Kindler Syndrome | Genodermatoses

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Author: Dr M Umair Memon - Ziauddin Hospital - Sukkur

In a free medical camp at a remote area of Sindh, you came across 9 children of the same family having a history of photosensitivity and variable degrees of blistering mainly over trauma-prone sites. On further examination variable degrees of oral mucosal ulceration were noted & older siblings were noted to have atrophic changes over dorsa of hands and feet with areas of hypo and hyperpigmentation (as shown in picture).

1 / 5

Q1. What is the most likely diagnosis ?

Porphyria cutanea tarda

Rothmund thompson syndrome

Werner syndrome

Dyskeratosis congenita

Kindler syndrome

2 / 5

Q2. What is the inheritance pattern of this disease that you need to discuss with their family ?

Autosomal recessive

Autosomal dominant

X-linked recessive

X-linked dominant

Acquired

3 / 5

Q3. What grave complication you might screen for in the affected children during followup visits ?

Basal cell carcinoma

Laryngeal cancer

Squamous cell carcinoma

Osteosarcoma

Thyroid cancer

4 / 5

Q4. While counselling the family how will you counsel regarding prognosis of disease ?

Self resolving

Blistering improves, poikiloderma progresses as age progress

Blistering worsens as age progresses

Good prognosis provided no cancer develops

Undetermined

5 / 5

Q5. Considering the physician has access to the best possible health facility, what pertinent finding can be picked up on skin biopsy sample (histopathology/electron microscopy) ?

Subepidermal split

Festooning of dermal papilla

Duplicated lamina densa

Non-specific dermal infiltrate

Interface dermatitis

Your score is

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