Pages 215 - 219 Volume 91, Issue 2
Back
Generalized idiopathic atrophodema of Pasini and Pierini misdiagnosed as relapse of disease in a patient with borderline tuberculoid leprosy

Idiopathic atrophoderma of Pasini and Pierini (APP) is a rare condition which is characterized by asymptomatic depressed hypopigmented patches with a classic ‘cliff drop’ edge, usually on the back. There are less than 100 cases reported in the literature till date. APP can mimic borderline tuberculoid (BT) leprosy due to its hypopigmented colour and loss of sensation as a result of damage to dermal nerves. We report a 51 years old man with BT leprosy who had already completed 6 months of PB-MDT adult regimen and subsequently developed new hypopigmented and hypoesthetic skin lesions over the trunk and extremities one year after completion of MDT. The lesions were misdiagnosed as relapse of BT leprosy and the patient was restarted MB-MDT. New lesions kept on appearing despite the intake of MDT. Later histopathology revealed the lesions to be atrophoderma.

Cite this article
Swetalina Pradhan, Gaurav Dash, Chandra Sekhar Sirka, Subhasini Naik;
Generalized idiopathic atrophodema of Pasini and Pierini misdiagnosed as relapse of disease in a patient with borderline tuberculoid leprosy; Leprosy Review; 2020; 91; 2; 215-219; DOI: 10.47276/lr.91.2.215
LEPROSY
Leprosy Review
0305-7518
British Leprosy Relief Association
Colchester, UK
Introduction
Idiopathic atrophoderma of Pasini and Pierini (APP) is a rare dermatological condition which is believed to affect dermal collagen organization, resulting in dermal atrophy.1 It is characterized by asymptomatic sharply demarcated depressed hypopigmented patches with a classic ‘cliff drop’ edge, usually on the back.2,3 It can mimic borderline tuberculoid (BT) leprosy due to its hypopigmented colour, loss of sensation and loss of hair, which can also be seen in atrophoderma due to involvement of the dermal nerves. We report a 51 year-old man who had already completed 6 months of PB-MDT and who subsequently presented with new hypopigmented and hypoesthetic skin lesions over the trunk and extremities after one year. These signs were misdiagnosed as relapse of BT leprosy, but turned out to be atrophoderma on histopathological examination.
Case report
A 51 year-old man presented with multiple hypopigmented and hypoesthetic skin lesions over the trunk and extremities, present for two and a half years. According to the patient, he initially noticed a hypopigmented skin lesion of around 3 cm × 4 cm on his left thigh for which he consulted a local physician and was diagnosed as borderline tuberculoid (BT) leprosy. He was started on PB-MDT, which he took for 6 months. One year after completion of MDT, the patient noticed the appearance of new hypopigmented skin lesions over the upper extremities, which gradually involved the trunk and lower extremities, within a span of one and a half years. He again consulted the physician and was diagnosed as a relapse of BT leprosy. He was restarted on MB-MDT. Despite being on MDT, new lesions kept on appearing. Dermatological examination revealed a hypopigmented patch with 70 to 80% loss of sensation over the left thigh, suggestive of BT leprosy (Figure 1). There were multiple skin coloured, hypopigmented, and hyperpigmented plaques ranging in size from 3 cm × 5 cm to 20 cm × 15 cm with atrophy and wrinkling over the extremities and trunk (Figure 2). There was hypoesthesia in all the plaques. The presence of wrinkling and hyperpigmention were not typical of leprosy. Peripheral nerve examination revealed enlargement of the left ulnar nerve and bilateral common peroneal nerves. Histopathology from the both hypopigmented and hyperpigmented plaques on the trunk and extremities revealed moderate pigmentation of the basal layer and dermis, showing sparse chronic inflammatory infiltrate and thick collagen bundles which were also better demonstrated by MAT stain (Figure 3). Slit skin smears for AFB were negative.
Basing upon clinical and histopathological features, diagnosis of healed BT leprosy with generalized atrophoderma was made. The patient was counselled regarding the inactive lesions of BT leprosy and MDT was stopped. He was counselled regarding the course and prognosis of atrophoderma. Hydroxychlroquine was proposed as treatment for atrophoderma, but the patient declined further treatment and follow up.
Figure 1.
Hyopigmneted, hypoesthetic patch over the left thigh suggestive of BT leprosy.
Figure 2.
(a) Hypopigmented plaques with atrophy, wrinkling and relative loss of hair over the upper back. (b) Hyperpigmented plaque on posterior aspect of the left thigh. (c) Hypopigmented plaques with atrophy and wrinkling over the anterior left thigh. (d) Hypopigmented plaques with atrophy and wrinkling over the arm.
Figure 3.
(a) H&E stain, 10× magnification: normal looking epidermis with focal loss of rete ridges. Dermis depicts adnexal structures, thickened collagen bundles and minimal inflammatory cell infiltrates. (b) H&E stain: 40× magnification showing minimal perivascular lymphocytes and histiocytes, with frequent melanin incontinence. (c) Massons trichrome stain 10×: Thickening of collagen bundles in papillary and reticular dermis, evidenced by decreased spacing.
Discussion
The classical presentation of borderline tuberculoid (BT) leprosy includes large, well to ill-defined, dry, icthyotic, hypopigmented patches with 80 to 100% loss of sensation to light touch. The border may start sloping outward and even merge into normal skin, with small extensions at the edge of the lesion (pseudopodium); satellite lesions are commonly seen.4 Nerves adjacent to plaques and peripheral nerve trunks are frequently enlarged. It is treated 6 months of MDT.5 Idiopathic atrophoderma of Pasini and Pierini is a rare disorder of collagen tissue. It usually occurs insidiously during the second or third decade of life. There are less than 100 cases reported in the literature till date.1 Generalized idiopathic APP has been rarely reported. The classic clinical manifestations include round-to-oval hyperpigmented or hypopigmented, depressed patches ranging from a few millimeters to several centimeters across, with an abrupt edge often exhibiting a ‘cliff-drop’ border.2 Another descriptive term commonly used is ‘footprints in the snow,’ or Swiss cheese–like appearance, depicting the common oval morphology of the depressed lesions.6 It most commonly occurs over the trunk and may gradually progress to the chest, arms, and abdomen. There can be associated pain, pruritus, or even paraesthesia in APP.1,7 Histopathologic changes include apparently an normal epidermis with flattened rete ridges. Varying degrees of homogenized collagen bundles and mild perivascular infiltrate consisting of lymphocytes and histiocytes may be present.8 No effective treatment is available till date. Other therapies with variable efficacy like topical and systemic steroids, antimalarials, D-penicillamine, and phototherapy have been used. Q-switched alexandrite laser (755 nm) was effective in diminishing the hyperpigmentation by 50% after three treatments in one case.9
Our case presented with a hypopigmented patch over left thigh for which he was treated with PB-MDT for 6 months. After completion of MDT, new asymptomatic hypopigmented skin lesions appeared, which were again treated with MDT without any response. Clinically, those lesions were both hypopigmented and hperpigmented plaques with wrinkling of the surface. Hypoesthesia was present in all the plaques, but the presence of atrophy in the form of wrinkling in all, and hyperpigmentation in a few patches, were against BT leprosy. Also despite being on MDT, new lesions were appearing, which supported the suspicion of a misdiagnosis. The lesions were described as atrophoderma on histopathology, using a special MAT stain. Also slit skin smears for AFB were negative. The patient was finally diagnosed as generalized atrophoderma with inactive or healed BT Leprosy. MDT was stopped. Misdiagnosis of an asymptomatic indolent skin disease, as an infectious and stigmatized disease like leprosy can have a large psychological impact on both the patient and his family. Careful clinical examination and detailed investigation are required in all such cases. Generalized atrophoderma presenting as large plaques all over body is very rare, which led to confusion in our case. We are reporting the case to increase awareness among dermatologists regarding such a rare combination of both Hansen’s disease and atrophoderma.
References
[1]MuntyanuA, RedpathM, RoshO, JfriA. Atrophoderma of Pasini and Pierini: case report and literature review. Clin Case Rep, 2018; 7: 258263.
[2]ZhiZR, YuanZW. Two uncommon cases of idiopathic atrophoderma of pasini and pierini: multiple and giant. Indian J Dermatol Venereol Leprol, 2011; 77: 402.
[3]GargA, KumarP. Atrophoderma of Pasini and Pierini. Indian Dermatol Online J, 2011; 2: 126128.
[4]PfaltzgraffRE, BrycesonA. Clinical leprosy. In: HastingsRC (ed.), Leprosy. New York: Churchill Livingstone, 1989; pp. 134176.
[6]AvanciniJ, ValenteNYS, RomitiR. Generalized lenticular atrophoderma of pasini and pierini. Pediatr Dermatol, 2015; 32: 389391.
[7]EichhoffG. Atrophoderma of Pasini and Pierini in a patient with concomitant psoriasis: response to methotrexate. JAAD Case Rep, 2019; 5: 277279.
[8]SalehZ, AbbasO, DahdahMJ, KibbiAG, ZaynounS, GhosnS. Atrophoderma of Pasini and Pierini: a clinical and histopathological study. J Cutan Pathol, 2008; 35: 11081114.
[9]ArpeyCJ, PatelDS, StoneMS, Qiang-ShaoJ, MooreKC. Treatment of atrophoderma of Pasini and Pierini-associated hyperpigmentation with the Q-switched alexandrite laser: a clinical, histologic, and ultrastructural appraisal. Lasers Surg Med, 2000; 27: 206212.