Table 1 Clinicopathological presentation and management of reticulate hyperpigmented scleroderma

Age (year) , sex

48, female

47, male

43, male

51, female

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

Right thigh

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

ANA

Negative

n/a

n/a

Positive, titer 1:640 (speckled pattern)

Anti-centromere Ab

Negative

n/a

n/a

Negative

Anti-Scl-70 Ab

Negative

n/a

n/a

Negative

Anti-cardiolipin Ab

Negative

n/a

n/a

Negative

Epidermis

Epidermal atrophy and basal pigmentation

Epidermal atrophy

n/a

Basal hyperpigmentation

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

n/a

Broad sclerotic collagen bundles in dermis replacing adventitious fat, superficial and deep perivascular lymphoplasmacytic infiltrate with few eosinophils, mild septal thickening

Treatment

n/a

Topical corticosteroids and antibiotics under a hydrocolloid dressing

Hydrocolloid dressing and topical antibiotics

Topical corticosteroid, colchicine, aspirin, nifedipine, vitamin E, hydroxychloroquin UVA1 phototherapy

Follow-up

n/a

Recovery of the ulcers

n/a

Improvement of thickened skin and Raynaud’ s phenomenon