Degos-Like Lesions In Association With Connective Tissue Diseases: A Report Of Three Cases And Literature Review

Figures & data

Figure 1 (A and B) Small porcelain-white papules and macules with peripheral telangiectasia. (C) Multiple atrophic porcelain-white macules with peripheral telangiectasia on reticulated erythematous to brownish background.

Figure 2 (A) Histopathology from case 1 showed hyperkeratotic epidermal atrophy, vacuolar alteration of basal keratinocytes, marked papillary edema with extravasated red blood cells, and telangiectasia (H&E, 100x). (B) Histopathology from case 3 showed wedge-shaped infarct in papillary dermis with sparse lymphoplasmacytic infiltrate and vacuolar alteration of basal cell layer. Homogenized eosinophilic altered collagen bundles within the entire dermis (H&E, 100x).

Figure 3 Direct immunofluorescence shows epidermal nuclear staining of immunoglobulin G (400x).

Table 1 Demographic Data, Clinical, Laboratory, Histopathologic, Immunopathologic Characteristics, Treatment, And Outcome Of Degos-Like Lesions In Patients With Connective Tissue Diseases

Reference	Disease	Age (y)/ Sex	Distribution	Histology Of Degos-Like Lesions	DIF	Lab	Treatment	Course
Case 1	Classic DM	50/F	Back	Epidermal atrophy with vacuolar alteration and dilatation of small vessels in dermal papillae	Epidermal nuclear staining	CK; 2637 ANA; positive 1:1280 fine speckled (anti-nRNP/anti-Sm 1+) MSA; neg APA; neg	IVIG, prednisolone, and methotrexate	Died from cervical cancer
Case 2	Classic DM	19/F	V-shaped region of the neck and upper chest	Epidermal hyperplasia, proliferation of blood vessels and fibroblast	Negative	CK; 34,950 ANA; neg MSA; neg APA; neg	IVMP, prednisolone, and azathioprine	DM well-controlled, skin lesions mostly resolved
Tsao et al^Citation5	Juvenile DM	26/M	Left fifth finger	Epidermal atrophy with mild interface dermatitis, dermal mucin deposition, and fibrinoid alteration of medium-sized vessel in deep dermis without vasculitis	Not done	CK; 2520 ANA; positive 1:640 speckled and cytoplasmic Jo-1; neg APA; neg	IVMP plus IVIG, prednisolone, azathioprine, methotrexate, and ASA	Skin lesions mostly resolved
High et al^Citation4	Amyopathic DM	39/F	Proximal upper and lower extremities	Focal necrosis, epidermal atrophy with vacuolar alteration, dermal mucin deposition, some focal thrombosis of blood vessels with smudging of erythrocytes within the lumen	Not done	CK; normal ANA; positive 1:320 MSA; NA APA; neg	Azathioprine and ASA	DM controlled
Case 3	dSSc/SLE	26/M	Back	Superficial and deep perivascular lymphoplasmacytic infiltration, homogenized thickened collagen bundles in whole dermis	Not done	ANA; positive > 1:1280 nucleolar and homogeneous (anti-Sm 2+, anti-Ro 3+, anti-Scl70 3+) Anti-DNA; pending APA; neg Hypocomplement Leukopenia ESR; 11	Cyclophosphamide, prednisolone, colchicine, ASA, and nifedipine	Lesions gradually improved over 4 weeks
Durie et al^Citation6	dSSc	34/M	Chest, abdomen, shoulders, arms, and face	Epidermal and dermal necrosis in the area of infarction, and large pale cells with foamy cytoplasm filled the lumen of the arteriole	Not done	ANA; positive homogenous Anti-DNA; NA APA; NA ESR; 85	Prednisolone	Died in 3 months from renal crisis
Liu et al^Citation7	dSSc	42/M	Extremities and right labia majora	Wedge-shaped, full-thickness, epidermal ulceration with mild-to-moderate neutrophils diapedesis, and seen thrombi within lumen	Not done	NA	Treprostinil	Lesions not improved
Dubin et al^Citation13	SLE with CNS involvement	29/M	Elbows, knees, hands, and feet	Some prominent endothelial proliferation and thickening of small vessels with fibrous tissue	Deposition of IgM and complement at BMZ	ANA; positive Anti-DNA; NA APA; NA Normocomplement Leukopenia and thrombocytopenia ESR; 44	Prednisolone	No new skin lesion in 5 months
Black et al^Citation14	SLE	39/F	Trunk and extremities	Epidermal atrophy with hyalinization of collagen in upper dermis, and mucin deposition	Thready granular deposition of IgG, IgM, C3 at BMZ	ANA; positive 1:50 Anti-DNA; NA APA; NA Leukopenia ESR; 14	Mepacrine then fibrinolytic therapy (phenformin, ethylestrenol) and prednisolone	Reduced lesions within 12 months
	SLE	37/F	Upper arms	Not done	Thready deposition of IgG, IgM at BMZ	ANA; positive 1:80 Anti-DNA; positive APA; NA ESR; 12	NA	Persistent joint involvement and weakness
Doutre et al^Citation15	SLE with LN involvement	25/F	Right wrist	Mononucleated cells around small vessels with prominent endothelial proliferation in upper and mid-dermis, and partially obliterated lumen of small vessels in deep dermis	Negative	ANA; positive 1:1000 homogeneous Anti-DNA; positive APA; NA Hypocomplement Leukopenia ESR; 71	Systemic corticosteroid	Satisfied clinical for 9-month follow-up
Török et al^Citation16	SLE	40/F	Abdomen and proximal extremities	Atrophic hyperkeratotic epidermis with underneath fibrous tissue and thick wall blood vessels with signs of hyaline degeneration	Granular IgA, IgG, C3 at BMZ IgM at BMZ and blood vessels	ANA; positive(anti-Ro, anti-La) Anti-DNA; NA APA; negativeESR; 17	Steroid, azathioprine, sulphone group, vasodilators, platelet aggregation inhibitors	Lesions not improved, no other system involvement in 16 years
NPY Chan et al^Citation17	SLE	25/F	Forehead, upper back and scalp	Epidermal atrophy with hyperkeratosis, basal cell vacuolation, focal epidermal necrosis with wedge-shaped area of dermal necrosis	Weak staining with fibrin in blood vessels	ANA; positive 1:160 Anti-DNA; positive APA; negative Normocomplement Leukopenia and thrombocytopenia ESR; NA	Prednisolone, ASA, hydroxychloroquine, and azathioprine	Old skin lesions had resolved, some leaving atrophic scars
Riyaz et al^Citation18	SLE	32/M	Back and proximal extremities	Dermal edema, mucin deposition and lymphocytic vasculitis, basal cell degeneration, interface dermatitis, periappendageal and perivascular infiltration	Not done	ANA; positive (anti-U1RNP, anti-Ro, anti-Sm) Anti-DNA; NA APA; NA ESR; NA	Hydroxychloroquine and ASA	NA
Jang et al^Citation8	SLE	29/F	Dorsal fingers and knees	Hyperkeratosis, epidermal atrophy, and dermal sclerosis, perivascular lymphocytic infiltration, fibrinoid necrosis, and seen thrombus in the lumen	Not done	ANA; positive 1:1280 (anti-Sm, anti-Ro/La) Anti-DNA; positive APA; neg ESR; NA	Hydroxychloroquine, prednisolone, cyclosporin, beraprost, and pentoxifylline	Skin lesions gradually improved within 2 months
Stephansson et al^Citation19	3 SLE	21.5^a/F	NA	NA	NA	ANA; positive Anti-DNA; NA APA; positive in 2/2 ESR; NA	NA	NA

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