|LETTER TO EDITOR
|Year : 2014 | Volume
| Issue : 2 | Page : 106-107
Schamberg's disease of upper extremity: An atypical site for a typical disease
Pooja Arora1, Mohd Jaseem Hassan2
1 Department of Dermatology, Dr. Ram Manohar Lohia Hospital, New Delhi, India
2 Department of Pathology, Hamdard Institute of Medical Sciences and Research, Jamia Hamdard, New Delhi, India
|Date of Web Publication||15-Dec-2014|
Department of Dermatology, Dr. Ram Manohar Lohia Hospital, New Delhi
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Arora P, Hassan MJ. Schamberg's disease of upper extremity: An atypical site for a typical disease. Pigment Int 2014;1:106-7
Pigmented purpuric dermatoses (PPD) are characterized by petechiae and pigmented macules that occur symmetrically on the lower limbs. These are mainly divided into five clinical types. Progressive PPD, also known as Schamberg's disease, is characterized by nonblanching pinhead sized reddish or pigmented macules on the lower extremities. The lesions resemble grains of cayenne-pepper and progress to form orange to brown pigmented plaques. The lesions of Schamberg's have been reported on trunk and arms, though involvement of these sites is rare. ,
We report a case of Schamberg's disease that started on the wrist that was followed by appearance of classical lesions on legs months later. Our case challenges the hypothesis that PPD is due to venous hypertension or gravitational dependency.
A 35-year-old male, security guard by profession, presented with mildly pruritic, pigmented lesions on the wrist that had been present for the past 6 months. He had similar lesions over the legs and ankles. However, these were of recent onset (1-month). Patient denied any history of intake of drugs prior to the appearance of these lesions. There was a history of prolonged standing (10-12 h/day). Past medical history (drug intake, infection) was unremarkable.
Examination revealed pinhead sized reddish macules becoming confluent to form reddish brown patches with new macules at the periphery. The lesions were present symmetrically on the inner aspect of wrists, legs and ankles [Figure 1]a and b. The lesions were nonblanchable with a glass slide. There were no varicose veins. A full blood count and peripheral blood smear were normal. Coagulation screen including bleeding time, clotting time and prothrombin time did not reveal any abnormality. Antinuclear antibody, anti-HbsAg antibody and anti-HCV antibodies were normal. Skin biopsy performed on a lesion over the right wrist showed perivascular infiltrate of lymphocytes in the papillary dermis accompanied by extravasation of red blood cells [Figure 2]. Direct immunofluorescence could not be done in our case due to cost issues.
|Figure 1: (a) Inner aspect of wrist shows hyperpigmented nonblanchable macules that have coalesced (b) Pinhead sized hyper pigmented macules on medial aspect of left ankle. Similar lesions were seen on right side|
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|Figure 2: Skin biopsy showing perivascular infi ltration of lymphocytes in the papillary dermis and extravasation of few red blood cells (H and E, ×400)|
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Based on the above findings a diagnosis of Schamberg's disease was made and the patient was started on topical steroid for the control of itching, tablet Vitamin C and tablet calcium dobesilate with advice regarding avoidance of prolonged standing.
Pigmented purpuric dermatoses have been traditionally classified into five main types though this does not affect management or prognosis. All types are characterized by similar histopathological features in the form of perivascular infiltrate of lymphocytes and macrophages around superficial blood vessels with endothelial cell swelling and narrowing of lumina. , This is accompanied by extravasation of erythrocytes and hemosiderin deposition in macrophages. Infiltrate is usually composed of CD4 + lymphocytes with occasional CD1a + dendritic cells and macrophages. Direct immunofluorescence may show deposition of IgM, fibrinogen, C3 in superficial blood vessels. 
The etiology of PPD is still unknown though factors such as gravitational dependency, venous hypertension, capillary fragility, exercise, focal infections, and drugs may be implicated in its disease progression. ,, Perivascular infiltrate of CD4, CD1 + dendritic cells suggests a role of cell-mediated immunity in the pathogenesis of this disease.  Immune complexes are also involved as direct immunofluorescence is positive in few cases. 
The unusual site in our patient challenges the well-known theory of vascular congestion or venous hypertension as the central cause. In our patient, the lesions started over the upper limb, which was followed by appearance of classical lesions on lower limbs. The atypical site prompted us to investigate further to rule out other common causes of purpura, though the clinical picture was classical. There are very few cases reported in the literature where Schamberg's disease has involved the upper extremity. , Our case points out the need for further studies on the pathogenesis of this common, but sparingly reported entity.
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[Figure 1], [Figure 2]