Dermatomyositis associated with malignancy: A report of 3 cases

Abstract

The association between dermatomyositis (DMS) and various types of malignancies has been reported in several studies, with an estimated frequency of 20-25%. DMS may precede, accompany or follow the diagnosis of malignancy. In the present report, we have discussed three cases of dermatomyositis associated with malignancy. In the first case, DMS preceded the diagnosis of gastric adenocarcinoma while in the second and third cases, it followed the diagnosis of ductal carcinoma of the breast and transitional cell carcinoma of the bladder respectively. In all three patients, cutaneous and musculoskeletal features of DMS showed very good response to the treatment, irrespective of the course of malignancy.

Keywords: Dermatomyositis; ductal carcinoma of breast; gastric adenocarcinoma; transitional cell carcinoma of bladder.

Figure 1

Cutaneous examination of case 1. (a and b) Heliotrope rash and periorbital edema, (c) “V” sign, (d) “Shawl” sign and (e and f) Gottron's papules

Figure 2

Case 1: (a) Skin biopsy, 100×; hematoxylin and eosin (H and E) stain: Atrophic epidermis, focal basal cell vacuolar degeneration, dermal edema, mild perivascular lymphocytic infiltrate and abundant mucin in the dermis. (b) Skin biopsy, 400×, H and E stain: Dermal edema and abundant mucin in the dermis. (c) Biopsy from gastric ulcer, 100×; H and E stain: Normal gastric glands and transition zone showing malignancy. (d) Biopsy of gastric ulcer, 400×; H and E stain: Tumor cells mainly arranged in solid sheets with paucity of glandular structure and mitotic activity suggestive of poorly differentiated adenocarcinoma

Figure 3

Improvement in skin lesions of dermatomyositis after 2 weeks of treatment with oral steroids. (a) Heliotrope rash and periorbital edema before treatment. (b) Resolution of lesions after treatment. (c) Erythema over neck, chest and arms before treatment. (d) Resolution of the erythema over neck, chest and arms after treatment. (e) Erythematous and poikilodermatous lesions on the back and shoulders (Shawl sign) before treatment. (f) Resolution of erythematous lesions on the back, leaving behind dyspigmentation after treatment

Figure 4

(a) Cutaneous examination of case 2 showing violaceous erythema over the periorbital area (heliotrope rash), forehead, malar area and chin. (b and c) Erythematous plaques on the pinnae and pre- and postauricular areas. (d) Erythema and edema of both the breast, more on the right side than on the left. (e) Scar of previous surgery on the right breast surrounded by erythema. (f) Skin biopsy from the erythematous lesion on the forehead. 400×, Hematoxylin and eosin showing atrophic epidermis, focal basal cell vacuolar degeneration and dermal edema

Figure 5

(a) Heliotrope rash, periorbital edema and erythematous lesions on the forehead, malar area and chin before treatment. (b) After treatment. (c) Erythema over the right pinna and pre- and postauricular areas before treatment. (d) After treatment. (e) Erythema over the left pinna and pre- and postauricular areas before treatment before treatment. (f) After treatment. (g) Erythema and edema over both the breasts before treatment. (h) After treatment

Figure 6

(a) Cutaneous examination of case 3: Heliotrope rash and periorbital edema. (b) Erythema over the “V” area of the neck (i.e., “v” sign) and upper chest. (c) Skin biopsy, 400×, hematoxylin and eosin (H and E): Showing atrophic epidermis, basal cell vacuolar degeneration, dermal edema and perivascular inflammatory infiltrate. (d) Skin biopsy, 400×, H and E: Showing atrophic epidermis, basal vacuolar degeneration, dermal edema and perivascular inflammatory infiltrate with abundant mucin in the dermis

Figure 7

(a) Cutaneous examination of case 3: Heliotrope rash and periorbital edema before treatment. (b) Resolution of heliotrope rash and periorbital edema after 2 weeks of treatment. (c) Erythema over the “v” area of the neck (i.e., “V” sign) and upper chest. (d) Resolution of erythema over the “v” area of the neck and upper chest after 2 weeks of treatment