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Reticulate hyperpigmentation in systemic sclerosis: a case report and review of the literature.

Chuamanochan M, Haws AL, Pattanaprichakul P - J Med Case Rep (2015)

Bottom Line: 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.

View Article: PubMed Central - PubMed

Affiliation: Department of Dermatology, Faculty of Medicine Siriraj Hospital, Mahidol University, 2 Prannok Rd, Bangkoknoi, Bangkok, 10700, Thailand. drmch117@gmail.com.

ABSTRACT

Introduction: 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.

Case presentation: 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.

Conclusions: 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.

No MeSH data available.


Related in: MedlinePlus

Histopathological findings. A biopsy from the hyperpigmented induration on the right thigh showed increased basal pigmentation with pan-dermal sclerosis. Mild superficial and deep perivascular and perieccrine lymphocytic infiltrate was observed with some dilated dermal blood vessels and decreased adventitious fat surrounding sweat glands. (Hematoxylin and eosin, ×10 objective)
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Fig2: Histopathological findings. A biopsy from the hyperpigmented induration on the right thigh showed increased basal pigmentation with pan-dermal sclerosis. Mild superficial and deep perivascular and perieccrine lymphocytic infiltrate was observed with some dilated dermal blood vessels and decreased adventitious fat surrounding sweat glands. (Hematoxylin and eosin, ×10 objective)

Mentions: Our patient, a 51-year-old Thai woman, presented with a 1-year history of progressively evolving skin pigmentation over her body, which had become accentuated over her trunk and extremities during the past few months. She also complained of appetite loss resulting in an unintentional weight loss of 8kg (17.6lbs) in 1 year, joint pain in her wrists and ankles, and bilateral hand swelling. Our patient also reported the presence of Raynaud’s phenomenon (RP) during the wintertime that had occurred about 5 years prior to the skin change of the hands. There were no other remarkable systemic symptoms. Our patient had been previously healthy and did not take any medications. There was no familial history of a similar skin condition or history of chemical exposure in this case. A physical examination revealed a generalized, reticulate hyperpigmentation and indurated erythematous plaques over her trunk and extremities. Additionally, there was sclerosis of her proximal fingers, sclerodactyly with pitted scars on some fingertips, and Raynaud’s phenomenon (Fig. 1a-c). Nailfold capillaroscopy was performed and showed few capillary loops dilatation without significant tortuosity. Telangiectasia and calcinosis cutis were absent in this patient. There was no evidence of additional systemic involvement. Laboratory investigations revealed normal white blood cell count, creatinine, electrolytes, fasting blood sugar, thyroid function, and liver function. An elevated erythrocyte sedimentation rate (ESR) of 40mm/hour (reference range 0–20mm/hour), a positive antinuclear antibody (ANA) titer of 1:640 (coarse-speckled pattern), and a positive rheumatoid factor (RF) >130IU/mL (reference range; <12.5IU/mL = negative, 12.5–20.5IU/mL = borderline, >20.5IU/mL = positive) were detected. Anti-centromere antibody (ACA), anti-topoisomerase I antibody (anti-Scl-70 Ab), anti-dsDNA antibody, anti-RNP antibody, anti-cardiolipin antibody (immunoglobulin G (IgG) and IgM), lupus anticoagulant, anti-β2-glycoprotein 1 antibody (anti-β2-GP1 Ab), and anti-cyclic citrullinated peptide antibody (anti-CCP Ab) tests revealed negative results. Urinalysis, chest X-ray, pulmonary function test, and upper gastrointestinal endoscopy results were normal. A skin biopsy specimen from her right calf corresponding to the hyperpigmented induration showed hyperpigmentation of basal keratinocytes and broad sclerotic collagen bundles involving full thickness of the dermis, replacing adventitious fat, and extending into the subcutis with mild septal thickening (Fig. 2). Some ectatic capillary blood vessels were found in the superficial and deep reticular dermis without evidence of vasculitis or vasculopathy. A superficial and deep perivascular infiltrate composed of lymphocytes, plasma cells, and some eosinophils was noted. Based upon the clinical combination of sclerodactyly with sclerosis of the proximal fingers, generalized reticulate hyperpigmentation, and histopathological finding of pan-dermal sclerosis, reticulate hyperpigmented systemic sclerosis was diagnosed.Fig. 1


Reticulate hyperpigmentation in systemic sclerosis: a case report and review of the literature.

Chuamanochan M, Haws AL, Pattanaprichakul P - J Med Case Rep (2015)

Histopathological findings. A biopsy from the hyperpigmented induration on the right thigh showed increased basal pigmentation with pan-dermal sclerosis. Mild superficial and deep perivascular and perieccrine lymphocytic infiltrate was observed with some dilated dermal blood vessels and decreased adventitious fat surrounding sweat glands. (Hematoxylin and eosin, ×10 objective)
© Copyright Policy - OpenAccess
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4584480&req=5

Fig2: Histopathological findings. A biopsy from the hyperpigmented induration on the right thigh showed increased basal pigmentation with pan-dermal sclerosis. Mild superficial and deep perivascular and perieccrine lymphocytic infiltrate was observed with some dilated dermal blood vessels and decreased adventitious fat surrounding sweat glands. (Hematoxylin and eosin, ×10 objective)
Mentions: Our patient, a 51-year-old Thai woman, presented with a 1-year history of progressively evolving skin pigmentation over her body, which had become accentuated over her trunk and extremities during the past few months. She also complained of appetite loss resulting in an unintentional weight loss of 8kg (17.6lbs) in 1 year, joint pain in her wrists and ankles, and bilateral hand swelling. Our patient also reported the presence of Raynaud’s phenomenon (RP) during the wintertime that had occurred about 5 years prior to the skin change of the hands. There were no other remarkable systemic symptoms. Our patient had been previously healthy and did not take any medications. There was no familial history of a similar skin condition or history of chemical exposure in this case. A physical examination revealed a generalized, reticulate hyperpigmentation and indurated erythematous plaques over her trunk and extremities. Additionally, there was sclerosis of her proximal fingers, sclerodactyly with pitted scars on some fingertips, and Raynaud’s phenomenon (Fig. 1a-c). Nailfold capillaroscopy was performed and showed few capillary loops dilatation without significant tortuosity. Telangiectasia and calcinosis cutis were absent in this patient. There was no evidence of additional systemic involvement. Laboratory investigations revealed normal white blood cell count, creatinine, electrolytes, fasting blood sugar, thyroid function, and liver function. An elevated erythrocyte sedimentation rate (ESR) of 40mm/hour (reference range 0–20mm/hour), a positive antinuclear antibody (ANA) titer of 1:640 (coarse-speckled pattern), and a positive rheumatoid factor (RF) >130IU/mL (reference range; <12.5IU/mL = negative, 12.5–20.5IU/mL = borderline, >20.5IU/mL = positive) were detected. Anti-centromere antibody (ACA), anti-topoisomerase I antibody (anti-Scl-70 Ab), anti-dsDNA antibody, anti-RNP antibody, anti-cardiolipin antibody (immunoglobulin G (IgG) and IgM), lupus anticoagulant, anti-β2-glycoprotein 1 antibody (anti-β2-GP1 Ab), and anti-cyclic citrullinated peptide antibody (anti-CCP Ab) tests revealed negative results. Urinalysis, chest X-ray, pulmonary function test, and upper gastrointestinal endoscopy results were normal. A skin biopsy specimen from her right calf corresponding to the hyperpigmented induration showed hyperpigmentation of basal keratinocytes and broad sclerotic collagen bundles involving full thickness of the dermis, replacing adventitious fat, and extending into the subcutis with mild septal thickening (Fig. 2). Some ectatic capillary blood vessels were found in the superficial and deep reticular dermis without evidence of vasculitis or vasculopathy. A superficial and deep perivascular infiltrate composed of lymphocytes, plasma cells, and some eosinophils was noted. Based upon the clinical combination of sclerodactyly with sclerosis of the proximal fingers, generalized reticulate hyperpigmentation, and histopathological finding of pan-dermal sclerosis, reticulate hyperpigmented systemic sclerosis was diagnosed.Fig. 1

Bottom Line: 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.

View Article: PubMed Central - PubMed

Affiliation: Department of Dermatology, Faculty of Medicine Siriraj Hospital, Mahidol University, 2 Prannok Rd, Bangkoknoi, Bangkok, 10700, Thailand. drmch117@gmail.com.

ABSTRACT

Introduction: 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.

Case presentation: 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.

Conclusions: 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.

No MeSH data available.


Related in: MedlinePlus