Table of Content

Current Issue

Summer 2019, Vol. 27 No. 2

Hong Kong J. Dermatol. Venereol. (2019) 27, 75-78


Views and Practice

Lichen planus pigmentosus: report of effectiveness of extracorporeal photochemotherapy in recalcitrant case

YV Molochkova,VA Molochkov, YA Pimenova

A 53-year old female patient presented with an eruption of itchy lesions on the region of trunk and limbs. Three years ago, the patient had a psycho-emotional trauma after which she noticed eruption of lesions on the mucous membrane of the genital area. Within two years, itchy papules appeared on the trunk and limbs and the patient was diagnosed with lichen planus. She was treated with intralesional betamethasone injections, systemic intravenous Cyclophosphamide and applied corticosteroids topically. After the treatment, healing process was noticed. However, within a month, new pruritic lesions erupted on different areas of trunk and limbs and the patient was hospitalised.

During the admission, she presented with diffuse, symmetrical and indurated lesions in ears, neck, limbs (Figure 1), trunk, axilla (Figure 2), and inguinal folds. The lesions consisted of light and dark coloured macules, brown-purple coloured shiny papules and oval shaped plaques. The size of the plaques varied from 0.5 cm to 4 cm with clear and distinct borders. In most lesions, the skin in the centre was thinned and atrophic giving a 'rice paper appearance.'

Figure 1 Lichen planus pigmentosus on the lower limbs.

Figure 2 Lichen planus pigmentosus on the axilla.

The histological analysis revealed atrophy of the epidermis with the presence of melanophages in the upper layers of dermis and vacuolar dystrophy in the basal layer of the epidermis along with lichenified lymphocytic infiltration (Figure 3).

Figure 3 Histological slide: (a) LPP with large amount of melanophages in the upper layers of dermis. Haematoxylin-eosin x 100. (b) LPP melanophages in papillary layers of dermis. Haematoxylin-eosin x 400.

Based on the histological and clinical findings, the patient was diagnosed as lichen planus pigmentosus (LPP) . The patient was treated with loratadine 10 mg daily, intramuscular chloropyramine 40 mg daily for 10 days and four sessions of extracorporeal photochemotherapy (ECP). Two hours before the ECP session, the patient was given 8-methoxypsoralen at a dose of 0.6 mg/kg. After gaining peripheral vascular access, 500 ml of blood was drawn from the patient and centrifuged for 5 minutes at 2600 rpm. Supernatant plasma enriched with leucocytes was transferred to a container and re-centrifuged for 15 minutes at 3000 rpm. Isolated lymphocytes were suspended in 200 ml of saline solution, thoroughly mixed, and then subjected to UV irradiation for 40 minutes and re-administered to the patient. The UV emission was wavelength of 320-400 nm, administered with 16 watt UV lamps. Course of treatment included 4 photopheresis sessions, each session on alternate days. After three days of treatment, there was decreased pruritus. After the fourth (ECP) session of ECP, the lesions had faded and we observed clinical improvement (Figures 4 & 5).

Figure 4 Hyperpigmentation in the axillary area after two weeks of treatment.

Figure 5 Hyperpigmentation in the lower limbs after two weeks of treatment

Discussion

LPP was first described in 1918 by J. Fabri and D. Pirilia. It is most commonly found in Asia, Latin America and Middle East.1 LPP is clinically characterised by the presence of hyperpigmented brown macules, in sun-exposed areas of skin, skin folds and flexural part of the limbs.2 One of the most important clinical presentations of LPP is the appearance of hyperpigmentation preceding the formation of lichenified papules; therefore papular lesions may not be initially evident. There may also be pigmentation and typical pink-purple elements. In LPP, the mucous membranes are rarely affected and pruritus is moderate or absent. LPP can be present in combination with the pemphigoid form of lichen planus (pigmento-pemphigoid form). LPP may also present in a linear distribution characterised by hyperpigmented dark brown macules with linear plaques.3

The histology of LPP is due to cytotoxic damage of epithelial basal cells by T-lymphocytes and degradation of the epithelial basement membrane.4 The histological studies of pigmented LPP reveal melanophages in the atrophic epidermis. The stratum basalis layer of the epidermis shows vacuolar dystrophy and slight histolytic lichenoid lymphocytic infiltration.5 The course of LPP is chronic with a tendency to remission and exacerbation.5 LPP should be distinguished from toxic lichenoid eruption and Kaposi's sarcoma.

In toxic lichenoid eruption, the lesions are generally present on sun-exposed areas of the skin. The pathological process of toxic lichenoid eruption is triggered by sun exposure. Sometimes the appearance of a rash is associated with exposure of drugs and toxic substances, such as oil fuel and other combustion products.6 Characteristic lesions of LPP on the mucous membrane are absent.

Kaposi's sarcoma can be easily differentiated from LPP. In Kaposi's sarcoma, there is gradual onset of eruptions on the limbs and characteristic cherry-coloured lesions on the mucous membrane. Unlike lichen planus, these lesions do not show a linear distribution. The regressive form of typical Lichen planus (LP) and LPP can be differentiated as follows: in typical LP, hyperpigmentation occurs on the place of former papular lesions and it is not accompanied by the appearance of new lesions, and Koebner phenomenon while in LPP is characterised by appearance of papules on hyperpigmentation background, sometimes lesions are lacy, and are combined with lichenoid rash on other areas.4

Erythema dyschromicum perstans may also be considered as differential diagnosis of LPP. Erythema dyschromicum perstans is generally seen in children and young adults and it is characterised by the formation of small, grey-coloured macules that do not become confluent which each other and are present on the trunk, neck and proximal parts of the upper limbs.7

Standard therapy of generalised LPP includes topical and systemic corticosteroids, retinoids, PUVA-therapy and cyclosporin in persistent cases.8 Most of the cases with defined lesions are treated with local corticosteroids of class I and II. To avoid any complications and secondary effects, children and adults are recommended to use non-halogen and non-fluorinated ointments twice daily for two to four weeks. Antihistamines may be given for pruritus.

In resistant cases of LP, biologics, in particular adalimumab have been given.9

Extracorporeal photochemotherapy (ECP, photopheresis) has been successfully used in conditions such as T-cell lymphoma, LP-like reaction, graft-versus-host disease and in a number of autoimmune diseases like rheumatoid arthritis, systemic lupus erythematosus, and systemic sclerosis. ECP (two sessions/week during the first 3 weeks with progressive reduction according to clinical improvement to one session every 4 months) was successfully used in the case of erosive oral LP,10 and in a recalcitrant case erosive cutaneous lichen planus.11 According to A. D. Guyot, the mechanism of action of ECP in LP is associated with a significant decrease in the number of blood T-lymphocytes with stable levels of B-lymphocytes and natural killers cells.10

Conclusion

There are numerous reports of the usage of ECP in LP which have reported that ECP is highly effective particularly for resistant cases of LP.

To our knowledge there have been no reports of the use of ECP in LPP. We have used this method in the treatment of resistant to Betamethasone and Cyclophosphamide LPP with good efficacy. Based on our findings, further study of the immunological changes in patients with LP and LPP following treatment with ECP will clarify the mechanism of action of this treatment modality.

References

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