Systemic Lupus Erythematosus Masquerading as Gangrene- an Infrequent Presentation

Dr. Aditi Singh

MBBS, DDVL

Consultant Dermatologist and Aesthetician

Dr Aditi Singh's Skin Clinic, Raipur

Government District Hospital, Raipur

Abstract

Systemic lupus erythematosus (SLE) is an auto-immune disease affecting multiple organs. Among the cutaneous features, digital gangrene is considered to be very rare, occurring in only 1.3% of SLE patients.[1] It is considered to be due to compromised perfusion of digits secondary to vasculitis, vasospasm and thomboembolism. This is the case report of a 35 year old female who presented with acute blackish discoloration of toes of both feet associated with pain. She had no other features of SLE. But serology came out positive for SLE. The patient responded well to steroids, hydroxychloroquine, anti-platelet and anticoagulant medications and is currently under follow-up.

This case report basically shows us that SLE should be considered as a possible differential diagnosis whenever there is a young patient presenting with digital gangrene without any other medical history.

Keywords: digital gangrene, systemic lupus erythematosus, infrequent

Introduction

Systemic lupus erythematosus is an auto-immune disease of unknown etiology wherein auto-antibodies are formed against various tissues of the body, leading to inflammation. Cutaneous manifestations include malar rash, photosensitivity, livedo reticularis, alopecia, discoid lupus erythematosus and digital gangrene. Out of these, digital gangrene is considered to be very rare, occurring in only 1.3% of SLE patients.[1] Furthermore, peripheral digital gangrene as the first presenting symptom of SLE is seldom reported.

Here we describe a case of a 35 year old female, who presented initially as an acute peripheral digital dry gangrene. She was then evaluated and showed positive ANA. We diagnosed her as a case of SLE and started her on Hydroxychloroquine, anti-platelet and anti-coagulant medications and she is currently under follow up.

Case report

A 35-year old female presented with painful blackish discoloration of toes of both feet since ten days. There was a history of exacerbation of this pain on exposure to cold, with diminution on rewarming. There was no history of smoking, facial rash, photosensitivity, oral ulcers, joint pains, fever prior to onset of symptoms. There was no history of recurrent spontaneous abortions. On examination, there was blackish discoloration of distal half of all toes on right side, and similar discoloration till mid-dorsum of left foot, and locally reduced temperature on both sides. Right anterior and posterior tibial arteries were palpable, dorsalis pedis was feeble. Left anterior tibial artery was palpable, posterior tibial was feeble, and dorsalis pedis artery was not palpable. On investigating, total leucocyte counts (TLC) were normal, with a neutrophilic shift and haemoglobin was 7g/ dL. Erythrocyte sedimentation rate (ESR) was elevated, Anti Nuclear Antibodies (ANA), Double stranded DNA (dsDNA) were positive. Bilateral lower limb arterial Doppler studies were normal. Computerised Tomography (CT) angiography showed left dorsalis pedis artery occlusion and hypoplastic left posterior tibial artery. Hepatic transaminases were mildly elevated. Anti-beta-2 glycoprotein and anti-cardiolipin antibodies were negative. A diagnosis of Systemic Lupus Eythematosus (SLE) was made and patient was started on intravenous Methylprednisolone 500mg in 100 ml normal saline once daily (OD) along with Hydroxychloroquine 200mg twice a day (BD), anti-platelet and anti-coagulant medications.

Discussion

This case has been reported in view of the sporadic presentation of systemic lupus erythematosus as gangrene. Peripheral digital gangrene has been described as a rare manifestation of SLE. It occurs only in 1.3% of SLE patients.[1] Furthermore, peripheral digital gangrene as the first presenting symptom of SLE is rarely reported. Only 0.2% of patients with SLE present initially as digital gangrene.[1] Former reports declared that it is seen mostly at a late stage of the disease. Rosato et al. claimed that acute digital gangrene is never the first presentation of SLE.[2] Here, this patient presented with digital gangrene as the first presentation of SLE. In 2009 Rajasekhar et al described that, out of 344 patients, 20 cases of digital lupus gangrene had been reported among the Indian population.[3]

According to a study conducted by Liu et al on 2684 lupus patients, a history of Raynaud’s phenomenon is a predictive factor for developing digital necrosis.[1] In this case too, the patient gave a prior history of Raynaud’s phenomenon prior to developing digital gangrene.

As per literature, the suggested treatment of digital gangrene includes corticosteroids, immunosuppressants, anticoagulants and hypolipidemic drugs.[4] This patient too responded well with corticosteroids and hydroxychloroquine, besides anticoagulants.

This case report enlightens us to consider SLE as a possible differential diagnosis whenever we encounter a young patient presenting with digital gangrene, without any other medical history and think of auto-immune etiology as a distinct possibility, so that

References

1. Alalawi ZM, Alkenany S, Almahroos F, Albloushi B. Peripheral Gangrene as the Initial Presentation of Systemic Lupus Erythematosus in Emergency Department. Cureus. 2020;12(1).

2. Rosato E, Molinaro I, Pisarri S, Salsano F. Digital ulcers as an initial manifestation of systemic lupus erythematosus. Intern Med. 2011;50(7):767-9.

3. Rajasekhar L, Jayachandran NV, Prabu VN, Narsimulu G. Comment on: Prevalence, serological features, response to treatment and outcome of critical peripheral ischaemia in a cohort of lupus patients. Rheumatology (Oxford). 2009 Apr;48(4):451-2.

4. Liu A, Zhang W, Tian X, Zhang X, Zhang F, Zeng X. Prevalence, risk factors and outcome of digital gangrene in 2684 lupus patients. Lupus. 2009 Oct;18(12):1112-8.

Acknowledgement: Horizon Hospital

Age-related atopic dermatitis phenotypes evaluated in study

Atopic dermatitis, also known as eczema, is a chronic skin condition characterized by itching, redness, and the formation of rashes and blisters. It is often seen as a long-term, recurrent skin disease and is more common in children, but can also occur in adults. Pediatric patients with atopic dermatitis (AD) are more likely to experience symptoms at classical sites such as the knees and have more associated and severe signs of AD, while older adults tend to present with less flexural eczema and the fewest associated signs. Those are key findings from a study conducted which aimed to identify the age-related clinical phenotypes of AD. Previous studies have found differences in the clinical characteristics of AD depending on age of AD onset, ethnic background, and AD severity. However, none have prospectively compared the clinical characteristics and associated signs by age group. Improved understanding of the clinical phenotypes of AD may help guide choice of treatment and improve health outcomes.

The reserchers reviewed self-reported questionnaires that were completed by 380 patients prior to their visit at clinic. Questions included age of AD onset, sociodemographics, Visual Analog Scale (VAS) itch and sleep for Scoring AD, and Numeric Rating Scale (NRS) for skin pain and itch. The researchers used the Eczema Area Severity Index to assess AD severity and a dermatologist conducted full body skin exams, noting the distribution of AD involvement as well as associated signs. Of the 380 patients, 6.1% were younger than aged 18 years, 46.3% were young adults aged 18-39 years, and 47.6% were older adults 40 years of age and older. Compared with pediatric patients, both young and older adults were less likely to experience AD on the ankles, moderate to severe AD lesions on flexures, pityriasis alba, oozing lesions, and moderate to severe excoriations.

In children, severe itch was more common, reported in 47.1%, compared with 43.4% of the young adults and 38.6% of the older adults, and itch was less severe among the young and older adults. Interestingly, despite increased itch in pediatric patients, reserchers found no difference in the severity of skin pain across all age groups, the researchers wrote. Moreover, pediatric patients reported skin pain less often than adult patients. This may be due to age-related differences of pain perception.

In other findings, compared with pediatric patients, young adults were more likely to experience AD around the eyes, while older adults were less likely to experience AD on elbows, nipples, knees, and less likely to have keratosis pilaris, and lichenification. The reserchers used latent class analysis to identify four classes for distribution of AD lesions. In this model, class 1 had low probabilities of AD involvement at all sites examined and class 2 had low probabilities of scalp, face, and foot involvement, and intermediate probability of all other AD sites. Class 3 had low probabilities of hand and foot involvement, high probability of facial erythema, and intermediate probability of all other AD signs, while class 4 had intermediate probability of postauricular and foot involvement, and high probability of all other AD sites examined.

Pigmentary disorder experts welcome research explosion

Atopic Pigmentary disorders typically indicate an increased amount of melanin, leading to darker color of the skin, called hypermelanosis or hyperpigmentation. Decreased or absent pigment makes the skin appear lighter or white, known as hypomelanosis or hypopigmentation. The disorders can be genetic or acquired. Research on pigmentary disorders has entered an overdue era of increased attention, resulting in more treatment options and the promise of improved quality of life for patients.

The arrival of ruxolitinib cream, a topical JAK inhibitor – and oral JAK inhibitors, including ritlecitinib, a JAK3/TEC (tyrosine kinase expressed in hepatocellular carcinoma) inhibitor in clinical trials – is a welcome development for treatment of vitiligo. Also in the pipeline is a kit for melanocyte-keratinocyte transplantation, which involves transplanting epidermal cells from one part of the body to another. This can be a challenging procedure but a kit would make it easier for a wider range of practitioners. (Topical ruxolitinib was approved by the Food and Drug Administration for treating nonsegmental vitiligo in July, 2022.) In the last 10 years, it’s just blown up and people care about vitiligo now, noting that vitiligo is more than a cosmetic issue, like gray hair or wrinkles. Vitiligo is an autoimmune disease and now is being treated as such.

The experts said that the increasing availability of treatment options for hyperpigmentation, aside from hydroquinone, which is associated with an increased risk of adverse effects. We have more and more nonhydroquinone agents which is really nice because it expands our treatment armamentarium and what we can use to cycle people off of hydroquinone. Some of these options include tranexamic acid and products containing azelaic acid or vitamin C. The hyperpigmentation has “profound effects on quality of life” for patients. Patients are often more bothered by the darkening of the skin than the primary process that caused it.

The experts offered suggestions for managing hyperpigmentation patients. Among experts tips for hyperpigmentation, physicians should not be afraid to biopsy the face – but suggested small, 2-millimeter specimens. In addition, “you can get common conditions in uncommon places, if you see something that looks like melasma off the face, it actually could be, so keep that in your differential. Clinicians need to use an algorithm for diagnosis, considering features such as localized or diffuse, scale or no scale, as well as patient history, and other factors. For instance, a hypopigmented area that is localized and has a reddish central papule might lead a clinician to a diagnosis of hypopigmented sarcoidosis.