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CASE REPORT
Year : 2019  |  Volume : 8  |  Issue : 1  |  Page : 59-61

Schamberg's disease; a forgotten dermatoses: A case report with review


Department of Pathology, IMS and SUM Hospital, Bhubaneswar, Odisha, India

Date of Submission17-May-2018
Date of Acceptance23-Jul-2018
Date of Web Publication26-Apr-2019

Correspondence Address:
Dr. Pranita Mohanty
Department of Pathology, IMS and SUM Hospital, Bhubaneswar - 751 003, Odisha
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/JDRNTRUHS.JDRNTRUHS_47_18

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  Abstract 


Schamberg's Disease (SD) also known as progressive pigmented purpuric dermatitis is the most common PPD (Pigmented Purpuric Dermatitis). PPD includes a spectrum of vascular diseases with an incidence of 0.18% of all skin and VD lesions. SD has Idiopathic aetiology, affects all races and ages (with mean age of 34 years). Males are affected more, especially the legs showing reddish brown purpuric patch/macules which has a chronic slow progression to other body parts. Long standing medications are thought to be one of the precipitating factor. Extravasation of RBCs from the superficial venous plexus of skin is thought to be the cause of skin discoloration. Histopapathology (HP) of the skin lesion show mild epithelial hyperplasia, occasional foci of spongiosis and lymphohistiocytic infiltrate with extravasation of RBCs. The disease has a cosmetic problem. No malignant transformation has been documented yet and to date there is no effective treatment. It is usually diagnosed by Clinical suspicion followed by HP confirmation.

Keywords: Macules, pigmented purpuric dermatitis, purpuric patch, Schamberg's disease


How to cite this article:
Mohanty P, Hota A, Chakraborty K, Mohapatra D. Schamberg's disease; a forgotten dermatoses: A case report with review. J NTR Univ Health Sci 2019;8:59-61

How to cite this URL:
Mohanty P, Hota A, Chakraborty K, Mohapatra D. Schamberg's disease; a forgotten dermatoses: A case report with review. J NTR Univ Health Sci [serial online] 2019 [cited 2019 Nov 20];8:59-61. Available from: http://www.jdrntruhs.org/text.asp?2019/8/1/59/257166




  Introduction Top


In 1901, Jay Frank Schamberg described a case of “Peculiar progressive pigmentation disease” of the skin on a 15 year old boy and since then this disease is named as Schamberg's Disease (SD).

SD is the commonest form of pigmented purpuric dermatoses (PPD) which includes six different types basing on clinical aspects:-

1--Progressive pigmentary purpura/Schamberg's dsease 2 – Pigmented purpuric lichinoid dermatitis of  Gougerot-Blum capillaritis More Details

3--Purpura annularis telangiectodes of Majocchi 4 – Eczematoid like purpura of Doucas and Kapetanakis

5--Lichen aureus 6 –Itching purpura of Lowenthal.

PPDs have 0.18% of incidence from all skin and VD lesions.[1] SD occurs in all races and ages ranging from 11– 66 years (mean age 34 years) with male preponderance, having M: F ratio 3.8:1.[1]

Persons affected show groups of small reddish brown spots which sometimes coalesce to form brownish macules or patches particularly on the legs which can either spontaneously regress or slowly spread to other parts of body. It has no specific effective treatment.

Here we report such a case of Schamberg's disease due to its extreme rarity with an aim to impress upon the fact that It should not be misdiagnosed as some of the common entities which are mimicker of it e. g. 1-Leukocytoclastic vasculitis, 2-Cutaneous T-cell lymphoma, 3-Purpuric generalized lichen nitidus and 4-Drug reactions.


  Case Reports Top


A 24 year maIe presented to the Dermatology department of our hospital with multiple discrete non palpable purpuric non-itchy macules only on the flexor aspect of the lower one third of left leg, developed over 3 months [Figure 1]a and b]. He was not on any medication/applied any emolient. Patient had no family history of such lesions/Diabetes Melitus/Hypertension/allergy. Punch biopsy was done and sent to the histopathology section of our institute. The routine microscopic examination with Hematoxylin and Eosin (H and E) stain revealed keratinized stratified squamous epithelium with focal basket weaving pattern of overlying horny layer, mild epithelial hyperplasia and occasional foci of spongiosis [Figure 2]a and b]. The underlying papillary dermis showed good number of dilated blood vessels with endothelial proliferation and perivascular lymphocyte and histiocyte infiltrate admixed with extravasation of RBCs.[2] There were no pigment dropout seen in the superficial dermis not even in the perivascular region [Figure 3]a and b]. The histopathology diagnosis, given was SD differentiating it from the mimickers and other forms of PPDs (named earlier).
Figure 1: (a and b) Purpuric spots in the lower third of the leg

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Figure 2: (a and b) Photomicrograph shows basket weaving pattern of horny layer (thin arrows) and mild epithelial hyperplasia with occasional spongiosis (H and E, ×100)

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Figure 3: (a and b) Photomicrograph shows dilated blood vessels with perivascular lympho-histiocytic infiltration admixed with extravasation of red blood cells (H and E, ×400)

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  Discussion Top


SD, the commonest among all types of PPDs, are idiopathic in nature but recent viral infection, hypersensitivity to an unknown causal agent and association of some medicines like Thiamine, Paracetamol, Aspirin, Chlordiazepoxide are postulated to have some influences. It usually runs a chronic persistent course with frequent exacerbations and remissions. Most disappear spontaneously. The characteristic appearance of the lesions which usually starts in the lower limbs particularly on the legs below the knees and sometimes on the arms or wrists are crops of orange-brown or reddish-brown spots (purpuric/punctate) which may coalesce to form irregular orange brownish macules/patches within and edges of the old lesions classically described as “cayenne pepper”. Neither the punctate nor the macules blanch on pressure and non-itchy.

The causes are thought to be due to inflammed small blood vessels (capillaritis) near to the skin surface though true vasculitis is not seen in the skin biopsy. Extravasation of RBCs occurs through these leaky capillaries and results in petechial hemorrhage. The colour of the petechie/purpuric spots fade away slowly depositing hemosiderin in the upper parts of the dermis hence producing the classic “cayenne pepper” appearance in older lesions.[3]

Considering the other forms of PPDs; In our case the lesions were progressive and not itchy. whereas in Eczematoid like purpura of Doucas and Kapetanakis _usually bilateral, intensely itchy, occurs in men and show lichenification, appears around the ankle and spread to leg.

Pigmented purpuric lichinoid dermatitis of Gougerot-Blum capillaritis _ less common form.

Lesions are red/brown papules or plaques, thickened and itchy rather like eczema. Pathology shows lichenoid reaction, affects middle aged men. Responds to PUVA therapy.

-Purpura annularis telangiectodes of Majocchi _ presents with ulcers over setting of brown patches and cayenne spots that are symmetrical, telangiectatic and atrophic in nature, coalesce to form annular patches/plaques which spread outwards. The patches can be linear/stellate/Serpiginous of 2-20 mm size. It has a predilection for young females and children in lower limbs and buttocks with mild/absent itchiness.

Lichen aureus _ show solitary brown yellow patch that's localised and very persistent; often overlies a varicose vein.

Itching purpura of Lowenthal/disseminated pruriginous angio dermatitis-rare form, accompanied by itching.

Hence all the above forms of PPDs were excluded.

Comparing with the mimikers: 1. Leukocytoclastic vasculitis-there will be inflammation of the vessel wall.

2. Cutaneous lymphoma-predominantly monoclonal lymphoid cells showing features of epidermotropism.

3. Purpuric generalized lichen nitidus-Lesions are monomorphic, pruritic flat, shiny red, non blanching papules densely clustered on feet, ankles, lateral sides of abdomen and forearms. Microscopy shows acanthosis, exocytosis and basal cell vacuolization mild elongation of rete ridges of epidermis. Upper dermis show dense lymphocyte and epithelioid histiocyte infiltrate.

The absence of aforesaid features excludes mimickers.

Treatment with colchicine dates back to the year 2000, which is low cost, effective yet prevents relapse but newer therapeutic modes with Advanced fluorescence technology (AFT) pulsed light therapy imparts favourable results, using a 570 mm handpiece with fluence of 12-14J/cm2, and pulse width of 12-15 ms (Harmony XL; Alma Lasers); given in 4-6 weeks intervals until patient's cosmetic satisfaction in 1-3 sittings without any adverse events/recurrence with 6 months of follow up.[4] Use of skin emollients and moisturizers is often employed variable results found with pentoxyfylline, griseofulvin, oral corticosteroids, topical corticosteroids and PUVA. Oral rutoside and vitamin C improved some patients after four weeks.[5]

Our patient was advised topical steroid use for three months and followed up for six months without any progress of lesions or relapse. The presentation of our case hopefully will impart some knowledge in one of the rarest dermatoses.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Sharma L, Gupta S. Clinicoepidemiological study of pigmented purpuric dermatoses. Indian Dermatol Online J 2012;3:17-20.  Back to cited text no. 1
[PUBMED]  [Full text]  
2.
Cavalcante ML, Masuda PY, Brito FF, Pinto AC, Itimura G, Nunes AJ, et al. Schamberg's disease: Case report with therapeutic success by using colchicine. An Bras Dermatol 2017;92:246-8.  Back to cited text no. 2
    
3.
DermNet NZ: Capillaritis: A/Prof Amanda Oakley, Dermatologist, Hamilton, New Zealand, 1999. Available from: https://www.dermnetnz.org/topics/capillaritis/. [Updated January 2016].  Back to cited text no. 3
    
4.
Manolakos DA, Weiss J, Glick B, Weiss KD, Weiss E. Treatment of Schamberg's disease with advanced fluorescence technology. J Drugs Dermatol 2012;11:528-9.  Back to cited text no. 4
    
5.
Sardana K, Sarkar R, Sehgal VN. Pigmented purpuric dermatoses: An overview. Int J Dermatol 2004;43:482-8.  Back to cited text no. 5
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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