Schamberg disease

Schamberg disease
Rust-colored spots typical of Schamberg disease on the lower left leg and left foot of a 26-year-old Caucasian male.
Classification and external resources
Specialty	dermatology
ICD-10	L81.7
ICD-9-CM	709.09
DiseasesDB	30753
eMedicine	derm/327
Patient UK	Schamberg disease
MeSH	D010859

Schamberg Disease, (also known as "Progressive pigmentary dermatosis of Schamberg",^[1] "Purpura pigmentosa progressiva" (PPP),^[1] and "Schamberg's purpura"^[1]) is a chronic discoloration of the skin found in people of all ages, usually affecting the legs. It slowly spreads throughout the body, and is most common in males.^[2]^:829 It is named after Jay Frank Schamberg, who described it in 1901.

Definition

Schamberg's disease, or progressive pigmented purpuric dermatitis, is a chronic discoloration of the skin which usually affects the legs and often spreads slowly. This disease is more common in males and may occur at any age from childhood onward.

Cause

Schamberg's disease is caused by leaky blood vessels near the surface of the skin, capillaries, which allow red blood cells to slip through into the skin. The red blood cells in the skin then fall apart and release their iron which causes a rust color, this accounts for the orange tint of the rash.

Symptoms

The lesions are most frequent on the lower limbs, but may occur anywhere on the body, including the hands, arms, torso and even the neck. They may vary in number and erupt in mass numbers. They consist of irregular patches of orange or brown pigmentation due to the iron complex hemosiderin, with characteristic "cayenne pepper" spots appearing within and at the edge of old lesions. There are usually no symptoms, although there may be some slight itching. The eruption may persist for many years. The pattern of the eruption changes, with slow extension and often some clearing of the original lesions.

Treatment

There is no cure for Schamberg's disease; however, the itching can be controlled by a cortisone cream, and Colchicine treatment has been successfully used to prevent recurrence of the symptoms.

References

1 2 3 Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 1-4160-2999-0.
↑ James, William D.; Berger, Timothy G.; et al. (2006). Andrews' Diseases of the Skin: clinical Dermatology. Saunders Elsevier. ISBN 0-7216-2921-0.

Pigmentation disorders/Dyschromia (L80–L81, 709.0)

Hypo-/
leucism

Loss of melanocytes

vitiligo:	Quadrichrome vitiligo Vitiligo ponctué

syndromic	Alezzandrini syndrome Vogt–Koyanagi–Harada syndrome

melanocyte development:	Piebaldism Waardenburg syndrome Tietz syndrome

Loss of melanin/
amelanism

albinism:	Oculocutaneous albinism Ocular albinism

melanosome transfer:	Hermansky–Pudlak syndrome Chédiak–Higashi syndrome Griscelli syndrome Elejalde syndrome Griscelli syndrome type 2 Griscelli syndrome type 3

other:	Cross syndrome ABCD syndrome Albinism–deafness syndrome Idiopathic guttate hypomelanosis Phylloid hypomelanosis Progressive macular hypomelanosis

Leukoderma w/o
hypomelanosis

Ungrouped

Hyper-

Melanin/
Melanosis/
Melanism

Reticulated	Dermatopathia pigmentosa reticularis Pigmentatio reticularis faciei et colli Reticulate acropigmentation of Kitamura Reticular pigmented anomaly of the flexures Naegeli–Franceschetti–Jadassohn syndrome Dyskeratosis congenita X-linked reticulate pigmentary disorder Galli–Galli disease Revesz syndrome

Diffuse/ circumscribed	Lentigo/Lentiginosis: Lentigo simplex Liver spot Centrofacial lentiginosis Generalized lentiginosis Inherited patterned lentiginosis in black persons Ink spot lentigo Lentigo maligna Mucosal lentigines Partial unilateral lentiginosis PUVA lentigines Melasma Erythema dyschromicum perstans Lichen planus pigmentosus Café au lait spot Poikiloderma (Poikiloderma of Civatte Poikiloderma vasculare atrophicans) Riehl melanosis

Linear	Incontinentia pigmenti Scratch dermatitis Shiitake mushroom dermatitis

Other/ungrouped	Acanthosis nigricans Freckle Familial progressive hyperpigmentation Pallister–Killian syndrome Periorbital hyperpigmentation Photoleukomelanodermatitis of Kobori Postinflammatory hyperpigmentation Transient neonatal pustular melanosis

Other
pigments

iron:	Hemochromatosis Iron metallic discoloration Pigmented purpuric dermatosis Schamberg disease Majocchi's disease Gougerot–Blum syndrome Doucas and Kapetanakis pigmented purpura/Eczematid-like purpura of Doucas and Kapetanakis Lichen aureus Angioma serpiginosum Hemosiderin hyperpigmentation

other metals:	Argyria Chrysiasis Arsenic poisoning Lead poisoning Titanium metallic discoloration

other:	Carotenosis Tattoo Tar melanosis

Dyschromatoses

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