Persistent pigmented purpuric lesions in a 14-year-old adolescent

The authors present the case of a 14-year-old girl with a macular, bilateral, pigmented purpuric lesions onto the lower part of legs, that had appeared three weeks prior to the time of the admission in the Pediatric Department II of the “Sf. Maria” Emergency Clinical Hospital for Children, Iaşi, Romania, apparently after an upper respiratory tract infection. The patient first presented to the family doctor, being prescribed only symptomatic therapy for cough (acetyl-cysteine-based medication). Two weeks later, the rash appeared, and the treatment was adjusted with antihistaminic drugs (desloratadine). As the cutaneous symptomatology persisted, she was admitted in the pediatric department for further investigations. Regarding the family history, we noted the mother and a sister with arthritis, and regarding the personal history, she was recently diagnosed with autoimmune thyroiditis, but the family didn’t provide any medical documents.

The clinical features included the extended, irregularly shaped patches of purpura and purpuric papules on the posterior area of both legs, which eventually formed a large eruption (Figure 1). The eruption did not blanch with diascope pressure. There was no itching or soreness when touching or pressuring the affected area.
 

The differential diagnosis ruled out at the time an allergic rash, an autoimmune vasculitis, and also an infectious-induced rash.

The nonspecific laboratory studies were within normal range (normal CBC, normal aspect of the peripheral blood smear, normal hepatic enzymes and renal markers, normal glycemic and lipidic status), the only modified biochemical marker being a low value of the ionic calcium. There were also no abnormalities in the coagulation profile for our patient.

The biological findings were also irrelevant for the allergic suspicion of the symptomatology (normal values of the eosinophil count, low IgE values, normal eosinophilic cationic protein and diamine oxidase values). Immunological study of Ig A, Ig G and Ig M, as well as the antinuclear autoantibody screening, anti-DNA autoantibody, and the anti-thyroid autoantibody (anti-TPO, anti-Rab) were within normal range. The patient had also no elevation of the inflammation markers (normal ESR, fibrinogen and CRP values), and the rheumatoid factor was negative. We also searched the infectious trigger (normal serum QuantiFERON, negative serology for Mycoplasma pneumoniae, parvoviral and Epstein-Barr infection, chronic viral B and C hepatitis, and also for Borrelia burgdorferi). The thyroid ultrasonographic study revealed normal aspect of the thyroid and the parathyroid glands, although the hormonal analysis showed a slightly low thyroxine level.

Given the history, the clinical aspect and the nonspecific biological profile, the patient was referred to the Pediatric Surgery Department to perform a skin biopsy. The positive diagnosis was outlined by the histopathological analysis, that revealed focally perivascular inflammatory infiltrates consisting of lymphocytes and conspicuous extravasation of red blood cells, mainly in the papillary and reticular dermis (Figure 2 a, b). There were slight pigmentations in the basal layer of the epidermis, as well as a swollen endothelium within the skin vessels. There were also spongy aspects within the profound layer of the epidermis. Hemosiderin deposits were evident in the papillary dermal layer. Rare aspects of granulomatous inflammation were also observed. The endothelial cells of the capillaries were swollen, but there was no histological evidence of vasculitis (Figure 2 c, d).
 

The immunohistochemical analysis was positive for CD45 in the perivascular areas, also with CD3 positive cells in the inflammatory infiltrate. We noted the presence of the CD68 in macrophagic cells, especially in the profound layers of the skin.

These histological findings were consistent with pigmented purpuric dermatosis, therefore the eruption was diagnosed as pigmented purpuric dermatosis, based on clinically purpuric pigmented lesions and the histological findings.

Pigmented purpuric dermatoses (PPD) include several skin diseases characterized by multiple petechial hemorrhage as a consequence of capillaritis. Since the histopathology is almost superimposable, they are classified on the basis of clinical presentation. There are four types (or clinical variants) of the disease: Schamberg disease or progressive purpuric pigmented dermatosis, purpura annularis telangiectodes of Majocchi, pigmented purpuric lichenoid dermatitis of Gougerot and Blum, and lichen aureus⁽¹⁾. Although elderly adults are more frequently affected, there are also reports of childhood PPD in literature, usually following an infectious trigger⁽²⁾.

The etiology of PPD is still unknown. Immunopathologic studies suggest that the vascular damage and erythrocyte extravasation are secondary to a localized cell-mediated immunologic event. However, pathogenesis pathways may include physical activity, venous hypertension, capillary fragility (especially in lower limbs) and local infection^(2,3). Some medication regimens, such as prolonged acetaminophen intake, nonsteroidal anti-inflammatory drugs, antibiotic therapy, and oral antidiabetic drugs, may appear as a trigger, in particular in Schamberg disease. PPD can also be associated with systemic diseases⁽²⁾, such as thyroid dysfunction, mycosis fungoides, hematological and solid neoplasms, liver disease, diabetes mellitus, rheumatoid arthritis, systemic lupus erythematosus, and hyperlipidemia.

There are no standard guidelines of treatment available for PPD, given the polymorphism of the etiological and the cli­ni­cal features of the disease^(2,3). Rashes are usually per­sis­tent and resistant to therapies, thus conservative or non­phar­­ma­co­lo­gic management should be considered in the case of asymptomatic patients. Therapeutic options include to­pi­cal (moisturizing agents, corticosteroid topic ther­apy)⁽⁴⁾ and oral drugs (ascorbic acid, rutoside, other an­ti­oxi­dants)⁽⁵⁾, phototherapy, and laser therapy⁽⁶⁾. Other ther­apies, such as colchicine, griseofulvin and/or cyclosporin A, were reported by authors in selected cases of pa­tients with no response to priorly used therapeutic measures^(7-9). Another valid option is laser therapy, particularly 595-nm vascular laser proved to be effective in Schamberg di­sease⁽¹⁰⁾.

Our patient successfully used topic treatment with moisturizing cream (twice a day, for 14 days), alternatively with topic corticotherapy (once a day, for 14 days), and also oral therapy with ascorbic acid 500 mg daily, for 30 days. The rash ameliorated in the first six days of treatment and completely disappeared after 12 days. However, the follow-up should include periodic clinical examination, along with biological and imagistic surveillance of the thyroid impairment. Although pigmented purpuric dermatoses are reported to be rare, the recognition of those histological changes is essential in order to ensure an appropriate diagnosis and an individual and efficient therapy. 

Autori pentru corespondenţă: Alice-Nicoleta Azoicăi E-mail: agrudnicki@yahoo.com

CONFLICT OF INTEREST: none declared.

FINANCIAL SUPPORT: none declared.

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