Aggressive squamous cell carcinoma in Kindler syndrome.

[kindler syndrome]

A 57 -year -old Hispanic man with a personal and family history of bullae and photosensitivity presented with a fungating , ulcerated squamous cell carcinoma on his left hand (Figure 1 ). Physical examination showed conjunctival injection , ectropion , symblepharon , urethral stricture , loss of teeth , short stature , and nail dystrophy . There was reticulated erythema , atrophy , hyperpigmentation and hypopigmentation , and telangiectasia of sun-exposed skin of the face , neck , and hands consistent with poikiloderma (Figure 2 ). In addition , there was foreshortening of the left thumb and sclerodermoid changes of his hands (Figure 3 ). Radiation therapy was applied to shrink the tumor before a local excision was performed . However , a local recurrence followed and axillary lymph nodes became clinically palpable , necessitating amputation and lymph node dissection . Extensive histologic evaluation of the specimen obtained following left arm amputation and lymph node dissection showed moderate -to -poorly differentiated deeply invasive squamous cell carcinoma . Two of 3 axillary lymph nodes were positive for metastatic carcinoma . A random biopsy of the trunk showed epidermal atrophy , telangiectasia , a perivascular lymphocytic infiltration , and pigment-laden macrophages consistent with poikiloderma . Electron microscopy illustrated extensive reduplication of the basement membrane , with loops , curls , and free extensions of the basal lamina in the superficial dermis ; reduced numbers of hemidesmosomes and anchoring fibrils ; and a basement membrane focally devoid of basal cells (Figure 4 ). On the basis of the clinical features and the characteristic basement zone changes , a diagnosis of Kindler syndrome was made .

Diseases
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Diseases presenting "basal cells" symptom

epidermolysis bullosa simplex

kindler syndrome

lamellar ichthyosis

omenn syndrome

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