An Unusual Case of Erythroderma in a 59-Year-Old Male

INTRODUCTION

Erythroderma is described as generalised redness and scales affecting at least 90% of the body surface area. It has various causes, such as psoriasis, drugs and cutaneous lymphomas. PL (pityriasis lichenoides) is a rare dermatosis with varied presentations, mainly PLC (pityriasis lichenoides chronica) and pityriasis lichenoides et varioliformis acuta (PLEVA) and febrile ulceronecrotic Mucha-Habermann disease, a severe form of PLEVA.

CASE REPORT

A male patient aged 59 years, previously diagnosed with type 2 diabetes mellitus controlled on oral hypoglycaemic agents, presented with multiple itchy, scaly, red, raised lesions over different parts of the body for 2 months. There was no fever, loss of appetite, significant unintentional weight loss, joint pain, or any other systemic symptoms. On examination, multiple erythematous, well-defined, scaly papules, plaques and some crusted lesions ranging in size between 0.5 and 1 cm in diameter were seen over different parts of the body, including the face, trunk, and bilateral upper and lower limbs, with 2–3 erosions over the glans penis of approximately 1 × 1 cm diameter, involving a body surface area of approximately 92% [Figures 1a–c]. There was no lymphadenopathy and organomegaly. Hair, nails, and mucosae showed no abnormalities on examination. There was no history of intake of drugs known to cause pityriasis lichenoides/erythroderma prior to the occurrence of skin lesions.

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Figure 1:

(a): A 59-year-old male patient with multiple erythematous macules and papules with mica-like scales over the trunk, (b): Presence of multiple erythematous papules with scaling (arrow) and some crusted lesions (line) over both knees and legs, (c): Similar type of lesions over the back, with multiple erythematous macules and papules with mica-like scales, with few crusted erosions.

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Two biopsy specimens, one from a scaly papule and another from a crusted skin lesion, were sent for histopathological examination. Both sections showed parakeratosis in the epidermis and focal interface vacuolar changes with lymphocytic infiltrates in the dermis with no atypia. Infiltration of lymphocytes and RBCs in the epidermis with RBCs in the papillary dermis was also seen in the section from the papule. The section from the crusted lesion also showed an eroded surface with thick scale made of parakeratotic cells, neutrophils, and clumps of bacteria [Figures 2a and b]. Routine blood and radiological investigations showed no abnormalities. The general blood picture did not show the presence of any atypical lymphocytes. A final diagnosis of PLEVA was made.

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Figure 2:

(a): Biopsy from crusted skin lesion showing vacuolar interface changes with lymphocytic infiltrate, parakeratosis, and eroded surface with thick scale. (haematoxylin and eosin, 100x), (b): iopsy from papule showing parakeratosis and red blood cells in papillary dermis. (haematoxylin and eosin, 400x).

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The patient was started on oral methotrexate 0.3 mg/kg/week (20 mg/week), and he showed significant improvement (> 75%) within 4 weeks [Figures 3a and b]. The dose was then tapered by 5 mg/week at 2-weekly intervals, with methotrexate being given for a total of 10 weeks, but the lesions relapsed when the dose was reduced to 5 mg/week. He was then switched to 8-methoxypsoralen (8-MOP) 0.6 mg/kg given twice weekly, followed by sun exposure for 10 minutes after 2 hours of intake of the drug, and asked for sun-protective behaviour for the rest of the day after sun exposure. The patient was in remission after 3 months of therapy [Figure 3c].

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Figure 3:

(a): Improvement of lesions with erythematous atrophic scars over the trunk after 4 weeks of treatment with methotrexate. (b): Resolution of lesions with erythematous atrophic scars over both knees and legs after 3 weeks of treatment with the presence of few crusted lesions. (c): Complete resolution of skin lesions post 3 months of PUVAsol therapy (PUVAsol: Psoralen and UVA obtained by solar light).

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DISCUSSION

Erythroderma is an inflammatory condition characterised by erythema and scales affecting 90% of the body surface area. In a prospective study conducted in a tertiary care centre in 309 patients, it was found that in the majority of patients, exacerbation of pre-existing dermatoses (eczema, psoriasis, atopic dermatitis) was the cause of erythroderma, with 40% of patients having a history of previous skin disease, and drug eruption was the second most frequent cause. Other causes include Sezary syndrome, mycosis fungoides, pemphigus foliaceous, dermatomyositis, pityriasis rubra pilaris, etc. In some cases, it may be idiopathic.^[1]

PL is a papulosquamous disorder of unknown aetiology affecting individuals of any age. Its presentation comprises a spectrum ranging from an acute form, PLEVA, and its severe form, Febrile ulceronecrotic Mucha-Habermann disease (FUMHD), to a chronic form, PLC. PLEVA, is a self-limiting condition, presenting as generalised papules undergoing central vacuolisation and haemorrhagic necrosis and healing with varioliform scars. FUMHD, a severe, potentially fatal form of PLEVA, presents with systemic symptoms such as high-grade fever, myalgia, arthralgia, and other organ involvement. PLC is characterised by red, scaly papules with micaceous scales that periodically relapse and remit over several years.^[2]

Histopathology shows degeneration of epidermal basal cells and lymphocytic infiltrate around the blood vessels in all cases of PL. Parakeratosis, epidermal necrosis, and extravasation of RBCs are additional features seen in PLEVA.^[3]

In the present case, histopathological features suggestive of psoriasis, such as hyperkeratosis, hypogranulosis, elongated rete ridges, tortuous dilated capillaries, and Munro microabscesses were absent; epidermotropism, a feature of mycosis fungoides, was also absent. The absence of an eosinophilic infiltrate in the dermis ruled out drug-induced erythroderma.

A systematic review that studied the different treatments for PL, published in 2020, found that phototherapy [broadband ultraviolet B (BBUVB), narrowband ultraviolet B (NBUVB), and ultraviolet A (UVA)], antibiotics (erythromycin and doxycycline), and corticosteroids (both topical and oral) had been studied the most. However, none of the included studies displayed robust evidence. Most of them did not differentiate between PLC and PLEVA, despite the two conditions being dissimilar in histopathology, morphology, and pattern of recurrence.^[2] The efficacy of PUVA is believed to be due to its role in altering the function of lymphocytes, both in circulating forms as well as those in the skin. Evidence for the efficacy of methotrexate in the management of pityriasis lichenoides has been demonstrated in several case series and reports.^[4,5]

CONCLUSION

The case above is that of a rare presentation of PL presenting as erythroderma in an Indian patient in his sixth decade of life, responding well to PUVAsol treatment.