Reticulate hyperpigmentation in systemic sclerosis: a case report and review of the literature
© Chuamanochan et al. 2015
Received: 26 January 2015
Accepted: 26 August 2015
Published: 28 September 2015
Systemic sclerosis is a systemic connective tissue disease with variable cutaneous presentations. Although pigmentary disturbances have been described in systemic sclerosis, a reticulate hyperpigmentation has only been reported in one case of systemic sclerosis to date.
We describe a previously healthy 51-year-old Thai woman who presented with a reticulate hyperpigmentation affecting her trunk and extremities, together with sclerodactyly and proximal sclerosis, resulting in a new diagnosis of systemic sclerosis.
To date, the pathogenesis of reticulate hyperpigmentation in systemic sclerosis remains unclear. Increased melanin synthesis and altered thermoregulatory mechanism are proposed to be involved in the pathogenesis of this presentation. This case represents an unusual cutaneous feature of reticulate hyperpigmentation in the setting of systemic sclerosis.
KeywordsSystemic sclerosis Scleroderma Reticulate hyperpigmentation
Systemic sclerosis (SSc) is a multisystem rheumatic disease with a variable clinical presentation. Clinical diagnosis is mainly based on the presence of skin thickening and variable involvement of internal organs. Various cutaneous pigmentary alterations have been described in SSc , including a diffuse, generalized hyperpigmentation with accentuation in sun-exposed areas, a vitiligo-like depigmentation with perifollicular hyperpigmentation, and a combined hyper- and hypopigmentation in the areas of sclerosis [2–6]. The pattern of reticulate hyperpigmentation in SSc has been rarely reported in the literature, and has an unclear pathogenesis. We report an unrecognized pigmentary abnormality in a 51-year-old patient with SSc who presented with a reticulate hyperpigmentation affecting the trunk and all extremities. To date, there have been only a few cases of reticulate hyperpigmented scleroderma reported in the English literature.
Reticulate hyperpigmentation is characterized by mottled patterns of cutaneous hyperpigmentation. The etiology is varied from congenital to acquired conditions. An initial approach to identify the causes of reticulate hyperpigmentation depends on the characteristic onset of the disease, the distribution of lesion, and the associated clinical findings. Further investigations including a skin biopsy may be helpful for the definitive diagnosis . There are several patterns of pigmentary alteration previously described in sclerodermic patients, which are: i) diffuse generalized hyperpigmentation, similar to Addison’s disease; ii) focal depigmentation with perifollicular hyperpigmentation, resembling vitiligo; iii) localized hypo- and hyperpigmentation in localized sclerotic skin; iv) streaky hyperpigmentation over blood vessels on a background of depigmentation on the legs and temporal scalp; and v) the most recent condition termed “reticulate hyperpigmented scleroderma” [2–6]. The pathogenesis of these hyperpigmentation abnormalities remains unclear, but some proposed hypotheses include increased keratinocyte-derived endothelin-1 (ET-1), increased melanin synthesis; increased secretion of melanocytic growth factors by fibroblasts and endothelial cells; and a thermoregulatory mechanism which results in a hyperpigmentation over vessels (“streaky hyperpigmentation”) on a background of depigmented patches [4, 7–9]. Histopathological findings to explain hyperpigmentation in SSc have been previously reported as increased epidermal melanin and pigmentary incontinence with an increased number of dermal melanophages in the superficial dermis . However, in this present case, the lesional skin biopsy showed prominent basal hyperpigmentation without evidence of melanin incontinence or increased dermal melanophages.
The presence of some indurated erythematous plaques over the trunk and extremities is quite uncommon regarding sclerotic skin change in SSc, and therefore the differential diagnosis with generalized morphea or other scleroderma-mimic conditions such as eosinophilic fasciitis, sclerodermiform genodermatoses, scleroderma-like syndromes induced by environmental factors, scleroderma diabeticorum, nephrogenic systemic fibrosis, graft-versus-host disease, and scleroderma-like lesions in malignancies also is concerned. Thus, the absent history of underlying systemic diseases including diabetes, chronic kidney disease, malignancy or substance exposures and the recent onset of cutaneous symptoms would render the diagnosis of SSc regardless of other etiology. Moreover, the presence of sclerodactyly, RP and abnormal nailfold capillaroscopy support the diagnosis of SSc in our case.
To date, there have been only three reported cases of reticulate hyperpigmented scleroderma. Of these reports, two cases were the result of melphalan-induced localized reticulate scleroderma, which occurred secondary to isolated limb perfusion for treatment of malignancies in patients who did not have a history of SSc or other connective tissue diseases [2, 3]. These patients presented with localized porcelain-white sclerotic bands in a fishnet pattern, which was due to melphalan-induced endothelial injury. Ee et al. reported the only other known case to date of reticulate hyperpigmented scleroderma arising in the setting of SSc, and was that of a 48-year-old woman . The pathogenesis remains unclear and further study is required to identify the mechanism of reticulate pigmentary change in SSc. The thermovascular influence, as suggested by Jawitz et al., is one of the possible mechanisms ; however, the skin biopsy in our patient did not reveal the presence of vasculitis or vasculopathy. The only vascular-related changes seen in the biopsy was the dilation of dermal vasculature, which was surrounded by sclerotic collagen within the dermis.
Clinicopathological presentation and management of reticulate hyperpigmented scleroderma
Previous case 
Previous case 
Previous case 
Age (year) , sex
Signs and symptoms
Raynaud’s phenomenon, dsyphagia, sclerodactyly, periungual telangiectasias, mask-like facies with perioral radial furrows; new diagnosis of systemic sclerosis
Porcelain-white, sclerotic bands in a reticulate pattern and painful ulcerations; associated with melphalan
White reticular sclerotic bands with painful ulcerations; associated with melphalan
Raynaud’s phenomenon, sclerodactyly
Area of scleroderma involved
Trunk, thighs, upper and lower limbs
Left thigh and upper calf
Proximal sclerosis involving trunk and extremities
Area of reticulate hyperpigmentation
Chest, abdomen and back
Medial aspect of the left thigh and upper calf
Medio-popliteal aspect of the right thigh
Trunk and extremities
Positive, titer 1:640 (speckled pattern)
Epidermal atrophy and basal pigmentation
Dermis and subcutis
Thickened sclerotic collagen, marked pigmentary incontinence with numerous melanophages in the upper dermis, septal thickening
Thickened, intensely eosinophilic and closely packed collagen bundles
Broad sclerotic collagen bundles in dermis replacing adventitious fat, superficial and deep perivascular lymphoplasmacytic infiltrate with few eosinophils, mild septal thickening
Topical corticosteroids and antibiotics under a hydrocolloid dressing
Hydrocolloid dressing and topical antibiotics
Topical corticosteroid, colchicine, aspirin, nifedipine, vitamin E, hydroxychloroquin UVA1 phototherapy
Recovery of the ulcers
Improvement of thickened skin and Raynaud’ s phenomenon
Reticulate hyperpigmented scleroderma is a distinctly rare cutaneous presentation in association with systemic sclerosis. The pathogenesis is uncertain. Unlike the previously reported case of reticulate hyperpigmentation in association with systemic sclerosis, our patient failed to demonstrate the presence of vascular alterations or melanin incontinence in the skin biopsy. Further studies are necessary to evaluate the mechanism of this peculiar pigmentary change in systemic sclerosis.
Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
None of the authors were funded for this particular case presentation.
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