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 Table of Contents  
LETTER TO EDITOR
Year : 2014  |  Volume : 5  |  Issue : 2  |  Page : 71-72

Purpura annularis telangiectodes in a child: An uncommon entity


1 Department of Dermatology, Venereology and Leprosy, Muzaffarnagar Medical College and Hospital, Muzaffarnagar, Uttar Pradesh, India
2 Department of Pathology, Muzaffarnagar Medical College and Hospital, Muzaffarnagar, Uttar Pradesh, India
3 Department of Dermatology, Godavari Foundation Medical College, DUPMC, Jalgaon, Maharashtra, India

Date of Web Publication19-Jul-2014

Correspondence Address:
Tarang Goyal
Department of Dermatology, Venereology and Leprosy, Muzaffarnagar Medical College and Hospital, Muzaffarnagar, Uttar Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1658-5127.137168

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How to cite this article:
Goyal T, Varshney A, Zawar V. Purpura annularis telangiectodes in a child: An uncommon entity. J Appl Hematol 2014;5:71-2

How to cite this URL:
Goyal T, Varshney A, Zawar V. Purpura annularis telangiectodes in a child: An uncommon entity. J Appl Hematol [serial online] 2014 [cited 2018 May 23];5:71-2. Available from: http://www.jahjournal.org/text.asp?2014/5/2/71/137168

Sir,

A 10-year-old boy presented with asymptomatic reddish-brown eruption on lower extremities present since last 4 years with slow progression. They were first noticed in the ankle region, then gradually progressed upwards. On examination, there were multiple annular, reddish-brown nonblanching macular lesions ranging from pin point to large 4 × 5 cm in maximum diameter, arranged bilaterally and symmetrically on both the lower extremities [Figure 1]a. They were present predominantly on lower legs with lesser lesions on thighs and trunk. Early lesions were follicular and punctuate, with slight scaling visible in them [Figure 1]b. The lesions were there on flexure as well as extensor aspects of lower extremities [Figure 2]a. There was no history of any drug intake before the onset of lesions, or no varicosities. There was a history of relapse and remissions present since last many months. All routine blood investigations, hepatitis serology, ultrasound abdomen were normal. Although diagnosis is clinically evident, to confirm the diagnosis biopsy was done, and it revealed perivascular lymphocytic infiltrate in papillary dermis with extravasated erythrocytes. Epidermis showed mild spongiosis and parakeratosis [Figure 2]b. He was thus diagnosed to be a case of purpura annularis telangiectodes (Majocchi purpura) and put on Vitamin C 500 mg/day and rutoside 50 mg/day orally. There was a significant regression in the cutaneous lesions after 12 weeks. There were no adverse effects to the drugs administered.
Figure 1: (a) Multiple erythematous to reddish-brown annular slightly scaly macular lesions on both lower extremities, (b) Close up view of the same showing annular lesions and slight scaling

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Figure 2: (a) Presence of lesions on extensor aspect of lower legs, (b) Histopathology (H and E, 4 × 10) showing epidermal spongiosis, parakeratosis, perivascular lymphocytic infiltrate with extravasated red blood cells

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The pigmented purpuric dermatoses are a group of benign chronic disorders sharing a common histological spectrum and subclassified due to varied clinical presentations. [1] The eponymic subgroups, include Shamberg disease, purpura annularis telangiectodes (Majocchi purpura), pigmented purpuric lichenoid dermatosis (Gougerot and Blum), eczematid like the purpura of Doucas and Kapetanakis, itching purpura, lichen aureus, unilateral linear capillaritis and granulomatous pigmented purpura.

Majocchi purpura is a lesser reported variant with the first case described by Majocchi in 1896 [2] in a 21-year-old male patient. It is characterised by annular follicular and punctuate reddish-brown macules with telengiectasia and purpura usually on lower extremities. There are conflicting reports regarding the age predilection for purpura annularis telangiectodes, although most reports support adolescent females. [3] Purpura annularis telangiectodes have also been described in newborn and elderly patients. [4] Honda et al. [4] and Ozturk et al. [5] have reported these cases in pediatric age group previously, very rare reported entity.

As far as pathogenesis of these disorders, there are three different views put together. First is that they are hypothesised to be due to weakness or disturbance in cutaneous blood vessels, fragility of capillaries and extravasation of red blood cells. Second mechanism put forth is humoral immunity as deposits of C3, C1q, IgM and IgA are seen on direct immune florescence studies positive in some cases. Recently, Aiba and Tagami [6] have demonstrated dermal infiltrate to be composed of helper-inducer T-cells and OKT-6 reactive cells and concluded that Langerhans cells cellular immune reaction may play an important role in the pathogenesis of these disorders. Drugs such as aspirin, carbamazepine, acetaminophen have been found to be a common cause as can be said about the role of gravity and increased venous pressure.

The differential diagnoses to be considered are drug eruptions, tinea corporis, urticaria, erythema annulare centrifugum, granuloma annulare, stasis dermatitis, and mycosis fungoides. Although no treatment is very effective, use of systemic and topical glucocorticoids, antihistamines, colchicine, griseofulvin, rutoside (50 mg/day twice daily), ascorbic acid (500 mg/day), cyclosporine A, methotrexate, psoralen ultraviolet A, and narrowband ultraviolet B have all been tried with variable success rates.

We present the case due to paucity in the literature of such cases in paediatric age group. Also, there is a need to recognise this entity so that unnecessary investigations are avoided in these presentations.

 
  References Top

1.Hoesly FJ, Huerter CJ, Shehan JM. Purpura annularis telangiectodes of Majocchi: Case report and review of the literature. Int J Dermatol 2009;48:1129-33.  Back to cited text no. 1
    
2.Majocchi D. Sopra una dermatosis non ancora descritta, "purpura annularis telangiectode". G Ital Mal Venereol 1896;31:263-4.  Back to cited text no. 2
    
3.Kim HJ, Skidmore RA, Woosley JT. Pigmented purpura over the lower extremities. Purpura annularis telangiectodes of Majocchi. Arch Dermatol 1998;134:1477-80.  Back to cited text no. 3
    
4.Honda M, Saijo S, Tagami H. Majocchi′s disease in a newborn baby: A familial case. Br J Dermatol 1997;137:655-6.  Back to cited text no. 4
[PUBMED]    
5.Ozturk P, Ataseven A, Ozturk U, Demiroren K, Dagli F. Majocchi disease in a child. Indian J Dermatol 2006;51:275-7.  Back to cited text no. 5
  Medknow Journal  
6.Aiba S, Tagami H. Immunohistologic studies in Schamberg′s disease. Evidence for cellular immune reaction in lesional skin. Arch Dermatol 1988;124:1058-62.  Back to cited text no. 6
    


    Figures

  [Figure 1], [Figure 2]


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