Loeffler's Syndrome Following Cutaneous Larva Migrans: An Uncommon Sequel.

Cutaneous larva migrans (CLM) is characterized by the formation of distinctive, tortuous, and serpentine skin lesions occurring as a result of epidermal burrowing by certain helminthic larvae. Although this condition is usually uneventful, rarely it may result in patchy pulmonary infiltration with peripheral eosinophilia, also called Loeffler's syndrome. This association is fairly uncommon and is thus being reported.

What was known?

Cutaneous larva migrans occurring as a result of epidermal infiltration by helminthic larva is a relatively rare condition. Its association with pulmonary infiltration called Loeffler's syndrome is even rarer.

Introduction

Cutaneous larva migrans (CLM) is a distinctive skin eruption, characterized by the formation of exceedingly itchy, slightly raised, and bizarre serpentine lesions; mainly caused due to infestation by different varieties of hookworms.[1] In the majority of cases, this condition heals by itself; however, occasionally this may result in Loeffler's syndrome, especially when there is a severe infestation. This is a rare complication of CLM. We report a case of CLM, which resulted in Loeffler's syndrome. To the best of our knowledge, this is one of the first reports from India, depicting this uncommon association.

Case Report

A 30-year-old nonsmoker, agriculturist, presented to us with exceedingly itchy lesions on both hands for the last 20 days. Initially, he noticed a small papule on his left palm that advanced proximally in a twisting streak within a few days. Subsequently, multiple similar lesions developed over both hands. He gave a history of having cattle and pets in his house and confessed to the habit of handling the fodder and cleaning the animal excreta with bare hands.

He also complained of intense, nonproductive cough for the last 7 days with occasional exertional breathlessness. However, there was no history of wheeze, hemoptysis, fever, or other allergic conditions like rhinitis, conjunctivitis, and eczema. His medical history was otherwise noncontributory. He had received a course of oral antibiotic (levofloxacin) and antihistamines, with no significant improvement.

Dermatological examination revealed multiple skin-colored, slightly raised, bizarre-shaped, serpiginous tracks about 3–7 cm in length, involving the palms and fingers of both hands with mild erythema around some lesions [Figure 1a and b]. A vesicle was noted at the advancing edge. Hair, nail, and mucosa were spared. The patient was referred to the Department of Pulmonary Medicine for evaluation of his respiratory problem; rest of the systemic examination was unremarkable.

Figure 1

Baseline picture showing multiple serpiginous tracks with vesicle at advancing end, one hand (a), both hands (b)

Blood biochemistry was normal except for the presence of leukocytosis (total leukocyte count – 14,500/cumm) with an elevated absolute eosinophil count (5200/cumm). Stool examination for ova, parasite, and cyst was negative. Chest skiagram showed the presence of a relatively well-defined homogenous opacity in the right middle and lower zone [Figure 2]. A repeat chest skiagram was carried out after 12 days showed the presence of similar opacity in the left middle and lower zone with the absence of opacity in the previous location [Figure 3]. The presence of such fleeting opacities on chest skiagram together with eosinophilia is suggestive of Loeffler's syndrome.

Figure 2

Baseline chest X-ray showing opacity involving the right middle and lower lung

Figure 3

Chest X-ray after 12 days, showing opacity involving the left lung with clear right lung; fleeting opacities

A diagnosis of CLM with Loeffler's syndrome was made. He was treated with oral albendazole (400 mg/day) for 5 days along with nebulization with levosalbutamol and budesonide as required. The skin condition improved after the stipulated period [Figure 4a and b], while he has been advised to attend a pulmonologist for assessment of his respiratory system.

Figure 4

(a and b) Posttreatment picture showing improvement of skin lesions after Albendazole therapy

Discussion

CLM, also known as creeping eruption, Plumber's itch or Duckhunter's itch[1] is a clinical term for a distinctive eruption that creeps or migrates to produce twisting, winding, serpiginous skin lesions, as a result of burrowing of the offending larvae.[2] The disease has been typically reported from tropical and subtropical areas and is endemic in the Caribbean, Central, and South America, Southeast Asia, and Africa.[1]

Numerous organisms have been reported to cause CLM viz. Ancylostoma braziliense, Ancylostoma caninum, Uncinaria stenocephala, etc., of which A. braziliense (dog tapeworm) is the most common cause in humans.[1] The adult hookworm resides in the intestine of dogs and cats, and their ova are deposited in the animal faeces. Man gets infected incidentally when the infective filariform larva penetrates the skin after contact with infected soil, producing a papule or nonspecific, itchy dermatitis at the site of the entrance. These larvae migrate through the epidermis, producing 2–4 mm wide, erythematous, elevated, bizarre-shaped serpentine tracks progressing at a rate of about 2 cm/day.[3] The lesions usually occur over feet, buttocks, abdomen, hands, and genitalia[2] but may occur anywhere on the body.[34] In our patient also, itchy, characteristic lesions developed on the hands.

The diagnosis of CLM is typically clinical; history of close contact with animals was suggestive in our case. Skin biopsy is usually unyielding. Optical coherence tomography is a noninvasive method to demonstrate the larva in vivo and confirm the diagnosis;[4] however, this facility is unavailable at our center.

Some differentials which were considered in our case included – larva currens (multiple pink urticarial plaques or threadlike lesions; usually present over perianal area and thighs that advance rapidly), migratory myiasis (cutaneous nodules often with central punctum that migrates but serpiginous tract is absent), scabies (excoriated papules in typical locations with nocturnal worsening of pruritus), cercarial dermatitis (typically nonmigratory, usually presents with diffuse maculopapular rash), and gnathostomiasis (painless migratory swelling or subcutaneous nodules usually associated with a history of eating raw or undercooked fish). All these conditions were ruled out in our patient based on the history and clinical examination.

CLM may be complicated by secondary bacterial infection on severely excoriated lesions, allergic reaction, eczematization, or very rarely Loeffler's syndrome may occur.[4]

Loeffler's syndrome also known as pulmonary infiltration with eosinophilia syndrome (PIE) was first reported in association with CLM by Wright and Gold.[5] Loeffler's syndrome has also been found to be associated with medications, parasitic infestations such as Ascaris, Trichuris, Strongyloidiasis, Taenia saginata, and Entamoeba histolytica and as a complication of chronic asthmatic states.[6]

The exact pathogenesis of pulmonary infiltrates remains unclear. Muhleisen detected A. braziliense larva in the sputum of a patient with CLM. Our patient was unable to produce sputum for analysis. However, currently, it is regarded as a hypersensitivity reaction to the larva or its soluble antigens. The resolution of the pulmonary shadows after treatment of skin lesions is consistent with the diagnosis of Loeffler's syndrome.[7]

Although the condition is primarily self-limited, appropriate treatment leads to a hasty resolution. Oral ivermectin in a single dose of 200 µg/kg is the main treatment.[8] Albendazole (400–800 mg/day for 1–7 days) has also been found to be equally effective.[9] Oral or topical 10% thiabendazole may be used as an alternative. Surgery and cryotherapy are rarely effective.[10] Adequate antihelminthic therapy usually improves pulmonary eosinophilia but sometimes additional supportive therapy like oxygen inhalation, systemic, or inhalational corticosteroids may be necessary to alleviate the symptoms.[11] Our patient was successfully treated with oral Albendazole.

CLM is a relatively common condition; however, its association with Loeffler's syndrome is extremely rare. To the best of our knowledge, this is the first report of this uncommon association from the Indian subcontinent.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

What is new?

Although cutaneous larva migrans has been reported before, this is one of the few reports of Loeffler's syndrome from the Indian subcontinent.