis affects the fingers the term "opera glass shortening" has been used to describe the telescopic shortening of the phalange bones. Other organ systems involved are: heart, lungs, liver, lymph nodes, liver, muscles and gastrointestinal tract. Approximately, 1/4 of all patients with Multicentric Reticulohistiocytosis also have an internal malignancy. Thus, the use of immunosuppresant therapy to treat this inflammatory disease must be weighed against lowering the body's defenses against fighting malignancy.

Multicentric Reticulohistiocytosis (http://archderm.ama-assn.org/content/vol140/issue8/images/medium/dce30011f1.jpg)

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Histiocytoses - Hereditary Progressive Mucinous Histiocystosis in Women

Dr. Perri — Sat, 07 Apr 2012 14:51:08 +0000

Hereditary Progressive Mucinous Histiocytosis in Women is a rare type of Non-X Histiocytosis that I occasionally encounter in both my The Woodlands dermatology and Conroe dermatology offices. In patients with this disease it is genetically derived and is transmitted either in an autosomal dominant or X-linked fashion. It is exclusively found in women. Clinically, the skin lesions appear as brown to red nodules and papules with a predilection for the face and extremities. Typically, the lesions begin in the teenage years and continue to accumulate throughout life and do not usually resolve spontaneously. Fortunately, this skin disease is strictly limited to the skin and does not have any systemic involvement.

Hereditary Progressive Mucinous Histiocytosis in Women (http://www.medicaljournals.se/acta/content/files/web/3118-web-images/3118fig2_opt.jpeg)

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Histiocytoses - Papular Xanthoma

Dr. Perri — Sat, 31 Mar 2012 17:52:29 +0000

Papular Xanthoma is a type of Non-X Histiocytosis that I occasionally encounter in both my The Woodlands and Conroe dermatology offices. Papular Xanthoma typically appears as multiple small yellow papules. The lesions in Papular Xanthoma can be found anywhere on the body and have a predilection for the extensor surfaces. In some instances, the lesions may resolve and leave scars. Papular Xanthoma does not have any systemic involvement and the lipid levels are usually normal.

Papular Xanthoma (http://archderm.ama-assn.org/content/vol143/issue5/images/medium/dlv60060f1.jpg)

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Histiocytoses - Progressive Nodular Histiocytosis

Dr. Perri — Sat, 24 Mar 2012 21:15:14 +0000

Progressive Nodular Histiocytosis is a type of Non-X Histiocytosis that I occasionally encounter in both my The Woodlands dermatology and Conroe dermatology offices. Clinically, a patient develops a nodule that has a superficial portion near the surface of the skin and a deeper portion that can extend into the fat layer of the skin. Multiple lesions can develop and are usually symmetrical. In most patients, Progressive Nodular Histiocytosis is limited to the skin with no internal involvement.

Progressive Nodular HIstiocytosis (http://binary-services.sciencedirect.com/content/image/1-s2.0-S1027811711000607-gr1.sml)

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Histiocytoses - Xanthoma Disseminatum

Dr. Perri — Sat, 17 Mar 2012 15:46:31 +0000

Xanthoma Disseminatum is a type of Non-X Histiocytosis that I occasionally encounter in both my The Woodlands dermatology and Conroe dermatology offices. Clinically, Xanthoma Disseminatum appears as yellow/red/brown papules and nodules that can occur anywhere on the skin but are most prevalent in the folds and creases as well as the eyelids. Over time, the papules and nodules become larger and can coalesce into larger plaques that encompass a significant body surface area. Most commonly this skin disease occurs in children and young adults. Approximately 50% of patients with Xanthoma Disseminatum have mucous membrane involvement. The larynx and oropharynx can be involved leading to airway obstruction and difficulty breathing and talking. The eyes can be involved as well leading to blindness. In some instances the pituitary gland may be involved and up to 40% of patients with Xanthoma Disseminatum have diabetes insipidus. Lipid levels are elevated in up to 20% of patients with Xanthoma Disseminatum. Although this condition may spontaneously resolve, patients need close internal medicine surveillance and medicines such as cyclophosphamide have been successful in some patients.

Xanthoma Disseminatum (http://archderm.ama-assn.org/content/vol0/issue2010/images/medium/dob05039f1.jpg)

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Histiocytoses - Generalized Eruptive Histiocytosis

Dr. Perri — Sat, 10 Mar 2012 21:46:11 +0000

Generalized Eruptive Histiocytosis is a rare Non-X histiocytosis skin disease that I occasionally encounter in both my The Woodlands dermatology and Conroe dermatology offices. Clinically, a patient develops multiple yellowish nodules on the arms, chest and back with no internal involvement. Generalized Eruptive Histiocytosis rarely occurs in children and is most often seen in adults. Occasionally, this disease may be in response to an underlying leukemia.

Generalized Eruptive Histiocytosis (http://www.medicaljournals.se/acta/content/files/web/3158-web-images/3158fig1_opt.jpeg)

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Histiocytoses - Benign Cepahlic Histiocytosis

Dr. Perri — Sat, 03 Mar 2012 08:59:42 +0000

Benign Cepahlic Histiocytosis is a type of Non-X Histiocytosis that I rarely encounter in my The Woodlands dermatology and Conroe dermatology offices as it occurs in children less than 5 years of age. Red yellow papules occur on the head first and later appear on the neck and trunk. The lesions in Benign Cephalic Histiocytosis are never solitary as they almost always occur in groups. Typically, the skin lesions are asymptomatic and resolve spontaneously over a few years leaving only residual hyperpigmentation.

Benign Cephalic Histiocytosis (http://accessmedicine.net/loadBinary.aspx?name=wolf7&filename=wolf7_c149f006at.jpg)

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Histiocytoses - Juvenile Xanthogranuloma

Dr. Perri — Sat, 25 Feb 2012 16:15:48 +0000

Juvenile Xanthogranuloma (JXG) is the most common Non-X Histiocytosis and I occasionally encounter this skin disease in both my The Woodlands dermatology and Conroe dermatology offices. Clinically, JXG appears as a small red/pink nodule with a yellowish tinge that usually occurs on the head and neck. 90% of all JXGs are solitary. In most cases, JXG lesions do not require treatment as they resolve spontaneously. When JXG lesions are multiple, there is a higher incidence of internal involvment of JXG lesions in the liver, bone and lungs. The most common extra cutaneous location for JXG is actually the iris of the eye. When JXG lesions are on the iris, patients have a higher risk of glaucoma and uveitis, thus an opthalmologic evaluation is prudent in patients with multiple JXG lesions. In Neurofibromatosis 1, JXGs are also more prevalent so workup for this genetic disease may be initiated with the discovery of JXG.

Juvenile Xanthogranuloma (http://www.dermis.net/bilder/CD054/550px/img0018.jpg)

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Histiocytoses - Juvenile Xanthogranuloma

Dr. Perri — Sat, 25 Feb 2012 16:15:48 +0000

Juvenile Xanthogranuloma (JXG) is the most common Non-X Histiocytosis and I occasionally encounter this skin disease in both my The Woodlands dermatology and Conroe dermatology offices. Clinically, JXG appears as a small red/pink nodule with a yellowish tinge that usually occurs on the head and neck. 90% of all JXGs are solitary. In most cases, JXG lesions do not require treatment as they resolve spontaneously. When JXG lesions are multiple, there is a higher incidence of internal involvement of JXG lesions in the liver, bone and lungs. The most common extra cutaneous location for JXG is actually the iris of the eye. When JXG lesions are on the iris, patients have a higher risk of glaucoma and uveitis, thus an ophthalmologic evaluation is prudent in patients with multiple JXG lesions. In Neurofibromatosis 1, JXGs are also more prevalent so workup for this genetic disease may be initiated with the discovery of JXG.

Juvenile Xanthogranuloma (http://www.dermis.net/bilder/CD054/550px/img0018.jpg)

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