Figure 2. Hyperpigmented depressed plaques on the arm
A 31-year-old male waiter presented to us with a non-healing ulcer on the back of 3-years duration. The lesion started as a skin-colored itchy papule that progressed to form a plaque with scale and crust. Soon he developed hyperpigmented scaly depressed plaques over the back of the arms and thighs. The lesion on the back progressed to an ulcer with purulent discharge and improved with a course of antibiotics. On further questioning patient gave a history of joint pains affecting the metacarpophalyngeal, knee, shoulder, and ankle joints for which he was on non-steroidal anti-inflammatory medication. There was no history of photosensitivity, oral ulceration, malar rash, or trauma prior to the onset of skin lesions. Cutaneous examination revealed a 8 × 7 cm ulcer with necrotic base and slough on the mid back with surrounding areas of erythema and depigmentation.
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| Figure 1 | Figure 2 |
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| Figure 1. Ulcer on the back. Figure 2. Hyperpigmented depressed plaques on the arm |
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| Figure 3 | Figure 4 |
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| Figure 3. Hyperpigmentation on the zygoma. Figure 4. Follicular pits in the ears |
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| Figure 5 | Figure 6 |
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| Figure 5. Low power photomicrograph Figure 6. Basal cell degeneration with pigment incontinence |
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| Figure 7 | Figure 8 |
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| Figure 7. Perivascular infiltrate Figure 8. Post treatment |
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Small scaly hyperpigmented plaques were present on the posterior arms and thighs. Hyperpigmented pits were seen in the triangular fossa of the ears. There was hyperpigmentation on the face particularly the zygomatic area and the palms and soles, nails were not affected.
The routine hematological and biochemical investigations were normal except for a raised erythrocyte sedimentation rate. His antinuclear antibody test was weakly positive for ribosomal-P protein. A skin biopsy from the edge of the ulcer showed hyperkeratosis with follicular plugging, liquifactive degeneration of basal cell layer, and perivascular and periappendageal lymphocytic infiltrate in the dermis. Lesional biopsy for direct immunofluorescence showed a strong basement membrane band. Based on the clinical and laboratory findings, a diagnosis of discoid lupus erythematosus was made.
The nomenclature and classification system derived by James Gilliam divides the cutaneous manifestation of lupus erythematosus (LE) into LE specific skin disease and LE non-specific skin disease. The term cutaneous LE is often used as an umbrella designation for acute cutaneous LE, subacute cutaneous LE, and chronic cutaneous LE. Chronic cutaneous LE or discoid LE is a relatively benign condition with characteristic histopathology [1, 2]. Females are affected twice as commonly as males with peak age of onset in the fourth decade. The DLE skin lesions account for 15-30 percent of SLE population; approximately 5 percent of patients presenting with isolated localized DLE subsequently develop SLE. DLE is an autoimmune disorder in which the environmental factors interfere with defense mechanisms and precipitate the disease. The triggering factors include sunlight, extremes of weather, premenstrual period, early pregnancy, viral infection, drugs, and cigarette smoking [3]. The pathogenetic effects of UV light in cutaneous LE are poorly understood. However it has been suggested to induce the expression of neo-antigens that could be the target of dysregulated immune attach. Ultraviolet light may be associated with an exaggerated release of immune mediators such as IL-1, TNF-α, prostaglandins, and oxygen-free radicals in genetically predisposed individuals with LE. The aberrant expression of adhesion molecules such a ICAM 1 could play a role in pathogenesis of LE photosensitivity [4].
Typical DLE lesions evolve into sharply demarcated coin-shaped erythematous plaques covered by a prominent adherent scale that extends into the orifices of hair follicles. When the adherent scale is removed keratotic spikes similar in appearance to carpet tacks can be seen to project from the undersurface of the scale (carpet tack sign). The lesions expand with erythema and hyperpigmentation at the periphery leaving atrophic central scarring, telangiectasia, and hypopigmentation. Localized DLE occurs on the head and neck, whereas generalized DLE can occur both above and below the neck. DLE lesions below the neck mostly are seen on the extensor aspect of forearms, arms and hands. The palms and soles can also be the site of painful at times disabling erosive DLE lesions. The lesions may spontaneously involute but relapses are common.
Non-healing ulceration on back, the peculiar presentation of our patient, was confirmed to be DLE only on immunofluorescence and histology. It has been said that DLE in certain ethnic backgrounds such as Indians can present with clinical findings limited to isolated hyperpigmented macules. Our patient also had macular hyperpigmentation over the zygomatic area, which, if taken into consideration along with the follicular pits in the ears and the small hyperpigmented depressed scaly plaques on the forearms could be the residual signs of disseminated DLE. Our patient has been under regular follow up and was treated with hydroxychloroquin and topical steroids for his skin complaints.
© 2006 Dermatology Online Journal