Dermatomyositis (DM) is characterized by several cutaneous features classified as pathognomonic (Gottron’s papules and sign), characteristic (heliotrope erythema, periungual telangiectasias and macular violaceous erythema), compatible (poikiloderma and calcinosis cutis), and others.6 The patient here described had Gottron’s sign and heliotrope erythema as cutaneous findings of JDM, but also presented with papules with hematic crusts and white atrophic scars, suggestive of Degos’ disease.1
Degos’ disease can be classified as classic or Degos-like disease.5 Classic Degos’ disease features typical skin lesions and multiple limited infarcts in other organs, such as bowel and central nervous system; approximately 15% of the cases are a benign form often limited to the skin. On the other hand, Degos-like lesions occur in patients with underlying diseases: SLE, antiphospholipid syndrome, SS, RA,3–5 and DM/JDM. 4,7−13
In both types, skin lesions are distinctively characterized by diffuse papules with porcelain-white centers and slightly raised erythematous telangiectatic rims.1,5 On histopathology, the lesions demonstrate hyperkeratosis, epidermal atrophy, and an underlying wedge-shaped area of dermal ischemia and necrosis with a prominent lymphocytic infiltrate; vessels in the papillary dermis show fibrinoid necrosis and thrombosis. Mucin deposition is seen in all stages, and fibrin deposition may be also observed.8,9 Several of these histologic features were seen in our patient’s skin biopsy.
The etiology of Degos’ disease is unknown, however a vaso-occlusive process, obliterating arteriolitis, necrotizing vasculitis, endovasculitis with secondary thrombosis, intravascular coagulation disorder, or a fibrinolysis disorder have all been considered. Several authors suggest Degos’ disease actually represents a clinical and histological endpoint reaction pattern to vascular injury derived from different disease processes, instead of a distinct clinical entity; which could explain its association with autoimmune disorders.3–5
There are only 10 previously reported cases of DM with Degos-like disease, 4,7−13 7 of them in adult patients (3 males and 4 females), with a median age at diagnosis of 37 years (20 to 50 years) and a fatal outcome in 4 (57%), as can be seen in Table 1. In this group of patients, cutaneous lesions typical of Degos were observed in 5 cases.
Table 1
Degos-like disease associated with Dermatomyositis
Author & Year | Gender & Age | Dermatomyositis features | Degos-like features | Treatment | Outcome |
ADULT PATIENTS |
Tsao et al.7 1997 | Female 26 years | Muscle weakness Paresthesias Dysphagia Heliotrope eruption Shawl-like poikiloderma Periungual telangiectasias Positive ANAs | 6 months after DM diagnosis Numerous small atrophic porcelain-white papules with a thin rim of erythema Esophageal ulcer | Prednisone Methylprednisolone Methotrexate Azathioprine IVIG Aspirin | Good |
High et al.4 2004 | Female 39 years | Shaw-like violaceous macular eythema Gottron’s papules Tendon streaking Periungual telangiectasias Positive ANAs | 5 years after DM diagnosis Crops of small erythematous papules that healed with white scars | Azathioprine Aspirin | Good |
Magro et al.8 2009 | Male 37 years | Muscle weakness and pain Heliotrope rash Gottron’s papules Reticular erythema Periungual erythema Positive ANAs | 3 months after DM diagnosis Depressed porcelain atrophic macules in a reticulated background Gastric and duodenal ulcers & intestinal perforation | Prednisone Methotrexate IVIG | Fatal |
Gupta et al.9 2011 | Male 50 years | Raynaud phenomenon Proximal muscle weakness Erythemato-edematous periorbital and neck rash, Positive ANAs, anti-Sm and anti-RNP antibodies | Before signs of DM Erythematous macules, papules, pustules, and small ulcers that healed with a white atrophic scar and a rim of erythema and telangiectasias Diarrhea and melena Diffuse cerebral atrophy Deep vein thrombosis | Dypiridamol Aspirin | Fatal |
Burgin et al.10 2014 | Male 32 years | Heliotrope rash Gottron’s papules Muscle weakness and pain Periungual edema | 6 months after DM Erythematous macules and papules with white sclera-atrophic centers, violaceous borders and peripheral hyperpigmentation Gastric ulcers Ischemic colitis | Prednisone Azathioprine Rituximab IVIG Aspirin Dipyridamole Eculizumab | Fatal |
Vardeh et al.11 2016 | Female 20 years Female 37 years | Polyarthralgias & myalgias Proximal muscle weakness Dysphagia Diffuse subcutaneous edema Positive ANAs Gradual proximal weakness Petechial & maculopapular rash in a “shawl” distribution Negative ANAs | Simultaneous appearance Nausea / vomiting GI tract bleeding GI tract perforation On HP vascular changes resembling systemic Degos disease Bowel perforation & colectomy On HP vascular changes resembling systemic Degos disease | Prednisone IV methylprednisolone IVIG Cyclophosphamide Corticosteroids Methotrexate IVIG | Fatal Good |
PEDIATRIC PATIENTS |
Olmos et al.13 1979* | Male 8 years | Muscle weakness and pain Dysphagia Heliotrope eruption Scattered telangiectasias Livedo reticular | Simultaneous appearance Atrophic lesions | Not defined | Not defined |
Vardeh et al.11 2016 | Female 6 years | Proximal muscle weakness Gottron papules Nail bed capillary changes | Two weeks after JDM diagnosis Bowel rupture & abscess formation On HP vascular changes resembling systemic Degos disease | IV methylprednisolone IVIG Cyclophosphamide | Good |
Day et al.12 2020 | Male 7 years | Proximal muscle weakness Heliotrope rash Gottron’s papules | 9 months after JDM diagnosis Coalescing porcelain macules on upper eyelids Scattered hypopigmented depressed macules on the arms GI tract perforations Superior vena cava thrombus | Methotrexate Hydroxychloroquine Prednisone Folic acid Ranitidine Montly IVIG Rituximab Cyclophosphamide | Good |
Current case | Female 12 years | Muscle weakness Heliotrope rash Gottron’s sign Dysphagia Positive ANAs | Simultaneous appearance Small papules with hematic crusts and white atrophic scars. Ptosis | Methylprednisolone Cyclosporine IVIG Acenocoumarin Prednisone Methotrexate Mycophenolate mofetil | Good |
Notes: *Diagnosis was retrospectively presumed based on the description of the case
Abbreviations: DM = dermatomyositis, JDM= juvenile dermatomyositis, ANAs = antinuclear antibodies; IVIG = intravenous immunoglobulin, IV= intravenous
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Regarding pediatric patients, Vadeh et al.11 reported a 6 year-old-girl whom 2 weeks after being diagnosed with JDM developed bowel rupture, and Day et al.12 described the development of gastrointestinal perforation and cutaneous features of Degos in a 7 year-old-girl with known JMD. Besides, we believe that the child with JDM and livedoid vasculitis described by Olmos et al.13 could actually have presented JDM with Degos-like disease, since the microcylinders of endoplasmic reticulum in histiocytes described in their patient were also seen in an adult with the classic features of Degos’ disease. In our patient, armpit and upper limb lesions, not typical for JDM, were clinically and histopathologically compatible with Degos-like disease. Ptosis is not a feature of JDM which could be attributed to Degos’ disease, since there are some case reports of Degos’ patients with ocular manifestations involving the eyelids14. This clinical manifestation resolved completely after treatment.
Treatment modalities for Degos and Degos-like disease have included antiplatelet agents, anticoagulants, immunosuppressant drugs, and eculizumab with varied results.1 Our patient responded to installed treatment and has remained free of new crops of white-porcelain papules and muscle involvement.
In conclusion, we present a unique case of JDM associated with Degos-like disease responsive to treatment and a long follow-up.