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SKINDIA QUIZ
Year : 2018  |  Volume : 9  |  Issue : 6  |  Page : 467-468  

SkinIndia Quiz 48: Blistering and mottled pigmentation in a young male


1 Department of Dermatology, AIIMS, Jodhpur, Rajasthan, India
2 Department of Pathology, AIIMS, Jodhpur, Rajasthan, India

Date of Web Publication5-Nov-2018

Correspondence Address:
Anupama Bains
Department of Dermatology, AIIMS, Jodhpur - 342 005, Rajasthan
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/idoj.IDOJ_245_17

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How to cite this article:
Bains A, Vedant D, Bhardwaj A, Elhence P. SkinIndia Quiz 48: Blistering and mottled pigmentation in a young male. Indian Dermatol Online J 2018;9:467-8

How to cite this URL:
Bains A, Vedant D, Bhardwaj A, Elhence P. SkinIndia Quiz 48: Blistering and mottled pigmentation in a young male. Indian Dermatol Online J [serial online] 2018 [cited 2018 Dec 12];9:467-8. Available from: http://www.idoj.in/text.asp?2018/9/6/467/240582



A 21-year-old male born of a non-consanguineous marriage presented with history of blister formation over the extremities and trunk since childhood. The lesions used to rupture in 1–2 days followed by crusting and healing with hyperpigmentation in 7–10 days. Lesions were recurrent and aggravated by trauma. The severity of blistering gradually improved with age. He also had a history of photosensitivity. At about 4 years of age he developed thinning of skin over the dorsa of hands. Diffuse mottled pigmentation was present on the neck and face since 10 years of age. There was no history of oral ulcers, difficulty in urination, defecation, dysphagia, and dysphonia. There was history of similar lesions in his brother. On cutaneous examination, there were multiple erosions over the trunk and extremities with crusting and few vesicles [Figure 1]a. Nikolsky's sign was negative. There was presence of poikiloderma over the neck and mandibular area of the face [Figure 1]b. Over the dorsa of hands and feet, atrophic, cigarette-paper-thin skin was present [Figure 2]. Mucosa, hair, and nails were normal. Skin biopsy from bullous lesion showed subepidermal vesicle filled with lymphocytes, few eosinophils, and proteinaceous fluid [Figure 3]a. Skin biopsy from the poikilodermatous skin showed thinned-out epidermis with loss of rete ridges. Superficial dermis showed dilated capillaries, melanin pigment incontinence, along with mild chronic inflammatory infiltrate [Figure 3]b.
Figure 1: (a) Multiple crusted lesions with black arrow showing vesicle over the back. (b) Poikilodermatous skin over the neck

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Figure 2: Atrophic cigarette-paper-thin skin over the dorsa of hands

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Figure 3: (a) Skin biopsy from bullous lesion showed subepidermal vesicle-filled with lymphocytes, few eosinophils, and proteinaceous fluid (H and E, ×10). (b) Skin biopsy from poikilodermatous skin showed thinned-out epidermis with loss of rete ridges. Superficial dermis showed dilated capillaries and melanin pigment incontinence. (H and E, ×10)

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What is your diagnosis?



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   References Top

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Gupta V, Dogra D, Gupta N, Parveen S. Kindler's syndrome with long thick cuticles and mottled hyperpigmentation. Indian J Dermatol Venereol Leprol 2011;77:66-8.  Back to cited text no. 5
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