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E-IJD® - CORRESPONDENCE
Year : 2021  |  Volume : 66  |  Issue : 2  |  Page : 224
Unusual pigmented purpuric dermatosis with linear distribution and granulomatous inflammation


1 Department of Dermatology, Complexo Hospitalario Universitario de Santiago, Santiago de Compostela, Spain
2 Department of Pathology, Complexo Hospitalario Universitario de Santiago, Santiago de Compostela, Spain
3 Department of Pediatrics, Complexo Hospitalario Universitario de Santiago, Santiago de Compostela, Spain

Date of Web Publication16-Apr-2021

Correspondence Address:
Gabriela Pita da Veiga
Department of Dermatology, Complexo Hospitalario Universitario de Santiago, Santiago de Compostela
Spain
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijd.IJD_264_18

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How to cite this article:
da Veiga GP, Rosón E, Sainz-Gaspar L, Suárez-Peñaranda JM, Pérez-Feal A, Vázquez-Veiga H. Unusual pigmented purpuric dermatosis with linear distribution and granulomatous inflammation. Indian J Dermatol 2021;66:224

How to cite this URL:
da Veiga GP, Rosón E, Sainz-Gaspar L, Suárez-Peñaranda JM, Pérez-Feal A, Vázquez-Veiga H. Unusual pigmented purpuric dermatosis with linear distribution and granulomatous inflammation. Indian J Dermatol [serial online] 2021 [cited 2021 May 11];66:224. Available from: https://www.e-ijd.org/text.asp?2021/66/2/224/313781




Sir,

Pigmented purpuric dermatoses (PPD) include different subtypes that share some clinical and histopathological features. The presence of granulomas and a linear distribution characterize two of the most unusual variants: granulomatous PPD and unilateral linear capillaritis (ULC).

A 7-year-old girl presented with a 6-month history of asymptomatic lesions on her left leg. Her medical history was unremarkable. Her mother related the appearance of the lesion with her sliding down a fireman's pole in the playground. Physical examination showed red-brown macules extending in a linear distribution on her left leg, which did not follow dermatome or Blaschko lines [Figure 1]. Routine investigation including ANA and TSH were normal. Histopathology showed superficial and deep perivascular lymphoid infiltrate with mild red cell extravasation in the papillary dermis [Figure 2]. Occasional lymphocytes extended to the epidermis, but a lichenoid infiltrate was absent. Around the mid and deep dermal vessels, non-necrotizing granulomas were noted. Perls Prussian blue stain demonstrated dermal siderophages around the blood vessels. She was treated with mometasone furoate 0.1% cream. At the 15-month follow-up hyperpigmentation was still evident [Figure 3].
Figure 1: Red-brown macules extending in a linear distribution on our patient's left leg (a). Close-up of the lesions (b)

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Figure 2: Low-power view showing superficial and deep perivascular inflammatory infiltrate. (H and E, ×20) (a). In the superficial dermis, interstitial extension was noted with mild erythrocyte extravasation without vasculitis (H and E, ×100) (b). Non-necrotizing granulomas were present in the deepest part of the dermis, around blood vessels, (H and E, ×100) (c). Finally, hemosiderin deposition was present, as demonstrated with Perls Prussian blue, (Prussian blue, ×200) (d)

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Figure 3: At the 15-month follow-up hyperpigmentation was still evident

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PPD are characterized by punctate purpura, reddish-brown pigmentation and/or telangiectasia, mostly on the lower extremities. Different subtypes share clinical and histopathological similarities, but each variant has distinctive features.

Granulomatous PPD shares clinical features with other subtypes of PDD but it is distinguished histopathologically by a lymphohistiocytic infiltrate with granuloma formation in the dermis. Only 27 cases have been reported in English literature, and only one in a child.[1] Although the etiology is unknown, a possible association with dyslipidemia and autoimmune dysregulation should be considered.[2] However, our patient had normal cholesterol, ANA, and TSH levels.

Due to the granulomatous histology, sarcoidosis, and other granulomatous diseases must be considered in the histological differential diagnosis.

Our patient had a linear distribution of lesions. Therefore, a differential diagnosis including ULC had to be considered. ULC, also known as quadrantic capillaropathy or unilateral PPD, is characterized by unilateral and/or linear distribution of pigmented purpuric macules, mostly on the legs. It usually affects adolescents and the lesions tend to resolve within 2 years. To date, 18 cases have been reported, 11 occurring in children.[3] Since our case shares many clinical features with ULC but also has granulomatous inflammation, it could be considered as the first case of granulomatous ULC.

Another differential diagnosis is lichen aureus (LA), although rarely occurring in a unilateral and linear distribution. It usually presents in young adults, but it has also been reported in children.[4],[5] At least 2 previous cases with a segmental distribution following trauma have been reported.[4] Because features of LA lesions include chronic nature, characteristic golden lesions, occasional pruritus, and a band-like dermal inflammatory infiltrate, which were absent in our case, this diagnosis was considered less likely.

We would like to emphasize that the lesions appeared following trauma, in a child and no other associated diseases were found.

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.
Morrissey K, Rosenbach M, DeHoratius D, Elenitsas R, Tetzlaff MT. Granulomatous changes associated with pigmented purpuric dermatosis. Cutis 2014;94:197-202.  Back to cited text no. 1
    
2.
García-Rodiño S, Rodríguez-Granados M-T, Seoane-Pose M-J, Espasandín-Arias M, Barbeito-Castiñeiras G, Suárez-Peñaranda JM, et al. Granulomatous variant of pigmented purpuric dermatosis: Report of two cases and review of the literature. J Dtsch Dermatol Ges 2017;15:565-9.  Back to cited text no. 2
    
3.
Yoon TY, Lee DY, Kim YJ, Kim YJ, Lee JY, Kim MK. Unilateral linear capillaropathy limited to the upper extremity in an infant. J Dermatol 2013;40:71-5.  Back to cited text no. 3
    
4.
Ruiz-Esmenjaud J, Dahl MV. Segmental lichen aureus: Onset associated with trauma and puberty. Arch Dermatol 1988;124:1572-4.  Back to cited text no. 4
    
5.
Mishra D, Maheshwari V. Segmental lichen aureus in a child. Int J Dermatol 1991;30:654-6.  Back to cited text no. 5
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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