|Year : 2020 | Volume
| Issue : 2 | Page : 96-98
Lichen planus pigmentosus in systemic sclerosis: a rare association
Pallavi Goyal1, Surabhi Dayal2
1 Assistant Professor, Kalpana Chawla Govt. Medical College, Department of Dermatology, Karnal, Haryana, India (Ex. Senior Resident, Department of Dermatology, Venereology and Leprosy, Pt. B. D. Sharma PGIMS, Rohtak, Haryana, India
2 Senior Professor & Head of the department, Department of Dermatology, Venereology and Leprosy, Pt. B. D. Sharma PGIMS, Rohtak, India
|Date of Submission||26-May-2019|
|Date of Decision||06-Dec-2019|
|Date of Acceptance||27-Jul-2020|
|Date of Web Publication||03-Dec-2020|
Dr. Pallavi Goyal
Address for correspondence: Dr Pallavi Goyal, Pawan Surgical Hospital, Red Road, Kurukshetra-136118.
Source of Support: None, Conflict of Interest: None
A 30 year old female came to outpatient department with gradually progressing asymptomatic hyperpigmented lesions over face and neck since 2 years. Patient was a known case of systemic sclerosis and hypothyroidism and was on treatment for the same since 8 years. Differential diagnosis included lichen planus pigmentosus (LPP), ashy dermatoses, drug-induced hyperpigmentation, Riehl’s melanosis. Histopathology confirmed the diagnosis of lichen planus pigmentosus. Patient was put on topical depigmenting agents and topical calcineurin inhibitors along with photoprotection and the response was good. Patient had been regular in follow up.
Keywords: Hyperpigmentation, lichen planus pigmentosus, systemic sclerosis
|How to cite this article:|
Goyal P, Dayal S. Lichen planus pigmentosus in systemic sclerosis: a rare association. Pigment Int 2020;7:96-8
| Introduction|| |
LPP (Lichen planus pigmentosus) is a condition characterized by asymptomatic, persistent and diffuse slaty‑gray pigmentation predominantly involving sun-exposed areas mainly affecting middle aged females., Several types of cutaneous pigmentary modifications associated with systemic sclerosis (SSc) that have been described in literature are diffuse, generalized hyperpigmentation with accentuation in sun-exposed areas; a vitiligo-like depigmentation with perifollicular hyperpigmentation; salt and pepper dyspigmentation in the areas of sclerosis etc. We are hereby describing a case in which there is rare co-occurrence of SSc and LPP which has not been mentioned in literature till date.
| Case Report|| |
A 30 year old female came to out patient department with gradually progressive asymptomatic hyperpigmented lesions over face and neck since 2 years. It started from pre auricular region and temples 2 years back and further progressed to involve whole face and then neck. History of application of henna dyes, amla and mustard oil was absent. Family history was not significant. Melanonychia of all the 20 nails was present since the age of 10 years. Patient was diagnosed with systemic sclerosis 8 years back. At that time, patient had history of skin tightening and thickening over both arms distal to elbow joint and face; with Raynaud’s phenomenon and joint pains since 1 year. Patient had history of dyspnoea and dysphagia since 6 months. Antinuclear antibodies and anti centromere antibodies were negative. Spirometry and HRCT chest showed restrictive lung disease changes. Barium swallow was normal. According to ACR-EULAR criteria, total score was 17 establishing the definite diagnosis of systemic sclerosis. Later patient developed hypothyroidism. Punch biopsy was performed that revealed mild collagenization in the deeper dermis ND subcutaneous tissue with entrapment of eccrine apparatus which was suggestive of systemic sclerosis. Patient was given dexamethasone pulse therapies 8 years back for 18 months which lead to improvement in skin tightening and thickening. Since then, patient was on oral drugs like nifedipine, pentoxifylline, losartan, nicotinate, thyroxine and bosentan on and off.
On cutaneous examination, multiple symmetrical ill defined non scaly, non atrophic, grey-black patches of variable size coalescing with each other were present over face and neck [Figure 1]a and b. Trunk, upper and lower limbs were spared. Erythematous elevated border and typical salt and pepper pigmentation were absent. Pitted scars were present on fingertips. All the finger and toe nails showed melanonychia. Oral mucosa was involved showing white lacy pattern but flexures, scalp and genital mucosa were not affected.
|Figure 1 Multiple symmetrical ill-defined non-scaly, smooth grey-black patches of variable size coalescing with each other present over face and neck.|
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Differential diagnosis included LPP, ashy dermatoses, drug induced hyperpigmentation. Punch biopsy was taken from lesion on the face to confirm the diagnosis. Histopathology revealed atrophic epidermis, mild spongiosis, hyperkeratosis with vacuolar degeneration of basal layer along with pigment incontinence in upper dermis and presence of mild perivascular AND periadenexal inflammatory infiltrate [Figure 2]. Features were suggestive of LPP. Other features that aid in confirming the diagnosis of LPP were middle-aged female, predominantly affecting sun exposed sites, involvement of oral mucosa, other sclerotic sites not showing any pigmentary changes, and absence of typical salt and pepper pigmentation. Complete blood count, liver function test, renal function test, random blood sugar, urine complete were normal. Hepatitis B antigen (HBsAg) and anti-HCV were non reactive. Patient was advised for photoprotection and was started on topical tacrolimus 0.1% ointment in morning and glycolic acid 12% at night over affected area. Patient had been regular with follow up and was satisfied with treatment.
|Figure 2 Photomicrographs showing atrophic epidermis, spongiosis, hyperkeratosis, vacuolar degeneration of basal layer along with pigment incontinence in upper dermis and presence of mild perivascular and periadenexal inflammatory infiltrate.|
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| Discussion|| |
LPP is considered as a pigmented variant of lichen planus due to its association with classical lichen planus and histopathological similarities and it was first reported from India in 1974 by Bhutani et al who also coined this term., LPP is a condition characterized by asymptomatic, persistent, symmetric and diffuse slaty‑gray pigmentation predominantly involving sun-exposed areas most commonly face and neck. It affects dark-skinned middle-aged individuals with female preponderance and is reported mainly from India, the Middle East, and South America. Contributory factors are mustard oil which contains potential photosensitizer allyl thiocyanate, amla oil, fragrances, cosmetic agents such as kumkum, hair dyes, etc., LPP has been reported to be associated with frontal fibrosing alopecia, acrokeratosis of Bazex, head and neck carcinoma (paraneoplastic LPP), hepatitis C infection and nephrotic syndrome.
SSc is a chronic multisystem autoimmune connective tissue disease that causes fibrosis in the skin and internal organs with associated vascular and inflammatory manifestations. Its two major subsets, the limited and diffuse forms have been based upon the extent of skin involvement. Skin changes in limited SSc occurs over head, neck and distally on the limbs. Main predisposing factors are genetic, infectious, and environmental.,
Various patterns of pigmentary alterations have been described in SSc, which include 1) diffuse generalized hyperpigmentation; 2) focal depigmentation with perifollicular hyperpigmentation; 3) localized hypo- and hyperpigmentation in sclerotic skin (salt and pepper dyspigmentation); 4) streaky hyperpigmentation over blood vessels on a background of depigmentation on the legs and temporal scalp; and 5) reticulate hyperpigmented scleroderma.
The pathogenesis of these hyperpigmentation abnormalities in SSc remains unclear, but some
of the proposed hypotheses includes increased keratinocyte-derived endothelin-1 (ET-1) which promotes melanogenesis by multiplication of melanocytes and melanin synthesis; secretion of melanocytic growth factors by fibroblasts and endothelial cells; and a thermoregulatory mechanism. Cytokines such as IL-1a may be overproduced in SSc patients, and therefore promote ET-1 production from keratinocytes., In a study conducted in 2015 by Marie et al., melanonychia was found in 8.5% of SSc patients in comparison to 2.5% in healthy controls.
LPP is thought to be a type hypersensitivity IV reaction caused by abnormalities in T lymphocyte functions, due to unknown antigen with lichenoid inflammation, leading to melanin incontinence and superficial dermal pigmentation. Type IV hypersensitivity reaction also called as delayed type hypersensitivity (DTH) involves cellular activation of T-helper cells (CD4+) and/or cytotoxic T cells (CD8+ CTLs) with subsequent cytokine secretion. It is becoming clear that there is a strong aetiological DTH based component associated with deleterious pathologies occurring during many autoimmune disorders.
In pathogenesis of SSc, early event is T-cell activation with infiltration in skin and internal organs and later soluble mediators made by T cells, B cells and other cells activate and damage the fibroblasts and endothelial cells.
We are hereby reporting a rare association of SSc with pigmentary abnormality like LPP which has not been described in literature before. This co-occurence of SSc and LPP in our case can be attributed to abnormalities in T cell activation and functions.
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Conflicts of interest
There are no conflicts of interest.
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