Two challenging cases of anti-MDA-5 dermatomyositis with rapidly progressive interstitial lung disease.

Anti-MDA-5 dermatomyositis (DM) is a subtype of idiopathic inflammatory myopathy, commonly presenting as clinically amyopathic dermatomyositis. It is associated with rapidly progressive interstitial lung disease and a poor prognosis. Here, we present two cases of anti-MDA-5 DM and discuss the challenges associated with timely diagnosis, and the importance of early and aggressive treatment.

Radiation-induced subacute cutaneous lupus erythematosus.

We present an unusual case of radiation-induced subacute cutaneous lupus erythematosus (SCLE) occurring in a patient with non-Hodgkin lymphoma. Dermatologists should be aware of the ability of low-dose radiation treatment to induce a first presentation or flare of SCLE, with the possibility of extension of disease outside of the radiation field.

Images of the month 3: The 'bull's head' sign of SAPHO syndrome.

Synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO) syndrome is a rare, chronic, inflammatory disorder with cutaneous and osteoarticular manifestations.1 The aetiology of SAPHO syndrome is unknown and therefore treatment is tailored towards the individual. Non-steroidal anti-inflammatory drugs, bisphosphonates, corticosteriods, antibiotics, disease modifying anti-rheumatic drugs and biologics have all been used with variable success.

Lichen Planus Pemphigoides Mimicking Toxic Epidermal Necrolysis.

Lichen planus pemphigoides (LPP) is an immunobullous disorder characterized by a combination of lesions resembling bullous pemphigoid and lichen planus (LP). Immunofluorescence studies typically show linear deposition of IgG and C3 along the basement membrane zone and circulating antibodies to the hemidesmosome protein BP180 also known as type XVII collagen. It is now recognized that drug-induced linear IgA disease may present with lesions mimicking toxic epidermal necrolysis (TEN). However, to date, there have been no reported cases of LPP presenting with TEN-like lesions. This report describes a 70-year-old woman with LPP who presented with extensive erosions mimicking TEN. This case also highlights the need to consider important etiologies such as drug-induced and paraneoplastic LPP.

Lesson of the month 1: A rash decision.

Cellulitis is a common condition and several mimics exist which should be considered in patients who fail to respond to antibiotics. We describe the case of a patient with anaplastic large cell lymphoma masquerading as a lower leg cellulitis. The patient had failed to respond to intravenous antibiotics and a skin biopsy confirmed her diagnosis. She received radical radiotherapy to the lower leg but later developed shortness of breath and was identified to have pulmonary infiltration of the lymphoma. She died shortly afterwards from lobar pneumonia. This case highlights the importance of regularly reassessing patients with suspected cellulitis and considering alternative diagnoses in cases that fail to respond to treatment.

Ten cases of drug reaction with eosinophilia and systemic symptoms (DRESS) treated with pulsed intravenous methylprednisolone.

Drug reaction with eosinophilia and systemic symptoms (DRESS) is a rare, life-threatening, drug-induced illness characterised by a widespread polymorphic eruption, fever and multivisceral involvement. There is little published on the management of DRESS. Prompt recognition and withdrawal of the causative drug is essential, along with supportive treatment. However, the condition commonly progresses despite these measures. Oral corticosteroids are usually given but the response can be suboptimal and result in a prolonged exposure to systemic glucocorticoid. We conducted a prospective single-centre study to determine the efficacy of pulsed intravenous methylprednisolone followed by a short reducing course of oral prednisolone in ten patients with confirmed DRESS. Rash and fever responded rapidly to methylprednisolone in all patients. Compared to pre-treatment assessments, there was a significant reduction in eosinophil count at day 14 and AST level at day 90 post-treatment. One patient developed acute hepatic failure, necessitating a liver transplant, and died 4 months later. In the immediate post-treatment phase, 1 patient developed type 1 diabetes and 1 patient developed a corticosteroid-induced psychosis. Long-term follow-up on 8/10 revealed all patients to be well, although one patient had persistent pruritus. An aggressive corticosteroid regimen in the management of DRESS is associated with good clinical outcome and acceptable tolerance.